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Biomedical subjects

G Chéron

Publications and source records attributed to G Chéron.

At least 55 records · Page 3Linked to original sources

[Congenital asplenia. A childhood immune deficit often detected too late].

We report a case of fatal pneumococcal sepsis in a previously healthy 22 month old child. At postmorten examination the patient was found to have an atrophic spleen. When other malformations are not associated, congenital asplenia is generally diagnosed after death related to pneumococcal infection.

Fatal Outcome↗

[Isolated neonatal dysfunction of brainstem].

BACKGROUND: Brainstem dysfunction in newborns (BDN) is an association of symptoms originally described in the Pierre-Robin sequence. BDN is thought to result from a deficiency of the sucking and swallowing embryonic organization. POPULATION AND METHODS: Between 1983 to 1993, 48 infants without cleft palate were referred for suck and swallow abnormalities. They were considered to have BDN because they presented three of the four following criteria: neonatal suck and swallow difficulties; pharyngeo-oesophageal uncoordination with abnormal oesophageal manometria; upper airway obstruction, either clinically obvious or detected on laryngoscopy; vagal overactivity, either clinically obvious or detected during Holter recording with ocular compression. RESULTS: Among these 48 infants, 30 were affected with polymalformative syndrome often involving embryonic fields derived from the neural crest. Three infants had a conotruncal cardiac malformation and 15 had no associated malformation. These latter 15 infants presented with facial dysmorphic features including reciding chin, glossoptosis. U-shape palate and a vertical tongue. From birth or the first weeks of life, they had suck and swallow difficulties with various functional symptoms: slow baby bottle intake, cough or velo-pharyngeal incoordination, upper airway obstruction or apparent life threatening events (ALTE). Diagnosis was confirmed by both clinical observation and three simple investigations namely: laryngoscopy, oesophageal manometria and Holter recording with ocular compression. Ten children were nasogastric tube or gastrostomy fed, one had a tracheostomy and one had a nightly O2 supplementation. While the overall functional prognosis was good whatever the initial symptoms, 50% of these children had mental retardation, mostly moderate. CONCLUSION: Examination of short-term follow-up in these children has stressed that BDN requires a specific management of both nutritional and respiratory troubles. Finally, BDN should lead to the active search of an underlying polymalformative syndrome and to an accurate neurologic evaluation.

Abnormalities, Multiple↗

[Treatment of acute heart failure in infants].

The treatment of congestive heart failure in infants must be etiological and symptomatic. Vasodilators, inotropic agents and diuretics are the main therapeutics used in the symptomatic treatment. Guidelines for the emergency treatment according to the main etiologies are presented.

Acute Disease↗

[Pneumococcal meningitis in children: should probabilistic antibiotherapy of infectious meningitis be modified?].

BACKGROUND: Since a significant proportion of Streptococcus pneumoniae strains is now resistant to penicillin and sometimes to third-generation cephalosporin, it is necessary to reevaluate the initial therapy of bacterial meningitis proposed before identification of the organism and its susceptibility pattern. POPULATION: From 1 January 1992 to 31 March 1994, nine children with acute S pneumoniae meningitis were treated with ceftriaxone plus aminoglycoside as conventional initial therapy. Eight children were less than 1 year-old (five from 3 to 6 months). Five S pneumoniae strains were penicillin-resistant; four had a ceftriaxone minimal inhibitory concentration (MIC) of 0.047 to 0.094 mg/L and one of 1.5 mg/L. Ceftriaxone was given intravenously at doses of 50 mg/kg twice a day to patients less than 12 months old and 100 mg/kg once a day to patients older than 12 months. Intravenous amikacin (7.5 mg/kg twice daily) or netilmicin (3 mg/kg twice daily) were administered in combination. Dexomethasone was given to all children as adjunctive therapy. Follow-up lumbar puncture was performed after 24 to 36 hours of treatment. RESULTS: For each of the nine patients, cerebrospinal fluid was sterile with normal glucose level. After 2 or 4 days, initial therapy had been modified according to antibiogram and MIC. Monotherapy with ceftriaxone was continued in five children. Rifampicin was associated with initial bitherapy in one case. In two other patients, initial empiric therapy was stopped and changed to chloramphenicol. CONCLUSION: No case of bacteriological failure was noted in our patients but evolution of epidemiology and emergence of decreased penicillin sensibility in S pneumoniae strains (55% in our study) suggests that a third antibiotic (vancocin or rifampicin) should be associated with the standard first-line drug when S pneumoniae is suspected.

Adolescent↗

[Convulsions and neonatal hypoparathyroidism revealing maternal hyperparathyroidism].

