Acute acalculous cholecystitis associated with severe EBV hepatitis in an immunocompetent child.

Acute acalculous cholecystitis (AAC) is an inflammation of the gallbladder in the absence of demonstrated stones, which is rarely seen in paediatric population. The diagnosis is accomplished mainly through abdominal ultrasonography in the appropriate but usually non-specific clinical picture. Complicated cases need surgical intervention; the medical management is mainly constituted by supportive and antibiotic therapy, as most AAC are observed in the setting of systemic bacterial or parasitic infections.

Long-chain polyunsaturated fatty acids profile in plasma phospholipids of hyperphenylalaninemic children on unrestricted diet.

Introduction: The aim of the present study was to examine whether hyperphenylalaninemic children on unrestricted diet (MHP) may exhibit a different LCPUFA profile from PKU or healthy children in plasma phospholipids.

Patients And Methods: Forty-five MHP children (age 9-14 years) were age and sex matched with 45 PKU and 45 healthy children. Fatty acids were determined and expressed as % of total fatty acids.

Anomalies of the kidney and urinary tract are common in de Lange syndrome.

Sixty-one patients affected by de Lange syndrome underwent a careful renal and urological evaluation including family and personal history, physical examination, urinalysis, renal tract ultrasonography, and serum creatinine. A voiding cystourethrography was performed in patients with urinary tract infections, in patients with renal ectopy, and in patients with small kidneys. Structural anomalies of the kidney and urinary tract were detected either by ultrasound or voiding cystourethrography in 25 patients (41%): absent or poor corticomedullary differentiation (N = 8; 13%), pelvic dilation (N = 6; 10%), vesicoureteral reflux (N = 5; 8%), small kidney (N = 3; 5%), isolated renal cyst (N = 3; 5%), and renal ectopia (N = 2; 3%).

Additional case of Tsukahara's syndrome or new syndrome: further delineation of the association of microcephaly and radio-ulnar synostosis.

In 1994, Giuffré et al. reported two unrelated families in which some of the members had microcephaly and radio-ulnar synostosis, suggesting a new condition. Since this first report, Tsukahara et al.