A: They prevent the recurrence of biliary strictures permanently.
B: They eliminate the need for immunosuppressive therapy.
C: They reduce the need for permanent external drains, enhancing quality of life.
D: They avoid the risk of restenosis at the biliary-enteric anastomosis.
C: They reduce the need for permanent external drains, enhancing quality of life.
Explanation:
Biodegradable biliary stents provide temporary support to maintain biliary patency while naturally degrading over time, reducing the need for external drains. This approach enhances patient comfort and quality of life, particularly in pediatric patients with complex biliary anatomy and recurrent strictures. Although not a permanent solution, they delay the need for retransplantation and can reduce the frequency of interventions.
Reference:
Malik A, Ng VL, Sayed BA, Siddiqui A, Parra DA.
Biodegradable biliary stents placement using a “kissing-stent” technique for management of a recalcitrant stricture post-live donor liver transplant.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14725.
A: Berardinelli–Seip syndrome patients commonly reach adulthood without significant health issues.
B: The main therapeutic approach includes recombinant leptin therapy, which reverses liver cirrhosis.
C: Liver transplantation in Berardinelli–Seip syndrome is rare but may be considered in severe liver disease cases.
D: The syndrome is characterized solely by metabolic issues without physical manifestations.
C: Liver transplantation in Berardinelli–Seip syndrome is rare but may be considered in severe liver disease cases.
Explanation:
Berardinelli–Seip syndrome is a severe, rare genetic disorder characterized by a near absence of adipose tissue and significant metabolic disturbances, such as insulin resistance, hypertriglyceridemia, and liver disease. Physical manifestations can include hirsutism, a muscular appearance, and facial anomalies. While management primarily involves symptomatic treatment (nutritional support, metformin, and lipid-lowering drugs), liver transplantation is not common but can be a life-saving intervention in severe cases of liver failure.
Reference:
Aliyev A, Samadov E, Ibrahimli A, et al.
Liver transplantation in patient with Berardinelli–Seip syndrome: A literature review and case report.
Pediatr Transplant. 2024;28:e14680. doi:10.1111/petr.1468.
A: BIt provides a readily available option to patch smaller vein defects.
B: It ensures a perfect caliber match with both native and donor vessels.
C: It reduces the need for anticoagulation post-transplant.
D: It simplifies biliary anastomosis in LDLT procedures.
B: It ensures a perfect caliber match with both native and donor vessels.
Explanation:
In cases of significant IVC thrombosis during LDLT, reconstruction can be complex. A cryopreserved pulmonary vein graft was chosen for its appropriate caliber, matching both the upper and lower cava orifices, and its "Y" shape facilitated the anastomosis with the donor’s hepatic vein. This approach ensured adequate vascular continuity and minimized the risk of anastomotic stricture.
Reference:
Yang XY, Le-Nguyen A, Alvarez F, et al.
Pediatric living donor liver transplant for Budd–Chiari syndrome using a cryopreserved pulmonary vein graft for retro-hepatic vena cava reconstruction: A case report.
Pediatric Transplantation. 2024;28:e14674. doi:10.1111/petr.14674.
A: It improves enzyme activity to stabilize MMA-related metabolic dysfunction.
B: It reduces the patient's dependence on immunosuppression.
C: It enables direct surgical resection of hepatic metastases.
D: It is a definitive cure for MMA and prevents all future metabolic crises.
A: It improves enzyme activity to stabilize MMA-related metabolic dysfunction.
Explanation:
In patients with MMA, LDLT can enhance propionyl-CoA catabolism, stabilizing metabolic function by providing enzyme activity from the donor liver. Although not a cure for MMA, liver transplantation helps reduce episodes of metabolic decompensation and improves quality of life. LDLT is especially considered when liver neoplasms like hepatocellular carcinoma are present, as it addresses both the metabolic disorder and liver pathology. MMA patients still require careful monitoring for metabolic stability post-transplant.
Reference:
Malik A, Ng VL, Sayed BA, Siddiqui A, Parra DA.
Living-donor liver transplantation for methylmalonic acidemia patient with hepatocellular carcinoma: A case report and literature review.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14719.
A: It helps rule out other cholestatic disorders like biliary atresia and Alagille syndrome.
B: It enables immediate bile duct reconstruction to improve liver function.
C: It eliminates the need for liver transplantation in severe cases.
D: It ensures normal growth and development through medical therapy alone.
A: It helps rule out other cholestatic disorders like biliary atresia and Alagille syndrome.
Explanation:
Early genetic testing in infants with high-GGT cholestasis is crucial for identifying rare genetic disorders such as PFIC Type 8, particularly with the involvement of genes like KIF12. This approach allows differentiation from other causes of cholestasis, such as biliary atresia or Alagille syndrome, guiding appropriate management. For cases with severe disease progression, such as this patient, liver transplantation is often necessary as medical therapy alone may be insufficient
Reference:
Gautam V, Panda K, Kumar V, et al.
Youngest Living Donor Liver Transplant for End-Stage Liver Disease in a 6-Month-Old With a Novel Aggressive Mutation in KIF12 Gene.
Pediatric Transplantation 2024;28. doi:10.1111/petr.14804.
A: It enhances liver regeneration and growth in pediatric patients.
B: It provides an alternative inflow source for the graft when portal venous flow is compromised.
C: It reduces the need for anticoagulation after liver transplantation.
D: It improves biliary drainage and reduces cholangitis risk.
B: It provides an alternative inflow source for the graft when portal venous flow is compromised.
Explanation:
CPHT is a salvage technique used in cases of complete portomesenteric venous thrombosis, allowing the liver graft to receive blood flow through the IVC. This technique circumvents the need for traditional portal vein inflow, which may be impossible in severe PVT. By establishing an alternative blood flow route, CPHT supports graft viability and function, even in the absence of native portal flow.
Reference:
Barron JO, Radhakrishnan K, Coppa C, et al.
Ten-year follow-up of cavoportal hemitransposition in pediatric liver transplantation for complete portomesenteric venous thrombosis: A case report and literature review.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14738.
A: It allows for complete tumor resection and symptom control.
B: It minimizes the need for long-term somatostatin analogs.
C: It prevents recurrence by enhancing systemic chemotherapy.
D: It reduces the risk of biliary cirrhosis without immune suppression.
A: It allows for complete tumor resection and symptom control.
Explanation:
Liver transplantation provides curative potential for pediatric patients with unresectable primary NETs when standard surgical options are inadequate. It allows complete tumor removal and management of symptoms like cholestasis, especially in rare cases involving primary biliary tract NETs. Given the tumor’s well-differentiated nature and lack of extrahepatic spread, liver transplant is a viable option to improve long-term outcomes in select cases.
Reference:
Rai A, Sproule L, Larman T, et al.
Liver transplant for primary biliary tract neuroendocrine tumor in a nine-year-old girl.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14732.
A: Primary non-function (PNF)
B: Hepatic artery thrombosis
C: Seventh-Day Syndrome (7DS)
D: Post-transplant lymphoproliferative disorder (PTLD)
C: Seventh-Day Syndrome (7DS)
Explanation:
Seventh-Day Syndrome (7DS) is a rare, early complication following liver transplantation, typically manifesting with fever, marked liver enzyme elevations, and graft dysfunction within the first week. Characterized by severe immune response and microvascular injury, 7DS often progresses rapidly, especially in patients with autoimmune predispositions. Unlike primary non-function, 7DS occurs in previously functioning grafts and frequently requires retransplantation due to high morbidity and mortality without intervention.
Reference:
Hartjes K, Koo D, Al-Ibraheemi A, et al.
Early Graft Loss with Suspected Seventh-Day Syndrome Following Pediatric Liver Transplantation.
Pediatric Transplantation 2024;28. doi:10.1111/petr.14818.
A: To improve systemic circulation and reduce pulmonary hypertension
B: To restore hepatic first-pass insulin metabolism and resolve hypoglycemia
C: To promote liver regeneration and decrease portal vein resistance
D: To eliminate the need for protein and lactose restrictions
B: To restore hepatic first-pass insulin metabolism and resolve hypoglycemia
Explanation:
In CPSS, hyperinsulinemic hypoglycemia occurs due to bypassing of the hepatic portal system, leading to insufficient first-pass insulin metabolism. In this case, LDLT was essential as it allowed hepatic processing of insulin and stabilized blood glucose levels. This approach is critical when medical and dietary measures are ineffective at controlling hypoglycemia and related metabolic issues.
Reference:
Kadohisa M, Okamoto T, Iwanaga K, et al.
Living Donor Liver Transplantation for Congenital Portosystemic Shunt Presenting With Hyperinsulinemic Hypoglycemia.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14871.
A: It reduces the likelihood of future liver graft rejection.
B: It provides a longer Roux limb option in patients with limited bowel length.
C: It enables spontaneous intestinal adaptation in short-gut syndrome.
D: It prevents portal vein thrombosis by improving venous return.
B: It provides a longer Roux limb option in patients with limited bowel length.
Explanation:
In pediatric liver transplantation, especially for patients with short-gut syndrome or extensive surgical history, standard Roux-en-Y jejunal limb reconstruction may not be feasible. Using a gastric sleeve as an "extra-anatomical" Roux limb provides a suitable conduit for biliary drainage when bowel length is limited, maintaining adequate blood supply through the preserved gastric mesentery. This approach can reduce the risks associated with bile reflux and recurrent cholangitis in complex cases.
Reference:
Hakeem AR, Gee H, Attia M, Raj Prasad K.
Gastric sleeve as an extra-anatomical roux for biliary reconstruction in a pediatric third liver transplant.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14769.
A: Enhanced biliary drainage with reduced risk of bile duct stricture
B: Prevention of large-for-size syndrome and abdominal compartment syndrome
C: Elimination of the need for immunosuppressive therapy in ABO-incompatible cases
D: Improved vascular anastomosis by reducing graft size
B: Prevention of large-for-size syndrome and abdominal compartment syndrome
Explanation:
Hyperreduction of a left lateral segment graft ensures appropriate size matching, reducing the risk of large-for-size syndrome, which can lead to graft perfusion issues, vascular complications, and abdominal compartment syndrome. This is particularly important in small pediatric recipients where standard-sized grafts exceed optimal GRWR limits. While hyperreduction does not eliminate the need for immunosuppression, it facilitates a safer transplantation process with fewer technical complications.
