26 research outputs found
Hepatic sinusoidal dilation in the course of histiocytosis X
No full textWe 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
Congenital hepatic fibrosis with gastrointestinal bleeding in early infancy.
No full textWe 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
Ischemic necrosis of femoral condyles in systemic lupus erythematosus
No full textAvascular 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
Non-Hodgkin's lymphoma in childhood presenting as thyroid enlargement
No full textThe 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
Rhabdomyolysis and coeliac disease: A causal or casual association? A case report and review of literature
No full textBackground: Rhabdomyolysis is a rare, potentially life-threatening condition, caused by multiple disorders. The association with Coeliac Disease (CD) has been rarely reported and in these cases muscular damage was imputed to hypokalemia. Herein we describe a new case of severe rhabdomyolysis in a child subsequently diagnosed as affected by CD, and review previous reports. Case presentation: A 3-year-old boy was referred for diarrhea, brown urine, muscular pain/weakness, and no history of muscular trauma. At entry, laboratory tests showed elevated levels of creatine kinase (CK) (x 100 unv) and aspartate aminotransferase (AST) (x 10 unv), alanine aminotrasferase (ALT) (x 5 unv); electrolytes were within the reference range. Twenty-four hours after admission serum CK peaked 115,000 U/L and transaminases increased up to 30 times unv. Hyperhydration treatment was started with renal function monitoring. Urine output decreased little, while serum creatinine and urea nitrogen stayed within the reference range. Serum potassium levels went down to 2.8 mEq/L at day 3, in spite of supplementation. The patient completely recovered at day 16. Main metabolic causes of rhabdomyolysis were ruled out by appropriate tests. Because of rarely reported cases of CD/rhabdomyolysis, anti-tissue transglutaminase (tTG) antibodies were measured and found positive (IgA 34 U/mL, unv <9). HLA typing was DQA1 05:02, DQB1 03:02. As jejunal biopsy showed patchy villous atrophy, gluten free diet (GFD) was prescribed. One year after starting GFD, histology was normal. Review of literature: Literature (search engines: PUB MED and GOOGLE SCHOLAR) from 1980 to 2016 retrieved 8 cases (age range: 12 to 75 years old) previously described. Conclusion: The present case suggests to check for CD in children with severe rhabdomyolysis. Because severe rhabdomyolysis itself may elevate the serum potassium levels, hypokalemia might go unrecognized as the cause of muscular damage
Sharp-Pointed Foreign Body Ingestion in Pediatric Age
No full textObjectives: To assess the clinical complications reported after the ingestion of sharp/pointed foreign bodies (FBs) in pediatric age, their incidence among all FB ingestions, and the features and clinical presentation of children. Study design: We have recruited all consecutive patients aged 0-14 years, admitted for sharp/pointed FB ingestion. Clinical data until hospital discharge were accurately recorded, including both children with esophagogastric FB retention who underwent endoscopic removal and children who were radiologically followed-up till spontaneous FB expulsion. Clinical outcomes were recorded for each patient, with special reference to possible prolonged retention and wall perforation during the intestinal passage. Results: We have enrolled 580 children (males/females: 292/288; age range: 11-180 months; mean age ± standard deviation: 50.5 ± 42 months). Sharp/pointed FBs mainly included fragments of metal 270 of 580 (46.55%) and glass 180 of 580 (31%). FBs were endoscopically removed in 79 of 580 (13.6%) children whereas the remaining FBs passed through the gastrointestinal tract over an overall mean time of 29 hours. No cases of intestinal perforation nor prolonged retention were observed. In 3 of 65 (4.6%) procedures the endoscopist faced an uncomfortable endoscopic removal due to the shape and size of the FB which hampered the retrograde passage through the esophageal sphincters. Conclusions: Our original and extensive data emphasize that accidental ingestion of sharp/pointed FB ingestion is a current issue in pediatric age, especially in toddlers. Metal and glass objects are the most involved FBs and their endoscopic retrieval may not be easy in about 5% of cases. Fortunately, in our pediatric sample no surgical intervention was needed
