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Biomedical subjects

J Cardona

Publications and source records attributed to J Cardona.

At least 19 recordsLinked to original sources
PubMed1995

Liver transplantation in children with Alagille syndrome--a study of twelve cases.

Cholestasis associated with Alagille syndrome may, in a few cases, be extremely severe and result in major impairment in the quality of life during early childhood and end up in cirrhosis eventually. We report the results of liver transplantation in 12 children with a severe hepatic form of Alagille syndrome. All children presented with cholestatic jaundice from birth, peculiar facies, stenosis of the peripheral pulmonary artery, and posterior embryotoxon; butterfly-like vertebrae were present in 9 children. At the time of transplantation (mean age 7 years 10 months) refractory pruritus was present in 9 children, xanthoma in 11, and height and weight retardation in 11. Total serum bilirubin ranged from 116 to 322 mumol/L and total serum cholesterol from 3.5 to 29 mmol/L. Systolic right ventricular pressure was moderately raised (36 to 48 mmHg) in 5 children; mean creatinine clearance was 99 ml/min/1.73 m2. Histologic examination of the removed livers showed cirrhosis, severe annular fibrosis, and moderate portal fibrosis in 4 children each. Follow-up in the 11 survivors has ranged from 14 months to 5 1/2 years. All lead normal lives. Pruritus and xanthomas disappeared. Increase in height was observed in 8 of the 10 survivors who had growth retardation prior to transplantation. School level is normal in 4 (median age at LT: 5 yr 9 mo) and below normal in 6 (median age at OLT: 9 yr 9 mo). Liver function tests are normal in 10 children. Mean creatinine clearance is 101 ml/min/1.73 m2. These results indicate that the quality of life can be considerably improved after liver transplantation in children with a severe hepatic form of Alagille syndrome and suggest that it could be carried out before these children attend elementary school.

Alagille Syndrome↗
PubMed1995

[Hyperexplexia].

Hyperexplexia is a disease of neonatal onset characterized by an exaggerated startle reflex. Early diagnosis is important to rule out epilepsy. Clinical findings are mainly hypertony and generalized startle reflex which is exaggerated by tiredness and some exogenous stimuli. Electroencephalogram is normal. The expression of the disease is variable including minor forms that may be unnoticed and major forms with arthrogryposis-like symptoms, orthopedic complications, false passages, apnea and even sudden infant death (SID). The evolution is generally benign and symptoms disappear within 2 or 3 years. A neuromotor retardation is often present, without intellectual deficit. In severe forms, the risk of SID requires a multidisciplinary follow-up including monitoring and treatment with clonazepam. A low GABA level in cerebrospinal fluid has been reported. Present etiological hypotheses include neuromediator and/or receptor dysfunction.

Humans↗
PubMed1994

[Intraosseous vascular access, a technic previously underestimated in France].

Intraosseous vascular access is a simple and very efficient technique for fluid and drug administration in any pediatric emergencies where the intravenous route is impossible or inadequate. Yet it remains unrecognized in France. Its pharmacokinetics is close to that of peripheral intravenous route, but it allows much greater infusion flow rates. In pediatric resuscitation it must be considered as the number one technique of intravascular access in infants, and rapidly as the first alternative after failure of attempt of intravenous route in children under 6. Provided that the technique is performed with careful asepsis, the risk of infectious complications is very low. However the intraosseous route must remain a transitory vascular access, and has to be stopped as soon as possible, its use never exceeding 24 hours.

Bradycardia↗
PubMed1993

[Accidental bleach ingestion in children: results of a survey in 11 anti-poison centres. Proposals for management].

Accidental bleach ingestion is frequent in children but there is no agreement on its management. The results of a survey among 11 French poison centres about their recommendations in this intoxication are reported. Most of the centres adapt their guidelines according to the quantity and the concentration of the ingested bleach. In case of diluted bleach, no centre recommends an hospitalization when the quantity is smaller than 100 ml, whereas four of the 11 centres recommend it when the quantity is greater than 100 ml. In case of concentrate bleach ingestion nine of the 11 centres recommend an hospitalization whatever the amount, eight of them performing an emergency upper gastro intestinal (GI) endoscopy if clinical signs are present. In case of large ingestion of concentrate bleach ten centres recommend the hospitalization, eight perform an upper GI endoscopy between 6 and 8 hours post ingestion according to clinical signs, and two perform a systematic emergency upper GI endoscopy. Tablets and new bleach are considered as concentrate bleach. From these informations and a review of the literature, the authors emphasize the importance of the clinical signs as criteria for prediction of GI lesions regardless of the quantity or the concentration of ingested bleach.

Endoscopy, Digestive System↗