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J L Demarquez

Publications and source records attributed to J L Demarquez.

At least 37 records · Page 2Linked to original sources
PubMed1996

[Increasing frequency and diagnostic difficulties in intestinal stenosis after necrotizing enterocolitis].

BACKGROUND: Stenosis after necrotizing enterocolitis (NEC) has increased from 15 to 57% over the last 10 years in our unit. The aim of this study is to point out the difficulty of diagnosis and treatment, and search for factors explaining this increase. PATIENTS AND METHODS: From 1986 to 1991, 42 newborns had NEC, followed by intestinal strictures in 19 of them (57% of the 33 survivors). Data from these 19 patients were compared with those of the 14 without intestinal strictures. The 33 survivors were also compared with those of an earlier study including 25 NEC seen from 1979 to 1986. RESULTS: After medical treatment (n = 12), intestinal stenosis led to occlusion after three weeks, was located to both small and large intestine and was short and tight. After surgical treatment (n = 7), stenosis was shown by opacification before digestive anastomosis (n = 5) or revealed by occlusion (n = 2); it stayed on the colon, was long or multiple, requiring extensive resections. No difference could be found between data from patients with or without stenosis. Although newborns were actually more premature, the risk of stenosis was more frequent when newborns of same gestational ages and/or weights were compared. CONCLUSIONS: Intestinal stenosis is a frequent complication after NEC; its diagnosis is often difficult and requires extensive digestive resections. No clinical or therapeutic factor could be found to explain the actual increase in frequency.

Birth Weight↗
PubMed1995

Respiratory mechanics before and after late artificial surfactant rescue.

OBJECTIVE: To assess the effect of late administration of synthetic surfactant (Exosurf) on the ventilatory function of premature infants with hyaline membrane disease (HMD). METHODOLOGY: Prospective non-randomized study in the Neonatal Intensive Care Unit (NICU) of a major referral hospital. The patients included two groups of premature infants with a birthweight between 750 and 2000 g who developed HMD. In group 1 with moderate to severe HMD, 2 x 5 mL/kg doses of Exosurf were given 12 h apart (first dose given at a mean age of 18.7 +/- 3.4 h [mean +/- s.e.m.]). In group 2 with milder HMD, no surfactant was given. RESULTS: Significant reductions (P < 0.05) in the fraction of inspired oxygen (FIO2) occurred 6 h after surfactant administration (24 h of life) and by 48 h (64 h of life) in group 2. These improvements in gas exchange preceded improvements in passive respiratory compliance which occurred 24 h after surfactant (42 h of life) and by 72 h (88 h of life) in group 2 (P < 0.01). In both groups pulmonary resistance increased and was significant (P < 0.05) by 48 h (66 h of life) in group 1. CONCLUSIONS: Synthetic surfactant given as late as a mean age 18.7 +/- 3.4 h still improves gas exchange but these early improvements cannot be completely explained by modifications of respiratory compliance.

Age Factors↗
PubMed1994

Pulmonary mechanics in ventilated preterm infants with respiratory distress syndrome after exogenous surfactant administration: a comparison between two surfactant preparations.

The effects of two surfactant preparations on lung mechanics have been studied on 24 ventilated premature infants with respiratory distress syndrome (RDS): 13 were given artificial surfactant (Exosurf Neonatal, Burroughs-Wellcome) and 11 natural porcine surfactant (Curosurf, Laboratoire Serono France). Measurements of respiratory system compliance (Cdyn, Crs) and resistance (Rrs) were performed immediately before surfactant administration and repeated 6, 18, 24, 48, and 72 hours later. With Exosurf treatment, 6 hours after surfactant administration inhaled O2 concentration (FlO2) could be lowered from (0.72 +/- 0.20, to 0.62 +/- 0.33; P < 0.05), whereas Crs did not change (0.37 mL/cmH2O/kg, +/- 0.14 vs. 0.39 +/- 0.12, NS). After 24 hours and during the following days a significant increase in Crs occurred (24 hours post-Exosurf: 0.51 +/- 0.18, P < 0.05). With Curosurf treatment, the improvement in oxygenation was greater and FlO2 could be lowered much more after 6 hours (from FlO2, 0.78 +/- 0.23 to 0.34 +/- 0.11, P < 0.01). This was associated with an increase in Crs (from 0.39 +/- 0.09 to 0.59 +/- 0.17, P < 0.05). During the following days, Crs was significantly higher in the group treated with Curosurf. Resistance was not altered by the type of surfactant preparation used except after 72 hours, when Rrs increased in the group treated with Exosurf. In conclusion, Curosurf appears to be more effective than Exosurf with regard to immediate pulmonary changes in ventilator treated premature infants with RDS. A rapid increase in Crs after Curosurf treatment indicates that recruitment of new functional areas of the lung is likely to be associated with a stabilization of small airways and alveolar units.