Reference:
Van der Schyff F, Britz RS, Strobele B, et al.
Hyperreduced left lateral living donor liver transplant in a 4.5 kg child—A first in Africa.
Pediatr Transplant. 2023;27:e14536. doi:10.1111/petr.14536.
A: It allows for the preservation of both lobes for dual recipients.
B: It reduces cold ischemic time, minimizing graft dysfunction risk.
C: It simplifies the surgical technique for pediatric recipients.
D: It decreases logistical challenges during organ transport.
B: It reduces cold ischemic time, minimizing graft dysfunction risk.
Explanation:
In situ split liver procurement is especially beneficial when donor distance is significant, as it reduces cold ischemic time by splitting the liver at the donor site rather than on a backtable. This method helps maintain graft viability and minimizes the risk of primary non-function, which is critical for critically ill pediatric patients requiring expedited organ transplantation.
Reference:
Kwon YK, Valentino PL, Saarela KM, et al.
Pushing the Limits of In Situ Split Liver Procurement to Overcome Donor Distance and Graft Size Challenges for 8-Week-Old Pediatric Recipient.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14848.
A: Complete eradication of microscopic tumor cells through vascular isolation
B: Enhanced liver hypertrophy allowing for safe two-stage resection
C: Immediate reduction of portal hypertension and liver function improvement
D: Elimination of the need for adjuvant chemotherapy post-transplantation
B: Enhanced liver hypertrophy allowing for safe two-stage resection
Explanation:
The ALPPS technique facilitates rapid hypertrophy of the future liver remnant (FLR), enabling safe resection in cases where conventional liver resection is unfeasible due to insufficient liver reserve. This staged approach was critical for managing the advanced hepatic MRT in this patient, allowing for subsequent LDLT. While ALPPS does not directly treat portal hypertension or obviate the need for chemotherapy, it expands the pool of patients eligible for curative liver resection or transplantation.
Reference:
Uesato Y, Ono S, Kawamata F, et al.
Associating liver partition and portal vein ligation for staged hepatectomy as bridging therapy for liver transplantation in an infant with an advanced hepatic rhabdoid tumor.
Pediatr Transplant. 2023;27:e14559. doi:10.1111/petr.14559.
A: MZ heterozygous donors are unaffected by A1ATD-related liver dysfunction.
B: Heterozygous grafts guarantee long-term immunity to liver-related A1AT complications.
C: Limited availability of deceased donor grafts necessitated alternative solutions.
D: MZ phenotype donors produce elevated A1AT levels compensating for recipient deficiency.
C: Limited availability of deceased donor grafts necessitated alternative solutions.
Explanation:
The shortage of deceased donor grafts often necessitates the use of living donors, even if they carry heterozygous mutations, as seen in this case. MZ donors typically have near-normal liver function and are suitable if they demonstrate adequate preoperative A1AT levels and lack histological evidence of liver disease. The use of heterozygous donors expands the donor pool, offering timely transplantation and survival benefits for critically ill patients. However, long-term monitoring for potential graft-related issues remains essential.
Reference:
Sood V, Lee EJ, Raghu V, et al.
Liver transplantation for alpha-1 antitrypsin deficiency (A1ATD) using a heterozygous donor: Outcomes and review of the literature.
Pediatr Transplant. 2023;27:e14488. doi:10.1111/petr.14488.
A: Reduction of portal hyperperfusion to prevent small-for-size syndrome
B: Avoidance of anterior and posterior graft compression in the abdominal cavity
C: Improved biliary drainage through enhanced anatomical alignment
D: Elimination of the need for immunosuppressive therapy
B: Avoidance of anterior and posterior graft compression in the abdominal cavity
Explanation:
Ex situ right posterior sectionectomy (RPS) reduces both graft volume and three-dimensional dimensions (anteroposterior thickness and cranio-caudal length), facilitating a better fit within the recipient’s abdominal cavity. This adjustment minimizes the risk of LFS complications such as compression-related vascular and biliary dysfunction. While portal hyperperfusion is relevant in small-for-size grafts, RPS addresses mismatches caused by excessive graft size. Immunosuppressive therapy remains necessary in all transplant cases, regardless of graft modifications.
Reference:
Rossignol G, Muller X, Dubois R, et al.
Optimizing graft-recipient size matching in adolescent liver transplantation: Don't forget ex situ right posterior sectionectomy.
Pediatr Transplant. 2023;27:e14510. doi:10.1111/petr.14510.
A: Tacrolimus-induced Th1 to Th2 immune shift
B: Persistent Epstein–Barr virus (EBV) viremia
C: Chronic allograft rejection with mucosal inflammation
D: Dietary allergens triggering systemic eosinophilia
A: Tacrolimus-induced Th1 to Th2 immune shift
Explanation:
Tacrolimus is known to selectively suppress Th1 lymphocytes, which shifts the immune response toward Th2 predominance, promoting atopy and eosinophilic inflammation. This patient exhibited multiple risk factors for de novo atopy post-transplant, including young age, high tacrolimus exposure, and elevated peripheral eosinophil counts. While dietary triggers and EBV can exacerbate eosinophilic inflammation, they were not primary factors in this case. Reduction of tacrolimus successfully controlled airway pathology but resulted in graft rejection, highlighting the challenge of balancing immunosuppression.
Reference:
Rajasekaran V, McCaffer C, Bishop J, et al.
Late airway complications following pediatric liver transplantation: A case series.
Pediatr Transplant. 2023;27:e14473. doi:10.1111/petr.14473.
A: Improved vascular anastomosis through robotic assistance
B: Enhanced cosmetic outcomes with reduced wound complications
C: Complete elimination of intraoperative bleeding risk
D: Increased graft perfusion due to pneumoperitoneum
B: Enhanced cosmetic outcomes with reduced wound complications
Explanation:
The hybrid approach for LDLT combines laparoscopic mobilization with a limited midline incision, avoiding extensive abdominal muscle disruption. This results in lower rates of wound-related complications and improved cosmetic outcomes, particularly beneficial in pediatric patients. While the procedure does not eliminate bleeding risks or involve robotic assistance, it provides a balance between minimally invasive techniques and surgical precision.
Reference:
Kosaka T, Soyama A, Fujita T, et al.
A hybrid procedure of living donor liver transplantation for a pediatric patient with citrin deficiency.
Pediatr Transplant. 2023;27:e14485. doi:10.1111/petr.14485.
A: Chronic cholangitis due to recurrent biliary infections post-LT
B: Impaired bile flow in the Roux-en-Y afferent limb due to poor peristalsis
C: Absence of prophylactic antibiotics post-LT for enteric reconstruction
D: Long-term use of tacrolimus leading to biliary stasis
B: Impaired bile flow in the Roux-en-Y afferent limb due to poor peristalsis
Explanation:
The Roux-en-Y reconstruction used during LT creates an afferent limb that may become a blind loop with low peristaltic activity, predisposing to bile stasis and stone formation. Over time, deposition of bile constituents, combined with bacterial infection, promotes the growth of pigment stones. The patient did not have a history of recurrent cholangitis or antibiotic prophylaxis issues. While tacrolimus may influence bile flow, it is not a primary factor in this context.
Komine R, Sakamoto S, Uchida H, et al.
Gallstone ileus at 17 years after living donor liver transplantation: A case report.
Pediatr Transplant. 2023;27:e14517. doi:10.1111/petr.14517.
A: Granulomatous inflammation caused by impaired lysosomal function in macrophages
B: Excessive T-cell activation leading to mass-forming lesions in immunodeficient hosts
C: Chronic fibrosis due to prolonged bacterial antigen exposure in transplant recipients
D: Autoimmune destruction of tissue macrophages by anti-phagocytic antibodies
A: Granulomatous inflammation caused by impaired lysosomal function in macrophages
Explanation:
Malakoplakia results from defective macrophage bactericidal activity due to low cyclic guanosine monophosphate (cGMP) levels, impairing lysosomal function. This defect allows bacteria to persist within macrophages, leading to the deposition of calcium and iron, forming Michaelis-Gutmann bodies, which are pathognomonic for the condition. Treatment includes prolonged targeted antibiotic therapy guided by culture and susceptibility, as surgical options may not always be feasible in immunocompromised patients.
Reference:
Gerard A, Mesa H, Danziger-Isakov L, et al.
Successful treatment of malakoplakia of the liver and skin in a pediatric liver transplant patient.
Pediatr Transplant. 2023;27:e14492. doi:10.1111/petr.14492.
A: Prevent exacerbation of bone marrow failure by preexisting liver disease
B: Minimize the risk of conditioning regimen-induced liver graft failure
C: Facilitate earlier engraftment by improving nutritional status
D: Avoid the need for immunosuppressive therapy during HSCT
B: Minimize the risk of conditioning regimen-induced liver graft failure
Explanation:
In patients with FA and concurrent severe liver disease, LT is prioritized to stabilize hepatic function before subjecting the patient to HSCT. The conditioning regimens required for HSCT can exacerbate preexisting liver disease, leading to potentially fatal complications. By resolving the hepatic dysfunction first, the patient can tolerate HSCT with reduced risk of liver-related morbidity. This strategy reflects a multidisciplinary approach to managing complex comorbidities in FA.
Reference:
Di Stasio F, Bravi M, Bonanomi S, et al.
Successful sequential liver and hematopoietic stem cell transplantation in a patient with Fanconi anemia.
Pediatr Transplant. 2023;27:e14503. doi:10.1111/petr.14503.
A: Direct antiviral activity against HHV8
B: Immunosuppression with antineoplastic properties
C: Induction of T-cell tolerance to HHV8
D: Restoration of natural killer cell function
B: Immunosuppression with antineoplastic properties
Explanation:
Sirolimus is an mTOR inhibitor with dual effects: immunosuppression to prevent organ rejection and antineoplastic activity that inhibits angiogenesis and tumor cell proliferation. These properties are particularly beneficial in managing KS, a tumor linked to HHV8 infection and immunosuppression. Unlike tacrolimus, sirolimus reduces the risk of KS progression without compromising graft function.
