We tested the hypothesis that patients with Prader-Willi syndrome (PWS) may be at lower risk of developing non-alcoholic fatty liver disease (NAFLD) because of a higher insulin sensitivity. Twenty-one PWS patients and 42 control subjects closely similar for age, gender, pubertal stage and body mass index (CNT), were studied. Metabolic profile and body composition were assessed. NAFLD was established by a validated method of US grading (range from G0 to G3). PWS patients showed a significantly better metabolic profile (lower waist circumference, fasting glucose levels, HOMA-IR, cholesterol, transaminase levels and trunk fat mass/fat mass ratio). Furthermore, NAFLD G1stage was significantly more frequent in PWS subjects (P < 0.05), whereas G2 stage was significantly more frequent in control patients (P < 0.05). NAFLD grading seems to correlate with body composition in PWS, also after adjustment for sex and GH treatment. To our knowledge, this is the first report suggesting a reduced risk of NAFLD in PWS children.
Prognosis concerning renal function seems to be mainly related to two factors: age at presentation and underlying diagnosis. Decompression of the urinary tract by urine extravasation produces the best results in terms of preservation of the renal function in LUT obstruction (75% in prenatal and 84% in postnatal cases); UUT obstruction however is associated with a good prognosis in the neonatal period (80% of preserved renal function) but with a severe impairment (only 20% of preserved renal function) in prenatally detected cases.
The Malone surgical procedure for antegrade continence enema (ACE) greatly improves the quality of life of many patients with fecal incontinence but has several complications. This report describes a technique of percutaneous endoscopic cecostomy (PEC) which was applied in three children with fecal incontinence secondary to anorectal malformation or spina bifida. Using a pediatric fiberscope, a colonoscopy was done which reached the right colon. An intraoperative ultrasound examination confirmed the position of the cecum just below the layers of the abdominal wall. The cecostomy tube was then percutaneously inserted, imitating the steps of percutaneous endoscopic gastrostomy (PEG) using the pull technique. The preliminary results are very encouraging: there are no procedure-related complications in this small series; all the patients are able to carry out the antegrade enema by themselves; and complete control of defecation has been obtained.
Subinguinal "artery- and lymphatic-sparing" microsurgical varicocelectomy, considered the gold standard treatment in adults, also proved to be successful in this preliminary experience with pediatric patients. The minimally invasive approach ensures the prevention of recurrences and/or postoperative complications, and is associated with a very low morbidity rate.
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