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C Le Pommelet

Publications and source records attributed to C Le Pommelet.

15 recordsLinked to original sources
PubMed2001

Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?

OBJECTIVES: The prognosis of hepatoportoenterostomy (the Kasai operation) for biliary atresia worsens when the age at surgery increases. This study examines whether the Kasai operation remains justified after 3 months of life. STUDY DESIGN: Records for all patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed, and patients were classified into 3 groups: group 1 (n = 30), no contraindication to the Kasai operation, but orientation to de novo transplantation; group 2 (n = 380), age at Kasai operation <90 days; and group 3 (n = 60), age at Kasai operation > or =90 days. Survival with native liver, survival after liver transplantation, and overall survival (Kaplan-Meier method) were compared by using the log-rank test. RESULTS: Five-year (10-year) survival with native liver was 35% (30%) in group 2 and 25% (22%) in group 3 (P =.03). Five-year overall survival was 57%, 74%, and 55% in groups 1, 2, and 3, respectively (P =.003). Poor results in groups 1 and 3 were mainly due to increased pre-transplantation mortality, but survival after transplantation was not significantly different in the 3 groups. CONCLUSIONS: Performance of the Kasai operation after 3 months of age is justified in selected cases, because it may obviate liver transplantation. Preoperative evaluation should exclude patients with advanced liver disease for whom liver transplantation should not be delayed.

Age Factors↗
PubMed1999

Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996.

Since the sequential treatment of Kasai operation with or without liver transplantation became available, the overall prognosis of biliary atresia remains unclear. This study examined the prognostic factors from diagnosis. All patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed. Actuarial survival rates were calculated for survival with native liver, survival after liver transplantation, and overall survival. Potential prognostic factors were analyzed using the logrank test and the Cox model. A total of 472 patients were identified. Ten-year overall survival was 68%. Independent prognostic factors for overall survival were (S = 10-year rates) performance of Kasai operation (performed: S = 69%; not performed: S = 50%), age at Kasai operation (</=45 days: S = 80%; >45 days: S = 66%), anatomical pattern of extrahepatic bile ducts, polysplenia syndrome, experience of the center (</=2 new biliary atresia [BA] patients/year [24 centers]: S = 54%; 3 to 5 [2 centers]: S = 60%; >/=20 [1 center]: S = 78%). Survival with native liver depended on the same independent prognostic factors. In conclusion (1) Kasai operation remains the first line treatment of BA, and (2) early performance of Kasai operation and treatment in an experienced center reduces the need for liver transplantation in infancy and childhood and provides children with the best chance of survival.

Biliary Atresia↗
PubMed1999

Epidemiology of biliary atresia in France: a national study 1986-96.

BACKGROUND/AIMS: The reported incidence of biliary atresia varies from 5 to 32/100000 live births. The existence of seasonality and/or clustering is controversial. Based on a large population analysis, we examined the incidence of biliary atresia in France, and the space-time distribution of cases. METHODS: All patients with biliary atresia living in France and born in the years 1986-96 were recorded. Geographic distribution, seasonality, time clustering and space-time clustering were analysed. Statistical analysis used the Chi square test, the Spearman nonparametric correlation test, the Walter and Elwood test for seasonality of events and Knox analysis for time and space-time clustering. RESULTS: We identified 461 patients: 421 born in metropolitan France (incidence 5.12 [4.63-5.61]/100000 live births), and 40 born in overseas territories. No significant regional variation in incidence was found in metropolitan France, while the incidence was higher in French Polynesia (incidence 29.4 [15.4-43.3]/100000 live births) (p<0.001). Seasonality, time-clustering and time-space clustering could not be demonstrated. CONCLUSIONS: 1) The incidence of BA was 5.7-fold higher in Polynesia than in metropolitan France. 2) Neither seasonal variation in incidence nor clustering was identified.

Biliary Atresia↗
PubMed1999

Paediatric liver transplantation with a split graft: experience at Bicêtre.

UNLABELLED: This study evaluates the results of paediatric liver transplantation (PLT) with split liver grafts at Bicêtre hospital. PATIENTS AND METHODS: Between January 1, 1988 and December 31, 1995, 205 PLT were performed in 180 children. One auxiliary PLT was excluded from the study. The graft was a whole (WLG), reduced (RLG) and split liver graft (SLG) in 76, 112 and 16 cases respectively. The SLG consisted of segments II + III in 14 cases, and II + III + IV in 2 cases. Results of PLT with SLT, RLT and WLG were retrospectively compared. Minimal follow-up was 12 months. RESULTS: In elective PLT, actual 1 year patient (graft) survival were 93.3% (84.4%) with WLG (n = 64), 84.1% (76.4%) with RLG (n = 72), 81.8% (81.8%) with SLG (n = 11). In urgent LT, actual patient (graft) survival were 100% (83.3%) with WLG (n = 6), 58.6% (52.5%) with RLG (n = 40), 25% (20%) with SLG (n = 5). Specific complications of the splitting technique were: 2 Budd-Chiari syndromes in 2 early patients, without any new case after modification of the technique of left hepatic vein to inferior vena cava anastomosis; 4 bile leaks (25%) from the left hepatic duct to Roux-en-Y loop anastomosis. DISCUSSION: In our experience, the results of PLT with SLG were satisfactory in the elective situation, but disappointing in urgent cases. In the current context of liver graft shortage, appropriate use of this sophisticated and demanding technique depends on the experience of the team, recipient's condition, and logistic considerations.

Adolescent↗
PubMed1998

[Neuro-intellectual prognosis at school age for 62 children born with a gestational age of under 32 weeks].

