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Background: Pediatric kidney transplantation is unique when compared to adults. They need optimum post-transplant care to achieve longer graft and patient survival. Infections, rejection, recurrence, malignancy, bone problems, hypertension, and surgical complications are the most important challenges that require prevention, early detection, and management. Chronic allograft nephropathy needs different approaches based on its related causes, allograft pathology, graft function, and availability of re-transplantation. Aim/objectives: This national adapted guideline aims to frame evidence-based post-transplant recommendations adopted or adapted from EB source guidelines to optimize post-transplant patient care. Methods: Our guideline is evidence based adapted from four national and international source guidelines with permissions [KDIGO 2020, RA/BTS 2022–2018, EAU 2018] that were appraised with AGREE II instrument tool. We followed the "adapted ADAPTE" CPG formal adaptation methodology that consists of 3 phases and 24 steps and tools. Virtual monthly meetings all through the year 2023 were activated for reviewing and validation of final adaptation of evidence-based guideline draft, considering all comments of external reviewers. This guideline was registered in the practice guideline registration for transparency (PREPARE), number: PREPARE-2023CN364. Results: Recommendation statements in guidelines stand for results in articles. Discussion of important recommendations and the rationale behind selection of adopted statements and tailoring of others to suit our local facilities, expertise, and renal native disease profile was presented in the text with reasons and references. Conclusion: The provided guidelines aim to improve graft and patient outcome through prevention and early management of complications and suggest research areas lacking validated research recommendations. [ABSTRACT FROM AUTHOR]

Background: Cystic fibrosis (CF) is a debilitating and time-consuming disease that can interfere with or impair typical daily life activities and social interactions. This study aims to identify and evaluate behavioral problems and cognitive dysfunction in children with CF. Methods and procedures: This study was carried out on a cohort of 40 children diagnosed with CF. Patients were assessed using a child behavior checklist (CBCL) and intelligence quotient (IQ) photograph test. The obtained results were compared with 40 matched controls. Results: The male population accounted for 62.5% of the patients included in the study. Patients with CF exhibited elevated somatic, anxious and aggressive behaviors relative to the control group with p values < 0.012, < 0.025, < 0.027 respectively. In addition; there was a statistically significant positive correlation between somatic problems with both age of the patients and disease duration (r value for age = 0.368; r value for duration = 0.468). Furthermore; there was positive correlation between anxious behavior with age of patients and disease duration (r value for age = 0.358; r value for duration = 0.351). Otherwise, patients had comparable IQs (median of 107.5) with those of the control group (median of 105) with p value = 0.544. Conclusion: Our findings indicate that CF has a substantial negative impact on the behavior of affected patients. However, it did not have an impact on the individual's level of intelligence. There is evidence indicating a connection between patients' age and disease duration with behavioral problems. [ABSTRACT FROM AUTHOR]

Background: Even though current therapeutic approaches have significantly increased the longevity and standard of living for children suffering from cystic fibrosis (CF), the crucial psychological components of this illness have not received the same level of attention as other chronic illnesses. The aim of the research was to assess depression, anxiety, and hopelessness in cystic fibrosis-affected children and their relation to the duration of illness. In this study, we compared 40 healthy control children between the ages of 7 and 12 with 40 cystic fibrosis children who were matched for age and sex. All patients had detailed clinical and psychometric evaluations using the Children's Depression Inventory (CDI), the Anxiety Scale for Children (ASC), and the Hopelessness Scale for Children (HSC). Results: The CDI, ASC, and HSC revealed a significant difference between both groups. Positive correlations between depression, anxiety, and hopelessness and disease duration were found. Conclusions: Patients with CF experienced significantly increased depression, anxiety, and hopelessness, and there is an association between these symptoms and the duration of the illness. We advise making psychiatric screening a standard part of evaluating and monitoring CF patients. [ABSTRACT FROM AUTHOR]

Background: Cystinosis is a rare autosomal recessive disease. Children with nephropathic cystinosis (NCTN) have evidence of intellectual dysfunction and behavioural abnormalities which are attributed to renal dysfunction, metabolic disarrangement, and gene mutation. This study aimed to characterize the cognitive functions and behavioural profiles in nephropathic cystinosis patients on cysteamine therapy, and determine its relation to cysteamine treatment duration. In this analytical cohort study, 20 children with nephropathic cystinosis aged 6 years or above were compared to 26 children with chronic kidney disease (CKD) matched in age, sex, and CKD stage. All patients were subjected to full clinical and psychometric assessment using the Child Behaviour Checklist (CBCL) and the Arabic language version of Stanford-Binet test (SB). Results: There was no significant difference between both groups regarding Stanford-Binet test (SB) and Child Behavioural Checklist (CBCL), apart from delinquent behaviour. Duration of cysteamine treatment was inversely correlated with short-term memory, thought, and sex problems. Conclusions: Children with cystinosis have a wide range of neurocognitive and behavioural problems that still present after cysteamine treatment and may be related to impact of genetic mutation on brain structure and function. Longer duration of cysteamine treatment could have beneficial effects on some behavioural problems. [ABSTRACT FROM AUTHOR]

Background: Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disorder characterized by kidney and extra-renal complications due to the accumulation of cystine crystals in various tissues and organs. Herein, we describe the early neuromuscular complications in a cohort of pediatric nephropathic cystinosis patients. Methods: We prospectively evaluated the clinical, biochemical, and neurophysiological data of 15 cystinosis patients. Neurophysiological evaluation was performed to confirm or exclude presence of neuropathy and/or myopathy. Results: Patients' age ranged between 20 and 216 months at time of examination. Nine patients were males. Three patients had early abnormal neurophysiological features consistent with neuromuscular involvement (clinically asymptomatic proximal myopathy with a patchy distribution in one patient and isolated asymptomatic sensory nerve conduction changes in two patients). A fourth patient had mixed abnormal motor and sensory axonal neuropathic changes associated with overt clinical features (predominantly motor symptoms). Patients with abnormal neuromuscular features were significantly older in age than the unaffected group (P = 0.005) and had a diagnosis of cystinosis with subsequent cysteamine therapy at a significantly older age than the unaffected group (P = 0.027 and 0.001, respectively). Conclusions: We expanded the recognized phenotypes of cystinosis neuromuscular complications with early proximal skeletal myopathy and symptomatic motor and sensory axonal neuropathy. Early asymptomatic neuromuscular complications could develop in pediatric patients and would require neurophysiological studies for early detection prior to development of overt clinical manifestations. Prompt diagnosis and timely initiation of cysteamine therapy with recommended dose can delay the development of neuromuscular complications. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]