BACKGROUND: Severe prolonged hypocalcemia may occur in neonates whose parathyroid hormone production has been blocked by maternal hyperparathyroidism. This report describes such a case. CASE REPORT: A 7 day-old girl was admitted suffering from dyspnea and repeated convulsions that had continued for 2 days. Her birthweight was 3,500 g and her height 50 cm. Her mother had been given an iron preparation and calcium during pregnancy. Clinical examination of the newborn at birth revealed a cleft palate and micrognathia. The baby had been given milk-formula plus ergocalciferol, 1,200 IU/day. At admission, the baby was hypotonic. Her serum total calcium was 1.27 mmol/l; total proteins 61 g/l; ionized calcium 1.1 mmol/l; phosphorus 2.14 mmol/l; intact PTH 21 pg/ml (N = 10-65) and 25(OH)D 8 ng/ml (N = 8-30). She was given intravenous calcium gluconate (1 g/m2/d), diazepam (0.5 mg/kg) rectally, intravenous phenobarbital (20 mg/kg) but intravenous phenytoin was needed to stop clinical and electrical seizures. Her blood calcium was normalized 5 days later. Her mother, who was clinically normal, had: total serum calcium: 2.72 and 2.77 mmol/l; total proteins: 71 g/l; phosphorus: 0.85 mmol/l; intact PTH: 73 pg/ml; 25(OH)D: 6 ng/ml; Ultrasonography showed an adenoma of the right parathyroid. Further studies on the baby showed no signs of Di George syndrome. CONCLUSION: Neonatal hypocalcemia always requires investigation of both the infant and mother. Measurements of vitamin D metabolites and intact PTH are required to recognize maternal hyperparathyroidism.

Female↗

[Neonatal trismus].

BACKGROUND: Trismus is caused by sustained contraction of the jaw-closing muscles. It is rare in the neonate and can be a part of developmental defects. PATIENTS AND METHODS: Twenty-four neonates, aged 1 to 12 days, were admitted from 1980 to 1992 because they suffered from trismus. All of them had severe difficulties of sucking and/or swallowing, some (12/24) had repeated episodes of apnoea requiring tracheotomy. Specialized investigations included transcutaneous PO2 and PCO2 monitoring, polygraphic recordings during sleep, 24-hour Holter monitoring, ocular compression test, barium swallow, manometry and 24-hour pH monitoring of the distal esophagus, electromyography of muscles involved in swallowing and several imaging techniques. RESULTS: Twenty-one patients had anomalies of the facies and brain stem dysfunctions. They were classified as Robin sequence (14 patients) with (five patients) or without (nine patients) micrognathia, cleft palate and glossoptosis, as Hanhart syndrome (four patients) and Moebius syndrome (three patients). The last three patients had arthrogryposis (two patients) and Stuve-Wiedeman syndrome. Twenty-two of 24 patients had severe gastroesophageal reflux, 15 of 20 had increased vagal reactivity and five of 24 had anomalies of the central nervous system. Eight patients died during the first year of life. CONCLUSION: Neonatal trismus is a poor prognostic sign, requiring specialized investigations and frequently resuscitation techniques.

Brain Stem↗

[Pharmacokinetics of diphemanil methylsulfate in infants].

BACKGROUND: Diphemanil methylsulfate is an atropin-like drug used in some infants suffering from vagal bradycardia. Its pharmacokinetic parameters are known for adults but not for infants. The report describes these parameters in six infants. POPULATION AND METHODS: Five infants aged 35 to 109 days (mean: 62 +/- 28) and weighing 3.5 to 5.3 kg (mean: 4.3) were included in the study with the formal consent of their parents. All suffered from vagal hyperreactivity. The sixth younger full-term infant was aged 10 days and weighed 4 kg. They were given a single dose (3 mg/kg) of diphemanil methylsulfate orally, after a minimal fast of 4 hours. Blood samples were collected at T0 and 3, 6, 8, 12 and 24 hours after administration. Urines were also collected from 1 hour before drug administration to 24 hours after. Plasma concentrations of diphemanil methylsulfate were measured by gas-exchange chromatography. RESULTS: The peak plasma concentration in the five infants occurred at 3.9 +/- 2.3 hours (range: 2.9-8 hours). Half-life was 8.6 +/- 2.4 hours and tended to decrease with age. All the other parameters were identical to those found in adults. The peak plasma concentration occurred in the sixth younger infant at 2.9 hours, with a half-life of 17.2 hours. Renal clearance was high (0.3 l/h/kg). CONCLUSION: The relatively long half-life of diphemanil methylsulfate allows this drug to be given every 8 hours. This longer interval is more comfortable for the patients and their parents. The high renal clearance suggests that this drug is excreted by both glomerular filtration and tubular secretion.

