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Myelomeningocele causes serious locomotor disability, osteoporosis and pathologic fractures. The aim of this study was to investigate the relationship between body composition, bone mineral density, walking ability and sport activity in myelomeningocele children. 60 patients aged between 5 and 14 yrs with myelomeningocele (22 ambulatory and 38 non-ambulatory), were studied. Fat mass and fatfree-mass were calculated by anthropometry. The bone mineral density at lumbar and femoral neck were evaluated. Bone mineral density at the lumbar and femoral neck was lower than in the normal population. In the non-ambulaty group, bone mineral density was ~1 SD lower than in the ambulatory one (p <0.01). Fat mass was greater than expected but without significantly differences between walking group (mean 26%) and wheel-chair users (25%). Patients practised sport activity had a better bone mineral density and body fat compared with other patients with the same disability. Patients with myelomeningocele have decreased bone mineral density and are at higher risk of pathologic bone fractures. All subjects showed an excess of fat as percentage of body weight and are shorter than normal children. The measurement of bone mineral density may help to identify those patients at greatest risk of suffering of multiple fractures. Walk ability and sport activity, associated with the development of muscle mass, are important factors in promoting bone and body growth, to reduce the risk of obesity and of pathological fractures. [ABSTRACT FROM AUTHOR]

Interventions directed to the recognition of abnormal bone mineral density, bone mineral content, and body composition in the pediatric age require the definition of factors influencing bone mass acquisition during growth. We have evaluated in a cross-sectional manner by dual-energy X-ray absorptiometry the impact of sex, age, puberty, and physical activity on total body areal bone mineral density, regional (lumbar and femoral) bone mineral densities, bone mineral content, and body composition (fat mass and lean mass) in a cohort of 359 healthy Italian children aged 3-14 years and investigated their specific contribution to bone mass accrual. Statistical multiple regression analysis was performed dividing the population in pre- and post-pubertal groups. Bone mineral density at the lumbar spine has resulted equally distributed in both sexes before puberty while has resulted higher at the femoral necks in males at whatever age. A significant effect on bone mass acquisition was exerted by male sex and lean mass. In the areas where the cortical bone is prevalent, males of the pre-pubertal group have presented the highest values; in the areas where the cancellous bone is prevalent, both sexes were equivalent until the age of 9 years, but after this age, females have presented higher increases, probably related to the inferior dimensional development of lumbar vertebrae. Conclusively, male sex and lean mass seem to represent independent predictors of bone mass accrual in the cortical bone of the examined children, while female sex and pubertal maturation are independent predictors of bone mass accrual in the trabecular bone.

A number of studies have focused on quality of life in children with spina bifida, concentrating for the most part on younger children. We assessed health-related quality of life and disability in adolescents with spina bifida and correlated these with the clinical and neurophysiologic picture. Twelve consecutive spina bifida cases were evaluated through a multiperspective protocol by means of (1) clinical and neurophysiologic assessment, (2) a self-administered questionnaire for general health--Short Form-36 (SF-36)--and (3) standardized disability measurements (Functional Independence Measure, Barthel Index, Deambulation Index). Relationships between disability measurement, the adolescents' perspective, and conventional clinical and neurophysiologic assessment were evaluated. The Functional Independence Measure is strongly related to the clinical measurement of muscle strength (P < .03, r > .6). The Barthel Index and Deambulation Index are related to clinical measurement of muscle strength (P < .03, r > .6), as well as to electromyographic findings in the proximal muscles of inferior limbs (P < .05, r > .6). Although the function of lower limb muscles, especially the proximal muscles, is closely related to impairment of physical aspects of quality of life, it is only partially related to the mental aspects (P < .03, r > .6). The findings at clinical examination, especially proximal deficit of inferior limbs, were usually related to higher disability and lower physical aspects of quality of life. The multiperspective evaluation of adolescents with spina bifida shows a high correlation between conventional neurologic-neurophysiologic measurements and the adolescents' own perspective of their quality of life. Our data demonstrated that the conventional clinical and neurophysiologic measurements are useful not only because they provide a measure of lower limb impairment but also because they appear to be related to physical aspects of the adolescent's quality of life.

