[Kallmann-de Morsier syndrome: about 3 cases]. / Le syndrome de Kallmann-de Morsier: à propos de trois cas.

Kallmann-de Morsier syndrome (KS) is a genetic disease of the olfactory system characterized by the association of hypogonadotropic hypogonadism also referred to as gonadotropin-releasing hormone (GnRH) deficiency and anosmia or hyposmia (with hypoplasia or aplasia of the olfactory bulbs). Apart from sporadic cases that occur most often, familial Kallmann's syndrome is being described with increasing frequency. Diagnosis is mainly made in adolescents with absence of spontaneous puberty associated with smell disorders with hypoplasia or even aplasia of the bulbs and/or of the olfactory lobes on MRI. Sometimes, the diagnosis may be suspected in early childhood due to the association of cryptorchidism and micropénis. A mutation in one of known genes is only found in less than 30% of cases and, therefore, many other genes are still to be found. Hormone therapy allows pubertal growth in all cases and fertility can be obtained in most of the cases. We here report 3 cases of patients with this syndrome.

La nécrobiose lipoïdique: à propos d'un cas: Necrobiosis lipoidica: about a case.

Necrobiosis lipoidica is a rare granulomatous dermatosis. It occurs in 0.3-1.2% of diabetic patients mainly at the level of the leg. The lesions appear as erythematous plaques with central depressions. We here report the case of a 21-year old female patient with a 6-year history of type 1 diabetes presenting with a 1-year history of well circumscribed, symmetrical and asymptomatic erythematous plaques with atrophic yellowish center at the level of the anterior compartments of the legs (A, B). The diagnosis of necrobiosis lipoidica was suspected, a biopsy was performed which confirmed the diagnosis. The patient had a granulomatous inflammatory reaction with palisade-like border disposed around poorly delimited foci of altered connective tissue on the entire dermis layer. The inflammatory reaction was also characterized by multinucleate giant cells, dendrocytes and lipid deposits. Local corticosteroid therapy was prescribed. Patient's evolution was marked by the persistence of the lesions.

[High grade insulinoma detected in a patient with a 5-year history of severe hypoglycemias]. / Insulinome de haut grade découvert après 5 ans d'hypoglycémies graves.

Functional endocrine tumors of the pancreas are rare. Among them insulinomas are the most common types. The majority of the patients with insulinoma are between 30 and 60 years of age and 59% of them are women. Most insulinomas are sporadic tumors while 10% of them develop in patients with multiple endocrine neoplasia (MEN). Diagnosis is based on clinical examination and laboratory tests. Preoperative imaging assessment (echo-endoscopy, CT, MRI) is essential for tumor localization in more than 80% of cases. Surgical excision is the treatment of choice. We here report the case of a 50-year old patient with high grade insulinoma developed on the anterior surface of the pancreas tail.

Exceptional Association Between Klinefelter Syndrome and Growth Hormone Deficiency.

Klinefelter syndrome (KS) is characterized in adults by the combination of a tall stature, small testes, gynecomastia, and azoospermia. This case is described in a North African population of the Mediterranean region of North Africa. We report the case of a male 16 years old, of Arab ethnic origin, and diagnosed with this syndrome, who had a small height in relation to a growth hormone (GH) deficiency and a history of absence seizures (generalized myoclonic epilepsy). The patient's size was <-2.8 standard deviation (SD) with weight <-3 SD. GH deficiency was isolated and confirmed by two dynamic tests (insulin - hypoglycemia tolerance test and clonidine) with normal hypothalamic magnetic resonance imaging (MRI). GH supplementation using recombinant GH was advocated, while gonadotropin treatment was deferred. Small size in children or adolescents should not eliminate the diagnosis of Klinefelter syndrome - on the contrary, the presence of any associated sign (brain maturation, delay in puberty, aggressiveness) should encourage one to request a karyotype for the diagnosis and appropriate care of any case of KS that can be associated with GH deficiency, or which is in a variant form (isochromosome Xq, 49,XXXXY).