Biomedical subjects
Publications and source records attributed to M Roussey.
The case of a 13 month-old-boy with visceral leishmaniasis acquired in Brittany, a region of France where leishmaniasis is not endemic, is presented. The mode of contamination remains unclear, although a transfusional origin through blood transfusions during the neonatal period appears the most likely.
A young Angolian boy who had emigrated to France at the age of 2, presented with a long history of fever. Gambian Trypanosomiasis was diagnosed with peculiar aspects: 1) evolution of adult sickness with a long hemolymphatic period (first stage) and a subacute worsening period with neurologic deficit and somnolence (second stage); 2) a possible post-transfusional contamination: the young boy, born in South Angola, a nor-highly endemic area, was transfused at the age of 10 months with the blood of a donor who was subsequently treated for Trypanosomiasis; 3) a suppurating adenopathy; 4) a predominance of IgG within the hypergammaglobulinemia while IgM are the predominant immunoglobulins in this affection; 5) a hepatic toxicity of Difluoromethylornithine.
Measles immunization with the Edmonston Zagreb stain was carried out in 71 six-month-old infants. Proportions of subjects with immunity were 91% among the 47 subjects retested before one year of age and 100% among the 28 subjects retested between two and three years of age. These results support the WHO recommendation that measles immunization should be given at the age of six months. The concerns expressed by some about possible adverse effects of early measles immunization (decreased immune defenses) are discussed, as well as the transfer of maternal antibodies and persistence of these antibodies in the child. The obstacles to such studies in developing countries, including the need for repeated phlebotomies with centrifugation of specimens and freezing of sera, could be circumvented by the use of filter paper dried blood spot samples which seem to provide reliable results although with values somewhat lower than those found in frozen sera.
An 18-month-old infant developed hemiseizure-hemiplegia syndrome following a booster dose of vaccine against diphtheria, pertussis, tetanus, and poliomyelitis. Clinical, CT scan, and electroencephalographic findings during the two-and-a-half-year follow-up are described. The timing of events and data from the literature suggest that hemiseizure-hemiplegia syndrome is related to post-immunization hyperthermia rather than to direct neurologic toxicity of the vaccine.
The authors report the case of an eleven-month old infant who was given maprotiline and clobazam. He was found in coma state and suffered from convulsions, that were treated with phenobarbital. The electrocardiogram showed a right bundle branch block and a left anterior hemiblock over 72 h. The child then recovered without sequelae.
We report a case of Sandifer syndrome with chronic torticollis and gastroesophageal reflux (GER). The infant exhibited regurgitations and vomiting from birth. Torticollis with a permanent tilt of the head towards the right developed at age six months. At 16 months, persistence of the vomiting and abnormal attitude of the head and neck led to a CT scan that outruled a brain tumor. Esophageal pH recordings disclosed severe gastroesophageal reflux (pH less than 4 for 46% of the time over 24 hours) and endoscopy showed ulcerated peptic esophagitis. Surgical treatment of the GER ensured both resolution of the reflux and disappearance of the torticollis, establishing the causal relationship between the former and latter manifestations.
We report on two sibs with a multiple congenital anomalies/mental retardation (MCA/MR) syndrome who have a first cousin with Möbius anomaly. This may represent a new MCA/MR syndrome.
Hydrocephalus is a frequent complication of spina bifida. We wanted to find out if the risk of isolated hydrocephalus was greater in families with NTD (anencephaly and spina bifida) from 424 families studied between 1975 and 1984 in Brittany. The risk of recurrence of NTD is 1.8% in these families, the risk of hydrocephalus 0.3% which represents a risk three times greater than that of the population at large. This risk seems all the higher if the proband is a spina bifida of the male sex. However, no X-linked heredity can be reasonably assumed in these cases. We conclude that these families present a higher multifactorial risk of having another malformed child.
A 14-month-old child was admitted to the hospital with acute pancreatitis, abdominal pain, and shock. An abdominal radiograph revealed a paper clip standing out at the duodenal level. The foreign body was removed by endoscopy, revealing an edematic and hemorrhagic duodenal papilla. The patient progressed favorably after the foreign body was removed. This observation underlines the necessity of digestive transit surveillance of a foreign body that has not been endoscopically removed and the relevancy of duodenal endoscopy for acute pancreatitis of indefinite etiology.
Home care of tracheostomized infants was studied through the experience of 4 families. Medical, social, financial, technical and psychological problems were reviewed. Common main outlines loomed out: after an initial defensive response against tracheostomy, parents were involved in the care of the child. They learned to suction the child and change the tube. Home comeback of the baby produced most anxiety to the parents for a few nights then they coped with it. The mothers had to leave their outside work so the family income decreased in all cases. The family's activities were most altered too. But babies' psychomotor development was excellent, language was delayed but finally normal in three cases, school attendance was obtained and all families considered lucky with the overall development. The knowledge of this common background permits to plan the parental education and the intervention of social workers, speech therapist, kinesiotherapist and psychologist.
