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Biomedical subjects

J Badoual

Publications and source records attributed to J Badoual.

At least 55 records · Page 3Linked to original sources

Alloimmune neonatal neutropenia.

In three neonates, the diagnosis of anti-NA1 alloimmune neutropenia related to maternal immunization against fetal polymorphonuclear leukocytes (PMN) antigens was achieved by serum antibody screening and PMN phenotyping. All the children were small for date and exhibited bacterial infection within days 2-13. Neutropenia persisted until days 20-50. High-dose intravenous immunoglobulin G (IVIgG) was ineffective. In one case, NA1-negative PMN collected from a normal donor were infused because of infection after thoracic surgery and resulted in a sharp but transient increase in PMN counts with resolution of infection. The natural history and the management of alloimmune neonatal neutropenia are discussed.

Adult↗

Caffeine acetylator phenotyping during maturation in infants.

Caffeine acetylator phenotype was studied during maturation in 54 8- to 447-d-old children hospitalized for minor disease (group A) and in five 3- to 630-d-old children with Pierre Robin syndrome (group B). In group A, the children received 2.5 mg/kg caffeine orally once between birth and 15 mo. Group B patients were chronically treated with caffeine (2.3 to 15.8 mg/kg/d) for prevention of apneas, and the acetylator phenotype was serially determined. Phenotyping was performed on a spot urine sample collected 2-6 h after drug administration. Caffeine metabolites [5-acetylamino-6-formylamino-3-methyl uracil (AFMU), 1-methylxanthine, 1-methyluric acid, 1,7-methyluric acid, and 1,7-methylxanthine] were measured using HPLC. Acetylator phenotype was determined on the basis of AFMU/1-methylxanthine (ratio 1) and AFMU/AFMU + 1,7-methyluric acid + 1-methylxanthine + 1,7- methylxanthine + 1,7-methyluric acid (ratio 2) molar ratios. In group A, all children were slow acetylators before 83 d of age (ratio 1 less than 0.4; ratio 2 less than 0.08), whereas older children included slow and fast acetylators. The acetylation molar ratios differed significantly between age groups and increased with age. The cumulative percentage of fast acetylators increased with age but the plateau was not yet reached at 15 mo. In three children, the phenotyping was repeated after 15 mo: the second determination was consistent with the first one. In group B, all children appeared as slow acetylators on the first phenotyping. Four of them appeared subsequently as fast acetylators; one remained a slow acetylator until 11 mo.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetylation↗

[Perinatal hemochromatosis].

Neonatal hemochromatosis is responsible for death in utero or severe hepatic insufficiency in the newborn, generally with rapid progression to death. Recurrence in sibships is frequent. Necropsy findings are characterized by siderosis in the liver, pancreas, heart, thyroid, but not in the reticuloendothelial system. Urinary cytology, serum iron concentration, iron-binding capacity, ferritin, and magnetic resonance imaging allow a diagnosis to be made before death. Liver transplantation probably offers the best chance of survival. The pathophysiology is unknown; there may be a variety of etiologies.

Female↗

[Viral infection in the neonatal period: diagnostic difficulties, the role of interferon alpha levels].

Serum and/or cerebrospinal fluid (CSF) alpha interferon (aIFN) levels were determined in 31 neonates hospitalized for suspected sepsis. Final diagnosis was bacterial sepsis in 15, viral infection in 13 and no infection in 3. Among the 13 neonates with viral infection, enterovirus was isolated 5 times and coxsackievirus 4 times. No aIFN was found in cerebro-spinal fluid and/or serum among the neonates with bacterial sepsis or without infection. By contrast, in neonates with viral infection, CSF and/or serum aIFN levels were always elevated except in one case in which serum aIFN determination was not available. We conclude that in neonates, elevated levels of CSF and serum aIFN appear to be specific of viral infection, and that this might be helpful in the differential diagnosis of suspected sepsis.

Coxsackievirus Infections↗

[Embryofetal diseases in the tropical zone].

The treatment and the prevention of acquired fetal diseases in developing countries are difficult. Nevertheless the deleterious consequences of fever, alcohol consumption, drug intake and malaria on the embryo and the foetus can be avoided by appropriate measures. As for the prevention and the diagnostic in utero of rubella and toxoplasmosis, they may be considered lately when the current priority objectives of public health are reached. Epidemiological studies on these diseases are therefore justified.

Bacterial Infections↗

[QT prolongation and circulatory arrest after an injection of erythromycin in a newborn infant].

