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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. [ABSTRACT FROM AUTHOR]

Primary hyperoxalurias (PH) comprise a group of three distinct metabolic diseases caused by derangement of glyoxylate metabolism in the liver. Recent years have seen advances in several aspects of PH research. This paper reviews current knowledge of the genetic and biochemical basis of PH, the specific epidemiology and clinical presentation of each type, and therapeutic approaches in different disease stages. Potential future specific therapies are discussed. [ABSTRACT FROM AUTHOR]

Background: Central venous catheters are frequently used as access for hemodialysis (HD) in children. One of the known complications is central venous stenosis. Although this complication is not rare, it is often asymptomatic and therefore unacknowledged. Superior vena cava (SVC) stenosis is obviously suspected in the presence of upper body edema, but several other signs and symptoms are often unrecognized as being part of this syndrome. Case-diagnosis/treatment: We describe four patients with various manifestations of central venous stenosis and SVC syndrome. These sometimes life- or organ-threatening conditions include obstructive sleep apnea, unresolving stridor, increased intracranial pressure, increased intraocular pressure, right-sided pleural effusion, protein-losing enteropathy and lymphadenopathy. The temporal relationship of these complications associated with the use of central venous catheters and documentation of venous stenosis, together with their resolution after alleviation of high venous pressure, points to a causal role. We suggest pathophysiological mechanisms for the formation of each of these complications. Conclusions: In patients with occlusion of the SVC, various unexpected clinical entities can be caused by high central venous pressure. As often the etiology is not obvious, a high index of suspicion is needed as in some cases prompt alleviation of the high pressure is mandatory. [ABSTRACT FROM AUTHOR]

Icodextrin is a starch-derived glucose polymer used in peritoneal dialysis dialysate to treat volume overload by increasing ultrafiltration in patients with end-stage renal disease. Reported adverse reactions to icodextrin are mild and rare and mainly consist of skin rash that resolves spontaneously after discontinuation of treatment. We describe a young patient with extreme eosinophilia that appeared with the use of icodextrin, disappeared after its discontinuation, and reappeared after a rechallenge with the drug. The eosinophilia was not associated with peritonitis, was asymptomatic, and fully resolved after discontinuation of the drug. Severe eosinophilia can potentially cause tissue damage in several organs, which would indicate that blood eosinophil count is recommended in routine complete blood counts while icodextrin peritoneal dialysis is being administered. [ABSTRACT FROM AUTHOR]