Reference:
Cordeiro C, Ferreira S, Nobre S, et al.
Kaposi sarcoma in three pediatric liver transplantation recipients.
Pediatr Transplant. 2023;27:e14469. doi:10.1111/petr.14469.
A: Use of a covered stent with controlled expansion to minimize hepatic encephalopathy risk
B: Recanalization of intrahepatic and extrahepatic portal veins using transhepatic access
C: Surgical intervention to bypass thrombosed portal segments prior to TIPS
D: Administration of long-term anticoagulation to prevent recurrent thrombosis
A: Use of a covered stent with controlled expansion to minimize hepatic encephalopathy risk
Explanation:
Using a controlled-expansion polytetrafluoroethylene (PTFE) stent allowed for precise regulation of shunt diameter, reducing the risk of hepatic encephalopathy by limiting excessive portosystemic shunting. The trans splenic approach enabled effective recanalization of thrombosed portal veins, facilitating TIPS placement. The minimally invasive procedure provided a critical alternative to surgical shunting, which carries higher morbidity. Long-term anticoagulation, though important, was a secondary consideration after the immediate resolution of GI bleeding.
Reference:
de Assis AM, de Carvalho Melo JA Jr, Kawakami WY, et al.
Life-threatening variceal bleeding after liver transplantation and extensive portal vein thrombosis: Desperate times call for desperate measures.
Pediatr Transplant. 2023;27:e14555. doi:10.1111/petr.14555
A: Enhanced contrast of the bile duct anatomy through direct injection into the biliary system
B: Real-time visualization of bile excretion via near-infrared fluorescence following hepatic clearance
C: Identification of vascular perfusion abnormalities associated with the leak site
D: Visualization of bile leaks using radiotracer uptake within the biliary tree
B: Real-time visualization of bile excretion via near-infrared fluorescence following hepatic clearance
Explanation:
ICG fluorescence imaging involves intravenous administration of ICG, which is exclusively taken up by hepatocytes and excreted into the bile. Using near-infrared fluorescence, ICG enables real-time visualization of bile excretion, allowing precise localization of leaks. In this case, it proved invaluable in identifying the small bile leak along the cut liver surface when conventional visual inspection under magnification failed. This technology does not directly visualize bile duct anatomy or use radiotracer uptake as in HIDA scans.
Reference:
Lemoine CP, Lautz TB, Superina R.
Indocyanine green fluorescence imaging as an adjunct for the localization of a bile leak after split liver transplantation.
Pediatr Transplant. 2023;27:e14431. doi:10.1111/petr.14431.
A: Availability of a size-matched graft for vascular reconstruction
B: Enhanced ability to treat systemic complications of IMTs
C: Ability to achieve complete tumor resection with free margins
D: Potential for spontaneous tumor resolution without surgery
C: Ability to achieve complete tumor resection with free margins
Explanation:
LDLT was selected to enable radical tumor resection and ensure free margins, critical for a favorable prognosis in IMTs, especially in cases involving critical structures like the hepatic hilum and IVC. The tumor’s benign nature and lack of effective chemotherapy options further supported surgical intervention. The success of the procedure also depended on complex vascular reconstruction, highlighting the role of LDLT as a curative strategy for advanced hilar IMTs.
Reference:
Costa CM, Neto JS, Benavidez MR, et al.
Liver transplantation for hilar inflammatory myofibroblastic tumor: Case report and review of the literature.
Pediatr Transplant. 2023;27:e14445. doi:10.1111/petr.1
A: High-dose sedation to reduce stress and oxygen demand
B: Frequent use of neuromuscular blockade to facilitate ECMO management
C: Implementation of early rehabilitation with minimal sedation
D: Complete bed rest to prevent further lung injury
C: Implementation of early rehabilitation with minimal sedation
Explanation:
Early rehabilitation and physical activity during ECMO, with optimized sedation protocols, help maintain physical conditioning and reduce deconditioning, which are critical for improving pre-transplant status and post-transplant outcomes. This approach requires a multidisciplinary team and family involvement. High-dose sedation or bed rest can lead to significant deconditioning, worsening outcomes. Neuromuscular blockade may only be used in specific situations and is not standard for optimizing outcomes.
Reference:
Herrera-Camino A, Sweet SC, Pendino R, et al.
Lung Re-transplantation after prolonged veno-venous extracorporeal membrane oxygenation (ECMO) in a child with chronic lung allograft dysfunction.
Pediatric Transplantation. 2024;28:e14579. doi:10.1111/petr.14579.
A: Conservative monitoring with serial imaging
B: High-dose corticosteroids to reduce inflammation
C: Immediate surgical exploration and repositioning of the herniated lung
D: Bronchoscopic airway stenting to alleviate compression
C: Immediate surgical exploration and repositioning of the herniated lung
Explanation:
ung herniation following HLT is a rare but potentially life-threatening complication due to the risk of vascular compromise and airway obstruction. Early recognition through imaging and prompt surgical correction is essential to prevent irreversible damage and maintain lung function. Conservative measures or non-surgical options are not sufficient in cases involving structural displacement.
Reference:
Kim D, Choi KH, Kim H, Lee JH, Kim Y, Byun J-H.
Right lower lung midline herniation as a rare complication in an infant with heart-lung transplantation: A case report.
Pediatric Transplantation. 2024;28:e14656. doi:10.1111/petr.14656.
A: The patient's age at the time of transplantation
B: The presence of pre-existing colonization by atypical fungi
C: Immediate onset of hypoxic episodes post-transplant
D: Resistance of Irpex lacteus to most antifungal agents
B: The presence of pre-existing colonization by atypical fungi
Explanation:
In immunocompromised patients such as lung transplant recipients, pre-existing colonization with atypical fungi like *Irpex lacteus* can progress to invasive fungal disease (IFD), particularly when coupled with declining clinical status and immunosuppressive therapy. Though initially asymptomatic, worsening respiratory function and imaging findings should prompt consideration for antifungal treatment. The risk of IFD must be balanced against potential treatment side effects.
Reference:
Atwood DT, Köhler JR, Vargas SO, et al.
Identification of Irpex and Rhodotorula on surveillance bronchoscopy in a pediatric lung transplant recipient: A case report and review of literature of these atypical fungal organisms.
Pediatric Transplantation. 2024;28:e14759. doi:10.1111/petr.14759.
A: It promotes rapid resolution of bowel obstructions.
B: It decreases TGF-β production, reducing fibrotic processes.
C: It acts as a direct anti-inflammatory agent in peritoneal tissue.
D: It enhances the immunosuppressive effect of corticosteroids.
B: It decreases TGF-β production, reducing fibrotic processes.
Explanation:
Tamoxifen is a selective estrogen receptor modulator that has shown benefits in EPS by inhibiting TGF-β production, a key mediator in fibrotic changes within the peritoneum. This helps control the fibrotic process and mitigate encapsulation. The use of tamoxifen in conjunction with steroids targets both the inflammatory and fibrotic components of EPS.
Reference:
Al Riyami MS, Altalebi A, Al Hashmi S, et al.
Improvement of encapsulating peritoneal sclerosis after medical treatment and successful deceased donor kidney transplant in a child: A case report.
Pediatric Transplantation. 2024;28:e14867. doi:10.1111/petr.14867.
A: They reduce the likelihood of surgical infection.
B: They allow extension of short renal veins, minimizing dissection.
C: They eliminate the need for post-operative immunosuppression.
D: They enable the use of arterial clamps for better control during surgery.
B: They allow extension of short renal veins, minimizing dissection.
Explanation:
In pediatric kidney transplantation involving anatomical challenges like short renal veins, utilizing iliac vein grafts can extend the renal veins effectively. This minimizes the extent of surgical dissection required, preventing transplant-related bleeding and lymphatic complications while facilitating a secure anastomosis. The technique supports graft function without increasing the risk of venous thrombosis or technical complications.
Reference:
Lledo E, Tabbara MM, Alvarez A, et al.
Venous vascular reconstruction of a robotically procured right kidney with two renal veins transplanted into a pediatric recipient.
Pediatric Transplantation. 2024;28:e14646. doi:10.1111/petr.14646.
A: Direct cytotoxicity of anti-A antibodies on intestinal endothelial cells
B: Activation of complement-mediated vascular thrombosis in mesenteric veins
C: Disseminated intravascular coagulation (DIC) triggered by severe hemolysis
D: Direct invasion of the bowel wall by donor-derived lymphocytes
C: Disseminated intravascular coagulation (DIC) triggered by severe hemolysis
Explanation:
Passenger lymphocyte syndrome (PLS) involves hemolytic anemia caused by donor-derived lymphocytes producing antibodies against recipient red blood cell antigens. In this case, hemolysis and a hypercoagulable state led to DIC, which contributed to venous-origin ischemic colitis. This complication emphasizes the systemic effects of severe hemolysis in PLS. While anti-A antibodies may activate complement, no direct cytotoxic or invasive mechanisms were observed in the colon.
Reference:
Yeom GE, Lim SH, Kim JH, et al.
Gastrointestinal involvement of passenger lymphocyte syndrome followed by minor ABO-incompatible renal transplantation: A case report.
Pediatr Transplant.. 2023;27:e14556. doi:10.1111/petr.14556.
A: Prevention of extra-renal complications, such as vascular abnormalities
B: Resolution of defective cellular immunity while maintaining graft function
C: Enhanced renal allograft survival through improved immune tolerance
D: Elimination of the need for lifelong immunosuppressive therapy
B: Resolution of defective cellular immunity while maintaining graft function
Explanation:
Sequential HSCT after KT addresses the underlying cellular immune deficiency in SIOD, reducing the risk of recurrent infections and cytopenias while preserving kidney graft function. HSCT replenishes hematopoietic and immune systems, offering systemic benefits for this multisystem disease. Although it may stabilize some extra-renal complications, it primarily targets the immunological dysfunction, without eliminating the need for immunosuppression entirely.
Reference:
Woo HA, Kim SH, Ahn YH, et al.
Clinical course of post-kidney transplant Schimke immuno-osseous dysplasia.
Pediatr Transplant. 2023;27:e14605. doi:10.1111/petr.14605.
