[Hepatic adenoma with high phlogosis indices: what relationship? Description of a pediatric clinical case.].

Hepatocellular adenoma is a benign neoplasm that accounts for 2-4% of pediatric liver tumors. The inflammatory variant of the adenoma can be expressed at the clinical level only with the evidence of persistent elevation of the inflammatory indices, since the hepatic mass is itself silent unless there are complications. The main complications relate to the bleeding risk, the chronic inflammatory state and the low risk of malignant evolution.

[Kawasaki disease shock syndrome: a case report].

Kawasaki disease is a systemic vasculitis that presents with stable vital signs. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during the acute stage, Kawasaki disease shock syndrome (KDSS) has recently been described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state.

The importance of disease prevalence in assessing the diagnostic value of a test: endoscopic markers in celiac disease.

Background/aims: We evaluated the diagnostic variability and reproducibility of endoscopic signs in two populations with a different pretest likelihood of celiac disease (CD).

Methods: We recruited 289 CD patients (both adults and children) in a multicenter prospective study. Group 1 (high risk) included 111 patients referred for positive serology.

Stunting is an independent predictor of mortality in patients with cystic fibrosis.

Background & Aims: Some studies have shown a direct relationship between nutritional status and survival in Cystic Fibrosis (CF) patients. Body wasting, defined as a percentage of the ideal body weight for age, has been shown to be an independent predictor of mortality in CF. With respect to height only two studies were performed and these studies suggested that stunting is an important determinant of survival but both did not adjust statistical analysis for confounding variables.

Validation of a predictive survival model in Italian patients with cystic fibrosis.

Background: In 2001 Liou published a 5-year survival model using CFF Registry data.

Aims: To evaluate its validity in predicting survival in Italian CF patients.

Methods: In a retrospective study on 945 patients, the 9 variables selected by Liou were analyzed, vital status on December 2008 recorded and observed and expected deaths compared.

Polymorphisms of tumor necrosis factor-alpha but not MDR1 influence response to medical therapy in pediatric-onset inflammatory bowel disease.

Aim: We investigated the contribution of variants of tumour necrosis factor (TNF)-alpha and MDR1 genes in the predisposition and response to medical therapy in a large pediatric cohort of patients with Crohn disease (CD) and ulcerative colitis (UC).

Patients And Methods: In this study, 200 patients with CD, 186 patients with UC, 434 parents (217 trios), and 347 healthy unrelated controls were investigated. Single-nucleotide polymorphisms -G308A and -C857T of the TNF-alpha gene and C3435T of the MDR1 gene were investigated and correlated with clinical subphenotypes and efficacy of medical therapy.