Many factors have been proposed as predictors of poor renal prognosis in children with hemolytic uremic syndrome (HUS), but their role is still controversial. Our aim was to detect the most reliable early predictors of poor renal prognosis to promptly identify children at major risk of bad outcome who could eventually benefit from early specific treatments, such as plasmapheresis. Prognostic factors identifiable at onset of HUS were evaluated by survival analysis and a proportional hazard model. These included age at onset, prodromal diarrhea (D), leukocyte count, central nervous system (CNS) involvement, and evidence of Shiga toxin-producing Escherichia coli (STEC) infection. Three hundred and eighty-seven HUS cases were reported; 276 were investigated for STEC infection and 189 (68%) proved positive. Age at onset, leukocyte count, and CNS involvement were not associated with the time to recovery. Absence of prodromal D and lack of evidence of STEC infection were independently associated with a poor renal prognosis; only 34% of patients D(-)STEC(- )recovered normal renal function compared with 65%-76% of D(+)STEC(+), D(+)STEC(-) and D(-)STEC(+ )patients. In conclusion, absence of both D and evidence of STEC infection are needed to identify patients with HUS and worst prognosis, while D(-) but STEC(+) patients have a significantly better prognosis.

The occurrence of interstitial nephritis in patients receiving antimicrobial therapy has frequently been reported in adults while it has rarely been described in children. We report the case of a patient treated with amoxicillin who presented hallucinations and serosanguineous blisters during treatment and developed renal failure a few days after discontinuation of the drug. On renal biopsy an interstitial nephritis with tubulitis was identified., (Copyright 2003 S. Karger AG, Basel)

Objectives: To assess the health-related quality of life (QOL) and disability in young patients with spina bifida and to correlate them with the clinical examination findings., Design: Prospective multidimensional study by means of (1) clinical assessment, (2) self-administered questionnaire for general health, and (3) standardized disability measurements. Relationships between disability measurement, patient-oriented examination, and conventional clinical assessment were evaluated., Setting: Pediatric department at a university hospital in Italy., Patients: Twelve consecutive young patients with spina bifida (mean age, 15.2 y; range, 14-18 y)., Interventions: Not applicable., Main Outcome Measures: The Medical Outcomes Study 36-Item Short-Form Health Survey, the FIM trade mark instrument, and the Barthel Index., Results: As expected, disability was inversely related (r=.72, P<.02) to the physical aspect of QOL. Unexpectedly, for the mental aspects of QOL, less disability was associated (r=-.70, P<.05) with higher psychologic distress and severe role disability because of emotional problems. The findings at clinical examination, especially proximal deficit of inferior limbs (r=-.70, P<.05), were usually related to higher disability and lower physical aspects of QOL., Conclusion: There was no linear inverse correlation between disability and QOL in patients with spina bifida. Patients with mild disability needed as much psychologic support as patients with severe whole disability., (Copyright 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation)

The main goal in the management of children with neurogenic bladder is to preserve renal function and assure a socially acceptable continence. Urodynamic testing based on cystomanometry evaluation of intravesical pressure and flow measurement, at present is considered the examination of first choice in the diagnosis of neurogenic micturition disorders to better classify the type of bladder based on the risk of upper urinary tract impairment and be able to plan a specific, personalized management.

The neurogenic bladder represents a vesical dysfunction secondary to a congenital or acquired neurologic lesion, spinal dysraphism being the most common cause in pediatric age. According to the lesion level and severity, neurogenic bladder can be hypertonic hyperreflexic, hypotonic hyporeflexic or of mixed type characterized by different clinical and urodynamic patterns and the potential cause of nephrourologic complications. These types of neurogenic bladder require diversified treatments as anticholinergic drugs, clean intermittent catheterization and antibiotic prophylaxis, separately or in various combinations. Close follow-up of children is necessary to control and prevent the onset of complications with changes in medical therapy or surgical treatment if required.