Airway Resistance↗
PubMed1994

Human airway smooth muscle responsiveness in neonatal lung specimens.

The mechanical activity of proximal airways isolated from human lung specimens obtained at autopsy from 11 neonates was studied in response to the following compounds: carbachol, histamine, potassium chloride (KCl), neurokinin A (NKA) (both in the presence and in the absence of the neutral endopeptidase inhibitor phosphoramidon) and isoproterenol. Isometric responses to the various concentrations of each of the compounds were expressed as both raw values of force normalized to smooth muscle cross-sectional area (SMCSA), i.e., muscle stress and percentages of the maximal response to acetylcholine. Maximal active muscle stress of human neonatal bronchi was induced by carbachol and averaged 95 +/- 25 mN/mm2 SMCSA (n = 8). The rank of maximal force induced by the contractile agonists was carbachol > histamine > KCl > NKA, and the rank of the concentration of drug producing one-half of the maximum effect (EC50) was NKA < carbachol < histamine < KCl. The EC50 value for isoproterenol was the lowest, although it generated the smallest mechanical response. When compared with results obtained under identical experimental conditions in the human adult lung, except for carbachol and isoproterenol, general trends were an increase in force generation with age and little changes in EC50 values. There was a decrease in carbachol-induced force with age, whereas the opposite was observed with isoproterenol. We conclude that most of the mechanisms that control airway tone in humans are already present in the neonate. Alterations in the response to agonists with the maturational process may have implications in the pharmacologic modulation of bronchial obstruction in neonates.

Carbachol↗
PubMed1993

Fatal neonatal liver failure and mitochondrial cytopathy (oxidative phosphorylation deficiency): a light and electron microscopic study of the liver.

Mitochondrial cytopathies are multisystemic disorders of extremely variable expression due to a deficiency in oxidative phosphorylation. Cases have recently been reported in which fatal liver failure with neonatal onset was the major clinical and biochemical syndrome. In this series we reviewed the liver histology of 10 such patients who died in the first weeks of life (from 3 days to 6 mo). In six cases the diagnosis was confirmed by study of the mitochondrial respiratory chain in the muscle, liver or both; in the other four, appropriate tests were not available for diagnosis but symptoms were identical and all other diagnoses were ruled out. In all 10 cases we noted significant steatosis, mostly microvesicular; widespread hepatocytic, canalicular and bile duct cholestasis with bile thrombi and cholangiolar proliferation; and different degrees of hepatosiderosis and glycogen depletion. Fibrosis took varying forms: perisinusoidal, periportal with the formation of septa, even precirrhosis. In the two cases of infants who died, one at 5 and one 6 mo, micronodular cirrhosis was also present. Mitochondria, either densely or loosely packed, were abnormal-pleiomorphic with few or no cristae and a granular fluffy matrix. Dense, large granules were seen in two cases. The association of neonatal liver failure and hyperlactacidemia should lead to immediate examination of the respiratory chain. The expression of this mitochondrial cytopathy can be lethal, associated with severe liver damage due to the deficiency in oxidative phosphorylation.

Female↗
PubMed1993

Ondine-Hirschsprung syndrome (Haddad syndrome). Further delineation in two cases and review of the literature.

Two unrelated children with congenital central hypoventilation syndrome (CCHS-Ondine syndrome) and long segment Hirschsprung disease are reported. Patient 1, a girl, is still alive at 3 years. Patient 2, a boy, died of viral pneumonia at 5.5 years. Continuous mechanical ventilation was necessary for months and those children could never be weaned from the respirator during sleep. Seventeen cases of this complex neurocristopathy are reviewed. Only six children (including our cases) survived beyond 2 years of age. Hypotonia, delay in developmental milestones or epilepsy were frequently observed. Ventilator dependency does not improve with time. Multifocal congenital neuroblastoma occurred in two children. Aetiology is unknown.

