A complex case of congenital cystic renal disease.

This case outlines the potential complexity of autosomal recessive polycystic kidney disease (ARPKD). It highlights the challenges involved in managing this condition, some of the complications faced and areas of uncertainty in the decision making process. With a paucity of published paediatric cases on this subject, this should add to the pool of information currently available.

An observational study of early neonatal biochemical parameters in twins.

Early neonatal biochemical values are generally considered to reflect maternal values. However, studies have been inconclusive due to the statistical methods used. We hypothesized that there would not be important differences in plasma biochemistry between newborn twins, suggesting a common mechanism of control, and that differences between unrelated infant pairs would be greater. All twins over a 5-year period who had plasma biochemistry measured within 6 hours of birth were identified retrospectively. An unrelated control infant was matched to one of each twin pair. The 95% limits of agreement for plasma urea, creatinine, and sodium were calculated for twin pairs and unrelated matched infant pairs. Fifty-three twin pairs were studied. For urea, creatinine, and sodium, 95% of differences between twins were less than or equal to 1.5 mmol/L (8.9 mg/dL), 9.2 µmol/L (0.1 mg/dL), and 5.1 mmol/L (11.7 mg/dL), respectively. In unrelated infant pairs, the corresponding values were 4.0 mmol/L (24.3 mg/dL), 33.9 µmol/L (0.4 mg/dL), and 8.2 mmol/L (18.9 mg/dL). Differences between unrelated infant pairs were significantly wider than differences between twins ( p < 0.001). This study has demonstrated a close and significant agreement in urea, sodium, and creatinine when measured soon after birth in twin pairs compared with unrelated infants, implying a common mechanism of control.

HSP or not HSP... that is the question.

The authors report on the case of a 10-year-old girl who presented with a vasculitic process primarily involving the skin, joints and kidneys, which was initially presumed to be a variant of Henoch-Schonlein purpura. When the disease process behaved in a more aggressive way than expected, with the rapid onset of acute renal failure, further investigation revealed the underlying diagnosis to be that of Wegener's granulomatosis.