Purpose: Urological management of spina bifida patients is controversial. The goals of therapy of neurogenic bladder are continence, prevention of infections and preservation of urinary tract. Desmopressin has been recently used in a spina bifida population that is dry during the day (daytime continence was achieved with clean intermittent catheterization and anticholinergics) but wet at night. The aim of this study was to assess plasma antidiuretic hormone (ADH) levels in these children., Materials and Methods: The study included 24 patients, 11 males and 13 females (mean age 6.4 years) referred to the Spina Bifida Centre of the Catholic University of Rome, and 57 normal age-matched controls. Morning (07.30-08.00 h) plasma ADH levels were measured using a specific radioimmunoassay., Results: Plasma ADH levels (normal range 5-11 microg/l) did not differ between spina bifida population and healthy controls. Serum ADH had a mean of 6.8 microg/l in affected children and a mean of 7.4 microg/l in the controls., Conclusion: We conclude that the use of desmopressin in children with spina bifida should be reserved only in patients with decreased secretion of ADH, or may be useful in patients with persistent nocturnal incontinence to reduce night wetting. Therefore, research with a larger population is needed., (Copyright 2002 S. Karger AG, Basel)

Purpose: The purpose of this study was to compare contrast-enhanced gray-scale voiding urosonography (CE-VUS) and contrast-enhanced color Doppler voiding urosonography (CE-CDVUS) with voiding cystourethrography (VCUG) to verify whether the use of color Doppler imaging improves the diagnosis and grading of vesicoureteral reflux (VUR)., Methods: In 74 patients, CE-VUS and CE-CDVUS were compared with VCUG, which was used as the gold standard. SHU 508 A (Levovist) was used as the echo-enhancing contrast agent. VUR was diagnosed if hyperechoic dots or color signals were visualized in the ureter on sonograms. VUR grading was based on morphologic and dynamic findings on CE-VUS and morphologic and color findings on CE-CDVUS. VCUG was performed conventionally, and grading by VCUG was in accordance with the international system of radiographic VUR grading. Patients who voided during 1 examination only (either CE-VUS and CE-CDVUS or VCUG) were excluded from the study. Agreement between the results of CE-VUS and VCUG and between those of CE-CDVUS and VCUG in diagnosing VUR was calculated by kappa statistics. CE-VUS and CE-CDVUS were compared for diagnostic accuracy by the McNemar test., Results: The agreement between CE-VUS and VCUG in predicting VUR was 90% (kappa score, 0.77; p < 0.001). The agreement between CE-CDVUS and VCUG was 96% (kappa score, 0.91; p < 0.001). CE-CDVUS showed a significantly higher diagnostic accuracy than did CE-VUS (96% versus 90% of cases correctly classified; McNemar chi2 = 4; p < 0.05). This was mainly related to the lower number of false-negative results for grade I and grade II VUR when CE-CDVUS was used., Conclusions: The use of color Doppler imaging significantly improves the accuracy of contrast voiding urosonography in the detection and grading of VUR.

The pathophysiological events regarding the atherosclerotic processes are present already in early childhood. Various clinical trials confirm tight correlation between dyslipidemia and cardiac and cerebrovascular complications. Over the clinical dyslipidemia genetically determined, various systemic diseases can lead to anomalies of the lipidic metabolism. The determination and analysis of the lipidic pattern is essential during childhood and in particular in patients with renal disease for the increased risk of progressive failure of renal function and for the central role of kidney in lipidic homeostasis. Cardiovascular event is the most important cause of mortality in patients with end-stage renal disease. In children with nephrotic syndrome, renal failure, hemodialysis or after renal transplantation, an increase of the serum values of triglycerides, cholesterol, LDL, VLDL is generally observed. The presence of anomalies in lipidic pattern must be considered during the course of renal disease for preventing the progression of renal damage. Thus, the pharmacological therapy and diet permit to prevent the atherosclerotic events.