Clinical practice recommendations for recurrence of focal and segmental glomerulosclerosis/steroid‐resistant nephrotic syndrome

Lutz T. Weber, Burkhard Tönshoff, Ryszard Grenda, Antonia Bouts, Rezan Topaloglu, Bora Gülhan, Nikoleta Printza, Atif Awan, Nina Battelino, Rasmus Ehren, Peter F. Hoyer, Gregor Novljan, Stephen D. Marks, Jun Oh, Agnieszka Prytula, Tomas Seeman, Clodagh Sweeney, Luca Dello Strologo, Lars Pape.

Recurrence of primary disease is one of the major risks for allograft loss after pediatric RTx. The risk of recurrence of FSGS/SRNS after pediatric RTx in particular can be up to 86% in idiopathic cases. There is a need for consensus recommendations on its prevention and treatment. The CERTAIN study group has therefore performed a thorough literature search based on the PICO model of clinical questions to formulate educated statements to guide the clinician in the process of decision‐making. A set of educated statements on prevention and treatment of FSGS/SRNS after pediatric RTx has been generated after careful evaluation of available evidence and thorough panel discussion. We do not recommend routine nephrectomy prior to transplantation; neither do we recommend abstaining from living donation. Special attendance needs to be given to those patients who had already experienced graft loss due to FSGS/SRNS recurrence. Early PE or IA with or without high‐dose CsA and/or rituximab seems to be most promising to induce remission. The educated statements presented here acknowledge that FSGS/SRNS recurrence after pediatric RTx remains a major concern and is associated with shorter graft survival or even graft loss. The value of any recommendation needs to take into account that evidence is based on cohorts that differ in ethnicity, pre‐transplant history, immunosuppressive regimen, definition of recurrence (eg, clinical and/or histological diagnosis) and treatment modalities of recurrence.

Early school‐age cognitive performance post–pediatric heart transplantation

Anna Gold, Bianca C. Bondi, Jenna Ashkanase, Anne I. Dipchand

Background

As survival in pediatric heart transplantation (HTx) has improved due to medical advances, the analysis of long‐term outcomes impacting quality of life such as cognition and development becomes increasingly important. Neuropsychological assessments provide a comprehensive understanding of individual needs, allowing for the development of tailored recommendations and interventions.

Methods

Routine neuropsychological assessment was completed between 5 and 7 years of age in this cohort of pediatric HTx recipients at our center (Jan 2014‐Oct 2018), including tests of general intellect (WPPSI‐IV, WISC‐V), academics (WIAT‐II/III), perceptual‐motor abilities (Beery VMI), and memory (CMS). Relevant medical variables were collected.

Results

Among 25 children, the median age at testing was 6.7 (IQR:5.8‐7.4) years, with a median time since HTx of 5.2 (IQR:4.8‐6.8) years. Medical diagnoses included congenital heart disease (CHD; 56%) and cardiomyopathy (44%). Cognitive functioning across the intellectual, academic, and perceptual‐motor domains fell within the low‐average range, while memory abilities fell within the average range. DSM‐5 clinical diagnoses were provided for 14 (56%) children: Intellectual Disability‐Mild (20%), Learning Disability (20%), Language Disorder (8%), and Attention‐Deficit/Hyperactivity Disorder (12%). The presence of neurological issues and/or CHD predicted poorer performance on various neuropsychological domains.

Conclusions

Over 50% of this cohort of pediatric heart transplant recipients seen for routine post‐HTx neuropsychological assessment received a clinical psychological diagnosis, notably higher than rates in the general population. This population requires monitoring to ensure that high risk children are identified and successfully supported in school and their community.

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