A 8 day-old full-term newborn showed severe cardiac disturbances after intravenous injection of erythromycin. The neonate, suspected of having Chlamydia pneumonitis because of tachypnea and rhinitis, had been given 5 injections of erythromycin without clinical effect. Pallor, vomiting and bradycardia developed a few minutes after the 6th injection, and ECG showed ventricular arrhythmia, prolonged QT interval and an atrioventricular block. The infant died in intensive care unit. This case and the analysis of other published cases of cardiac disturbances following the parenteral use of erythromycin, indicate the potential arrhythmogenic risk of this drug. It is suggested that newborns treated with erythromycin should be monitored by ECG.

Erythromycin↗

[Superficial pseudophlebitis in a HIV seropositive child].

A case of "hyperalgesic pseudothrombophlebitis" in an hemophilic child with acquired immune deficiency syndrome (AIDS) is reported. Known causes such as associated blood clotting defect, collagen vascular disease, infection and homocystinuria were ruled out. Five similar cases were reported in 1985, in homosexual American adult males with AIDS, in whom venography did not show evidence of venous occlusion. Recovery occurred spontaneously and progressively, while the patients were given an anticoagulant therapy. Despite our hemophilic patient did not receive any anticoagulant treatment, a similar favorable course was observed. This AIDS related syndrome has not been reported in children yet, and its pathogenesis remains unknown.

Child↗

[Benign hypertrophic gastritis associated with cytomegalovirus infection].

A new case of benign hypertrophic gastritis associated with cytomegalovirus infection in a 7 year-old boy is reported. The main symptoms were complete digestive intolerance and protein loss, resulting in major hypoalbuminemia and anasarca. The alpha-1-antitrypsin clearance was very increased. Diagnosis was established by upper gastrointestinal x-rays which displayed important thickening of the gastric rugae, and by endoscopic examination with biopsy. A cytomegalovirus infection attested by seroconversion and viruria probably played a role in its origin. Symptomatic treatment and albumin infusions led to recovery within one month. In the last 25 years, 31 cases of benign hypertrophic gastritis have been reported. Vomiting is the main symptom with abdominal pain and G-I bleeding. The protein loss is constant, often severe. In most cases, the disease recedes within a few weeks, unlike the adults' Ménétrier disease which proves to be chronic and severe. The cause remains unknown. In 11 cases, a cytomegalovirus infection was reported and, so, is probably not fortuitous.

Child↗

[Perinatal hemochromatosis].

Perinatal hemochromatosis (PHC) is a rare disorder presumably autosomal recessive, responsible for foetal death or severe liver failure during the neonatal period. It is fatal in nearly all cases. Diagnosis relies on histologic examination, generally post mortem, which shows numerous iron deposits in the liver as well as other organs (pancreas, heart, thyroid...). The study of iron and of its ligands may have a diagnostic interest. The authors report 2 cases of PHC in one family, of whom one concerned child presented with biological signs evoking PHC on foetal blood at 31 weeks of pregnancy.

Female↗

[Sublethal microcephalic chondrodysplasia. Taybi-Linder syndrome, primordial microcephalic nanism types I and III].

The authors describe a case of microcephalic dwarfism observed in a newborn until 10 months of age and discuss the diagnostic challenge. They show that the Taybi-Linder syndrome and the primordial dwarfism type I and type III of Majewski are an identical recessive autosomal entity. The radiological evolution explains the initial separation of type I and type III. Because of the skeletal lesions, lacking in the Seckel syndrome, the name of sublethal microcephalic chondrodysplasia is proposed for this disease.

Abnormalities, Multiple↗

[Delayed measles encephalitis in a leukemic child].

A case of measles encephalitis of the delayed type in a 5 year-old girl is reported. The encephalitis occurred 6 months after a measles which supervened just after the 18th monthly reinduction treatment for acute lymphoblastic leukemia. The child died one month later. This measles inclusion body encephalitis (MIBE) was confirmed by the presence of intracellular inclusions in the brain cells visualized by electron microscopy. The evidence of viral related intracellular nucleocapsides was confirmed by in situ hybridization. These nucleocapsides were identical to those seen in subacute sclerosing panencephalitis.

Child, Preschool↗

Nitrofurantoin excretion in human milk.

