[Hypoplasia adrenal congenita of anencephalic type: two cases with pituitary abnormalities and review of literature]. / L'hypoplasie surrénale congénitale de type anencéphalique: deux cas avec anomalies hypophysaires, sans mutation de DAX-1 et SF-1 et revue de la littérature.

Hypoplasia adrenal congenita is an extremely uncommon disease of early onset. This condition can be lethal in the absence of treatment. Some forms are due to the congenital adrenal hypoplasia of anencephalic type whose origin is even unknown. Here, we present two cases of congenital adrenal hypoplasia of anencephalic type with pituitary abnormalities. The two male newborns died because adrenal insufficiency in the neonatal period. The adrenal glands were hypoplastic with a histological structure of anencephalic type Immunocytochemical study of the pituitary revealed an absence of the gonadotrophs. No mutation of DAX 1 and SF-1 was found.

[Development of the human adrenal glands]. / Le développement de la surrénale humaine.

The human adrenal is an endocrine gland located at the superior part of the kidney. Composed of the adrenal cortex of mesoblastic origin and the adrenal medulla of neuroectoblastic origin, the human fetal adrenal grows considerably during the first three months of development. From 12 to 18 weeks of development (WD), the weight of the adrenals increases seven-fold. The gland's weight doubles from 18 to 28 WD and from 28 to 36 WD. At birth, the two adrenals weigh on average 10 g. At the 8th week, two zones are individualized in the adrenal cortex: the definitive zone and the fetal inner zone. At the second trimester, according to ultrastructural and biochemical studies, a third zone, called the transition zone, is individualized between the definitive zone and the fetal inner zone. The definitive zone persists, but the origin of the three zones (glomerular, fascicular and reticular) of adult adrenal cortex is not known. The fetal inner zone regresses from the 5th month of gestation and disappears totally one year after birth. At the 8th week, the immature neuroblasts migrate to the definitive zone, then to the fetal inner zone to compose the adrenal medulla, which develops essentially after birth and during the first year. Before the 10th week, the human fetal adrenal is able to produce steroid hormones, in particular dehydroepiandrosterone sulfate (DHEA-S); the secretion of cortisol remains discussed. The development of the human fetal adrenal is complex and is under the control of hormones (ACTH, LH and betaHCG), growth factors (ACTH essentially) and transcription factors (essentially SF1 and DAX-1). Knowledge of morphological and molecular phenomena of this development permits to understand the pathophisiology of congenital adrenal deficiencies.

[Histological and molecular study of fetal human adrenal cortex (12-36 wk)]. / Etude histologique et moléculaire de la corticosurrénale foetale humaine (12e-36e SD). La corticosurrénale foetale humaine (12e-36e SD).

Histological and functional characteristics of the fetal human adrenals was studied in 119 normal fetuses aged 12 to 36 weeks development (WD). Immunocytochemical detection of steroidogenesis enzyme (3beta-HSD and P450 c21) and evaluation of cell proliferation using two nuclear markers (Ki-67 and PCNA) were performed in 70 of them. The human fetal adrenal cortex is composed of two morphologically distinct zones: the definitive peripheral zone and the fetal inner zone. From the 12th WD, we observed expression of an adherence protein (NCAM) and two steroidogenesis enzymes (3beta-HSD and P450 c21) in the definitive zone cells, attesting to the capacity of these cells to synthesize mineralocorticoids and/or cortisol. In the fetal zone, only P450 c21 immunoreactivity was detected. From the 14th WD, a transitional zone between the definitive zone and the fetal zone was identified by immunocytochemistry, with expression of 3b-HSD from the 21st WD. Only cells of the definitive zone proliferated from the 12th to 25th WD. The indexes of proliferation of PCNA and Ki-67, 40% and 25% respectively, decreased gradually and were lower than 1% at the 25th WD.

[Epidemiologic and etiologic features of urinary infections in children at the pediatrics service of the CHU-Campus de Lome (Togo)]. / Aspects épidémiologiques et étiologiques de l'infection urinaire de l'enfant dans le service de pédiatrie du CHU-Campus de Lomé (Togo).

From 1st January 1989 to 31th December 1997, 175 infants (108 females and 67 males) were hospitalised and treated at the pediatric service of CHU-Campus for urinary tract infection; this study follows the observation of the increasing of urinary tract infection in several centers of health in Togo; the aim of this study was to have a list the contributing factors, to understand the mechanism of such infection in order to reduce its frequency and the high percent of the mortality; the diagnosis of urinary tract infection was given by the result of the cytobacteriological exam of the urine which shows the pathological germ; others forms of the investigation, as abdominal echography were used also to look for the etiology of the urinary tract infection; but, the deficit of the of the medical imagery or the old material of the laboratories limited the searching of urinary tract infection etiology; cured infants were declared on the basis of absence of pathological germ in the result of the cytobacteriological exam control of the urine; the prevalence of the urinary tract infection was 8.29% with an incidence of 7.84% at the pediatric service of CHU-Campus; clinics symptoms were atypic and polymorphic; but the fever was the first clinical sign in the newly born and the urological signs were clear only from two to thirty months; 141 children (80.57%) were cured and 34 presented the complications with 3.43% of mortality; preventive measures on the urinary tract infection in infancy were proposed for the children parents and the practical physicians; these measures included information, education and communication (IEC) on the urinary tract infection, the symptomatology and the cytobacteriological exam of the urine.

[Ulcerative colitis: a case in Togo]. / La rectocolite hémorragique : une observation au Togo.

Ulcerative colitis seems to be rare in sub-Saharan Africa. Because its clinical and radiological signs are non-specific, anatomopathologic studies are necessary for definitive diagnosis after exclusion of parasitic, bacterial, and viral causes. The purpose of this report is to describe a fatal case of ulcerative colitis in a 68-year-old woman in Togo.