[Hemihypertrophy and scoliosis revealing a Chiari 1 malformation with syringomyelia]. / Hémi-hypertrophie et scoliose révélatrices d'une malformation de Chiari de type 1 avec syringomyélie.

We report the case of a 9-year-old boy with progressive thoracic scoliosis and crossed hemihypertrophy who was discovered with a Chiari 1 malformation and syringomyelia. These disorders are connected by complex physiopathological mechanisms; their association deserves attention. This observation reviews the importance of the clinical examination, particularly the neurological exam, in childhood scoliosis. The features suggesting a neurogenic background of spine deformation should be sought. Scoliosis with hemihypertrophy can be the sign of an underlying neurological abnormality.

[Kwashiorkor after an exclusion diet for eczema]. / Kwashiorkor après régime d'éviction pour eczéma.

BACKGROUND: Nutritional disorders observed in children with eczema may be aggravated by inappropriate diet, sometimes with dramatic effects. CASE REPORT: A child aged 3 years 7 months presented a state of severe malnutrition with edema (kwashiorkor). The child had chronic eczema and had been given a highly restrictive diet based on a principle of exclusion without medical advise. Cure was achieved without sequelae with a normal diet. DISCUSSION: This case illustrates the fragility of nutritional status in children with chronic widespread skin disease due to the risk of cutaneous loss of protein. Kwashiorkor is an exceptional observation in developed countries and, in this context, might be on the rise due to the popularity of exclusion diets for presumed allergic disease.

Prenatal transmission of dengue: two new cases.

Dengue is commonly observed in most tropical countries, but its transmission from mother to fetus has not been frequently described. We report two such cases. The first signs of dengue in the infants appeared on the 3rd and 9th days of life. In both, a bacterial infection was suspected initially. In areas where it is endemic, the diagnosis of dengue should be considered in the neonate with signs of bacterial infection. When dengue is suspected in a pregnant woman, laboratory investigation and extended observation of the newborn are advised.

[Therapeutics of the future for allergy]. / Thérapeutiques du futur en allergologie.

Future improvement in the therapeutic arsenal in allergy must in part be by improvement of the existing, by the development of new molecules that are more powerful and more selective amongst the antiH1, chromones, Beta-2 mimetics, corticoids as well as the development of new routes of already-know products. Specific Immunotherapy (ITS) will undoubtedly benefit from progress in geni-genetics, from improvement in adjuvants and development of new routes of administration. Control of the environment will assist industry for the manufacture of anti mite bedding and household materials. Primary prevention will be based on better identification of populations at risk and on rationalisation of the alimentary diversification of infants, so controlling the environment of populations at risk. Finally, better understanding of the complex interaction of cytokines will allow consideration of creation, following the recent example of antileukotrienes, of new products of intervention and in particular regulation of the TH1-TH2 balance.

Congenital heart disease diagnosed in Guadeloupe.

An epidemiologic survey of congenital heart disease was performed in Guadeloupe, French West Indies, during the 3-year period 1988-1990. This survey, the first on congenital heart disease from the Caribbean, found an overall rate of 6.08 congenital heart disease cases per 1,000 live births, a high rate of hypoplastic left heart disease, and a rate of coarctation of the aorta similar to rates reported in developed countries.

PIP: Between January 1988 and December 1990 health workers referred 139 infants suspected of heart disease to a skilled pediatric cardiologist at the University Hospital of Pointe-a-Pitre, Guadeloupe. The physicians considered patent ductus arteriosus of prematurity to be a congenital heart disease if it still existed after the neonatal period. The incidence of congenital heart disease was 6.08 cases/1000 live births with a sex ratio of 1:1. 12% of chromosomal abnormalities, especially Down's syndrome. Physicians diagnosed 92% of all cases in the 1st year of life. 35% (49 infants) went to the Pediatric Cardiologic Department of Hospital Necker in Paris, France, to undergo catheterizations. 32 of these infants also underwent chest surgery. Prevalence of coarctation of the aorta (3.1/10,000 live births) essentially equalled that of developed countries. The prevalence of hypoplastic left heart disease was considerably higher than the rate in the literature (3.5/10,000 live births vs. 0.5-2.7/10,000 live births). It was the 4th ranking congenital heart disease in this population (5.7% of all cases). All the infants with hypoplastic left heart disease died.

[Leprosy in Guadeloupe 1980-1990: development and management in the general hospital]. / La lèpre en Guadeloupe 1980-1990: évolution et prise en charge a l'Hôpital général.

