Ghizlane El Mghari

Association of newly diagnosed type 1 diabetes and autoimmune pancreatitis

Summary Autoimmune pancreatitis is a new nosological entity in which a lymphocytic infiltration of the exocrine pancreas is involved. The concomitant onset of autoimmune pancreatitis and type 1 diabetes has been recently described suggesting a unique immune disturbance that compromises the pancreatic endocrine and exocrine functions. We report a case of type1 diabetes onset associated with an autoimmune pancreatitis in a young patient who seemed to present a type 2 autoimmune polyglandular syndrome. This rare association offers the opportunity to better understand pancreatic autoimmune disorders in type 1 diabetes. Learning points: The case makes it possible to understand the possibility of a simultaneous disturbance of the endocrine and exocrine function of the same organ by one autoimmune process. The diagnosis of type 1 diabetes should make practitioner seek other autoimmune diseases. It is recommended to screen for autoimmune thyroiditis and celiac diseases. We draw attention to consider the autoimmune origin of a pancreatitis associated to type1 diabetes. Autoimmune pancreatitis is a novel rare entity that should be known as it is part of the IgG4-related disease spectrum.

Un cas de phéochromocytome découvert au cours d’une grossesse gémellaire: un diagnostic à ne pas méconnaitre et revue de littérature

The pheochromocytoma is a rare, but potentially serious, which can be revealed by pregnancy. Less than 250 cases described in the literature .The rarity of this association and similarity with pregnancy-induced hypertension explains the frequency of unknown diagnoses during pregnancy. A privileged time of discovery, the prognosis associated with early diagnosis, a multidisciplinary management. Hence the need to explore all arterial hypertension poorly defined or family nature during pregnancy. The certainty diagnosis is conducted by biological tests provided are thought of, a review of localization by ultrasound or magnetic resonance imaging (MRI), a medical preparation can choose according to the term to remove the tumor before or after delivery. We related a case of pheochromocytoma diagnosed during a twin pregnancy of 26 weeks of amenorrhea (SA), bringing us the elements of the clinical and laboratory diagnostic imaging, treatment and maternal and fetal prognosis.

Malignant external otitis in diabetic patients

Malignant external otitis (MEO) or necrotising otitis is a rare entity, the starting point is a violation of the external ear canal (EEA) and dissemination of the infection to the soft parts and the adjacent bone, hard adiagnostiquer and whose evolution can be very serious. Elective on debilite ground, such as immunocompromised, or more often the elderly diabetic, is a necessary diagnostic criterion. The symptoms and signs may include severe earache, otorrhoea and achievement of various crannies, often caused by Pseudomonas Aeruginosamais also of other agents can rarementinclure to other bacteria (Staphylococcus aureus, Proteusmirabilis, Klebsiellaoxytoca, Pseudomonascepacia) or fungi (Aspergillus, Pseudallescheria, Candida, Pityrosporum).1 The presence of tissue granulation in the EEC, a high rate of sedimentation of red, and abnormalities on imaging studies are also diagnosis parameters. It is so very important to make the diagnosis of MEO as soon as possible, at the beginning it is very similar to severe acute external otitis, but MEO is developing a severe osteomyelitis of the temporal bone, attaquantles cranial nerves adjacent (VII, XII), the blood vessels and soft tissue, and finally, if it is not treated, it causes death due to the expansion osteomyelite of the skull and thromboembolism septic disseminated brain. Therapy should be should be conducted by the otolaryngologists in collaboration with the endocrinologist, internist, neurologist, radiologist and microbiologist. Local treatment (with removal of bone sequestered) and systemic antibiotic treatment according to the results of bacteriological tests (aminoglycosides, semisynthetic Penicillins, thirdand fourthgeneration cephalosporins, fluoroquinolones) are generally accepted.

Paragangliome tympanique simulant un cholestéatome: à propos d’un cas

Tympanic paragangliomas are common benign tumors of the middle ear, made up of neuroendocrine cells dispersed along the major vascular axes of the head, the neck and the vertebral column. The revealing symptoms are hearing loss and a pulsatile tinnitus. Otoscopy often shows pulsatile retrotympanic reddish mass. MRI and somatostatin analogue scintigraphy (OctreoScan) are the imaging modality of choice. OctreoScan allows physicians to detect other tumor locations. The dosage of urinary methoxyl derivatives is determined, complemented by the screening of other components of multiple endocrine neoplasias (MEN). Biopsy is contraindicated because of the risk of hemorrhage. Surgery is the treatment of choice. We report the case of F. A., a 75-year old patient, admitted with tympanic paraganglioma revealed by hearing loss in his right ear (occurred 4 years before) associated with pulsatile tinnitus concomitant with the heart beating in his ear. He underwnt otoscopy showing retrotympanic reddish pulsatile mass in his right ear (A). (B) MRI of the brain showed total involvement of the middle ear and of the mastoid cells, without identifiable tumoral process, with distinct hyperintensity on diffusion-weighted imaging and strong contrast-enhancement. MEN assessment was without abnormalities, including 24h methoxyl derivatives, parathyroid hormone and calcitonin. The diagnosis of paraganglioma was retained because of the strong contrast-enhancement. OctreoScan was indicated before surgery.

