[Diffuse arterial calcified elastopathy. A case report]. / Elastopathie calcifiante artérielle diffuse. A propos d'un cas.

Diffuse arterial calcified elastopathy is a very rare and little known hereditary disease, characterized by diffuse calcifications of the arterial wall. It seems common in North Africa and in the Caucasian region. Its incidence appears to be underestimated in Morocco. Clinical pattern is dominated by renovascular hypertension often associated with symptoms of heart failure. Risk of sudden death from myocardial infarction is particularly important. Thus, the diagnosis of diffuse arterial calcified elastopathy must always be suspected in front of an apparently unexplained heart failure or renovascular hypertension occurring in an infant. We report a case of diffuse arterial calcified elastopathy discovered in a neonatal intensive care unit, during management of a cardiogenic shock in a 3-months old infant. This observation demonstrates the importance of systematic measurement of the arterial tension, family screening and the impact of the ultrasound in the detection of vascular calcifications. Treatment remains essentially symptomatic.

[Oesophageal duplication with neonatal revelation. About 2 cases]. / Duplication oesophagienne à révélation néonatale. A propos de 2 cas.

UNLABELLED: Oesophageal duplication is a rare form of digestive duplication. This congenital malformation can be asymptomatic or manifest itself through respiratory signs due to airway compression. We report 2 cases of oesophageal duplication discovered in the neonatal period. CASE REPORT: In both cases, symptoms were dominated by respiratory distress and vomiting. Diagnosis was confirmed by oesophageal contrast X-rays, which revealed a total tubular form in the 1st case and a cystic form in the 2nd case. CONCLUSION: Oesophageal duplication is a rare abnormality of benign nature, which can be revealed in neonatal period by a noisy compression picture. Diagnosis of this anomaly should trigger a search for other digestive duplications, as well as associated malformations, in particular vertebral.

[Natal teeth: apropos of five cases]. / Dents natales: à propos de cinq cas.

INTRODUCTION: The normal eruption of primary teeth begins with mandibular incisors about the age of 6 months. There are several qualifiers used to describe prematurely erupted teeth. Massler and Savara defined "natal teeth" as teeth present at birth and "neonatal teeth" as teeth erupted within the first month of life. The aim of this study, based on 5 cases, is to present clinical and structural characteristics, etiology, management techniques, complications and a review of the literature for natal teeth. MATERIAL AND METHODS: Our study is a retrospective study of 17,000 infants who were examined in the Neonatal Department of Children Hospital between 1984 and 2001. The material consisted of 5 infants with natal teeth, identified by the pediatrician. We analyzed the family history, the pregnancy history, the gender, the etiology, the complete examination of the infant, the clinical, the structural characteristics, the complications and the management of the teeth. RESULTS: We found 4 boys and 1 girl, 2 of the newborns were premature, all of them observed to have natal teeth. The incidence was 1: 3,400 births. There were 14 natal teeth, 10 incisors (70%) and 4 canines (30%), no molar was found. Nine of the teeth (6 incisors and 3 canines) were maxillary (65%) and 5 (4 incisors and 1 canine) were mandibular (35%). No morphological syndrome was discovered. Most of the teeth were mobile in all directions and were extracted because of the possibility of aspiration, the difficulty in feeding and the ulceration of the ventral surface of the tongue. DISCUSSION: According to the literature, this phenomenon is rare and the incisors are the teeth most commonly involved. Natal teeth are more common than neonatal teeth and nearly 90% of these teeth are the normal primary teeth. The presence of natal teeth is due to several factors related to an unknown cause of disturbed biological chronology. There is no conclusive evidence of a correlation between early eruption and systemic disorders, but some investigators suggest that natal teeth may be associated with certain syndromes. We must keep in mind that radiographic examination is essential for the differential diagnosis between supernumerary and normal primary teeth. The supernumerary teeth should always be extracted but the decision to extract a normal mature natal tooth should be done according to scientific knowledge, mobility of the tooth, local or general complications and parental opinion.

[Consanguinity and public health in Morocco]. / Consanguinité et santé publique au Maroc.

This study concerns the evolution of consanguinity in Morocco in its historical, religious, legal, and sociological context with regards to Moroccan customs and undertakes to evaluate its relationship to public health. Thus it attempts to specify if consanguinity, in its present state in Morocco, poses a problem for public health. With this goal in mind, methodology has been concentrated on two approaches. The first, based upon examination of documents and oral research interviews, has made theoretical assessment possible. The second, substantiated by the study of notarial marriage acts and the analysis of 4773 medical files concerning consanguineous marriages compiled throughout the seven regions of Morocco, has enabled us to specify the importance and the evolution of consanguinity and its impact on health. This study shows a marked decrease in consanguineous unions predominantly found in rural and mountainous zones and frequent between first cousins, especially the paternal cousin. From the medical standpoint, our study has revealed the absence of pathology in 97.13% of cases studied, the transmission of various gifts in 1.08% and that of degenerative traits in 1.79% of the descendants. Consequently, in our country, a country in perpetual evolution and in contact with the outside world, consanguinity which is disappearing of its own accord does not present a preoccupying problem for public health. Its future would seem to be limited to the relation between the physician and marriage partners in search of genetic counsel.