[Fatal neonatal respiratory distress in Niemann-Pick C2 and prenatal diagnosis with mutations in gene HE1/NPC2].

Unlabelled: We report the fifth case of neonatal form of type C2 (NP-C2) Niemann-Pick disease with early and fatal respiratory distress. Eleven families presenting such cases are known to date in the world. Since December 2000, isolation of the underlying gene HE1/NPC2 and its mutations has allowed major advances in diagnosis.

[Congenital pulmonary lymphangiectasia revealed by cardiac arrest].

Unlabelled: Congenital pulmonary lymphangiectasia is a rare cause of respiratory distress in the neonatal period. Cardiac arrest may be its first manifestation.

Case Report: We report the case of a full term newborn who suffered at 30 min of life a sudden cardiac arrest.

[Non-medical factors in perinatal health. A study of socioeconomic anc cultural features of women admitted to the maternity hospital in Lens (Pas-de-Calais)].

Background: The aim of this study was to determine the influence of medical and non-medical factors on perinatal health in an underpriviledged area, the ex-coal mines of Lens (Pas-de-Calais).

Population And Methods: This prospective study concerned 1,000 women who delivered in the maternity hospital of Lens between January 5, 1993 and May 13, 1993. The relationships between preterm delivery, low birth weight and maternal socioeconomic and cultural characteristics were analyzed.

[An outbreak of allergy to horses in children. A review of 56 recent cases].

Over the past 11 years, signs of allergy were observed in 56 children and adolescents in contact with horses. The cases consisted of 35 boys and 21 girls, 35 of them were under 10 years of age. The main clinical signs were ocular symptoms (36), asthma (30) and rhinopharyngitis (24).

[Does the allergic child become sensitized to small domestic mammals (guinea pig, hamster, rabbit?)].

Our own works show among allergic children a frequency of sensitization of 30% to cats and of 17% to dogs. But, the proportion of sensitizations to other small domestic mammals such as guinea pigs, hamsters of rabbits seems to be unknown. Among atopic children with a known contact with those pets we noted: for guinea pig: 29% positive cutaneous or RAST tests 21% clinical signs in presence of animals; for hamster respectively 28% and 6.

[Is it necessary to test children having summer respiratory problems with cereal pollens?].

We tested 100 children with clinical signs that occurred in spring or summer. Prick tests were made with extracts of wild grass pollens (Dactylis, Phleum, Lolium) and with separate cereal pollens (Wheat, oats, Sweet-corn Barley and Rye). In 20 children whose test was negative for wild grass pollen, we saw no sensitization to cereals.

[Comparison of specific IgE levels versus various milk proteins in 76 eczematous children].

Among 76 eczematous children, we realized a RAST test for whole milk and milk specific proteins: lactalbumin, lactoglobulin and casein. One of these RAST tests, at least, was positive for 27 children. Whole milk RAST test is the most frequently positive (77%) but some non specific reactions seem to interfere: only three children are clinically milk sensitive.

[Can Acarus siro be considered an allergenic mite in children? Evaluation of 248 cases].

Among 248 allergic children we noted 44 children (21%) with strongly or moderately positive cutaneous tests (In seven out of these tested children respiratory provocation tests are very positive). They are urban children and their houses are not different from other allergic children. They are mostly boys (72%), more than 5 years old (91%), with an asthma (84%), a radiological sinusitis (45%), an hypereosinophilia (88%) and elevated total IgE (90%).

[Role of rhinomanometry in childhood mite allergy apropos of 76 cases].

Place for Passive Anterior Rhinomanometry (PAR) in mite allergic children. PAR is a simple method to investigate chronic or recurrent rhinopathies in children. Among 59 investigations with a Dermatophagoides pteronyssinus extract, we found 27 positive responses, 3 doubtful results and 32 negative explorations.

[Does mugwort pollenosis occur in children in the northern region?].

Is Mugwort pollenosis frequent among children in North of France? In North of France where no ragweed grows, mugwort (artemisia) is after grasses and plantago, the third pollenosis in children. Among 184 children with summer clinical signs, positive cutaneous tests with a mugwort extract are noted in 40 patients (21%). RAST are positive only in 23 on 34 children with positive cutaneous test.

[Does immediate-type respiratory allergy occur regarding Stemphylium? Evaluation of 39 challenge tests].

Does immediate respiratory allergy with stemphylium exist? About 39 provocation tests. We present 39 children with positive cutaneous and/or RAST tests for stemphylium mold. Provocation tests individualize 19 patients (14 boys and 4 girls, 10 to 16 years old in 17/19) with a respiratory sensitivity: 6/10 by passive anterior rhinomanometry and 13/15 by bronchial tests.

[Are the left-handed more often allergic?].

Are left handed more frequently allergic patients? With the same evaluation clinical criteria, we find a Non Right Handedness (left handed + mixed handedness) in 48/403 children (11.9%) at school in a town near our consultation place and in 152/625 (24.32%) children of the same age with a mites allergy (p less than 0.

[Neonatal epileptic encephalopathy with suppression bursts (author's transl)].

In a neonate who had seizures in the second day of life, the EEG showed - during waking and sleep alike - periodic bursts of asymmetrical high voltage on a low amplitude background tracing. From the third week head jerks occurred, followed by repetitive spasms and then adversive tonic seizures. A severe psychomotor retardation was noted.