Background Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease.

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IntroductionAim of our study was to identify total costs of COVID-19 inpatients treatment in an upper-middle income country from Southeast Europe.MethodsThis retrospective, observational cost of illness study was performed from National Health Insurance Fund perspective and included a cohort of 118 males and 78 females admitted to COVID-19 ward units of a tertiary center, during the first wave of epidemics.ResultsThe median of total costs in the non-survivors’ subgroup (n=43) was 3279.16 Euro (4023.34, 355.20, 9909.61) which is higher than in the survivors (n=153) subgroup 747.10 Euro (1088.21, 46.71, 3265.91). The odds ratio of Charlson Comorbidity Index total score and every 100-Euros increase of patient’s total hospital treatment costs for fatal outcome were 1.804 (95% confidence interval 1.408-2.311, p<0.001) and 1.050 (1.029-1.072, p<0.001), respectively.ConclusionsDirect medical treatment costs for COVID-19 inpatients represent significant economic burden. The link between increased costs and unfavorable final outcome should be further explored.

Gastrointestinal complications are common among patients on peritoneal dialysis. Risk factors for the development of gastrointestinal complications in this patient population include: toxic effects of uremic toxins, frequent use of nonsteroidal anti-inflammatory drugs, Helicobacter pylori infection, angiodysplasia, increased intra-abdominal pressure, use of bioincompatible solution for peritoneal dialysis, increased glucose in solutions for peritoneal dialysis, secondary hyperparathyroidism (hypercalcemia), a disorder of lipid metabolism (hypertriglyceridemia), and the duration of peritoneal dialysis treatment. The most important non-infectious gastrointestinal complications in patients on peritoneal dialysis are: gastrointestinal bleeding, herniation and leaking of the dialysate from the abdomen (increased intra-abdominal pressure), impaired lung function (intra-abdominal hypertension), acute pancreatitis, and encapsulating sclerosis of the peritoneum. Intra-abdominal hypertension is defined as IAP ≥ 12 mmHg. Pouring the peritoneal dialysis solution leads to increased intra-abdominal pressure, which results in the development of hernias, pleuro-peritoneal dialysate leakage (hydrothorax), and restrictive pulmonary dysfunction. Risk factors for the development of acute pancreatitis in this patient population include: uraemia, secondary hyperparathyroidism with hypercalcemia, hypertriglyceridemia, features of the peritoneal dialysis solution (osmolarity, acidity, glucose, chemical irritation, and calcium in the solution for peritoneal dialysis lead to “local hypercalcemia”), toxic substances from the dialysate, the bags and tubing, and peritonitis and treatment of peritonitis with antibiotics and anticoagulants. Encapsulating sclerosis of the peritoneum is rare and is the most serious complication of long-term peritoneal dialysis. It is characterized by thickening of the peritoneum, including cancer, and signs and symptoms of obstructive ileus. Diagnosis is based on clinical, laboratory and radiological parameters. Encapsulating sclerosis of the peritoneum can be indicated by an AR-CA-125 concentration of less than 33 U/min and a concentration of AR-IL-6 greater than 350 pg/min in the effluent of patients with ultrafiltration weakness. Treatment consists of stopping peritoneal dialysis, using anti-inflammatory (corticosteroids) and anticicatricial drugs (tamoxifen), while surgical treatment includes enterolysis and adhesiolysis.