Child nephrology and urology最新文献

Clearance studies were performed in 12 patients with cystic fibrosis (CF) during maximum water diuresis. The fractional diluting segment reabsorption was calculated from chloride clearance (CCl) and maximum free water clearance (CH2O) as 'CH2O/CCl+CH2O'. Fractional delivery to the diluting segment and from the proximal tubules was estimated by maximum urine flow during water diuresis (Vmax/GFR) and lithium clearance (CLi/GFR), respectively. The data were compared with results obtained in young healthy subjects. To estimate free water generation beyond the medullary diluting segment, CH2O/GFR was also measured during acute furosemide administration in 5 patients. Values for the term CH2O/CCl+CH2O were significantly lower in patients with CF (79.1 +/- 5.1%) as compared with healthy controls (89.4 +/- 4.4%). This was also true for the terms Vmax/GFR (CF: 9.5 +/- 1.6%; control subjects: 13.8 +/- 2.5%) and CLi/GFR (CF: 22.2 +/- 4.1%; control subjects: 30.7 +/- 5.1%). Compared to data derived from the literature, the solute-free water generation during administration of furosemide (CH2O/GFR) was significantly reduced in patients with CF (6.8 +/- 1.5%) as compared with healthy subjects (13.3 +/- 6.1%). We conclude that reabsorption in the diluting segment is impaired in patients with CF, perhaps at a site beyond the thick ascending limb of Henle's loop. Enhanced proximal reabsorption, suggested by reductions in Vmax/GFR and CLi/GFR, appears to be a compensatory phenomenon to maintain sodium and chloride balance.

Statural growth was studied in 16 glomerulopathic children, 12 boys and 4 girls, treated with alternate-day prednisone for more than 2 years. Five patients were older than 12 years when started on therapy. Growth velocity was significantly slower in boys who received therapy during peripubertal age than in younger boys taking similar amounts of prednisone. In boys, pubertal growth spurt was delayed and shorter than normal, but growth continued with a velocity greater than normal for some years and kept on after 19 years of age. The ultimate height of the patients followed up after 19 years of age ranged within the normal limits of genetic height potential.

We evaluated the policy changes of 10 pediatric departments in the Friuli Venezia Giulia region following the implementation of a new protocol for the diagnosis and treatment of urinary tract infection. Clinical data concerning 1,059 infants and children with a first episode of urinary tract infection were recorded in three periods, before (A), during (B), and after (C) implementation of the protocol application. We observed a significant increase in the total number of urinary tract infections (288 in period A, 464 in C) from period A to C, with particular regard to pyelonephritis (36.8% in period A, 64.3% in C). As radiological investigations were carried out more frequently, there was a significant increase in the number of cases of vesicoureteral reflux recorded (from 23 in period A to 76 in period C). Finally, a better therapeutic approach was progressively achieved: errors in the choice of antibiotics and the duration of treatment have been almost completely avoided in period C. In our opinion, these results demonstrate a long-term improvement of pediatricians' awareness of this frequent pediatric pathology and their policies to control it.

Three boys with urethral obstruction are described. To facilitate voiding, each boy compressed the shaft of his penis prior to initiating the urinary stream, a sign previously unreported in association with urethral obstruction.

The effect on growth of long-term treatment with prednisolone was studied in 12 patients with steroid-sensitive nephrotic syndrome. Our patient's heights were found between the 10th and 25th percentile both at the first and last height measurement. There was no statistical difference between the first and last height standard deviation score (Ht SDS) (p greater than 0.05). When compared with chronological age, growth velocity (GV), GV SDS and bone age were found low but within the normal range for this age group. There was not any correlation between the last Ht SDS and relapse number, total doses and duration of daily and alternate-day steroid therapy (p greater than 0.05). Growth hormone (GH) responses to pharmacological stimuli were obtained as severe deficiency in 10 patients, partial deficiency in 1 patient and normal level in 1. There was statistical difference between the pulse number of the overnight GH profile of the patients and control group (p less than 0.05). But no statistical difference was found between GH pulse amplitude and GH concentration in patients and control group (p greater than 0.05).

The genetically determined acetylator phenotype in diabetic children with and without increased urinary albumin excretion was investigated. Acetylator phenotype was determined according to Evans, and 24-hour albumin excretion rate (AER) was measured by immunoturbidometry in 86 children and adolescents with type 1 (insulin-dependent) diabetes mellitus and in 100 age-matched healthy controls. In diabetics, the fast acetylator phenotype was found in 36 (41.9%) patients and the slow one in 50 (58.1%); the control group had 52 (52%) fast and 48 (48%) slow acetylators. There were no significant differences in acetylator phenotypes between diabetic patients and control subjects (chi 2 = 1.0, NS). Among patients with normal albumin excretion (n = 70, mean age: 12.9 +/- 3.5 years, mean diabetes duration: 5.3 +/- 3.8 years, AER < 20 micrograms/min), 35 (50%) fast acetylators and 35 (50%) slow acetylators were found. In patients with elevated albumin excretion (n = 16, mean age: 14.0 +/- 3.2 years, mean diabetes duration: 4.9 +/- 3.0 years, AER > 20 micrograms/min), 1 (6.3%) patient was a fast acetylator and 15 (93.7%) were slow acetylators. A significant difference has been found between the two groups in the rate of fast/slow acetylators (chi 2 = 8.79, p < 0.01). The strong correlation between the slow acetylator phenotype and microalbuminuria in diabetics suggests that: (a) genetic factors may play a role in the development of diabetic nephropathy; (b) the acetylator status could be a useful tool to detect patients 'at risk' of nephropathy.

Residential summer camps exist for children with all varieties of chronic illness with the goal of improving their quality of life. This paper describes the development and implementation of a summer camp for children 9-18 years old who receive long-term peritoneal dialysis or who have received a kidney transplant. Thirty-five to forty children regularly participate in activities such as water olympics, survival hikes and campouts while continuing to receive their medical needs from trained personnel. A study to evaluate the impact of a summer camp revealed less patient hopelessness and improved self-esteem following the 1-week camping experience. Attendance at camp provided the medical staff with a unique perspective of childhood illness, while the period of respite for the parents was uniformly welcomed and may contribute to the prevention of parent burnout. It is hoped that the success of this camp and others like it will lead to the development of similar experiences for other children with chronic disease.