Abnormalities, Multiple↗
PubMed1993

Helium-oxygen mixture in respiratory distress syndrome: a double-blind study.

In a randomized, controlled trial, the lungs of infants with respiratory distress syndrome were ventilated with either a helium-oxygen mixture or a nitrogen-oxygen mixture. In the helium-oxygen group, infants required a lower inspired oxygen concentration and a shorter duration of ventilation. There were also fewer deaths and fewer cases of bronchopulmonary dysplasia in the helium-oxygen group.

Airway Resistance↗
PubMed1993

[Apple-peel jejunal atresia. Nutritional management].

Apple-peel jejunal atresia is a rare digestive tract congenital defect often accompanied with a short gut. Two cases managed by a three-stage medical and surgical approach are reported. A protein hydrolysate-jejunal secretion mixture was drip-fed through the distal ileostomy to stimulate trophicity of the distal gut.

Enteral Nutrition↗
PubMed1993

[Transient intestinal pseudo-obstruction syndrome in premature infants].

BACKGROUND: A chronic intestinal pseudo-obstruction is sometimes seen in premature neonates who are fed early and subsequently suffer from digestive intolerance for several weeks. PATIENTS: Seven premature babies (mean gestational age: 30.5 weeks, mean birthweight: 950 g) suffered from abdominal distension and failure to pass stools at a mean age of 2.5 days (extremes: 1 to 6 days); 2 of them also had vomiting. X-ray examination showed dilated loops of bowel throughout the abdomen without obstructive changes; barium or Gastrografin studies demonstrated inertia of the colon without obstructive changes or abrupt changes in caliber. Histological examination of enteric nerve cells in 2 cases showed normal maturation. Parenteral nutrition was necessary in all patients for 30 to 78 days (mean: 47), followed by continuous enteral feeding for 24 to 48 days (mean: 37). Septicemia complicated parenteral nutrition in 4 babies and was responsible for the death of 1 of them. Normal evacuation of stools occurred between day 27 and day 91 (mean day 46) allowing normal enteral feeding. All 6 patients, now aged 2.5 to 8 years (mean 3.5 years) are in good health, and have no digestive problems. CONCLUSIONS: Immaturity of intestinal motility may occur in some premature neonates suffering from intestinal ileus. Parenteral nutrition may be necessary for several weeks, but spontaneous recovery is usual. This transient intestinal pseudo-obstruction of premature babies is different from the classical chronic pseudo-obstruction for which surgery is often needed.

Chronic Disease↗
PubMed1993

[Unusual form of Noonan syndrome: neonatal multi-organ involvement with chylothorax and nevoid Cutis verticis gyrata].

We report the case of an unusual form of Noonan's syndrome with multisystemic involvement identified in the early neonatal period. Apart from the common association of dysmorphia, cardiopathy and lymphatic vessel anomalies, a tumor-like naevoid cutis verticis gyrata of the scalp was observed. This type of cutaneous lesion has never been described in previous reports of the syndrome.

Chylothorax↗
PubMed1992

[Malaise in infants].

Near-miss is the term used by English-speaking authors to define a sudden accident suggestive of imminent infant death. This is one of the most worrying problems, due to its frequency and its multiple possible causes, the most common of which are gastro-oesophageal reflux and vagal hypertonia. These accidents occur in the same age-group as the sudden infant death syndrome and in similar circumstances, even though they more often occur during waking. Near-miss therefore may constitute an abortive form of sudden infant death syndrome, which would make its study a good way for understanding the syndrome. Yet one should wait before making this assimilation as it might induce unwarranted medical procedures. The risk of recurrence (about 10%), sometimes lethal, exists, but is must be discussed after full investigation in search of a cause and a possible treatment. Electronic home monitoring is only one of the possible preventive measures; it must be decided upon and applied by a specialized medical team.

Home Nursing↗
PubMed1991

Neonatal Noonan syndrome with a molluscoid cutaneous excess over the scalp.

The Noonan syndrome is a multiple congenital anomalies syndrome with variable expressivity and autosomal dominant inheritance. We report an observation of a newborn with Noonan syndrome and an unusual molluscoid cutaneous excess over the scalp that might represent a new skin manifestation in Noonan Syndrome rather than a consequence of lymphatic dysplasia.

Chromosome Aberrations↗