AIM: The aim of this study was to detail the incidence of cerebral palsy in children born before 32 weeks of gestation and to evaluate the scholastic and intellectual performance in non-handicapped children. POPULATION AND METHODS: The population included 63 survivors hospitalized in 1984-85 (gestational age: 30.06 +/- 1.21 weeks; birth weight: 1386 +/- 267 g; inborn 60%; male: 38.7%; small for gestational age: 4.8%; hyaline membrane disease: 32%; European: 98.4%). Neurodevelopmental assessment was performed by pediatricians and psychologists using Wisc-R, visual screening by Monoyer scale, hearing by audiometry realized by oto-rhinolaryngologists. RESULTS: There were 62 survivors (one child dead by sudden infant death syndrome). Fifty children evaluated at a mean age of 9.3 +/- 0.7 years and written data available for another eight. Twelve children presented with cerebral palsy. Risk factors were ultrasound abnormalities of parenchymal brain and male gender. In children without cerebral palsy, we observed 12 visual and two hearing impairment. Three were in special education, 32 were in an age-appropriate level, nine with one year below. Neonatal events were not associated with the Wisc-R results except for ultrasound abnormalities of parenchymal brain. Wisc-R was strongly correlated with familial economic and education level. CONCLUSION: School performances in non-handicapped children born before 32 weeks is satisfying. Intellectual performance is mainly correlated with familial economic and education level.

Brain↗
PubMed1998

Haemophilia A: two cases showing unusual features at birth.

We report two patients with severe haemophilia A, factor VIII < 2 u dL-1, diagnosed at birth, through isolated bleeding in the adrenal gland (case 1) and through a haematoma in the liver (case 2). In these two clinical cases, the vital emergency, with haemorrhagic shock, required early diagnosis of the hereditary coagulation defect. Generally at birth, any unexplained bleeding should prompt screening for haemophilia. In the newborn period, the PTT is inadequate for many reasons and the laboratory evaluation must include factor VIII and IX levels.

Adrenal Gland Diseases↗
PubMed1997

[Current aspects of acute respiratory distress syndrome in children].

Acute respiratory distress syndrome (ARDS) is a frequent condition in pediatric intensive care units. The mortality remains high despite advances in conventional mechanical ventilation and aetiological treatment. Several animal studies have documented lung injury during mechanical ventilation with high tidal volume, and clinical investigations have shown that in human ARDS, most ventilation is distributed to the small areas of remaining aerated lung resulting in overdistension of these areas and lung injury ("baby lung" theory). Nevertheless the usefulness of extrapulmonary gas exchange remains much debated. New ventilatory strategies have been developed in order to reduce ventilator-induced lung injury and to improve systemic oxygenation but multicentric randomized clinical trials are needed before these strategies can be validated.

Acute Disease↗
PubMed1997

[Agranulocytosis after application of silver sulfadiazine in a 2-month old infant].

BACKGROUND: A granulocytosis associated with the use of silver sulfadiazine has been described in burn patients. This agranulocytosis may be due to either an allergic reaction or a bone marrow toxicity by silver sulfadiazine. CASE REPORT: A 1-month old baby was hospitalized in pediatric intensive care unit after a stage one repair of a tracheoesophegeal fistula. Gastroesophageal reflux and tracheomalacia appeared in the post operative period. When she was 2-months old, she developed a perineal erythema. A topical therapy was begun, with daily applications of silver sulfadiazine and ketoconazole. Five days later, agranulocytosis (granulocytes = 0.21 x 10(9).L-1) occurred. Silver sulfadiazine therapy was immediately stopped. Granulocyte count returned to normal over a few days. No other etiology than silver sulfadiazine could be found to explain agranulocytosis. The small surface of administration, the chronology of the events and the rapid correction of the disorders after silver sulfadiazine interruption argued for an immuno-allergic reaction to this drug. Medical problems were easily controlled and the patient recovered well. CONCLUSION: This event occurred when sulfadiazine was applied in a small cutaneous area in a young baby. This may be an argument for an allergic reaction; alternatively, it could be explained by an increased cutaneous absorption of a cytotoxic drug in younger people.

Agranulocytosis↗
PubMed1996

[Basedow disease following metastatic thyroid cancer].

OBJECTIVES: Metastatic thyroid carcinoma rarely provokes hyperthyroidism. We describe here the main characteristics of this association observed in a 48-year-old woman. CASE REPORT: The patient had presented signs of hyperthyroidism for one year. Clinical examination revealed severe Graves' disease with nodular goiter. Presurgery investigations demonstrated thyroid carcinoma and lung metastasis. Thyroid stimulating immunoglobulins were elevated. After total thyroidectomy, hyperthyroidism persisted, indicating the functional nature of the lung metastases. DISCUSSION: The effect of thyroid stimulating immunoglobulins on the clinical course of thyroid carcinoma and distant metastases remains unknown.

Female↗
PubMed1995

[Diabetes insipidus disclosing metastasis of breast adenocarcinoma].

We are reporting on a case of diabetes insipidus (DI) and anterior pituitary failure revealing a breast cancer metastasis. Ten years after being diagnosed with a unilateral breast cancer, the patient presented with asthenia, thirst, polyuria and nocturia improved by subcutaneous DDAVP. MRI revealed a thickened pituitary stalk. DI is uncommon, late and usually asymptomatic in breast cancer. The association with an anterior pituitary failure is even more rare. In our patient the metastasis is in the pituitary stalk and seems to be due to meningeal deposits. MRI appears to be the best procedure to perform, showing a thickening stalk. Extension to the pituitary gland is related to direct tumor invasion from adjacent structures rather than haematogenous spread.

Adenocarcinoma↗