Congenital reflux nephropathy: a follow-up of 108 cases diagnosed perinatally.

OBJECTIVE: To determine: (i) the proportion of vesicoureteric reflux (VUR) associated with congenital renal damage and whether it can be severe enough to cause renal impairment from birth: (ii) to evaluate the distribution of males and females affected; and (iii) to describe the course of congenital damage in the first years of life. PATIENTS AND METHODS: A total of 108 children (76 male and 32 female, M:F 2.3:1), whose VUR was diagnosed before any infection, were followed from birth for a mean (range) of 4.3 (1-10) years. Renal damage was defined by serum creatinine concentration, creatinine clearance and renal imaging (ultrasonography and renal scintigraphy) performed within the first month of life and periodically thereafter. RESULTS: Of the 108 children, 58 had bilateral and 50 unilateral reflux (total number of refluxing units, 166). High-grade VUR (grade > or = 4) was found in 96 (58%) refluxing renal units (RRUs). Males had a prevalence of bilateral severe (> or = grade 4) reflux (M:F 5.2:1), while in those wit unilateral VUR, the M:F ration was 1.5:1. At birth, mild to moderate damage was present in 56 (36%) RRUs and only associated with VUR of grade > or = 3. Bilateral reflux of grade > or = 4 was associated with congenital moderate/severe renal failure in nine neonates (seven males). In infants with grade > or = 4 VUR who underwent surgical correction, VUR resolved in 92% of cases. In infants with VUR of grade > or = 4 followed medically, the reflux spontaneously resolved in 42% and ameliorated in 16% after 18 months. Serial renal scans during the follow-up showed no progression of renal damage. CONCLUSIONS: VUR diagnosed at birth on prenatal ultrasonography is associated with congenital damage, with males affected more often than females. The damage involves both kidneys in a consistent proportion and is an important cause of chronic renal impairment from birth. It does not progress in the first years of life if infections are prevented. It is suggested that males with this condition may constitute a major group at risk of developing chronic renal failure in later life.

Phagocyte function and immunological findings in a Wiskott-Aldrich syndrome long-term survivor.

The case reported concerns a 19-year-old man who presented with clinical and laboratory findings compatible with the Wiscott-Aldrich Syndrome. Our patients is the eighth reported case of a long-term survivor with this syndrome. Immunologic studies revealed, in spite of a normal lymphocyte number, an impaired delayed hypersensitivity and a failure of response to mitogens and irradiated allogeneic cells. IgE and IgA levels were high while IgM levels were low. Studies of phagocytic cells showed normal phagocytosis, candidacidal activity, IgG receptors and phagocytic metabolic burst. However, the patient's neutrophils and monocytes responded poorly to chemoattractants and the serum generated less chemotactic activity than normal sera. Detailed studies revealed the presence in the patient's serum of 2 different inhibitors of chemotaxis: a cell-directed inhibitor and an inhibitor of chemotactic factors.

[The mesenteric artery syndrome]. / La sindrome del compasso mesenterico.

We describe a case of superior mesenteric artery syndrome in a 12 years old girl. The patient had anorexia, emesis and weight loss. Rx-abdomen without and with contrast medium showed duodenum obstruction. The syndrome was associated with application of body cast.