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Background Systemic oxalosis is a severe complication seen in primary hyperoxaluria type I patients with kidney failure. Deposition of insoluble calcium oxalate crystals in multiple organs leads to significant morbidity and mortality. Methods We describe a retrospective cohort of 11 patients with systemic oxalosis treated at our dialysis unit from 1982 to 1998 (group 1) and 2007-2019 (group 2). Clinical and demographic data were collected from medical records. Imaging studies were only available for patients in group 2 (n = 5). Results Median age at dialysis initiation was 6.1 months (IQR 4-21.6), 64% were male. Dialysis modality was mostly peritoneal dialysis in group 1 and daily hemodialysis in group 2. Bone disease was the first manifestation of systemic oxalosis, starting with the appearance of sclerotic bands (mean 166 days, range 1-235), followed by pathological fractures in long bones (mean 200.4 days, range 173-235 days). Advanced disease was characterized by vertebral fractures with resulting kyphosis, worsening splenomegaly, and adynamic bone disease. Two patients developed pulmonary hypertension, 4 and 8 months prior to their death. Four of 11 patients developed hypothyroidism 0-60 months after dialysis initiation. Only one patient survived after a successful liver-kidney transplantation. Four patients died after liver or liver-kidney transplantation. Conclusions This is the first comprehensive description of the natural history of pediatric systemic oxalosis. We hope that our findings will provide basis for a quantitative severity score in future, larger studies. Graphical abstract, Author(s): Efrat Ben-Shalom [sup.1] , Ruth Cytter-Kuint [sup.2] , Choni Rinat [sup.1] [sup.3] , Rachel Becker-Cohen [sup.1] [sup.3] , Shimrit Tzvi-Behr [sup.1] , Jenny Goichberg [sup.1] , Vardit Peles [sup.1] [...]

Introduction: Approximately a third of men worldwide and over 90% of Israeli men are circumcised. The procedure carries a low rate of complication, mainly including surgical and infectious complications. Urinary tract obstruction (UTO) is a rare complication of circumcision., Aims: The aim of this study was to define the incidence of UTO following circumcision in the last 20 years and describe the characteristics of the affected babies., Methods: Study participants were identified from a list consisting of all male babies aged 7-30 days treated at the Shaare Zedek Medical Center during the years 2000-2020. Files of patients with serum creatinine ≥ 1 mg/dl were reviewed. Clinical and laboratory data were collected from patients' records., Results: Ten babies with acute kidney injury due to UTO after circumcision were identified. Average age at admission was 10.1 days (8-13). Only two babies had an uncomplicated postnatal course. The main findings on physical examination were distended abdomen, abdominal wall discoloration and leg edema. Average creatinine on admission was 1.76 mg/dl (1.0-3.28). Additional findings were hyperkalemia 6.2 mEq/L (4.5-7.6) and hyponatremia 125 mEq/L (118-134). All hospitalized patients developed post-obstructive diuresis. Kidney function and laboratory abnormalities completely resolved in all of our patients. There was no evidence of kidney damage in six children with long-term follow up., Conclusions: UTO with acute kidney injury is a rare severe complication of circumcision. Prompt identification and proper treatment can result in complete resolution of kidney function.