Myélolipome de la surrénale: à propos d’un cas

Adrenal myelolipoma is a rare benign non secreting tumor. It is often unexpectedly detected. Pathophysiologically, it is adrenal cortex cell metaplasia into reticuloendothelial cells, resulting from infection, chronic stress or adrenal gland degeneration. The mean age at diagnosis is late forties. Histologically, the tumor is composed of mature fat tissue associated with normal haematopoietic tissue. Tumor echogenicity depends on the predominance of the greasy or myeloid component. Its diagnosis is based on CT scan that identifies the fat percentage. However, these radiological aspects may lead to diagnostic confusion with kidney angiomyolipoma, lipoma and liposarcoma, hence the key role of MRI. Surgical resection of myelolipoma is usually performed and is indicated when tumor is voluminous, compressive or at risk of hemorrhage. We here report the case of AL A, 75 years old, hospitalized for adrenal mass revealed by right back pain radiating to the right hypochondrium, without signs of endocrine hypersecretion. Clinical examination showed sensitivity of the right lumbar fossa. Pheochromocytoma and corticosurrenaloma assessment was without abnormalities, including 24-hours urinary methoxyl derivatives and dexamethasone suppression test. The patient underwent coelioscopy; anatomo-pathological examination showed adrenal myelolipoma.

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.

Kallmann syndrome with short stature and pituitary hypoplasia: a case report

Kallmann syndrome (KS) is a rare disease in which hypogonadotropic hypogonadism and anosmia co-exist. In KS, the gonadotropic deficiency is isolated, the other pituitary hormones, especially the growth hormone, are preserved. We report the case of a 17 year old male having a sporadic case of KS associated with growth retardation. The diagnosis was based on hormonal workup and specific features on the MRI. The pituitary gland was hypoplastic. The patient was diagnosed to have Kallmanns syndrome with short stature associated to pituitary hypoplasia. To the best of our knowledge, this is the first case to be described in the literature combining KS, short stature and hypoplastic pituitary gland.

Diabetes distress in transitional age evaluated by 'problem areas in diabetes' in type 1 diabetic patients from Marrakech.

Type 1 diabetes (T1D) represents 5.3% of all types on diabetes. Its incidence is increasing around the world as it is in the Middle East and North Africa Region, where the incidence is at 1/100 000. T1D touches young subjects and is then established in a growing body. Transitional period is a crucial phase with physical and emotional distress. Psychosocial difficulties are an additional challenge for these young patients. Aim of the study: Evaluate diabetes distress in transitional age using lsquoproblem area in diabetes (PAID) in its Arabic transcultural adaptation. Materials and methods: Problem area in diabetes questionnaire was self-administered in 50 type 1 diabetics that were followed up in the department of endocrinology in University medical hospital of Marrakech. Results: Over the population evaluated 54% were female. The median of age was 17, 54 years. 32% were younger than 15 years old and 68% were older. 84% were living in urban area. 78% of them were students. 48, 6% had duration of diabetes lt5 years and 56% had an A1c above 9%. 18% of patients had a PAID score above 40, which indicates a diabetic distress. 8% had a PAID score less than 10. And it was between 10 and 40 in 74% of the patients. Conclusion: A high prevalence of diabetes distress had been observed in our population of patients. Non-access to care, limited resources, and social problems explain this result. This kind of studies allows a better understanding and support of those patients and should lead toward an improvement of transitional care in diabetes. nbsp

Wolfram Syndrome: Report of Two New Cases

Wolfram Syndrome (WFS) is a rare autosomal recessive disease. It is a progressive neurodegenerative disorder in which patients present with diabetes mellitus, optic atrophy, diabetes insipidus, sensorineural deafness, urologic abnormalities and multiple neurological abnormalities. This study reports two sisters with late diagnosed wolfram syndrome with diabetes insipidus, diabetes mellitus, optic atrophy, deafness and urological abnormalities. The condition should be evaluated in a multidisciplinary attitude and specific tests are necessary to make a precise diagnosis of the syndrome.

Physiology of the Hypothalamic-Pituitary-Testicular Complex: A View by Stages in the Light of Recent Advances

The male gonadotropic axis consists of the hypothalamus, pituitary gland and testes. Testosterone is produced by the Leydig cells in the presence of pituitary luteinizing hormone (LH). LH and FSH (follicle stimulating hormone) are themselves regulated by gonadotropin-releasing hormone or GnRH, released in pulses by the anterior hypothalamic neurons. The embryonic migration of GnRH neurons, which is a critical step in this process, is now better understood thanks to the identification of new genes that are involved. The regulation of the gonadotropic function has itself been illuminated by the identification of new peptide regulatory factors which include kisspeptins. This review traces the physiology of male gonadotrope axis based on new knowledge relating to its establishment, its operation and its regulation, allowing a better understanding of the congenital hypogonadotropic hypogonadism.