A: T-cell depletion due to anti-thymocyte globulin
B: Persistent hypogammaglobulinemia after rituximab therapy
C: Subtherapeutic tacrolimus levels early post-transplant
D: Absence of prophylactic antibiotics targeting atypical organisms
B: Persistent hypogammaglobulinemia after rituximab therapy
Explanation:
The patient’s prior rituximab therapy resulted in prolonged hypogammaglobulinemia, leaving her vulnerable to infections with fastidious organisms like Ureaplasma parvum. Although ATG and tacrolimus contributed to immmunosuppression, her inability to recover adequate humoral immunity was the key predisposing factor. Prophylactic antibiotics do not routinely cover Ureaplasma, and the infection's onset was not related to early subtherapeutic tacrolimus levels.
Reference:
Schwartz S, Aldrich A, Kessler E, et al.
Disseminated Ureaplasma polyarthritis in a renal transplant recipient
Pediatr Transplant. 2023;27:e14538. doi:10.1111/petr.14538.
A: Migration of the stent causing renal vein thrombosis
B: Acute ureteral obstruction due to clot formation in the collecting system
C: Increased venous pressure leading to renal artery compression
D: Hemodynamic instability from excessive blood loss during stent deployment
B: Acute ureteral obstruction due to clot formation in the collecting system
Explanation:
Acute anuria in this patient was caused by obstruction of the renal pelvis due to clot formation, a known complication of stent placement. This may result from venous stasis or mechanical irritation during the procedure. The placement of a JJ stent effectively relieved the obstruction, restoring urine output and preventing further graft damage. While venous pressure changes or stent migration can complicate such interventions, these were not observed in this case.
Reference:
Reis J, Bogart AM, Healey PJ, et al.
Transplant renal vein stent placement complicated by obstructive hematuria: A case report.
Pediatr Transplant. 2023;27:e14607. doi:10.1111/petr.14607.
A: Direct cytopathic effect of CMV in glomerular epithelial cells
B: CMV-mediated immune activation leading to podocyte injury
C: Graft dysfunction secondary to CMV-induced vasculitis
D: Reactivation of latent lupus nephritis in the graft
B: CMV-mediated immune activation leading to podocyte injury
Explanation:
CMV-associated collapsing FSGS is proposed to occur through immune-mediated mechanisms rather than direct viral invasion, as suggested by negative CMV staining and absence of viral inclusions on biopsy. CMV infection triggers systemic immune activation, leading to cytokine release and podocyte injury, culminating in collapsing FSGS. This mechanism differs from recurrent lupus nephritis, which was excluded in this case. Timely antiviral therapy and adjusted immunosuppression were pivotal in reversing renal dysfunction.
Reference:
Parr MFE, Hidalgo G, Goldstein MJ, et al.
CMV-associated collapsing focal segmental glomerulosclerosis after kidney transplant in a pediatric patient
Pediatr Transplant. 2023;27:e14535. doi:10.1111/petr.14535.
A: Advanced immunosuppressive protocols preventing rejection
B: High regenerative capacity of pediatric kidneys with acute injury
C: Routine use of en-bloc transplantation to improve outcomes
D: Pre-transplant histological evaluation of all pediatric grafts
B: High regenerative capacity of pediatric kidneys with acute injury
Explanation:
Pediatric kidneys have a remarkable regenerative potential due to their lack of chronic structural damage and high cellular adaptability. These features enable successful transplantation even when donors exhibit acute kidney injury (AKI), including anuric states. Selecting low-risk adult recipients further enhanced outcomes. The kidneys were transplanted as single grafts rather than en-bloc, demonstrating their capacity for functional recovery despite AKI. Pre-transplant histological evaluation was not performed, highlighting the reliance on clinical judgment and donor history.
Reference:
Koch M, Kraus D, Boedecker-Lips S, et al.
Successful transplantation of four kidney grafts from two small pediatric donors with anuric acute renal failure into adult recipients.
Pediatr Transplant. 2023;27:e14542. doi:10.1111/petr.14542.
A: Suboptimal prophylaxis with TMP-SMX during the first 6 months post-transplant
B: Ongoing immunosuppressive therapy combined with eculizumab for aHUS
C: CMV co-infection leading to opportunistic fungal proliferation
D: Pre-existing obstructive lung disease misdiagnosed as asthma
B: Ongoing immunosuppressive therapy combined with eculizumab for aHUS
Explanation:
This patient’s immunosuppressive regimen, including tacrolimus, mycophenolate mofetil, prednisone, and eculizumab (a complement inhibitor), created a highly immunosuppressed state, predisposing him to late-onset PJP. Although TMP-SMX was appropriately used for prophylaxis during the initial 6 months, long-term immunosuppression significantly increased the risk of opportunistic infections. CMV infection was ruled out, and his restrictive lung pattern was secondary to PJP, not asthma.
Reference:
Grewal M, Srivastava R, Ang JY, et al.
Unique presentation of late-onset Pneumocystis pneumonia in a pediatric kidney transplant recipient.
Pediatr Transplant. 2023;27:e14576. doi:10.1111/petr.14576.
A: Enhanced prevention of opportunistic infections and malignancies
B: Improved graft perfusion through reduced endothelial damage
C: Elimination of the need for antiviral prophylaxis post-transplant
D: Rapid recovery of naïve T-cell populations after transplantation
A: Enhanced prevention of opportunistic infections and malignancies
Explanation:
Patients with SIOD have a predisposition to T-cell deficiency, increasing their risk for opportunistic infections and post-transplant lymphoproliferative disease (PTLD). The use of a low-dose immunosuppressive regimen, including tacrolimus monotherapy, minimizes immunosuppression-related complications while maintaining graft function. This approach is critical in managing patients with SIOD, where standard immunosuppressive regimens can significantly elevate morbidity and mortality risks.
Reference:
Finsen SH, Tepel M, Neland M, et al.
Successful low-dose immunotherapy after kidney transplantation in a 10-year-old girl with Schimke immuno-osseous dysplasia
Pediatr Transplant. 2023;27:e14495. doi:10.1111/petr.14495.
A: Direct inhibition of T-cell-mediated autoimmunity
B: Neutralization of circulating nephrin autoantibodies
C: Depletion of long-lived plasma cells producing pathogenic antibodies
D: Enhanced removal of immune complexes via complement activation
C: Depletion of long-lived plasma cells producing pathogenic antibodies
Explanation:
Daratumumab targets CD38, a surface marker on plasma cells, leading to their depletion and reducing the production of long-lived pathogenic antibodies. This approach is particularly effective in cases like post-transplant SRNS where conventional therapies fail. Plasma cells are key contributors to the production of autoantibodies, such as those targeting nephrin in INS, and their depletion significantly improves outcomes. This targeted therapy is distinct from the effects of traditional immunosuppression and B-cell depletion alone.
Reference:
Delbet J-D, Hogan J, Parmentier C, et al.
Successful global anti-B-cell strategy with daratumumab in a patient with post-transplant nephrotic syndrome recurrence unresponsive to immunoadsorption and obinutuzumab
Pediatr Transplant. 2023;27:e14544. doi:10.1111/petr.14544
A: Direct inhibition of complement activation at the C1q level
B: Suppression of plasma cell antibody production through proteasome inhibition
C: Blocking of cytokine signaling in memory T cells
D: Elimination of alloreactive T-cell populations via apoptosis induction
B: Suppression of plasma cell antibody production through proteasome inhibition
Explanation:
Carfilzomib, a second-generation proteasome inhibitor, reduces antibody production by targeting plasma cells, which are responsible for the persistence of DSAs in AMR. This approach is particularly useful in cases refractory to first-line treatments like bortezomib due to its higher specificity and reduced neuropathy risk. While it does not directly block complement or target memory T cells, its efficacy lies in disrupting the pathogenic antibody response central to AMR.
Reference:
Cody EM, Varnell C Jr, Lazear D, et al.
Carfilzomib-based antibody mediated rejection therapy in pediatric kidney transplant recipients.
Pediatr Transplant. 2023;27:e14534. doi:10.1111/petr.14534.
A: Inadequate immunosuppression post-transplant
B: Development of HLA donor-specific antibodies
C: Heterologous immunity due to prior viral infections
D: Graft rejection due to ABO incompatibility
C: Heterologous immunity due to prior viral infections
Explanation:
The patient’s history of COVID-19 infection and seropositivity for multiple viruses likely contributed to the activation of memory T cells with cross-reactivity to alloantigens, a phenomenon known as heterologous immunity. This may have exacerbated the alloimmune response and contributed to the rapid onset of rejection. Additionally, high levels of non-HLA autoantibodies (AT1R and ETAR) likely aggravated the rejection process. There were no donor-specific HLA antibodies or ABO incompatibility identified, and immunosuppression protocols were appropriately intensified to manage the rejection.
Reference:
Al Attas RA, Alshami A, Mohamed N, et al.
Severe delayed graft function in a living-related kidney transplant recipient due to combination of alloimmunity, autoimmunity, and heterologous immunity: A case report.
Pediatr Transplant. 2023;27:e14424. doi:10.1111/petr.14424.
A: Pre-operative use of reversal agents for anticoagulation
B: Implementation of extracorporeal heart preservation for prolonged ischemic times
C: Routine use of enoxaparin instead of apixaban for thrombosis prevention
D: Avoidance of antiviral treatment for post-transplant infections
B: Implementation of extracorporeal heart preservation for prolonged ischemic times
Explanation:
The use of the extracorporeal organ care system (OCS) was essential in maintaining the viability of the donor heart during the extended travel time, reducing cold ischemic time to 51 minutes and supporting warm perfusion for 428 minutes. This approach, combined with careful donor evaluation and antiviral treatment post-transplant, allowed successful outcomes despite donor HCV status and size mismatch concerns.
Reference:
Torpoco Rivera DM, Hollander SA, Almond C, et al.
An integrated program to expand donor utilization in pediatric heart transplantation: Case report of successful transplant with multiple donor risk factors.
Pediatric Transplantation. 2024;28:e14584. doi:10.1111/petr.14584.
A: It eliminates the need for antiviral therapy.
B: It expands the donor pool, reducing waitlist mortality.
C: It increases immunity against HCV-related complications post-transplant.
D: It decreases the risk of coronary allograft vasculopathy.
B: It expands the donor pool, reducing waitlist mortality.