One hundred children (67 boys, 33 girls) underwent early fiberoptic endoscopy (without general anesthesia in 96 cases) for caustic ingestion between January 1985 and June 1988. The intended use of the product was household (83) industrial (4) farm (6) or medicinal (7). Caustic burns were classified as grade 1 (mucosal hyperaemia), grade 2 (ulceration) and grade 3 (necrosis). Endoscopy was negative in 52, grade 1 in 41 who had evidence of esophagitis and/or gastritis, grade 2 and/or grade 3 in 7 cases, 4 of whom had ingested farm products. Eighty-seven children were discharged after examination, 6 were hospitalized for 24 hours. Outcome was favorable for the 5 cases with grade 2 lesions after total parenteral nutrition for an average period of 79 days. The 2 cases with grade 3 injuries went on to develop an esophageal stricture requiring endoscopic dilation and an antral stricture which was treated by antrectomy. Authors emphasize the severity of lesions secondary to the ingestion of dairy pipeline cleaners, the advantages of fiberoptic endoscopy and the role of parenteral nutrition.
The authors describe 2 new vaccines now available in France: one is the GenHevac, an hepatitis B vaccine, the first virus recombinant vaccine; the other one is the Typhim Vi, a polysaccharide typhoid vaccine. Three other vaccines are currently used in foreign countries and will be soon available: the Hemophilus influenzae vaccine, the acellular pertussis vaccine and the varicella vaccine. Rotavirus and Cytomegalovirus vaccines are studied for their clinical efficacy.
The authors have made the census of all the Medical Terminations of Pregnancy (MTP) which have been carried out in the Department of Ille et Vilaine from 1982 to 1986, i.e. 222 cases, in order to precise the different indications and the diagnosis tools which were used. 132 MTP concern women who live in the Department of Ille et Vilaine. By referring this figure to the total number of pregnancies in this area, one can see that the average incidence is of 1.9%; MTP account for 1% of the total number of Terminations of Pregnancies. Foetal indications are more frequent (188 cases; i.e. 84.7%) than maternal ones (34 cases, 15.3%); these figures remained stable over the 5-year period of study. Chromosomal aberrations and closing defects of the neural tubule are the main causes of MTP (22.9% of foetal indications). Among the 43 chromosomal aberrations, trisomies are the most frequent ones (34.9%) because all women aged 38 or more are proposed a detection. The diagnosis of trisomy was made in 24 cases after tests were programmed either because of the age of the mother or because of family antecedents (amniocentesis: 22 times, punction of foetal blood: once, biopsy of chorion villosities: once), in 6 cases after tests were carried out on the basis of suspect clinical signs amniocentesis: once, punction of foetal blood: 5 times), and in 13 cases after the echography had revealed a major syndrome. Closing defects of the central nervous system mainly concern anencephaly (17.6% of foetal indications) since the echography enables an easy diagnosis. All anencephaly have actually been detected during the reference period of pregnancy.(ABSTRACT TRUNCATED AT 250 WORDS)
Truncus arteriosus is an uncommon heart malformation; it is not reported that recurrence is high; nevertheless authors report three families with two or more cases; in the third there is a very high consanguinity (two uncle-niece marriages). The authors compare the situation with hypoplastic left heart and think that some cases of truncus arteriosus would have an autosomal recessive inheritance. That is an another argument for suggesting an echocardiographic survey of the pregnancies in these families.
The authors describe a case of neonatal Proteus mirabilis septicemia accompanied by cerebral abscess formation despite the presence of therapeutically effective antibiotic levels utilised to treat the disorder. The occurrence of such brain abscesses during the course of effective antibiotic therapy raises the question of the mechanism behind their formation. Cerebritis may occur very early in the clinical course of the infection without being due to failure of antimicrobial therapy.
The case of a 4 years old boy, hospitalized for an unexplained coma, is reported. He is the first child of a non-consanguin couple. The psychomotor development of this child was considered as normal up to the age of 18 months; then, a delay in language development, behaviour disorders with an important instability interrupted by episodes of somnolence, were observed. This child was treated for psychotic disorders. At the age of 3 and half, he had two episodes of seizures associated with fever. He was hospitalized for a 24 hours coma (4 years old). An hepatomegaly and a dry, brittle hair were then observed. Hyperammonemia was made obvious by a protein tolerance test. The diagnosis of argininosuccinate lyase (ASAL) deficiency was based on the increased levels of ASA in plasma and urine. The deficiency was proved by a fibroblast culture. With protein restriction, hepatomegaly disappeared, hair became normal, the behaviour disorders and the delay in language development was improved. However, some school difficulties persist. This case shows that an hereditary metabolic syndrome can be revealed by psychotic like symptoms in childhood.
The study of stillbirths is a way to approach foetal medicine and a good opportunity to foster a closer relationship between obstetricians, pediatricians and public health physicians, which will lead to preventive measures known to be effective in decreasing mortality rates. Four hundred and ninety pregnancies which ended in stillbirths were investigated: 61 before 27 weeks of amenorrhea, 228 between 28th and 36th weeks, 194 after 37 weeks. The different causes were identified: intrauterine growth retardation and pathological pregnancies remain the most important causes but the authors attract attention to a category of foetal deaths occurring at the end of the pregnancy without any evident cause and for which "postmaturity" was likely. The data attract attention on the necessity to carry out research on stillbirths through a closer collaboration between obstetricians and pediatricians and suggest that appropriate preventive measures may lead to a significant reduction of the 25% of avoidable deaths.
Two further cases of congenital diaphragmatic hernia with delayed presentation are reported: a 6-month-old male presented a posterolateral diaphragmatic hernia with small bowel in left hemithorax masquerading as pleural effusion; an 11-year-old boy with Down's syndrome presented a retrocostoxyphoid hernia revealed by vague faintness. The authors emphasize the deceptive clinical aspect, the different means for diagnosis, the risk of wrong diagnosis and pleural drain, the usual good outcome of these late-onset diaphragmatic herniation.