Hyperuricosuria in children: clinical presentation and natural history.

OBJECTIVE: Idiopathic hyperuricosuria (HU) was previously reported in only a limited number of children with hematuria. We aimed to outline the clinical presentation and natural history of HU not only in children with hematuria, but also in those with dysuria and/or recurrent abdominal/flank pain and a family history of urolithiasis. STUDY DESIGN: Retrospective analysis of data at diagnosis from 102 consecutive children with HU and outcome analysis of 26 of them who were followed >/=1 years (mean: 3.1) with no specific therapy. RESULTS: Sixty-one participants had HU and 41 had HU + hypercalciuria. Fifty-two patients had no hematuria among the presenting symptoms, more than one third had normal urinalysis at our first examination, one half had microcalculi (<3 mm in diameter) at renal sonography, and 12% had stones (4-18 mm). Thirty participants of the 39 with no hematuria at our first examination (77%) showed microcalculi or calculi at renal sonography. The patients with microcalculi were significantly older than were those without microcalculi. During the follow-up, 4 of 26 children never had hematuria and 8 had no hematuria during most of the follow-up period. Two patients who had a calculus at first visit and 3 who formed calculi 4 to 12 mm in diameter, after 1 to 3.5 years subsequently passed them in the urine. CONCLUSIONS: The lack of hematuria is not predictive of absence of urolithiasis. Therefore, it may be misleading to judge on the efficacy of a given therapy only based on disappearance of hematuria. HU and hypercalciuria have to be suspected in children with dysuria and those with recurrent abdominal/flank pain and familial history of urolithiasis, although they have no hematuria.

[Dental enamel hypoplasia in a group of celiac disease patients. Clinico-epidemiologic correlations]. / Ipoplasia dello smalto dentario in un gruppo di soggetti celiaci. Correlazioni clinico-epidemiologiche.

BACKGROUND: The aim of present study was to carry out a personal clinical-epidemiological research concerning a possible correlation between coeliac disease and enamel hypoplasia. MATERIALS AND METHODS: Forty-five patients of Cam-pania, aged between 2 and 26 years old, with a diagnosis of coeliac disease (diagnosis in accordance with recent protocol ESPGAN), were subjected to a careful dental examination: at the same time a control-group formed by 105 healthy subjects, of the same province, age- and sex-matched with coeliac patients was examined. RESULTS: The finding of enamel hypoplasias was more significant in coeliac patients (11 cases with a percentage of 2.4%) than in controls (5 cases with a percentage of 4.7%). CONCLUSIONS: The percent rate, if on the one hand is lower than in Finnic studies, on the other hand fundamentally reflects the results of studies on the same topic carried out by other Italian groups. Furthermore also in this research the involvement of milk-teeth is observed as reported by other authors. This appears to confirm a clear association between coeliac disease and dental hard tissues alterations.

Restoration of body composition in celiac children after one year of gluten-free diet.

Restoring protein, fat, and bone compartments in celiac children on a gluten-free diet (GFD) is not yet well understood. Hence, anthropometric, biochemical, and bone densitometric assessment was performed in 23 celiac children, 8 boys and 15 girls, aged 1 to 12 years (mean 4.7), at diagnosis and 1 year after GFD. At diagnosis the patients had height, arm muscle area, triceps skinfolds, subscapular skinfolds, fat area index, and bone mineral content significantly lower than in an age- and sex-matched control group. After 1 year on GFD, no significant difference was found between patients and controls in all the parameters studied except in height and arm muscle area, which, however, were very near to the normal expected. Serum hemoglobin, iron, and zinc values were below the normal range in more than one half of patients at diagnosis and within the normal range in almost all of them after 1 year of GFD. Serum hemoglobin, iron, zinc, triglycerides, proteins, albumin, and calcium values significantly rose during the year of GFD. A year of GFD in celiac children allows a virtually complete return in body mass composition.

Height and weight in children with vesicoureteric reflux and renal scarring.

