Renal functional reserve after acute poststreptococcal glomerulonephritis.

We evaluated renal functional reserve (RFR) in 36 patients aged 5-21 years, who had recovered from an acute episode of poststreptococcal glomerulonephritis (PSGN) 1-16 years previously, without apparent sequelae, as evidenced by normal serum creatinine, blood pressure, and urinary sediment. The control group consisted of 12 children aged 2-12 years with recurrent urinary tract infections or nocturnal enuresis, without active infection or anatomical anomalies. The basal creatinine clearance was similar in the PSGN and control groups: 140.0 +/- 27.4 ml/ min per 1.73 m2 and 142.9 +/- 15.5 ml/min per 1.73 m2, respectively. The RFR in the PSGN group was significantly reduced compared with that of the control group: 18.6 +/- 12.9 ml/min per 1.73 m2 and 41.1 +/- 25.3 ml/min per 1.73 m2, respectively (P < 0.02). In 7 PSGN patients (19.4%), no RFR was found. In 69% of patients who had recovered from PSGN more than 10 years before the protein loading tests, a significantly reduced RFR (less than 10% of baseline) was found. The same degree of reduction in RFR was found in only 26% of patients who had suffered from PSGN less than 10 years ago.

Gaucher's disease and mesangiocapillary glomerulonephritis in childhood--a coincidence?

A 6-year-old boy, presenting with a nephritic syndrome, was diagnosed as suffering from Gaucher's disease (GD) and mesangiocapillary glomerulonephritis (MCGN). GD was suspected because of aseptic necrosis of the femoral heads on X-ray and later confirmed by bone marrow aspiration and a lack of glucocerebrosidase activity in white blood cells; MCGN was documented on renal biopsy. The child was treated with prednisone, dipyridamole and aspirin, and recovered completely clinically. A second biopsy was not performed. The connection between these two rare diseases, and between nephritis and GD in general, is discussed.

Acute chest syndrome in children with sickle cell anemia.

Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). Those patients are frequently admitted with chest signs and symptoms suggesting an infective process. The term acute chest syndrome (ACS) is used to describe those chest episodes in SCA patients, and it is postulated that a primary bone vaso-occlusive crisis may be the cause of the acute chest syndrome in SCA patients. In this study we report 52 episodes of ACS in a group of 22 children with SCA. Chest pain, fever, and leukocytosis were a constant clinical finding. The hematological, radiological, and bacteriological studies are reported. There is a constant and significant fall in hemoglobin levels from 88 +/- 10 g/L (8.8 +/- 1.0 g/dl) to 68 +/- 15 g/L (6.8 +/- 5 g/dl). Unilateral or bilateral pulmonary basal infiltrations were found in 50 episodes. Pleural effusion was noticed in 60 episodes, and it was bilateral in three. No significant bacteriological findings were present.