Ischemic necrosis of femoral condyles in systemic lupus erythematosus

Avascular necrosis (AN) of bone is a complication of systemic lupus erythematosus (SLE). In the present paper we describe two patients with SLE who presented an AN of femoral condyles 18 months and 2 years respectively after the diagnosis of the disease. In the second case, together with the destructive change of the symptomatic knee, X-ray films showed an initial involvement, still asymptomatic, of the contralateral knee

Congenital hepatic fibrosis with gastrointestinal bleeding in early infancy.

We report a case of a 19-month-old infant with congenital hepatic fibrosis. The clinical features consisted of portal hypertension with massive gastrointestinal bleeding, recurrent cholangitis, and cystic dysplasia of the kidneys, without impairment of renal function. The dramatic course of the disease required surgical treatment. Congenital hepatic fibrosis with life-threatening gastrointestinal bleeding is extremely rare below three years of age. The purpose of the present report is to stress the need of looking for varices in all cases of congenital hepatic fibrosis, even when occurring in early infanc

Hepatic sinusoidal dilation in the course of histiocytosis X

We present a case of disseminated histiocytosis X with hepatic involvement characterized by sinusoidal dilation. This histological feature regressed in connection with the clinical remission of the disease. The present report suggests that sinusoidal dilation might represent a feature of hepatic involvement in the course of histiocytosis

Non-Hodgkin's lymphoma in childhood presenting as thyroid enlargement

The authors report a case of a 10-year-old girl with early involvement of the thyroid gland by non-Hodgkin's lymphoma, an uncommon site of presentation of childhood lymphoma

Diagnostic accuracy of the atopy patch test in children with food allergy-related gastrointestinal symptoms.

BACKGROUND: Little is known about the diagnostic accuracy of atopy patch tests (APT) in the clinical practice of pediatric gastroenterology. Moreover, APTs containing purified food extracts have recently become available, but their diagnostic accuracy is largely undefined. PATIENTS AND METHODS: We evaluated the diagnostic accuracy of food challenge, skin prick test (SPT), serum specific IgE determination, and APT using fresh food and commercial food extracts in parallel in children referred for suspected food allergy-related gastrointestinal symptoms. RESULTS: Eighty-nine food challenges were performed in 60 patients (38 boys, median age 23 months, range 3-48 months): 31 tested positive for cow's milk (CM), 19 for hen's egg (HE), and two for wheat. Specific immunoglobulin E (IgE) determination, and SPT, respectively, were positive in 7/31 and 14/31 of patients with cow's milk allergy (CMA), and in 7/19 and 7/19 with HE allergy. The results of APT with fresh food vs a commercial assay were (1) CM: sensitivity: 64.5%vs 6.4%, specificity 95.8%vs 95.6%, positive predictive value (PPV) 95.2%vs 66.6% and negative predictive value (NPV) 67.6%vs 43.1%; (2) HE: sensitivity 84.2%vs 5.2%, specificity 100%vs 100%, PPV 100%vs 100% and NPV 75.0%vs 33.3%. CONCLUSIONS: Atopy patch test is a useful tool in the diagnostic work up of children with food-allergy-related gastrointestinal symptoms. The diagnostic accuracy of ATP was higher with fresh food than with commercial food extracts