Explanation:
Utilizing HCV NAT-positive donor hearts, combined with prompt initiation of direct-acting antiviral (DAA) therapy, safely broadens the donor pool and reduces waitlist times in pediatric heart transplantation. While antiviral therapy is required, studies show excellent outcomes with DAAs, enabling HCV-negative recipients to maintain good graft function and viral suppression. This approach is essential in high-risk cases with limited donor availability.
Reference:
Kushner LE, Puckett L, Lee J, et al.
Utilization of Hepatitis C Virus-Infected Donor Hearts in Two Children and Two Young Adults: Initial Experience at a Pediatric Transplant Center.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14879.
A: Direct cytotoxic effect on EBV-positive B cells
B: B-cell depletion through CD20 binding
C: Stimulation of EBV-specific T cells to target infected cells
D: Enhancement of systemic antibody production against EBV
C: Stimulation of EBV-specific T cells to target infected cells
Explanation:
EBV-directed T-cell therapy provides cytotoxic T cells that specifically recognize and eliminate EBV-infected B cells, addressing cases of rituximab-refractory PTLD. This therapy aims to restore immune surveillance against EBV by delivering T cells programmed to target EBV antigens, thus reducing viral burden and lymphoma progression in immunosuppressed patients.
Reference:
Work E, Gupta D, Slayton WB, et al.
Epstein Barr virus-directed T-cell therapy for refractory EBV-PTLD in a toddler post Orthotopic heart transplantation.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14707.
A: Reduced risk of transplant-related complications in neuromuscular disorders
B: Stable skeletal and respiratory muscle function over time
C: Immediate reversal of restrictive pulmonary syndrome post-transplant
D: High likelihood of spontaneous cardiac recovery in congenital myopathies
B: Stable skeletal and respiratory muscle function over time
Explanation:
In patients with neuromuscular disorders, heart transplantation is considered when the cardiac phenotype is disproportionately severe compared to skeletal and respiratory muscle involvement. Stable neuromuscular function supports the decision to transplant, as progressive weakness or respiratory insufficiency could limit post-transplant outcomes. This patient demonstrated stable motor development despite significant cardiac dysfunction, making him a suitable candidate. Transplant-related complications and spontaneous cardiac recovery are less predictable and were not central to the decision-making process.
Reference:
Wacker J, Di Bernardo S, Lobrinus JA, et al.
Successful heart transplant in a child with congenital core myopathy and delayed-onset restrictive cardiomyopathy due to recessive mutations in the titin (TTN) gene.
Pediatr Transplant. 2023;27:e14561. doi:10.1111/petr.14561.
A: Subclinical tuberculosis reactivation due to immunosuppression
B: Inadequate pre-transplant screening for latent tuberculosis
C: Delayed initiation of corticosteroid therapy for neuroinflammation
D: Use of rifampicin interfering with tacrolimus metabolism
A: Subclinical tuberculosis reactivation due to immunosuppression
Explanation:
Immunosuppression following solid organ transplantation, particularly with calcineurin inhibitors like tacrolimus, significantly increases the risk of reactivating latent tuberculosis, leading to opportunistic infections such as cerebral tuberculoma. While pre-transplant latent tuberculosis screening is essential, this patient had no reported history or screening for latent TB. Rifampicin was managed effectively with tacrolimus dose adjustments, minimizing interactions. The presentation and progression underscore the need for high clinical suspicion and rapid treatment initiation.
Reference:
Oliveira PC, Corbi MJA, Siqueira AWS, et al.
Brain tuberculoma in a pediatric heart transplant recipient.
Pediatr Transplant. 2023;27:e14496. doi:10.1111/petr.14496.
A: Prevention of coronary allograft vasculopathy
B: Enabling physical and nutritional rehabilitation as a bridge to transplant
C: Restoration of aortic valve function in the setting of severe dilation
D: Long-term cardiac support eliminating the need for transplantation
B: Enabling physical and nutritional rehabilitation as a bridge to transplant
Explanation:
The use of BiVAD support provided critical circulatory stabilization and facilitated extensive nutritional and physical rehabilitation, which were essential for improving candidacy for heart transplantation in this patient with neonatal Marfan syndrome. This strategy addressed the severe heart failure and multisystem deconditioning commonly associated with this condition, enabling a successful transplant. While BiVAD did not directly address CAV or long-term cardiac function, it served as an effective bridge to transplantation.
Reference:
Laks JA, Lynch A, Honjo O, et al.
Heart transplantation in neonatal Marfan syndrome: Saving life in a rare and fatal condition.
Pediatr Transplant. 2023;27:e14560. doi:10.1111/petr.14560.
A: Direct eradication of gastrointestinal pathogens
B: Inhibition of IL-2 receptor signaling on activated T cells
C: Suppression of tumor necrosis factor-alpha (TNF-α) activity
D: Reduction of intestinal permeability through epithelial repair
B: HSCT provides curative potential for refractory lymphomas with a possible underlying immune deficiency.
Explanation:
HSCT is considered in pediatric cases of refractory or recurrent lymphoma, particularly when there is an aggressive disease course and potential immunodeficiency. It allows for immune reconstitution and eradication of lymphoma cells through graft-versus-lymphoma effects. In this case, the patient’s lymphoma did not respond to conventional chemotherapy, and HSCT offered curative potential.
Reference:
Memon S, Almudayfir Z, Elbashir E, et al.
A Rare Case of Refractory Nodal Marginal Zone Lymphoma in a Child, Successfully Cured by Allogeneic Stem Cell Transplant.
Pediatric Transplantation. . 2024;28. doi:10.1111/petr.14835.
A: NMZL typically resolves with HSCT in adults, so it was used in this pediatric case.
B: HSCT provides curative potential for refractory lymphomas with a possible underlying immune deficiency.
C: HSCT is effective in all cases of pediatric non-Hodgkin lymphoma (NHL), regardless of disease type.
D: HSCT allows the reduction of chemotherapy in pediatric patients with nodal lymphomas.
B: Inhibition of IL-2 receptor signaling on activated T cells
Explanation:
Basiliximab is a chimeric monoclonal antibody targeting the CD25 subunit of the IL-2 receptor on activated T cells, thereby reducing T-cell activation and proliferation. In this patient, basiliximab addressed the immune dysregulation underlying her bowel disease, which was refractory to conventional treatments. While it does not target pathogens directly or TNF-α, its ability to modulate T-cell responses made it effective in managing her condition.
Reference:
Kiskaddon AL, Wilsey M, Gonzalez-Gomez I, et al.
Basiliximab therapy for immune-mediated bowel disease in a pediatric heart transplant patient.
Pediatr Transplant. 2023;27:e14443. doi:10.1111/petr.14443.
A: Complete remission without any adverse events.
B: Initial remission followed by severe graft-versus-host disease.
C: Complete remission with manageable Grade 1 cytokine release syndrome (CRS).
D: Partial remission with severe multiorgan failure.
E: No response, leading to disease progression.
C: Complete remission with manageable Grade 1 cytokine release syndrome (CRS).
Explanation:
In the presented case, the patient achieved complete remission after CD7 CAR T-cell therapy, which served as a successful bridge to a second allo-HSCT. The treatment led to Grade 1 CRS, characterized by mild symptoms such as fever, but these were managed effectively without severe complications. This approach provided durable remission and a significant survival benefit.
Reference:
Fanqiao M, Chen X, Ren X, Li L, Wu T.
CD7 CAR T bridging to allo-HSCT in R/R T-ALL: A case report.
Pediatric Transplantation. . 2024;28. doi:10.1111/petr.14367.
A: Acute intestinal obstruction
B: Sepsis-related shock
C: Severe food protein-induced enterocolitis syndrome (FPIES)
D: GVHD recurrence involving the gut
C: Severe food protein-induced enterocolitis syndrome (FPIES)
Explanation:
Severe FPIES is a non–IgE-mediated food allergy that can present with vomiting, diarrhea, lethargy, and hypotension, often mistaken for sepsis. In this case, the patient experienced these symptoms post-HCT, unrelated to infections or GVHD recurrence. Awareness of FPIES as a differential diagnosis in post-transplant patients with immune dysregulation is essential for reducing morbidity.
Reference:
Alonso García L, Panesso Romero M, García Macías E, Segarra Cantón O, Diaz de Heredia Rubio C.
Severe food protein-induced enterocolitis syndrome after hematopoietic stem cell transplantation: Pediatric case report.
Pediatric Transplantation. . 2024;28:e14810. doi:10.1111/petr.14810.
A: It directly reverses renal tubular acidosis by correcting CA2 enzyme deficiency.
B: It halts osteopetrosis progression by providing functional osteoclasts derived from donor cells.
C: It promotes rapid cerebral calcification resolution in patients with CA2 mutations.
D: It eliminates the need for immunosuppressive therapy in osteopetrosis.
B: It halts osteopetrosis progression by providing functional osteoclasts derived from donor cells.
Explanation:
HSCT is essential for treating osteopetrosis associated with CA2 deficiency as it enables the generation of functional osteoclasts, which can restore normal bone resorption and prevent further skeletal abnormalities. Although HSCT does not fully resolve RTA, it significantly improves bone density and reduces disease symptoms. This approach is critical for patients with severe, progressive forms of osteopetrosis.
Reference:
Shamsian BS, Momtazmanesh N, Saneifard H, et al.
Allogenic hematopoietic stem cell transplantation in an Iranian patient with osteopetrosis caused by carbonic anhydrase II deficiency: A case report.
Pediatric Transplantation. 2024;28. doi:10.1111/petr.14689.
A: Complete eradication of central nervous system metastases
B: Sequential dose escalation with distinct chemotherapeutic agents
C: Preservation of functional vision in bilateral retinoblastoma
D: Long-term control of extraocular disease with minimal toxicity
B: Sequential dose escalation with distinct chemotherapeutic agents
Explanation:
Tandem HDC-ASCT allows for increased chemotherapy intensity by delivering sequential high-dose regimens using different agents, which enhances tumor control in high-risk pediatric cancers like trilateral retinoblastoma. While it does not guarantee CNS metastasis eradication or vision preservation, the approach improves disease-free survival compared to single HDC. Relapse in this patient highlights the need for multimodal therapy and underscores the aggressive nature of trilateral retinoblastoma.
