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C Largillière

Publications and source records attributed to C Largillière.

35 records · Page 2Linked to original sources
PubMed1990

[The bronze baby syndrome Apropos of 3 cases].

Bronze baby syndrome is an infrequent complication of phototherapy for neonatal jaundice which develops in infants with liver disease. The characteristic feature is a grayish-brown discoloration of the skin, plasma and urine. Three cases are reported. There was one case each of bile duct atresia, galactosemia, and parenteral nutrition with cholestasis. The pigment responsible for the discoloration has not been identified and pathogenesis remains unelucidated. The bronze color disappears spontaneously and the prognosis is dependent on the causative liver disease for which investigations should be performed routinely.

Biliary Atresia↗
PubMed1989

[Mitochondrial myopathy. Encephalopathy with lactic acidosis and cerebral infarction].

A 21 year-old man presented with a history of sudden onset of aphasia and headache. CT showed a left parietal hypodensity and pallidal calcifications. The ECG showed a Wolff-Parkinson-White's syndrome. The patient then developed successively focal epileptic seizures, temper disorders, a cardiomyopathy, a pepper and salt retinopathy with hemeralopia, a left hemiplegia, deafness, and fever of unexplained origin. Left carotid angiography showed thin, irregular or occluded branches of the middle and anterior cerebral arteries. Blood muscle enzymes, lactate and pyruvate, were elevated with acidosis. Muscle biopsy revealed a mitochondrial myopathy and blood chemistry showed a severe deficiency of respiratory chain enzymes. Death occurred after 28 months. This case showed the diagnostic features of Melas, with some elements of the Kearns-Sayre syndrome. To our knowledge, this is the first case were serial angiographies allowed demonstration of arterial changes capable of explaining cerebral infarctions.

Acidosis, Lactic↗
PubMed1988

Free malondialdehyde determination in human plasma by high-performance liquid chromatography.

A high-performance liquid chromatographic method was developed for quantification of malondialdehyde (MDA) in human plasma. Deproteinized samples were injected onto a Waters carbohydrate analysis column which was eluted with 20% (v/v) 0.03 M Tris buffer, pH 7.4, in acetonitrile. Peak absorbancy was measured at 267 nm. In contrast to data already published, we did not detect any free MDA in normal human plasma. This suggests that the classical thiobarbituric acid test is not suitable for the determination of MDA in human plasma.

Adult↗
PubMed1988

[Partial ornithine carbamyl transferase deficiency].

The author reports the case of a 8-month-old girl with a partial ornithine carbamyl transferase deficiency revealed by an acute hepatic failure. The first diagnosis was hereditary fructose intolerance, corrected after the finding of an hyperoroticuria. Despite the treatment based on hypoprotidic diet, sodium benzoate and arginine, the child presented several hyperammoniemic comas. Then, dermatological manifestations, presumably due to protein denutrition, were successfully treated with amino acids keto-analogs.

Acute Disease↗
PubMedDate not supplied

First-trimester diagnosis of sirenomelia. A case report.

We report a case of sirenomelia diagnosed at 13 gestational weeks. This rare malformation sequence is characterized by fusion and rotation of the lower limbs to various degrees and anorectal atresia, usually associated with absence of bladder and agenesis or dysgenesis of the kidneys. Diagnosis is commonly made later in the second trimester of pregnancy with oligohydramnios as the alerting sign. Survival is extremely rare, and only possible in the absence of bilateral renal agenesis. In view of the dismal prognosis, early diagnosis allows for earlier and less traumatic therapeutic abortion.

Adult↗
PubMedDate not supplied

Prospective evaluation of uterine artery flow velocity waveforms at 12-14 and 22-24 weeks of gestation in relation to pregnancy outcome and birth weight.

OBJECTIVE: Uterine artery flow velocity was prospectively assessed using Doppler ultrasound at 12-14 and 22-24 weeks of gestation in the prediction of subsequent complications related to uteroplacental insufficiency: preeclampsia, pregnancy-induced hypertension, fetal growth restriction, fetal death and placental abruption, and to elucidate its relationship with birth weight. METHODS: Uterine artery Doppler assessment was obtained during routine ultrasound screening in 263 unselected women. Flow velocity waveforms were coded according to the number of notches present at each scanning, respectively: none (0, 0), uni-/bilateral notches that disappeared (1, 0) or (2, 0), uni-/bilateral notches that persisted unilaterally (1, 1) or (2, 1), and persistent bilateral notches (2, 2). RESULTS: Complete outcome data was obtained for 243 (92.4%) women. Of these women, 55 (22.6%) and 84 (34.6%) women had uni- and bilateral notches, respectively, at 12-14 weeks' gestation; 14 (5.8%) and 21 (8.6%) patients had uni- and bilateral notches, respectively, at 22-24 weeks' gestation. Analysis of complication rates for the four groups showed that they increased with notch persistence (5.7, 13.5, 57.1 and 76.2%), while the corresponding mean birth weight declined (3,273, 3,180, 2,698 and 2,418 g). CONCLUSION: The absence or early disappearance of uterine artery notches is associated with fewer complications related to uteroplacental insufficiency and normal birth weight, whereas their late and partial disappearance or bilateral persistence tends to compromise the prognosis.

Adult↗
PubMedDate not supplied

[Characteristics of a case of hepatic fructose-1,6-diphosphatase deficiency].

The authors report a new case of hepatic fructose-1.6-diphosphatase deficiency, diagnosed in a 14 month-old girl on the occasion of an episode of prostration associated with acidosis, hyperlactacidemia and hypoglycemia without hepatomegaly. The unusual features of this case, especially the glucagon induced hyperglycemia after a 12 hour fast, emphasize the clinical and biological polymorphism of this enzymopathy.

Female↗