[Multiple endocrine neoplasia type 1: about a case]. / Néoplasie endocrinienne multiple type 1: à propos d'un cas.

Multiple endocrine neoplasia type 1 (MEN1) is a rare disease, defined as a tumor developing in at least two endocrine glands including the anterior pituitary gland, the parathyroid glands and the duodenopancreatic endocrine tissue. This disorder, inherited in an autosomal dominant pattern, is caused by mutations in the MEN1 gene encoding the tumor suppressor menin and located on chromosome 11q13. However, sporadic cases account for 8-14%. The first endocrine lesion may be solitary in approximately 75% of cases. However, all major alterations can be inaugural. We here report a case of multiple endocrine neoplasia type 1 revealed by aggressive somatoprolactinic pituitary adenoma which didn't respond to conventional treatment. The detection of primary hyperparathyroidism as well as neuroendocrine tumor of the pancreas seven years later make this a very particular case. Therapeutic options are discussed within the multidisciplinary team specialized in endocrine diseases.

[Difficulties in managing Graves' disease in children: about a case]. / Difficultés de prise en charge de la maladie de Basedow chez l'enfant: à propos d'un cas.

Adequate management of Graves' disease in children is an area of controversy in pediatric endocrinology, while optimal duration of medical treatment inducing remission in disease as well as indications for therapeutic alternatives still needs to be determined. We report the case of a 11-year old female child with no particular past medical history., presenting with progressive weight loss associated with diarrhea. Her parents brought the child to hospital because neighbours noticed anterior cervical swelling on her. Clinical and paraclinical data helped to retain the diagnosis of Graves' disease. The patient underwent medical treatment with carbimazole. Patient's evolution was marked by the occurrence of thrombocytopenia, suggesting prudent administration of medical treatment. Iratherapy was indicated due to the absence of remission after 4 years of treatment. Although rare, Graves' disease is the first cause of hyperthyroidism in children. Positive diagnosis is easy but its management may pose enormous problems. Medical treatment is based on synthetic antithyroid drugs, but they are not always innocuous, as in the case of our patient. Then subtotal thyroidectomy or radioactive iodine treatment are two therapeutic alternatives. Graves' disease is a rare and severe disease in children posing considerable management difficulties.

[Epidermoid cyst in the sella turcica: about a case]. / Kyste épidermoide de la selle turcique: à propos d'un cas.

Epidermoid cyst (EC) or cholesteatoma is a benign tumor, often of congenital origin, occurring in the sub-arachnoidian spaces. Its treatment is based on surgery. We report the case of a 38 year-old patient presenting with progressive intracranial hypertension syndrome associated with anterior pituitary insufficiency signs of the corticotropic, thyrotropic, gonadotropic axis occurring 1 year before. Brain MRI showed a craniopharyngioma. Anatomopathologic evaluation of the specimen showed epidermoid cyst.

[Hyperprolactinemia: unusual association between peripheral hypothyroidism and microprolactinoma]. / Hyperprolactinémie: coexistence rare d'une hypothyroïdie périphérique et d'un microprolactinome.

We report a rare case of hyperprolactinemia revealing the association between peripheral hypothyroidism and prolactin pituitary macroadenomas. The patient was a 43-year old woman, presenting with spontaneous bilateral galactorrhea over a period of 1 year. Hyperprolactinemia was confirmed and etiologic investigation revealed peripheral hypothyroidism secondary to autoimmune thyroiditis. Therapy consisted of administration of thyroid hormone, with clinical stabilization and hormonal normalization three months later. The evolution was marked by the persistence of hyperprolactinemia and galactorrhea. The diagnosis of microprolactinoma was objectified by pituitary MRI which showed microadenoma, justifying the administration of antidopaminergic therapy Six months later, the evolution was marked by normalization of prolactin levels and disappearance of pituitary microadenoma image.

[Etiology of endocrine arterial hypertensions: about a series of cases]. / Etiologies des hypertensions artérielles endocrines: à propos d'une série de cas.

Arterial hypertensions (HTA) of endocrine origin are a rare cause of hypertension; HTA overall prevalence don't exceed 4% of hypertensive patients. Research interest in endocrine HTA is due to the severity of some life-threatening, potentially curable and reversible forms of HTA. The aim of our study was to determine the clinical, paraclinical, etiological and therapeutic profile of secondary HTA of endocrine origin in patients treated in endocrinology department at the University Hospital Mohamed VI in Marrakech. We conducted a prospective, descriptive study spanned 4 years, enrolling 45 patients with endocrine HTA. The average age was 44.89 years, with a clear predominance of women (sex ratio 0.49). Etiology of endocrine HTA was dominated by pheochromocytoma (17 cases), hypercorticism (11 cases) and acromegaly (8 cases). HTA were paroxysmal in 24.4%. HTA were immediately classified as grade 3 severe in 40% of cases. HTA were complicated by heart disease in 24% of cases and by renal disease in 20% of cases. Curative treatment cleared up HTA in 60% of cases (27 cases). The diagnosis of secondary endocrine HTA is sometimes difficult because of the lack of clinical specificity. It is not unusual for HTA to be the only manifestation of the disease. In our study we noted the paroxysmal and severe nature of HTA. The potentially curable nature of HTA in more than two thirds of cases, demostrates the importance of early diagnosis of each severe HTA resistant to treatment or in the presence of suggestive clinical, biological or radiological signs.

Jaw tumor in primary hyperparathyroidism is not always a brown tumor.

Primary hyperparthyrodism (PHPT) is a common endocrine disease. It results from an inappropriate parathyroid hormone (PTH) secretion relative to serum ionized calcium level. Clinical manifestation of severe PHPT include bone disease called osteitis fibrosa cystica which reflects an increase osteoclastic resorption and osteoblastic activity. This high bone turnover is responsible of the occurrence of osteoclastomas, also named "brown tumors" (1). Rarely, PHPT may occur in inherited forms with association to fibrous jaw tumor that are unrelated to hyperparathyroidism. In this uncommon disease: hyperparathyroidism-jaw tumor (HPT-JT) syndrome, parathyroid tumor is frequently malignant and usually associated with nonendocrine malignancies (2). We report a case of a HPT-JT syndrome to focus on the differential diagnosis with brown tumors.