Reference:
Toret E, Ozdemir ZC, Zengin Ersoy G, et al.
Tandem high-dose chemotherapy followed by autologous stem cell transplantation: An infant with trilateral retinoblastoma.
Pediatr Transplant. 2023;27:e14504. doi:10.1111/petr.14504.
A: Insufficient antibody production following the varicella vaccine
B: Short interval between live vaccine administration and immunosuppression
C: Delayed initiation of antiviral prophylaxis post-transplant
D: Lack of protective T-cell immunity against wild-type varicella zoster virus
B: Short interval between live vaccine administration and immunosuppression
Explanation:
The early initiation of intensive chemotherapy only 20 days after the live-attenuated varicella vaccine likely impaired the development of a robust immune response, predisposing the patient to vaccine strain reactivation. Prophylactic antiviral treatment was not initiated earlier because the patient met criteria for starting antivirals only post-HSCT. T-cell-mediated immunity plays a critical role in controlling varicella reactivation, but the timing of immunosuppression in this case disrupted the balance, leading to complications.
Reference:
Coralie R, Chebel Z, Renaud C, et al.
Varicella vaccine meningoencephalitis in a child receiving autologous bone marrow transplantation.
Pediatr Transplant. 2023;27:e14562. doi:10.1111/petr.14562.
A: Myeloablative conditioning using busulfan and fludarabine
B: Use of rituximab for B-cell depletion in conditioning
C: Switching from sirolimus to cyclosporine A and later to MMF
D: Extracorporeal photopheresis for chronic GVHD
B: Use of rituximab for B-cell depletion in conditioning
Explanation:
Rituximab, an anti-CD20 monoclonal antibody, was used to deplete B cells, which not only helped control her pre-HSCT lymphoma but also reduced the risk of cGVHD by targeting donor B cells that can contribute to alloreactivity. Although other interventions, such as adjusting immunosuppression and ECP, were pivotal in managing GVHD, rituximab's inclusion in the conditioning regimen played a central role in balancing disease control and GVHD risk in this high-risk STK4-deficient patient.
Reference:
Uygun V, Keleş S, Daloğlu H, et al.
Hematopoietic stem cell transplantation in serine/threonine kinase 4 (STK4) deficiency: Report of two cases and literature review.
Pediatr Transplant. 2023;27:e14439. doi:10.1111/petr.14439.
A: Pancreas transplantation eliminates the autoimmune process of type 1 diabetes.
B: It provides endogenous insulin secretion, avoiding hypersensitivity reactions caused by exogenous insulin components.
C: Immunosuppression directly resolves insulin hypersensitivity.
D: Euglycemia post-transplant reduces the burden of chronic rejection and improves pancreatic function.
B: It provides endogenous insulin secretion, avoiding hypersensitivity reactions caused by exogenous insulin components.
Explanation:
This patient's hypersensitivity reactions were linked to preservatives in exogenous insulin formulations, such as polysorbates and metacresol. By providing endogenous insulin secretion, the pancreas transplant resolved her hypersensitivity reactions and stabilized her glycemic control, leading to significant quality-of-life improvements. While the autoimmune process of type 1 diabetes remains active, the transplanted pancreas can function effectively under immunosuppression. Immunosuppression prevents graft rejection but does not directly treat hypersensitivity, and euglycemia does not directly impact chronic rejection risk.
Reference:
Adamusiak AM, Ramanathan K, Moe T, et al.
Effective treatment of diabetes, improved quality of life and accelerated cognitive development after pancreas transplantation in a child with type 1 diabetes and allergy to manufactured insulin preparations.
Pediatr Transplant. 2023;27:e14447. doi:10.1111/petr.14447.
A: Replacement for histological diagnosis of ACR
B: Non-invasive tool for initial detection and response monitoring of ACR
C: Diagnostic confirmation of mesenteric vascular occlusion in transplant rejection
D: Primary imaging modality for viral enteritis in transplant recipients
B: Non-invasive tool for initial detection and response monitoring of ACR
Explanation:
Intestinal ultrasound (IUS) is a promising non-invasive tool for detecting and monitoring acute cellular rejection (ACR) in intestinal transplant recipients. It can identify changes such as bowel wall thickening, increased vascularity, and mesenteric inflammation, which correlate with histological findings. While IUS does not replace biopsy for definitive diagnosis, it provides a practical adjunct for tracking treatment responses, particularly in pediatric patients, reducing the need for repeated invasive procedures.
Reference:
Couper MR, Valamparampil J, Thyagarajan M, et al.
Intestinal ultrasound may be a useful tool in monitoring acute rejection following intestinal transplantation.
Pediatr Transplant. 2023;27:e14574. doi:10.1111/petr.14574.
A: Reduced antigen presentation by donor B cells and monocytes
B: High levels of circulating donor-specific antibodies
C: Persistent donor chimerism in the blood and lymph nodes
D: Complete absence of viral immune responses post-transplant
A: Reduced antigen presentation by donor B cells and monocytes
Explanation:
Operational tolerance was associated with donor-specific hyporesponsiveness in T-cytotoxic memory cells and reduced antigen presentation by donor B cells and monocytes. Additionally, the absence of DSAs and intact immunity to cytomegalovirus (CMV) and Epstein–Barr virus (EBV) supported a balanced immune state. Persistent donor chimerism was not observed in this case, and antiviral immunity was maintained. These findings suggest that reduced antigen presentation and donor-specific T-cell apoptosis contributed significantly to tolerance development.
Reference:
Remaley L, Ashokkumar C, Soltys KA, et al.
Operational tolerance after intestine re-transplantation in childhood and immunological correlates.
Pediatr Transplant. 2023;27:e14455. doi:10.1111/petr.14455.
A: SARS-CoV-2 cannot survive transplantation procedures.
B: SARS-CoV-2 infection in non-lung tissues is non-infectious.
C: SARS-CoV-2 RNA detection does not always indicate active infection.
D: Recipients' immunosuppressive therapies prevent SARS-CoV-2 replication.
C: SARS-CoV-2 RNA detection does not always indicate active infection.
Explanation:
Detection of SARS-CoV-2 RNA in donor tissues or fluids, especially outside the respiratory tract, does not confirm the presence of live, infectious virus. This case demonstrates that pediatric non-lung organ donors with SARS-CoV-2 RNA may not pose a significant risk of transmission to recipients, provided there is no evidence of systemic infection or organ dysfunction. The absence of clinical or laboratory signs of transmission further underscores the importance of careful donor evaluation.
Reference:
Clark JD, et al.
SARS-CoV-2 RNA positive pediatric organ donors: A case report.
Pediatr Transplant. 2023;27:e14452. doi:10.1111/petr.14452.
A: The donor's low Ct value and absence of recent symptoms
B: Pre-transplant SARS-CoV-2 vaccination and recipient antibody positivity
C: Use of IVIg and alemtuzumab to mitigate immune activation
D: Absence of any history of symptomatic COVID-19 in the recipient
B: Pre-transplant SARS-CoV-2 vaccination and recipient antibody positivity
Explanation:
The recipient's vaccination status and pre-existing anti-spike IgG antibodies likely reduced the risk of donor-derived SARS-CoV-2 transmission and severe post-transplant disease. The donor's low viral load (high Ct value) further minimized the risk of transmission. IVIg was administered as a precautionary measure, but the recipient's immunity played a central role in the favorable outcome. This case highlights the importance of assessing donor viral load and recipient immunity when considering organs from SARS-CoV-2-positive donors.
Reference:
Pizzo H, Soni PR, Nadipuram S, et al.
Utilization of SARS-CoV-2-positive donors in pediatric renal transplantation.
Pediatr Transplant. 2023;27:e14451. doi:10.1111/petr.14451.
A: It is recommended for all patients with recurrent FSGS
B: It is only useful in pediatric patients
C: It is useful to differentiate between genetic and non-genetic forms of FSGS
D: It has no role in the management of recurrent FSGS
A: Rituximab is considered the first-line therapy
B: Rituximab should be used only if plasma exchange fails
C: Rituximab is not recommended due to lack of efficacy
D: Rituximab is used in combination with corticosteroids as the initial treatment
A: Every 2 SDS decrease in height is associated with 30% increased risk of mortality.
B: Every 1 SDS decrease in height is associated with 14% increased risk of mortality.
C: Every 2.5 SDS decrease in height is associated with 14% increased risk of mortality.
D: Every 1 SDS decrease in height is associated with 14% increased risk of mortality.
A: Every 2 SDS decrease in height is associated with 30% increased risk of mortality.
B: Every 1 SDS decrease in height is associated with 14% increased risk of mortality.
C: Every 2.5 SDS decrease in height is associated with 14% increased risk of mortality.
D: Every 1 SDS decrease in height is associated with 14% increased risk of mortality.
A: Need for bilateral lung transplant
B: Presence of Burkholderia infection
C: Cardiac ejection fraction of 50%
D: Substantial limitation of daily activities
B: Presence of Burkholderia infection
A: Need for bilateral lung transplant
B: Presence of Burkholderia infection
C: Cardiac ejection fraction of 50%
D: Substantial limitation of daily activities
B: Presence of Burkholderia infection
A: Patient is on intermittent positive pressure ventilation
B: FiO2 demand of 40% to maintain saturation
C: Arterial PCO2 of 45mm hg
D: Pulmonary Hypertension leading to cardiac index of 2l/min/m2
D: Pulmonary Hypertension leading to cardiac index of 2l/min/m2
A: Age of the donor 50 years
B: Smoking history of 15 pack years
C: Past medical history of resolved community acquired pneumonia
D: Past medical history of CABG
D: Past medical history of CABG
A: Patient is put on cardiopulmonary bypass (CPB) and both of the lungs are removed together.
B: Patient is put on CPB and the lung with poorest function is removed first and first graft is implanted followed by the other.
C: Patient is put on CPB and only the diseased part of both lungs is replaced by the recipient graft.
D: Patient is put on CPB and lung with comparatively better function is removed first and implanted followed by the other.
B: Patient is put on CPB and the lung with poorest function is removed first and first graft is implanted followed by the other.