Age Factors↗

Pharmacokinetics of diphemanil methylsulphate in infants.

The pharmacokinetics of diphemanil methylsulphate was evaluated after oral administration of a single 3 mg.kg-1 dose to 5 infants being treated for symptomatic bradycardia. The mean pharmacokinetic parameters of oral diphemanil methylsulphate in infants were similar to those in adults. The mean half-life was 8.6 h. This would allow administration three times a day in infants instead of four to six times a day, as currently prescribed. The mean residence time decreases significantly with age (Spearman's r' = -1), and there is a trend for the half-life to decrease with age (r' = -0.9; NS), suggesting an influence of maturation on its elimination.

Administration, Oral↗

[Sudden crib death. Experience in a referral center 1986-1991].

BACKGROUND: Sudden infant death syndrome (SIDS) may have several etiologies, all of which must be identified in order to recognize those infants believed to be at risk. One of the best ways to do this is by prospective studies on a large population of infants who died of SIDS. METHODS: A total of 171 infants who died from SIDS between January 1, 1986 and June 30, 1991, were studied. 154 infants were completely investigated, including a post mortem examination. They were assigned to one of 4 groups, according to whether death was due to diseases of poor prognosis (group A), diseases that are occasionally fatal but potentially treatable (group B), minor diseases not normally fatal (group C), or was essentially unexplained (group D). RESULTS: The classical risk factors for SIDS were found in this population: incidence peaked in males (sex-ratio 1.5), during the cold seasons (62%), between 1 and 6 months of age (94%), mainly between 1 and 4 months of age (84%). Symptoms were definitely present during the 2 days before death in 50%. 20% of cases had clinical histories of congenital disease, complicated or recurrent postnatal disease, or fulminant recent disease. Group A included 107 infants (69% of the 154 completely investigated patients). Premature birth (17.5%) and low birth weight for gestational age (10.5%) were more frequent in our series than in the normal population. CONCLUSION: The cause of death was identified in about 75% of cases. This possibility improves management of further siblings of SIDS victims even though the variety of risk factors makes prevention of SIDS difficult.

Autopsy↗

[Myxoma of the pulmonary infundibulum as the cause of sudden death in an 18-month-old infant].

BACKGROUND: Myxoma, a benign cardiac tumor, develops in intracavitary locations. It is most frequent in the left atrium, but rare in the pulmonary infundibulum. CASE REPORT: An 18 month-old girl suddenly became cyanotic during playing and lost consciousness. Resuscitation was ineffective. Post-mortem examination showed diffuse pulmonary edema and multiple small pulmonary emboli. There was a myxoma of the septal wall of pulmonary infundibulum and histological examination showed that the infundibulum and the pulmonary valve were completely infiltrated by myxoid tissue. CONCLUSION: The death of this girl was probably due to a sudden embolism of blood clots developed in contact with the myxoma. This case emphasizes the need for pathologic investigation in all cases of sudden death.

Female↗

[Examination and management of facial malformation].

Facial malformations are embryonic morphological accidents involving the prosencephalic frontonasal process (facial cleft), the branchial arches (laterofacial malformations) or the rhombencephalic process. Knowing the future development of these clusters of cells helps in the search for neurological brain abnormalities associated with facial cleft and the respiratory, cardiac, gastrointestinal and brainstem malformations associated with laterofacial malformations and Pierre Robin syndrome. Taking care of the newborn already in the maternity unit prevents the consequences of vital distress and enables the child to be placed under conditions of normal psychomotor development.

Abnormalities, Multiple↗

[Transient erythroblastopenia in young children].

A case of transient erythroblastopenia of childhood is reported. This syndrome occurred in a 13 month-old girl. Initial signs were a 6.1 g/dl hemoglobin level and severe erythroblastopenia. Spontaneous recovery occurred after a few weeks. No etiology could be found.

Anemia, Aplastic↗

Cardiac involvement in cystic fibrosis revealed by a ventricular arrhythmia.

Cardiac involvement in cystic fibrosis (CF) is characterized by acute cardiac failure in the infant with myocardial fibrosis found on pathological examination. In the older child with chronic pulmonary disease, cor pulmonale tends to predominate the clinical picture. We report the case of an adolescent with CF presenting with ventricular arrhythmia. Cardiac scan, repeated echocardiograms and Holter monitoring were suggestive of an infiltrative process. We propose this case to be similar to those of CF in infants with cardiac involvement.

Adolescent↗