[Comparative study of psychometric results of 2 french speaking populations with hypothyroidism detected at birth]. / Etude comparative des résultats psychométriques de 2 populations francophones d'enfants hypothyroïdiens dépistés à la naissance.

The authors compare the data from the psychological assessment of a cohort of 191 French speaking hypothyroid children detected by neonatal screening: 99 were followed in Quebec and 92 in Lille, according to two independent programs. Three age levels were selected: 12-18 months, 5 and 7 years. Data were similar and, in both studies, results of the later ages were slightly lower than the norms of the tests or results obtained in control groups. In both populations correlations were found between some results and two factors related to hypothyroidism: delayed bone surface development and thyroxinemia at the time of diagnosis. On the other hand, etiology and time of treatment had no repercussions on any group results.

[Growth and bone maturity during congenital hypothyroidism screened in the neonatal period. Apropos of 82 cases]. / Etude de la croissance et de la maturation osseuse au cours de l'hypothyroïdie congénitale dépistée en période néonatale. A propos de 82 observations.

Systematic neonatal screening of congenital hypothyroidism (CH) allowed to detect 82 children (62 girls and 20 boys) including 73% with ectopic thyroid, 18% with athyreosis and 9% with eutopic thyroid. They were followed for 1 to 7 years according to the same protocol. Skeletal maturity was studied according to 2 methods: TW2 and Sempé's numerical. Height growth was compared to the standards for French children. T4 values were normal at all ages, TSH values were slightly higher than standard. Concerning the skeletal maturity the delay observed in boys lessened from age 6 years (TW2 method: less than 25th percentile until 3 years, = 25th from 3 to 5 years, between P25 and P50 by 7 years of age). This delay was less important in children who, at the time of diagnosis (day 15) had an epiphyseal surface greater than or equal to 5 mm2. In girls, it was always above the 25th percentile. The expression of the delay was greater with Sempé's method than with the TW2 method. Thus, early substitutive treatment of CH allows a normal height growth. The skeletal maturity is somewhat abnormal in boys. This could be explained by the high percentage (66.6%) of boys with a small epiphyseal surface during the neonatal period and by a slight under-treatment.

[Prognostic value of bone surface in hypothyroidism detected in neonatal period]. / Etude de la valeur pronostique de la surface osseuse dans l'hypothyroïdie dépistée en période néonatale.

Bone maturation at the time of diagnosis has been considered as a prognostic factor of congenital hypothyroidism. Using bone area as an index of bone maturation, we have compared the psycho-intellectual outcome of infants with high bone area (group I) at time of diagnosis, with those with low area (group II), in a population of infants with congenital hypothyroidism detected by a neonatal screening program. We found that children from group I had better performances than those from group II whatever the age of the evaluation (1, 2, 5 or 7 years). Nevertheless, the verbal performances of children from group I remained weaker than their non verbal performances and in 7 among 54, the IQ was found to be lower than 90 with a possible relationship with the thyroid insufficiency (bad compliance to treatment) in only one of them.

[West's syndrome. A clinical, therapeutic and prognostic study. Apropos of 66 cases]. / Le syndrome de West. Etude clinique, thérapeutique et pronostique. A propos de 66 observations.

The charts of 66 children suffering from West syndrome followed by the paediatric department of the University Central Hospital, Lille, between 1957 and 1984 were reviewed: 24 cases were followed for more than 10 years; 20 cases from 5 to 10 years; 22 cases from 1 to 5 years. Age at onset of the disease ranged from 3 to 5 months (37.8%) and 6 to 8 months (30.3%) with a slight male predominance. Prenatal causes were the most important (42.4%) with anomalies and cerebral malformations in 15.2% and neurocutaneous syndromes in 9.1% of cases. Etiology remained unknown in 27.3% of cases. Some clinical aspects were characteristic (flexion or extension spasms, psychomotor retardation) in 84.8% of cases. Hyparrhythmia occurred in 62.1% of cases. There were no significant differences in the results of the varying therapies used (ACTH, Synacten, hydrocortisone) or its associations. The best results were obtained with a treatment started as early as possible in the cryptogenic forms and in older infants (6 to 8 months).

[Treatment of type II familial hypercholesterolemia by portacaval anastomosis]. / Traitement de l'hypercholestérolémie familiale de type ii par anastomose porto-cave

Termino-lateral porto-caval anastomosis seems at present to be the most effective treatment for severe familial hypercholesterolaemia. The mechanism by which it acts has not been determined and its long term results are not known. One success and one failure confirm the immediate action of anastomosis in a condition with a fatal prognosis.