A: Motilin receptor agonist
B: Inhibition of calcineurin
C: B cell receptor (CD 20R) antagonists
D: Interleukin receptor (IL-1R) antagonists
A: Motilin receptor agonist
A: Hep A vaccine
B: Influenza vaccine
C: Hep B vaccine
D: BCG
E: INF-gamma
A: Transpilmonary pressure gradient (TPG) of more than 12mm Hg
B: Pulmonary vascular resistance (PVRi) of 14 woods
C: Oxygen saturation of 88%
D: Right atrial pressure(RAP) of 10mm Hg
A: Continue methylprednisolone for 1 more week
B: Switch to ATG+ IVIg
C: Start a calcineurin inhibitor therapy
D: Consider mechanical circulatory support
D: List him for retransplantation
A: B blockers
B: Aspirin
C: Statins
D: Nitrates
A: Readiness for Transition Questionnaire- provider version (RTQ-Provider)
B: STARx Program
C: Readiness Checklist the Readiness Checklist is for providers who prefer a "quick" assessment that is self-administered by the AYA using a short form checklist, rather than a qualitative assessment.
D:Transition Action Plan the Action Plan can be used to set individual goals for each domain and to guide educational interventions and counseling to help the AYA achieve self-efficacy.
E:Parent Action Form This form parallels the Transition Action Plan and will guide the parent/guardian in helping their adolescent achieve independence and improve self-efficacy.
E:Transition Protocol: RISE to Transition -Recognition of their disease process, reason for transplant, and the healthcare system. Insight into the short- and long-term impact of their disease, therapy, non-adherence, and emotional needs. Self-reliance in scheduling and attending appointments, refilling medications, and identifying urgent/emergent changes to their health on their own. Establish healthy lifestyle choices, life-long adherence to medications and follow-up, psychosocial skills, and educational/vocational goals.
Do you think that a pre-transplant genetic screening is needed?
A: Yes, it is appropriate to guide the post-transplant management and to exclude the disease in the donor.
B: No, as this is clearly a typical HUS.
C: It is an expensive evaluation. It should be performed in case of evidence of relapse.
D: It should be performed if the diagnosis is uncertain.
E: Genetic diagnosis is unnecessary due to eculizumab availability.
A: Yes, it is appropriate to guide the post-transplant management and to exclude the disease in the donor.
Thrombotic microangiopathy (TMA) defines a histopathological lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis and obstruction of the vessel lumen. Consumption of platelets and erythrocytes occurs in the microvasculature of kidney, brain and other organs, which causes laboratory features of thrombocytopenia and microangiopathic hemolytic anemia. Depending on whether brain or renal lesions prevail, two clinical entities have been described: the thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (HUS) (Noris American Journal of Transplantation 2010; 10: 1517–1523)
Typical HUS is caused by strains of E. coli (STEC) that produce Shiga-like toxins (Stx) and cause a hemorrhagic colitis. There are atypical forms, which are unrelated to STEC and account for less than 10% of cases (aHUS). These forms can occur sporadically or within families. The clinical outcome of aHUS is unfavorable (Noris American Journal of Transplantation 2010; 10: 1517–1523)
However, diarrhea may be an initial trigger of the disease in 39% of children (Clin J Am Soc Nephrol 8: 554–562, 2013). Thus, diarrhea at onset does not exclude an atypical HUS.
Various hereditary or acquired deficiencies in the complement alternative pathway proteins have been identified, including inactivating mutations in the genes coding for regulatory proteins of the alternative pathway C3 convertase (C3bBb), factor H (CFH), factor I (CFI), membrane cofactor protein (MCP), or thrombomodulin (THBD), anti-CFH antibodies associated with homozygous CFHR1-CFHR3 deletion and gain-of-function mutations in the genes coding for two components of the C3bBb convertase, factor B (CFB) and C3 (Clin J Am Soc Nephrol 8: 554–562, 2013.)
Risk of recurrence is significantly correlated with the type of mutation. The risk is highest (approximately 80 %) in patients with CFH, C3 or CFB mutations, and approximately 50% in patients with CFI mutation, compared to approximately 20% in patients with no identified complement mutation. The risk of post-transplant recurrence in patients with MCP mutation has been shown to be low. No post-transplant recurrence has been observed to date in patients with DGKE mutation. The recurrence risk is low in anti-CFH antibody-associated HUS if the antibody titer is low (< 500–1,000 AU/ml) at the time of transplantation, while substantial if elevated. THBD mutation may be followed by post-transplant recurrence (Pediatr Nephrol (2016) 31:15–39)
In aHUS patients with high risk of recurrence, treatment with anti-C5 therapy proved to be highly effective for preventing and treating posttransplant aHUS recurrence (American Journal of Transplantation 2012; 12: 3337–3354) and Eculizumab alone, without plasma therapy (plasma infusion and/or plasma exchange), is sufficient to prevent recurrence of aHUS and to maintain long-term graft function American Journal of Transplantation 2012; 12: 1938–1944 American Journal of Transplantation 2012; 12: 3337–3354
Live-related donation in patient with aHUS and identified mutation is a possible option, provided that complete genotyping of the donor is performed excluding the mutation identified in the patient or other possibly predisposing mutation American Journal of Transplantation 2010; 10: 1517–15238
It is therefore evident that genetic mutations should be identified prior transplantation in order to stratify the risk of recurrence and guide the best treatment.
A: Doppler ultrasound of aorta and IVC
B: Doppler ultrasound of aorta, IVC and iliac vessels
C: MRA/MRV (or CTA or formal angiography)
D: Angiography only if abnormal or inadequate ultrasound
E: 3D printing
B: Doppler ultrasound of aorta, IVC and iliac vessels.
Which of the following options would be an appropriate recommendation for the family at this time?
A: You recommend an immunosuppressive regimen, which is the current standard of care in adult renal transplant recipients (standard-dose tacrolimus with mycophenolate mofetil and glucocorticoids), because differences in the side effect profiles of immunosuppressive drugs between a pediatric and adult patient are minor and can be neglected.
B: You recommend an immunosuppressive regimen consisting of low-dose tacrolimus in conjunction with everolimus and steroid elimination at month 5, because there is recent scientific evidence that this regimen has the best long-term efficacy-safety profile in this patient population.
C: You recommend an immunosuppressive regimen consisting of standard-dose tacrolimus in conjunction with mycophenolate mofetil and steroid elimination either at an early point in time post-transplant (requiring induction therapy with an interleukin 2 receptor antagonist or anti-thymocyte globulin) or late, because this regimen has a good immunosuppressive efficacy regarding the prevention of acute rejection with an acceptable side effect profile, and steroid elimination allows post-transplant catch-up growth in the majority of prepubertal children with CKD-associated growth failure.
D: Defer listing and continue aggressive nutritional management and treatment with recombinant human growth hormone while the child grows to at least 40 kg prior to transplantation as the outcomes are much better in larger children.
E: List for deceased donor transplantation only from a pediatric donor, as this is the best way to achieve normal growth rates post-transplant irrespective of the immunosuppressive regimen.
C: You recommend an immunosuppressive regimen consisting of standard-dose tacrolimus in conjunction with mycophenolate mofetil and steroid elimination either at an early point in time post-transplant (requiring induction therapy with an interleukin 2 receptor antagonist or anti-thymocyte globulin) or late, because this regimen has a good immunosuppressive efficacy regarding the prevention of acute rejection with an acceptable side effect profile, and steroid elimination allows post-transplant catch-up growth in the majority of prepubertal children with CKD-associated growth failure.
In children, an immunosuppressive regimen allowing steroid elimination at some point in time post-transplant has the important advantage to allow normal longitudinal growth and avoid other steroid-associated side effects. Glucocorticoids (steroids) in pharmacological doses interfere with normal longitudinal growth by suppressing the secretion of endogenous growth hormone from the pituitary gland and induce resistance to the action of growth hormone in the growth plate; hence, steroid-free immunosuppressive regimens are important for pediatric patients after renal transplantation. While the 1 year data of the CRADLE study on a reduced dose tacrolimus regimen in conjunction with everolimus and steroid elimination at month 5 are promising (1), they do not provide currently sufficient scientific evidence that this regimen allows better longitudinal growth, while other previous randomized studies both from North America and Europe have documented improved growth rates with either steroid avoidance (2) or early (3, 4) or late (5) steroid elimination. There is no rationale to defer listing for renal transplantation in a 7 year old boy because renal transplantation at this age is standard of care and long-term dialysis in children is associated with multiple medical complications and psychosocial drawbacks. A renal allograft from a pediatric donor is not associated with better longitudinal body growth compared to an allograft from an adult donor.
Which are the therapeutic options in this situation, which you can offer to your patient and his parents?
A: Tremor is a well-known neurological side effect of standard-dose tacrolimus, and there is nothing that can be done against it
B: Treatment with a beta-blocker, for example metoprolol, is an established pharmacological therapy for tacrolimus-associated tremor
C: Because the tremor is likely to be tacrolimus-associated, stopping tacrolimus therapy and giving dual immunosuppressive therapy with mycophenolate mofetil and glucocorticoids is an appropriate solution
D: Because the tremor is likely to be tacrolimus-associated, reducing the tacrolimus exposure aiming at a trough level between 2 to 4 µg/L in conjunction with everolimus could be a therapeutic option. There is recent evidence from the pediatric CRADLE study and the adult TRANSFORM study that reduced-dose tacrolimus in conjunction with everolimus is non-inferior to standard-dose tacrolimus and MPA regarding immunosuppressive efficacy and preservation of renal graft function in the first year post-transplant.
E: A tacrolimus-free, belatacept-based immunosuppressive therapy in conjunction with MMF and steroids is the treatment of choice in this situation.
D: Because the tremor is likely to be tacrolimus-associated, reducing the tacrolimus exposure aiming at a trough level between 2 to 4 µg/L in conjunction with everolimus could be a therapeutic option. There is recent evidence from the pediatric CRADLE study and the adult TRANSFORM study that reduced-dose tacrolimus in conjunction with everolimus is non-inferior to standard-dose tacrolimus and MPA regarding immunosuppressive efficacy and preservation of renal graft function in the first year post-transplant.
