Bile duct to portal space ratio and ductal plate remnants in liver disease of infants aged less than 1 year.

AIM: To validate the bile duct to portal space ratio as an independent factor useful for the prognosis of neonatal liver disease. METHODS: We assessed the maturation of the intrahepatic bile duct system (IBDS) in 87 consecutive infants aged less than 1 year undergoing non-subcapsular, adequate (at least six portal tracts), liver needle biopsies because of hepatomegaly and/or cholestasis. The maturation of the IBDS was evaluated by immunohistochemistry with an antibody directed to cytokeratin 7 (CK7), a biliary-type intermediate filament of the cytoskeleton, and a schema showing the IBDS remodelling. We used five categories to fit the different patterns of the IBDS remodelling using the ratio between the number of bile ducts and the number of portal tracts (BD/PT) and the presence of abnormal reaction patterns (marked intra-acinar pseudorosettes and/or periportal ductular proliferation): (A) abnormal reaction patterns with any BD/PT; (B) BD/PT = 0; (C) 0.1 < or = BD/PT < 0.5; (D) 0.5 < or = BD/PT < 0.9; and (E) BD/PT > 0.9 (B-E categories: no abnormal reaction patterns). Further, we evaluated cholestasis, portal fibrosis (PF), portal inflammation (PI), giant cell transformation (GCT), and extramedullary haematopoiesis (EMH). RESULTS: We identified A-E categories in 24, 14, 17, 8, and 24 biopsies, respectively. B and C categories were frequently observed in biliary atresia (BA), A category in neonatal hepatitis (NH), A-C categories in paucity of intrahepatic bile ducts (PIBD), and E category in 'other liver diseases' (OLD). Cholestasis, PI, GCT, and EMH were more frequent in A and C, while PF was variably seen in all categories. The lowest survival rate occurred in B (Kaplan-Meier estimator). CONCLUSIONS: (1) Biliary epithelial cell patterns recapitulate the primitive stages of the IBDS maturation; (2) abnormal reaction patterns occur mainly in NH, whilst BD/PT < 0.5 in BA; and (3) lack of intrahepatic bile ducts in infants aged less than 1 year is an adverse prognostic factor independent from aetiology of neonatal liver disease.

Failure to thrive in childhood.

BACKGROUND: Failure to thrive impairs children's weight gain and growth, their defenses against infection, and their psychomotor and intellectual development. METHODS: This paper is a review of pertinent articles that were published from 1995 to October 2010 and contained the terms "failure to thrive", "underweight", "malnutrition", "malabsorption", "maldigestion" and "refeeding syndrome". The articles were retrieved by a search in the PubMed and Cochrane Library databases. RESULTS: In developed countries, failure to thrive is usually due to an underlying disease. The degree of malnutrition is assessed with anthropometric techniques. For each patient, the underlying disease must be identified and the mechanism of failure to thrive understood, so that proper medical and nutritional treatment can be provided. Nutritional treatment involves either giving more food, or else raising the caloric density of the patient's food. Liquid formulas can be given as a supplement to normal meals or as balanced or unbalanced tube feeds; they can be given orally, through a nasogastric tube, or through a gastrostomy tube. Severely malnourished children with poor oral intake should be treated with parenteral nutrition. To avoid refeeding syndrome in severely malnourished children, food intake should be increased slowly at first, and phosphate, magnesium, and potassium supplements should be given. CONCLUSION: The proper treatment of failure to thrive in childhood consists of treatment of the underlying illness, combined with nutritional treatment that addresses the mechanism of the accompanying failure to thrive.

Tuberculous pericarditis in an infant evolving during triple chemotherapy.

UNLABELLED: A 36-month-old girl was treated for pulmonary tuberculosis (Mycobacterium tuberculosis) with isoniazid, rifampin and pyrazinamide. Four weeks after starting chemotherapy, she developed high fever and clinical signs of acute pericardial tamponade. Pericardial effusion was shown by echocardiography and subsequently removed by pericardiocentesis. M. tuberculosis was demonstrated in the pericardial fluid by microscopy, polymerase chain reaction and specific culture. After pericardial drainage, the actual therapy was extended to include streptomycin and prednisone. Follow-up examinations demonstrated complete recovery without signs of constrictive pericarditis. CONCLUSION: infants treated for tuberculosis should be followed closely in order to monitor not only side-effects of antituberculous drugs but also to detect early extrapulmonary spread that may occur even with adequate chemotherapy. Rapid intervention and treatment adjustment in infants with tuberculous pericarditis may prevent pericardial constriction and may lead to full recovery.