Pheochromocytome a localisation para-renale revelee par une hypertension arterielle maligne: a propos d'un cas

Les auteurs rapportent un cas de pheochro-mocytome ectopique. Cas clinique : patiente de 29 ans; hypertendue connue depuis 8 ans et diabetique depuis 7ans; admise en urgence pour perte de connaissance. L'examen clinique a montre une pression arterielle a 190/130mmHg; un pouls a 116 battements/minute et un Glasgow a 7 sans deficit hemi corporel. L'examen du fond d'oil a mis en evidence des signes de retinopathie hypertensive stade III. Le dosage plasmatique de la normetanephrine etait de 3917ng/l (normale170) et celui de la metanephrine de 59ng/l(normale73). Le scanner abdominal a montre un processsus tumoral tissulaire para-renal anterieur et polaire superieur gauche; mesurant sur une coupe axiale 73;8 X 51;3mm. Le diagnostic de pheochromocytome a ete pose. Le traitement a consiste a une exerese de la tumeur. L'evolution est favorable. Conclusion : le pheochromocytome para-renal est une cause rare d'hypertension arterielle maligne

[One step surgery for severe African endocrine exophtalmia: clinical case]. / Chirurgie en un temps d'une exophtalmie basedowienne africaine sévère: présentation d'un cas clinique.

INTRODUCTION: The association hyperthyroidism-exophtalmia is pathognomonic of the Graves disease. Classically, the treatment is based on a pluridisciplinary step-by-step approach with a precise chronology. In some African places, these optimal conditions are not present. When confronted to such particular situations, it can be proposed to treat surgically at the same time the endocrine disease and the ophthalmologic complications. OBSERVATION: The authors report the case of a 42-year-old women suffering of a great thyreotoxic goiter with severe bilateral exophtalmia, treated by thyroidectomy and orbital decompression in the same session with a good result after 18 months. DISCUSSION: This non-conventional approach can be useful in particular situations when the medical environment is not optimal. It permits the radical treatment of their disease and improves the prognosis of patients with poor capacities for survey.

[Hypothyroid goiter with tracheal compression in a child in Cote d'Ivoire]. / Goitre hypothyroidien compressif chez une enfant en Côte d'Ivoire.

The purpose of this report is to describe a case involving a primary form of hypothyroid goiter with tracheal compression discovered late in a four-year-old child. Slowing of height and weight gain and mental retardation was irreversible. The child was treated using L-thyroxin. Systematic screening for hypothyroidism during the neonatal period is recommended in developing countries.

High precursor level in maternal blood results from the alternate mode of proopiomelanocortin processing in human placenta.

OBJECTIVE: ACTH-producing non-pituitary tumours are often associated with altered precursor processing, particularly in the most aggressive ones. Since placental tissue is characterized by its ability to express the proopiomelanocortin (POMC) gene and rapid cellular proliferation, we examined whether intact POMC could be released physiologically during human gestation. SUBJECTS: One hundred and fifty six normal pregnant women, 12 with multiple pregnancies, and 23 non-pregnant controls. Twenty-eight women were studied in the immediate postpartum period. MEASUREMENTS: We measured plasma POMC levels with a specific immunoradiometric assay (IRMA) using a combination of antibodies directed against ACTH and beta endorphin. Results obtained with this first IRMA were confirmed in 22 subjects with a second assay using the same beta endorphin antibody and a more distal antibody directed against the N-terminal fragment of POMC. Reverse transcription-PCR detected full length, pituitary-like, POMC mRNA in human placenta. RESULTS: Plasma POMC was undetectable (< 60 U/ml) in 23 normal subjects. In normal monofetal pregnancies, POMC became detectable in most women by the third month and then increased steadily until midgestation: 168 +/- 108 (U/ml; mean +/- SD) between 12 and 15 weeks, 190 +/- 103 between 16 and 19 weeks, 324 +/- 180 between 20 and 23 weeks, 276 +/- 171 between 24 and 27 weeks, 292 +/- 177 between 28 and 31 weeks, 290 +/- 235 between 32 and 35 weeks and 308 +/- 210 between 36 weeks and parturition. Plasma POMC was significantly higher in multiple pregnancies with very high levels in three triplet-bearing mothers: 671, 941, and 1731 U/ml at 31, 33 and 32 weeks, respectively. POMC levels felt quickly in post partum, becoming undetectable in five of 13 women on day 1, seven of eight on day 2 and five of six on day 3. Plasma POMC displayed no diurnal variation, was not suppressed by glucocorticoid administration and did not correlate with plasma ACTH or cortisol. In contrast, plasma POMC positively correlated with plasma CRH. CONCLUSIONS: Pregnancy is the only condition in which POMC is produced and released physiologically, similar in some respects to the ectopic ACTH syndrome. POMC is derived solely from the placenta, with no interference from maternal pituitary secretion, and is thus a new and specific placental marker.

Acute renal failure in a diabetic patient.

We report the case of a young insulin-dependent diabetic patient in whom acute renal failure led to detection of ureterohydronephrosis secondary to neurogenic bladder. The persistence of a high daily urine volume revealed diabetes insipidus, suggesting Wolfram syndrome, which was confirmed by the diagnosis of optic atrophy. The main features of Wolfram syndrome, particularly urologic ones, and their treatment are discussed in the light of our findings in this patient.

[Ectopic ACTH secretion: a heterogeneous entity]. / Sécrétion ectopique d'ACTH: une entité hétérogène.

OBJECTIVES: ACTH-secreting non-pituitary tumors are a rare cause of Cushing's disease. We report the clinical course, prognostic aspects and molecular analysis data in three patients for whom the diagnosis was confirmed but who had variable clinical features and laboratory results. CASE REPORTS: Patient n degree 1 had severe hypercorticism which rapidly progressed to death 13 months after diagnosis. In patient n degree 2, signs of hypercorticism severe, leading to death 5 years after discovery of the causal carcinoid tumor. Patient n degree 3 had moderate hypercorticism and has survived for more than 25 years. DISCUSSION: These 3 ectopic tumors are representative examples of a wide range of possible ACTH-secreting ectopic tumors. In highly malignant poorly-differentiated tumors such as small-cell anaplastic carcinomas, ACTH production is aberrant and poorly controlled, and thus not particularly effective. At the other extreme, typical benign bronchial carcinomas have a high degree of neuroendocrine differentiation and secrete ACTH in a well-controlled manner difficult to distinguish from corticotropic adenomas, further exaggerating the diagnostic pitfalls.