27 leprosy patients (19 adults, 8 children) from Basse Terre (Guadeloupe, FWI) were referred to General Hospital from 1975 to 1989: for diagnostic purpose in half the cases, for starting or modifying treatment regimens, or for complications (ENL). Lepromatous type was predominant (16/27), whereas ENL was the most serious diagnosis and therapeutic problem (7/27). Some epidemiological and psycho-social aspects are discussed.

[Infantile acropustolosis: unusual manifestation of scabies in the infant?]. / Acropustulose infantile: expression inhabituelle de la gale chez le nourrisson?

Three pediatric cases of vesiculopustules persisting for several months or years after eradication of Sarcoptes scabiei are reported. This particular course of scabies in infants is underrecognized although probably common. Its clinical manifestations, natural history and histologic features are identical to those seen in infantile acropustulosis, a recently individualized syndrome whose pathogenesis is unelucidated. In the light of these cases and a review of the literature, the possible relationship between scabies and infantile acropustulosis is discussed.

[Follow-up of HIV-I seropositive children at the Saint-Hyacinthe Hospital in Basse-Terre (Guadeloupe)]. / Suivi des enfants séro-positifs pour le VIH I à l'Hôpital Saint-Hyacinthe de Basse-Terre (Guadeloupe).

Between 1985 and 1989, we have followed twenty (20) newly born babies whose mothers were HIV seropositive. Eight of them were contaminated. Two of them died. We have studied the particular aspects of this infection in Guadeloupe.

[Marshall syndrome. 2 new cases]. / Syndrome de Marshall. Deux nouveaux cas.

Two new familial cases of Marshall syndrome are reported. The main features of this rare syndrome are outlined. Its possible relationship with Stickler syndrome is discussed.

[8 cases of diphtheria observed in Guadeloupe from 1979 to 1984]. / A propos de 8 cas de diphtérie observés en Guadeloupe de 1979 à 1984.

From 1979 to 1984, 8 cases of diphtheria were diagnosed in Basse-Terre, in children aged 11 months to 6 years. All of them were badly or not vaccinated at all. They showed the common clinical features; two patients had only pharyngitis. Corynebacterium diphtheriae was isolated from the throat in 6 patients. All children were successfully treated by early administration of antitoxin and antibiotics.

[Brain abscess in the neonatal period]. / Abcès du cerveau en période néonatale.

Cerebral abscess is considered as a rare lesion with severe prognosis in neonates. The present means of cerebral imaging allow for a better estimation of its frequency, a more precise diagnosis of the evolutive stage and the estimation of the efficacy of the treatments. Incision-drainage associated with antibiotic treatment was successful in children. Two cases of neonatal cerebral abscesses treated and cured according to this protocol are reported.

[Acute poisonings in children in Guadeloupe. 10 years' activity at a general pediatric service]. / Intoxications aiguës chez l'enfant en Guadeloupe. Dix années d'activité d'un service de pédiatrie générale.

The authors have studied all the files concerning intoxications admitted in their department throughout ten years. They stress on the epidemiologic aspects particular to this tropical country, in order to plan a program for preventing children intoxications and accidents.

[Reinfection after rubella and congenital polymalformation syndrome]. / Rubéole de réinfection et syndrome polymalformatif congénital.

A case is reported of a term newborn with intra uterine growth retardation and numerous malformations such as complex heart disease, abnormalities of distal limbs, cleft palate. Death occurred after two days. The diagnosis of rubella embryopathy was confirmed by the following criteria: a high level of rubella antibodies in mother and newborn (1/1000) an isolation of rubella virus from the infant's urine. Diagnosis of rubella after reinfection was documented by a high level of antibodies in the mother three years before this pregnancy. Other observations reported in literature confirm the extreme rarity of congenital rubella after reinfection.

[Pulmonary valve agenesis and giant lobar emphysema. A not uncommon association]. / Agénésie des valves pulmonaires et emphysème lobaire géant. Une association non exceptionnelle.

A case of neonatal giant lobar emphysema secondary to a complex cardiac anomaly with pulmonary valvular agenesis is presented. Clinic and radiologic findings are sufficient to suggest diagnosis. Right heart catheterization is required to demonstrate the pulmonary valvular agenesis. Such an investigation should be recorded when observed a giant neonatal emphysema. Is failure occurs in the neonatal period the prognosis of this association is poor.

[Congenital hydrocephalus with gigantism]. / Hydrocéphalie congénitale avec gigantisme.

Fetal ultrasonography supplied the diagnosis of hydrocephalus after 35 weeks of pregnancy. After birth, the diagnosis was confirmed by clinical examination and CT Scan. Psychomotor development was normal with stabilization of the hydrocephalus without any treatment. In view of this case, the authors discussed the indications for antenatal surgical treatment of hydrocephalus cases diagnosed in utero.