Six lactating white healthy women (26-36 years old, weighing 45-58 kg) were treated with 50 mg nitrofurantoin tablets, a urinary antiseptic. They received either 50 mg (group I; n = 3) or 100 mg (group II; n = 3) 3 times a day (09.00, 16.00, 19.00 h) for 24 h, 2-5 days after the delivery of a full-term neonate. The study was performed on the 4th dose at 09.00 h just before breakfast. Milk samples were collected before, 3 and 6 h after the nitrofurantoin administration with an Egnell SMB breast pump. The complete milk samples were collected from each breast, and pooled. 5 ml venous blood samples were drawn before, 1, 2, 3 and 6 h after nitrofurantoin administration. Plasma and milk nitrofurantoin concentrations were measured by HPLC. Apparent elimination half-life and apparent plasma clearance were the same in both groups, 0.8 +/- 0.09 h and 27.6 +/- 5.57 l/h, respectively. Nitrofurantoin was not detectable in the milk just before the 4th administration. The amount excreted in the milk within 6 h after nitrofurantoin administration was 22-57 micrograms (I) and 61-284 micrograms (II) which represents 0.05-0.11% (I) and 0.06-0.28 (II) of the nitrofurantoin dose. The nitrofurantoin concentration ratio of the breast milk to the plasma collected at 3 h was 2.2 +/- 1.2 (I) and 2.3 +/- 1.6 (II). These results show that nitrofurantoin excretion in human milk is low: below 0.12 (I) and 0.29% (II). It suggested that breast-fed newborn infants from mothers treated with nitrofurantoin would be exposed to small amounts of drug.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Familial forms of gastro-esophageal reflux].

Gastroesophageal reflux is a common disorder that can be responsible for respiratory disease and is not considered as an inherited condition. We report five families with several affected members. Other familial cases have been published and some authors have suggested that transmission occurs on an autosomal dominant basis. Only prospective studies could determine whether GER is an inherited disorder, but at present their feasibility is limited since currently available diagnostic investigations are not suitable for screening.

Child↗

Maturation of AFMU excretion in infants.

The maturation of the N-acetyltransferase-dependent AFMU production from caffeine was studied during infancy. The group of children (N = 14) consisted of 4 premature newborn infants and ten 1-19 month-old infants who received caffeine citrate solution for the treatment and prevention of apnea. Caffeine, AFMU, 1X and 9 other metabolites were measured in urine using HPLC. The AFMU/1X ratio did not vary significantly in this population with increasing age. In one of the infants serially studied, the AFMU/1X ratio increased dramatically between 6 and 12 months of age. This observation suggests that the maturation of N-acetyltransferase activity is not completed before 1 year of age implying that acetylator status cannot reliably be determined before that age. Patients studied before 1 year of age whose AFMU/1X ratio was below 0.4 may be either true slow acetylators or still immature fast acetylators.

Acetylation↗

[Use of the labelling of drugs with stable isotopes in clinical pharmacology in children].

There are only few studies available in the literature, using drugs labelled with stable isotopes in pediatric clinical pharmacology. However, the stable isotope methods are of great potential interest. These sensitive methods allow to quantitate drugs in small volumes of biological fluid with great specificity. They are innocuous and allow in vivo drug metabolic studies. Using 1-3 15N, 2 13C labelled theophylline, it has been possible to demonstrate N7-theophylline methylation into caffeine in premature neonates while this metabolic pathway is virtually not existent in adults. Stable isotope techniques allow to study intraindividual changes in kinetic parameters of drugs during maintenance therapy without discontinuing the drug. For instance, auto-induction of carbamazepine metabolism was demonstrated in children using stable isotope-labelled carbamazepine in serial pharmacokinetic studies during maintenance therapy. They also make bioavailability studies possible in children. These methods make possible new non invasive studies like CO2 breath test. Using 1-3-7 13C-caffeine we demonstrated the feasibility of the CO2 breath test in young infants and described the maturation of the N-demethylation pathway for caffeine. The correlation between the rate of 13C-CO2 elimination and caffeine plasma clearance indicates that the CO2 breath test is a non invasive method useful for measuring caffeine N-demethylation and that this test might theoretically be used for monitoring caffeine treatment.

Child↗

[Esophagogastroduodenitis in the newborn. Apropos of 32 cases].

Inflammatory lesions involving esophagus, stomach and duodenum are frequent in neonates and run a benign course. Thirty-two cases of esophagogastritis associated with duodenitis in 28% of cases were studied. Presenting symptoms included nonspecific symptoms such as feeding difficulties (15 cases), G-I bleeding (14 cases), regurgitation (14 cases) and/or impaired weight gain (4 cases). No precipitating factor could be identified. The diagnosis was established by endoscopy. Gastro-esophageal reflux, which seemed to be secondary to the mucosal lesions, required an anti-reflux treatment, which led to a rapid clinical recovery. Repeat endoscopy invariably showed an improvement or complete recovery of the mucosal lesions which did not seem to be influenced by antacid treatment. The etiology and pathogenesis of neonatal esophagogastroduodenitis remain undetermined.

Adult↗