Tremor that interferes with school and sports activity has significance for the patient and should not be neglected. Since the tremor is likely to be tacrolimus-associated, stopping this drug would be straight-forward, but dual therapy with mycophenolate mofetil and glucocorticoids has too little immunosuppressive activity for the majority of patients (1, 2). Belatacept-based immunosuppressive therapy could be an option, but belatacept is not yet licensed for pediatric patients and is contraindicated in EBV-seronegative patients. Reduced-dose tacrolimus in conjunction with everolimus is a therapeutic option, because this regimen is non-inferior to standard-dose tacrolimus and MPA regarding immunosuppressive efficacy and preservation of renal graft function. Another option would be to switch the patient from tacrolimus to the other calcineurin inhibitor cyclosporine, which is associated with less neurotoxicity.
A: Donor and recipient are highly likely to benefit
B: Surgical risk for donor is extremely low
C: Emotional and psychological risks to donor are minimized
D: All other deceased and living donor options have been exhausted (no adult donor, deceased donor unlikely)
E: The minor freely assents to donate without coercion
C: Emotional and psychological risks to donor are minimized
Answer C was added as a 5th condition proposed by the American Academy of Pediatrics in 2008.
Families need to be educated about the psychological risks that the donor may feel, particularly if most of the family’s resources remain focused on the ill recipient. Families must also be educated about the importance of affirming the donor’s role and the discomfort that some of the procedures may cause. Data in the bone marrow transplantation literature suggest that the risks can be minimized by preparing future donors through medical role-playing, allowing them to ask questions, and including them in the decision-making process.
Of the following new physical exam findings, which one is most concerning for allograft rejection?
A: Petechiae on his right foot
B: Splitting of the first heart sound
C: Dry mucous membranes
D: Gallop rhythm
E: Soft 1/6 systolic murmur at the left upper sternal border
D: Gallop rhythm
Of all exam findings listed above, the presence of a new gallop rhythm is the most sensitive for rejection, though there typically are a constellation of findings. There may also be tachycardia, new murmurs of mitral regurgitation or tricuspid regurgitation, or evidence of congestion (hepatomegaly, jugular venous distension, abnormal chest x-ray, etc). Early after transplant, the patient may be anemic resulting in the soft flow murmur as in answer (E).
Which of the following is the most likely medication that was started?
A: Trimethoprim/sulfamethoxazole
B: Phenytoin
C: Loratadine
D: Fluconazole
E: Metoprolol
D: Fluconazole
The antifungal medications are a consistent cause of increased calcineurin inhibitor levels in transplant patients. As such, any time any of these medications are considered being started, close monitoring of tacrolimus/cyclosporine is required. Other medications that may increase tacrolimus/cyclosporine levels include amiodarone, macrolide antibiotics, calcium channel blockers, and metoclopramide. Medications that may decrease tacrolimus/cyclosporine levels include octreotide, some anti-convulsants (phenytoin, phenobarbital, carbamazepine), and some antibiotics (nafcillin, IV Bactrim). Beta blockers have little effect on tacrolimus/cyclosporine levels. Patients who have tacrolimus toxicity have irritability, tremulousness, and may have seizures if levels are high enough.
Which of the following is the most likely cause of his symptoms?
A: Post-transplant lymphoproliferative disorder
B: Cytomegalovirus viremia
C: Bronchiolitis obliterans
D: Congestive heart failure
E: Tuberculosis
C: Bronchiolitis obliterans
Bronchiolitis obliterans is chronic inflammation of the bronchioles that results in fibrous deposition, ultimately obstructing airways. It is considered a form of chronic rejection in lung transplant recipients. Clinical presentation can be non-specific and subtle, and may resemble a upper respiratory infection at first. An increase in exertional dyspnea may be common, as well as noted decreases in daily spirometry values. While this may not seem relevant to a pediatric cardiology board review, the ABP lists knowledge of bronchiolitis obliterans as a complication of heart-lung transplant in their content specifications for the cardiology exam.
1. Rosen JB, Smith EO, Schecter MG, et al. Decline in 25% to 75% forced expiratory flow as an early predictor of chronic airway rejection in pediatric lung transplant recipients. J Heart Lung Transplant. 2012 Dec;31(12):1288-92
Which of the following medications likely is causing this degree of tremulousness in this patient?
A: Prednisone
B: Mycophenolate mofetil
C: Amlodipine
D: Tacrolimus
E: Sirolimus
D: Tacrolimus
Irritability and tremulousness are common side effects of tacrolimus, that tend to happen when serum levels are high. At high enough levels, tacrolimus toxicity can cause seizures to occur. The most common complication of azathioprine and mycophenolate is leukopenia, though many patients may have gastrointestinal side effects as well (constipation, diarrhea, nausea). The most common side effects of sirolimus are diarrhea and the development of mouth sores. The side effects of prednisone are well documented, including mood changes, increased appetite, increased blood glucose, weight gain, and a Cushingoid appearance. Long-term use is associated with the development of osteoporosis.
B: Mycophenolate mofetil
The most common complication of azathioprine and mycophenolate is leukopenia, though many patients may have gastrointestinal side effects as well (constipation, diarrhea, nausea). Irritability and tremulousness are common side effects of tacrolimus that tend to happen when serum levels are high. The side effects of prednisone are well documented, including mood changes, increased appetite, increased blood glucose, weight gain, and a Cushingoid appearance. Long-term use is associated with the development of osteoporosis.
D: Tacrolimus
Irritability and tremulousness are common side effects of tacrolimus, that tend to happen when serum levels are high. At high enough levels, tacrolimus toxicity can cause seizures to occur. The most common complication of azathioprine and mycophenolate is leukopenia, though many patients may have gastrointestinal side effects as well (constipation, diarrhea, nausea). The most common side effects of sirolimus are diarrhea and the development of mouth sores. The side effects of prednisone are well documented, including mood changes, increased appetite, increased blood glucose, weight gain, and a Cushingoid appearance. Long-term use is associated with the development of osteoporosis.
Considering the results of the serologic testing, which of the following would most likely be recommended to reduce the likelihood of the patient developing post-transplant lymphoproliferative disorder (PTLD)?
A: Minimize immunosuppression therapy due to EBV mismatch
B: Start antiviral therapy directed at CMV immediately post-transplant
C: Identify and treat early rejection
D: Close monitoring of CMV titers in the first year following transplant
E: Early transition of the primary immunosuppressant medication from a calcineurin inhibitor to mTOR inhibitor
A: Minimize immunosuppression therapy due to EBV mismatch
Post-transplant lymphoproliferative disorder is a significant cause of graft loss and death after transplant. Reduction in immunosuppression early after transplant has been recommended and led to improved survival. While monitoring for CMV is important, the majority of lymphomas after heart transplant have been found to be related to EBV. Re-transplantation for survivors of PTLD continues to be controversial and institution-dependent.
The most appropriate next step in management for this patient is:
A: Administration of antiobiotics
B: Administration of pulsed steroids
C: Conversion of tacrolimus to cyclosporine
D: Plasmapheresis
E: Evaluate the patient for cardiac re-transplantation
E: Evaluate the patient for cardiac re-transplantation
The patient is presenting with severe coronary artery vasculopathy. Options for management of the patient after this diagnosis are limited, but may include using aspirin, a statin drug such as pravastatin, and/or switching the patient from a calcineurin inhibitor (CNI) to an mTOR inhibitor such as sirolimus or everolimus. Stenting can be considered in certain situations, but typically does not have long-term benefit due to a very high incidence of re-stenosis. As such, listing the patient for re-transplantation is the best option. Steroids or plasmapheresis are treatments for rejection, and in absence of pathological findings or other evidence of acute rejection are not indicated. This being said, many patients will often receive presumptive treatment for rejection in this setting, in the hope of clinical improvement, though it should not be done in lieu of listing for re-transplantation.
A: More CMV and BKPyV infections
B: A higher number of acute rejections
C: More Proteinuria
D: A higher rate of de novo Donor specific antibodies
E: None of the above
E: None of the above
Studies in pediatric transplantation have shown, that mTOR inhibitors are protective for viral diseases. In combination with a low-dose CNI, there are no differences in acute rejections, proteinuria or the development of DSAs.
Which of the following is associated with the lowest risk for relapse?
A: Patient with undetectable soluble urokinase receptor (suPAR) serum level
B: Identified podocin mutation
C: Patient receiving peri- transplant plasmapheresis to prevent recurrence
D: Patient receiving living donor kidney
E: Patient who had showed an initial steroid response
B: Identified podocin mutation
suPAR was not confirmed as a reliable marker to predict FSGS relapse, the incidence and time to recurrence of FSGS in the kidney allograft are not significantly different between patients who did and did not undergo prophylactic plasmapheresis. Although, living donor kidneys may be used to transplant children with FSGS they are not associated to a reduced risk of relapse and children who had had an initial steroid response are at higher risk for relapse.
Which of the following is an absolute contraindication for kidney transplantation?
A: Malnutrition
B: HIV
C: Active Infection
D: Oxalosis
E: All of the above
C: Active Infection
While we would like all of our patients to be at the optimal nutritional status at the time of transplantation, this is not always the case. It is definitely not a contraindication. HIV was an absolute contraindicated until the recent antivirals were developed. There are centers that will transplant HIV + patients with good outcomes. Oxalosis was an absolute contraindication until recent years because of the risk of reoccurrence. Now it can be treated with combined liver kidney transplantation. Active infections should be treated before transplantation and immunosuppression to prevent increased morbidity and mortality from infection.
Which of the following is a true statement regarding these two CNIs?
A: Tacrolimus is more potent than cyclosporine on a molecular weight basis
B: Cyclosporine is a macrolide antibiotic
C: Tacrolimus increases expression of TGF-beta
D: Both have better absorption if taken with a fatty meal.
A: Tacrolimus is more potent than cyclosporine on a molecular weight basis
Tacrolimus is more potent than cyclosporine on a molecular weight basis hence 1-10mg tables of tacrolimus and 100s for cyclosporine. Tacrolimus is a macrolide antibiotic, not cyclosporine. Cyclosporine increases expression of TGF-beta and not tacrolimus. Both Cyclosporine and tacrolimus have some decreased absorption when ingested with a fatty meal, and it is recommended that they be taken on an empty stomach, if possible.
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