Height standard deviation scores (HSDS) and weight-for-height index (WHI) at diagnosis were evaluated in 156 children aged 2 months to 10.8 years (mean 3.7 years) with vesicoureteric reflux (VUR) and normal creatinine clearance, and in 156 age- and sex-matched healthy controls. Forty-three patients had bilateral VUR with scintigraphic signs of renal scarring (B SCAR+), 25 had bilateral VUR without renal scarring (B SCAR-); 40 had unilateral VUR with (U SCAR+) and 48 unilateral VUR without (U SCAR-) renal scarring. B SCAR+ patients had an average HSDS of -0.5 +/- 1.4 (SD) which was significantly (P = 0.02) below that of controls (0.05 +/- 1 HSDS) and an average WHI of 100.6% +/- 16% which was significantly (P = 0.007) below that of controls (108% +/- 12%); 14% of B SCAR+ patients had a height below -2 HSDS. B SCAR-, U SCAR+, and U SCAR- patients had heights near to O HSDS which was not different from that of controls, as well as WHI between 104% and 107.9%, which was not different from that of controls. HSDS and WHI were significantly (P = 0.00001) correlated in patients but not in controls. B SCAR-, U SCAR+, and U SCAR- patients are similar to healthy controls in weight and in height growth and have, on average, some excess weight as do the latter. In contrast, B SCAR+ subjects have a significant decrease of the relative height and normal WHI.

Altered body water distribution in subjects with juvenile rheumatoid arthritis and its effects on the measurement of water compartments from bioelectric impedance.

OBJECTIVE: To assess the reliability of bioelectric impedance analysis (BIA) for predicting total body water (TBW) and extracellular water (ECW) in children affected by juvenile rheumatoid arthritis (JRA). SUBJECTS: Thirty-nine children affected by JRA and 23 healthy children of similar age (11.0 +/- 3.6, range 3.0-19.0 y) were recruited for the study. METHODS: TBW and ECW were measured by deuterium oxide and bromide dilution, respectively. Bioelectric impedance (Z) was measured at frequencies of 5, 50 and 100 kHz. The prediction of TBW and ECW from BIA was based on the impedance index (ZI = height2/Z, cm2/omega). RESULTS: TBW standardized per kg of body weight and ECW standardized per litre of TBW were significantly higher in JRA as compared to control patients (59.7 +/- 2.4 vs 57.7 +/- 2.7% and 44.5 +/- 4.6 vs 38.1 +/- 7.9%, with P < 0.005 and P < 0.0001, respectively). Moreover, intracellular water standardized per litre of TBW was significantly lower in JRA than in control subjects (55.5 +/- 4.6 vs 62.5 +/- 8.1, with P < 0.0001). In both controls and patients, the use of ZI at 5kHz offered the more accurate prediction of ECW. However, the use of ZI at 100 kHz did not offer a better prediction of TBW as compared to its value of 50 kHz. Control-generated formulae for predicting water compartments from BIA [TBW = 0.716 x ZI at 100 kHz-1.504, r = 0.934, s.e.e. = 2.2 l;:ECW = 0.430 x ZI5-3.652, r = 0.869(7) s.e.e. = 1.7 l] underestimated TBW and ECW in JRA patients. However, population-specific formulae [TBW (1) = 0.766 x ZI at 100 kHz-0.053, r = 0.939, s.e.e. = 2.8 l; ECW (l) = 0.399 x ZI at 5 kHz-0.283, r = 0.886, s.e.e. = 1.7 l] allowed an accurate prediction of TBW and ECW in JRA patients, taking into account their altered body water distribution. CONCLUSIONS: Altered water distribution impedes the use of formulae developed on healthy children to predict TBW and ECW from BIA and JRA patients. It is hypothesized that chronic inflammation and subclinical malnutrition may be responsible for the altered body water distribution of JRA patients. Traditional body composition models may require adjustments for use in JRA children due to their altered body hydration and water distribution.

Pubertal growth in children with chronic renal failure on conservative treatment.

The pubertal growth spurt was followed for at least 3 years in 5 boys and 6 girls with chronic renal failure on conservative treatment. The peak height velocity averaged 8.6 cm/year (range 5.8-10.1 cm/year) in males and 8.2 cm/year (range 6.4-11.5 cm/year) in females. In none was the pubertal growth spurt below the 3rd percentile for chronological age. At the end of the follow-up period, all patients but 2 had stature within the normal limits of parental target. The relative variation of height averaged - 0.013 standard deviation scores per year. On the whole, the pubertal growth spurt was normal in subjects with chronic renal failure on conservative treatment.