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Pediatric patients on kidney replacement therapy (KRT) are among the most vulnerable during large-scale disasters, either natural or man-made. Hemodialysis (HD) treatments may be impossible because of structural damage and/or shortage of medical supplies, clean water, electricity, and healthcare professionals. Lack of peritoneal dialysis (PD) solutions and increased risk of infectious/non-infectious complications may make PD therapy challenging. Non-availability of immunosuppressants and increased risk of infections may result in graft loss and deaths of kidney transplant recipients. Measures to mitigate these risks must be considered before, during, and after the disaster including training of staff and patients/caregivers to cope with medical and logistic problems. Soon after a disaster, if the possibility of performing HD or PD is uncertain, patients should be directed to other centers, or the duration and/or number of HD sessions or the PD prescription adapted. In kidney transplant recipients, switching among immunosuppressants should be considered in case of non-availability of the medications. Post-disaster interventions target treating neglected physical and mental problems and also improving social challenges. All problems experienced by pediatric KRT patients living in the affected area are applicable to displaced patients who may also face extra risks during their travel and also at their destination. The need for additional local, national, and international help and support of non-governmental organizations must be anticipated and sought in a timely manner.

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Systemic lupus erythematosus (SLE) is an autoimmune disorder which is less often observed in children than adults.It is uncommon in children younger than ten years, especially in boys. In all age groups, nearly 15% of all cases have onset of disease over 16 years of age. Lupus nephritis is one of the most serious organ involvements of the disease and its symptoms are generally related to hypertension, proteinuria, and renal failure. But it may be also asymptomatic. Renal disease occurs in 90% of patients in the course of the disease. During its course serious complications as thrombotic microangiopathy. may develop. Recent reports suggest that renal involvement is more frequent in children than in adults. Childhood-onset lupus nephritis can be more severe than the late-onset disease. We present a 7-year-old boy with lupus nephritis who was previously misdiagnosed as Henoch -Schönlein Purpura (HSP).

Background and Aims Alport syndrome (AS) is an inherited glomerular basement membrane disease caused by mutations in COL4A3, COL4A4 or COL4A5 genes. Recently, it has been reported that focal segmental glomerulosclerosis (FSGS) can be seen in AS and COL4A mutations can be detected in FSGS. In this study, we aimed to define the clinical characteristics of patients with genetically confirmed AS, in order to establish genotype-phenotype correlation and investigate the effects of different treatment regimes. Method A total of 87 pediatric AS patients included in this multicenter study. We retrospectively collected data from medical records and requested other centers to fill out a questionnaire, which included data regarding patient demographic features, family history, clinical and laboratory characteristics at first presentation, histopathological (if available) and genetic tests results, treatments and yearly follow-up results. Results A total of 87 (41 female, 46 male) genetically confirmed AS patients (COL4A5, n=43; COL4A3, n=25; COL4A4, n=19) were studied. Mean age at first presentation was 7.6±4.1 years and the median follow-up duration was 4.3 years (IQR 1.9–7.3). 14 (16.1%) of 87 patients presented with nephrotic syndrome (NS); renal biopsy findings showed FSGS in 11 (79%) of 14 patients with NS, and COL4A3 mutations were the most common (n=7, 50%) in this group. Of 14 NS patients, 12 received steroid, 11 received cyclosporine (CsA) and 4 received other immunosuppressives prior to genetic diagnosis. The inheritance pattern of the patients with NS was consistent with ARAS in 10 patients (71.4%), XLAS in 3 patients (21.4%), and ADAS in 1 patient (7.2%). During follow up, glomerular filtration rate (GFR) decreased below 90 ml/min/1.73 m2 in 24 of 87 patients (27.5%). COL4A3 mutations (n=14, 58.3%) were the leading genetic abnormality in patients who progressed to chronic kidney disease (CKD). At the last visit, GFR loss was significantly higher in patients with COL4A3 mutations when compared to patients with COL4A4 and COL4A5 mutations (p=0.04). Among patients with NS, 9 of 14 (64.2%) progressed to CKD. Genetic results of patients with NS who progressed to CKD were COL4A3 in 6 (66.7%), COL4A4 in 2 (22.2%) and COL4A5 in 1 (11.1%) patients. In survival analysis, renal survival rate without CKD was 12.1 years (95% CI: 6.7-17.5). After the first presentation, the 5-year cumulative risk of CKD was 51.8%, 12.6%, and 12.9% in patients with COL4A3, COL4A4 and COL4A5 mutations, respectively (p=0.001). We observed that patients with COL4A3 mutations, ARAS inheritance pattern, histopathology of FSGS or NS presentation progressed to CKD earlier (p<0.001 for COL4A3, p=0.01 for ARAS, p=<0.001 for FSGS, p=0.01 for NS presentation) when compared to those without. CsA treatment did not improve renal survival. Conclusion Detailed analyses of data from genetically confirmed Turkish patients with AS provided important clues regarding to the presentation, course and outcomes of the disease. COL4A3 mutations, ARAS inheritance pattern, NS presentation and FSGS finding in renal biopsy are major risk factors for progression to CKD. We recommend genetic testing for patients suspected of having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid resistant NS. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications.