Transient hyperphosphatasemia after liver transplantation in infancy.
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Biomedical subjects
Publications and source records attributed to M Hermier.
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The purpose of this paper is to evaluate the efficacy and safety of CT-guided needle biopsies and to determine the optimal indications for this technique. The case histories of 118 patients who underwent a CT-guided biopsy for brain lesions during a six-year period, from November 1986 to September 1992, were reviewed. During a preliminary CT-scan, the entry site was determined and localized using a radio opaque marker and the safest route to the lesion was chosen. One hundred and thirty four procedures were performed in 118 patients. A positive diagnosis of tumour was obtained in 106 patients (89.8%). Repeat procedures were required in 18 patients. High-grade gliomas were the more common lesions (55.1%). Morbidity and mortality was assessed over the 30-day period after the procedure. Nine patients died during this time. Eight patients from day 3 to day 30 in the expected course of their disease and one within 48 first hours from neurological deterioration following the procedure. We found that CT-scan guided biopsies are a safe and accurate way to obtain brain tissue specimens for pathological diagnosis in selected cases. For superficial and large tumours it is a simple, fast and effective procedure.
We report the radiological features, including Doppler sonography and magnetic resonance angiography (MRA) findings, of a nongalenic arteriovenous fistula diagnosed in the neonatal period. Hypertensive hydrocephalus developed in infancy. Emergent percutaneous transarterial embolization with n-butyl-2-cyanoacrylate was successfully performed and lead to clinical improvement. MRA allowed a noninvasive follow-up.
UNLABELLED: In this study, 144 consecutive percutaneous liver biopsies performed with a 1.6 mm Menghini needle, during a 2-year period were reviewed. All the children were aged under 15 years, 57 patients less than 1 year and 87 more than 1 year. All biopsies were adequate and the mean number of portal tracts examined was 17.6 per biopsy (14.3 in patients weighing less than 10 kg and 19.1 in the others). There were no deaths and we observed only bleeding complications. In patients with normal coagulation (128 cases), 1 bleeding requiring transfusion occurred; and in patients with abnormal coagulation (16 cases), we observed 2 bleeding cases requiring transfusion. CONCLUSION: Percutaneous liver biopsy can be performed with 1.6 mm needles in children. For increased safety, ultrasound-guided biopsies are recommended.
Blunt abdominal trauma is common in children and induces significant morbidity and mortality. Trends in management of solid viscera lesions have changed over the past 15 years and a conservative approach is now preferred whenever possible. Such management has become possible in selected cases due to advances in pediatric intensive care and medical imaging. The authors provide a review-based on international literature data-of the respective roles of medical imaging studies in the initial management of blunt abdominal trauma in children. The place of these investigations is discussed for each organ, with emphasis on the diagnostic value of sonography and computed tomography. Diagnostic pitfalls and limitations in prognostic accuracy are discussed.
Percutaneous endoscopic gastrostomy (PEG) has become a good alternative to nasogastric tube feeding and surgical gastrostomy. The procedure requires two practitioners, one of them being an endoscopist. The technique can be performed either under local or general anesthesia, at bedside or in an operating room or endoscopic room, using a one step button or first setting of a catheter and secondly a button, by pull technique in which the button is pulled through esophagus or push technique were the button is pushed through the parietal wall. Its is used to deliver enteral nutriments in patients who need tube feeding. In paediatrics practice, morbidity rate related to PEG is less than 1% and morbidity less than 10%. PEG appears as a simple, safe, practical, esthetic and cheap procedure.
BACKGROUND: The diagnosis of Tangier disease in childhood is based on the specific aspect of tonsils or by screening relatives of affected subjects. CASE REPORT: A moderately enlarged liver associated with splenomegaly was found upon routine physical examination of a 3 month-old breast-fed boy, born in Turkey from consanguineous parents. Laboratory studies disclosed moderate increase in serum alanine aminotransferase activity (ALAT 52 UI/l, N < 30). The diagnosis of Tangier disease was confirmed by studies of plasma cholesterol and apolipoprotein A. By 8 months of age, the patient had enlarged orange tonsils. Small cervical, axillary and inguinal lymphadenopathies were present. The tonsilar and adenoidal tissues were removed at 18 months of age because the patient suffered from chronic airway obstruction. Colonoscopic examination revealed tiny flat orange spots, 1 to 2 mm in diameter, scattered throughout the rectosigmoidal and colonic mucosa. Survey of the family led to the discovery of one sister, with asymptomatic apolipoprotein Al deficiency and a normal sister, while the parents were heterozygotes for Tangier disease. CONCLUSION: Enlarged liver associated with a moderate level in serum aminotransferase may be an early manifestation of Tangier disease in infants. Rectosigmoidal and colonic lesions may be convenient for biopsy when tonsillectomy is not indicated.
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We present a case of aneurysm of the anterior communicating artery associated with a pituitary adenoma. Recognition of this association has important clinical implications, leading to modification of the surgical approach. The aneurysm was shown on MR angiography and confirmed with contrast arteriography and at surgery.
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Today, the duration of parenteral nutrition (PN) is unlimited. PN is used in digestive tract chronic disease (the digestive tract is either unusable or is at rest) or in oncology, hematology and renutrition before transplantation. Thanks to technical advances, PN, although sophisticated, may be applied at home if an active involvement of one parent is obtained. Home PN is indicated when it is planned for more than 3 months. The indications as a function of the disease, socio-cultural background and the distance to the PN centre are reviewed. The technical modalities such as vascular access, the choice of a catheter, the nutriments to be perfused, the parents' training and the logistic support are studied. Complications of home PN are identical to those of prolonged PN; infections is the most frequent. Results of PN as well as the quality of life of the children are briefly reported.
BACKGROUND: A common biliopancreatic channel can be revealed by cholestatic episodes. Its early removal avoids the development of liver cirrhosis and the risk of carcinomatous change. CASE REPORT: A 2 year 9 month-old girl had suffered from jaundice plus dark urine and pale stools for 2 months. A similar episode occurred 7 months later. Ultrasonography showed moderate dilatation of the intra and extrahepatic bile ducts that disappeared a few weeks later. A third episode of cholestasis with moderate dilation of the bile ducts occurred at the age of 3 year 11 months, complicated 2 weeks later by abdominal pain, vomiting and abdominal distension. Her serum amylase activity was 1,380 IU/l (N < 82). Ultrasonography and CT scan showed moderate dilation of the common hepatic duct. Liver biopsy showed pathological features consistent with bile obstruction. Endoscopic retrograde choledocopancreatography showed a long common channel with dilated extra and intrahepatic bile ducts, an incomplete pancreas divisum and numerous intracanalar stones. A sphincterotomy was performed and stones were extracted. The patient is well 18 months after surgery, with normal laboratory and ultrasonographic profiles. CONCLUSION: A common biliopancreatic channel is often associated with choledocal cyst. Whether development of the cyst is preceded by intermittent dilation of the bile ducts, as in this case, remains to be determined.
A 7 1/2-year-old patient with glycogenosis type lb had chronic neutropenia, recurrent bacterial infections, and severe hypoglycemia in spite of continuous nasogastric feeding. She was treated with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation. Absolute neutrophil counts and platelet counts rapidly reached normal values, and infectious episodes were reduced. She resumed oral feeding after transplantation and had no subsequent hypoglycemic episodes.
We report a case of intrahepatic cholestatis with normal serum levels of gamma GT who was similar to the benign recurrent cholestasis (BRC). The diagnosis of cholestasis in infants may be difficult and the distinction between BRC and Byler's disease should be made as early as possible.
Thanks to the technical progresses during the last decade and to the active participation of parents, parenteral nutrition may be undertaken at home in patients with chronic digestive or extra-digestive diseases with serious undernutrition. This technique being highly sophisticated and demanding, we have undertaken a study of the quality of life of 44 children submitted to home parenteral nutrition and followed by the four registered French centres of pediatric nutrition. This study shows that home parenteral nutrition improves the quality of life of both children and parents as compared with the preceding period in the hospital. However, because of its constraints, it requires a careful evaluation of the parents' capacity to cope with, before to be undertaken.
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