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One hundred fifty-four children aged eighteen years or younger from 83 families with autosomal-dominant polycystic kidney disease were studied by ultrasonography or excretory urography. Twenty-three children had bilateral renal involvement with at least five cysts (ADPKD), 28 children were classified as suspicious (SADPKD), and 103 children had no renal involvement (NADPKD) detected by ultrasound. Seventy-four percent of the ADPKD children had signs or symptoms compatible with the diagnosis of ADPKD, compared to 34% of the NADPKD and 36% of the SADPKD children (both P less than 0.05). Three of the 23 ADPKD children had elevated serum creatinines at the time of diagnosis, while all of the NADPKD and SADPKD children had normal renal function. Renal area by ultrasonography (width X depth) was greater among the ADPKD children compared to the SADPKD and NADPKD groups (P less than 0.05). On follow-up 30 of the 37 NADPKD children remained NADPKD, three were reclassified as SADPKD, and four progressed to ADPKD after 18 years of age. All of the NADPKD children had normal renal function on follow-up. Overall, 14 children had suspicious ultrasounds at some point with follow-up ultrasonography and ten (71%) progressed to ADPKD. All SADPKD children maintained normal renal function. Eight of 18 ADPKD children had progression of the disease manifested by development of hypertension and/or decreased renal function. Three children progressed to end-stage renal disease. Five ADPKD children were diagnosed before one year of age, two of them via prenatal ultrasonography. One fetus was aborted after documentation of oligohydramnios.(ABSTRACT TRUNCATED AT 250 WORDS)

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Various agents have been tried in subjects with newly diagnosed Type 1 (insulin-dependent) diabetes mellitus in an attempt to preserve Beta-cell function. In this double-blind study, nicotinamide or placebo were given for one year to 35 children and adolescents with newly-diagnosed Type 1 diabetes. All subjects were within six weeks of diagnosis and were between the ages of 6 and 18 years. Nicotinamide, a poly-(ADP-ribose) synthetase inhibitor, was given in a dose of 100 mg/year of age up to a maximum of 1.5 g/day. There were no initial differences between the 17 control and the 18 test subjects in relation to mean age, sex distribution, or severity at onset. Mean insulin dosages and HbA1 values were similar for the two groups during the year of study. Fasting and glucagon-stimulated C-peptide levels were similar for the control and nicotinamide treated groups at the beginning and after 4 and 12 months. There were no differences in remission rates between the two groups. Nicotinamide, at this dosage, does not preserve residual insulin secretion in subjects with newly diagnosed Type 1 diabetes.

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