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In this report, we describe a newborn infant who presented with congenital nephrotic syndrome and renal insufficiency, as well as bilateral microcoria. This constellation of findings is a hallmark of Pierson syndrome, a newly recognized genetic disorder that is caused by a deficiency of beta2 laminin in the basement membrane. Our patient demonstrated classic histopathologic findings of Pierson syndrome on renal biopsy, including absence of beta2 laminin on immunofluorescent staining, and genetic testing confirmed the diagnosis. We conclude that Pierson syndrome should be included in the differential diagnosis for congenital nephrotic syndrome, especially in patients with ocular abnormalities. [ABSTRACT FROM AUTHOR]

Background: Children receiving maintenance dialysis exhibit high cardiovascular (CV) associated mortality. We and others have shown high prevalence of cardiac calcifications (CC) in children with endstage renal disease (ESRD). However, no pediatric study has examined modality difference in CC prevalence. The current study was conducted to assess for a difference in CC prevalence between hemodialysis (HD) and peritoneal dialysis (PD) in children with ESRD., Methods: 38 patients (19 female, 19 male; mean age 15.5 ± 4.1 years) receiving dialysis (21 HD, 17 PD) were included in the study. CC were assessed by ultrafast gated CT and quantified by Agatston score. Patients received thrice weekly HD for 3 - 3.5 hours or daily continuous cycler PD (CCPD). FGF 23, IL-6, IL-8, and CRP levels were obtained at time of CT. Time-averaged (6 months prior to CT) serum Ca, P, Alb, iPTH, and cholesterol levels were obtained. Patients on aspirin, with evidence of infection, underlying collagen vascular disease were excluded., Results: CC were present in 11/38 patients, but more prevalent in HD vs. PD (9/21 vs. 2/17, p = 0.04). Subjects with CC were older (p = 0.0003), had longer dialysis vintage (p = 0.02) and higher serum phosphorus (p = 0.02) and FGF 23 levels (p = 0.03). HD patients also had significantly higher phosphorus (p = 0.02), FGF 23 (p = 0.009), and IL-8 levels (p = 0.02) when compared to PD patients. Residual renal function was not different between modalities or patients with CC. On a multinomial regression model, modality, and age remained independent associations for CC prevalence., Conclusion: We have shown that pediatric patients receiving CCPD have lower CC prevalence conferring lower CV risk. The better control of mineral imbalance in patients receiving PD may play an important role in lower CC prevalence.

Obturator hernia is a type of external abdominal hernia. Site of hemiation is the obturator canal, which is the superolateral aspect of the obturator foramen. The herniation sac exits the pelvis near the obturator nerve and vessels. Herniated loops of small bowel extend between the pectineus and obturator muscles. In this report, we present a case of obstructed obturator hernia.

Accumulation of air in the stomach increases the gastric volume, which activates receptors in the gastric wall. This results in a reflex that relaxes the lower oesophageal sphincter, whereby the intragastric air can escape through the oesophagus. Ventilation of the stomach via the oesophagus is known as belching (ructus). Belching often occurs in combination with reflux symptoms and dyspepsia. In these cases, other symptoms are often more predominant, and it is advisable to treat these first. In patients with aerophagia, belching is the most common reason for medical consultation. These patients belch frequently, up to 20 times per minute, and often during consultation. Aerophagia results from air being sucked into the oesophagus or injected by pharyngeal contraction, after which it is expelled immediately. In contrast to the described gastric belching, aerophagia is therefore a form of supragastric belching. Aerophagia is a behavioural disorder, and behavioural therapy or logopedics appears to be most common therapeutic approach.