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Purpose Allgrove syndrome, also known as “triple A” syndrome, is characterized by adrenal insufficiency, achalasia, and alacrimia. When neurological signs are also present, the condition is referred to as “4 A” syndrome. Methods We conducted a retrospective analysis of three patients with 4 A syndrome confirmed genetically. A complete neurological exam was carried out by an experimented neurologist. Results Herein, we describe the neurological characteristics often associated with this condition, through the clinical and electrophysiological analysis of three patients. All patients exhibited a mutation in AAAS, the gene coding for ALADIN. While these individuals presented with the classic features of triple-A syndrome, neurological symptoms were not prominent. Conclusion The neurological manifestations of Allgrove syndrome have historically been overlooked and inadequately explored. Due to the condition’s rarity and substantial phenotypic heterogeneity, only recently have a variety of symptoms been recognized and described.

Abstract Background Pituitary apoplexy (PA) is defined as the hemorrhage or the infraction of a pituitary adenoma. Aiming to determine the epidemiological, clinical, paraclinical characteristics as well as management and outcomes of PA in our population, we conducted this cross-sectional study. Methods This cross-sectional study was conducted at the Department of Endocrinology of Hedi chaker university hospital, Sfax. Data was collected from medical charts of patients with pituitary apoplexy admitted in our department between 2000 and 2017. Results We included 44 patients with PA. Their mean age was 50 ± 12.6 years. Among them, 31.8% had a known pituitary adenoma, and it was in all cases a macroadenoma, predominantly a prolactin secreting tumor (42.8%). A triggering factor of PA was encountered in 31.8% of cases and it was mainly: head trauma, dopamine antagonists, and hypertension. The clinical presentation of PA encompassed headaches (84.1%), visual disturbances (75%), and neurological signs (40.9%). Gonadotropin deficiency was the most frequent form of hypopituitarism noted (59.1%), followed by corticotropin deficiency (52.3%), thyrotropin deficiency (47.7%), and somatotropin deficiency (2.3%). Hormonal assessment at PA onset, concluded that 23 had a secreting adenoma: 18 prolactinomas, 3 ACTH-secreting adenomas, and 2 GH-secreting adenomas. In the 21 remaining cases, the tumor was non-functioning (47.7%). Pituitary MRI was performed in 42 cases (95.5%), revealing infraction and or hemorrhage in the pituitary gland in 33 cases; a heterogenous signal or a fluid level within the adenoma, in nine cases. Urgent administration of intra venous hydrocortisone was required in 19 cases. Mannitol administration was mandatory in a patient who had severe intracranial hypertension. Surgical management of the PA was imperative in 24 patients (54.5%): 15 suffered from severe visual impairment, 4 had an intracranial hypertension, 2 cases demonstrated an impaired consciousness, 2 patients experienced a tumor enlargement and one case had a severe Cushing’s disease. Operative complications found were rhinorrhea attributable to cerebral spinal fluid leakage, insipidus diabetes associated with rhinorrhea, isolated insipidus diabetes, and hydrocephalus in one case each. Long-term follow-up concluded that headaches persisted in five cases, owing to the tenacity of a macroprolactinoma regardless of cabergoline treatment in one case, the recurrence of an adenoma in two cases and its persistence despite the medical and the surgical treatment in two patients. Concerning the visual acuity defects, only two patients had persistent diminished visual acuity at long-term follow-up. Among 25 patients, 13 were diagnosed with definitive thyrotropin deficiency. Similarly, 14 patients had persistent corticotropin deficiency (CD). Additionally, CD was de novo diagnosed in two patients. Otherwise, gonadotropin deficiency prevailed in all cases. Persistent prolactin deficiency was seen in two patients. Disappearance of the pituitary tumor was encountered in 11 out of 24 cases at long-term follow-up. Overall, surgery was associated with better outcome than conservative management. Pituitary apoplexy is a challenging condition due to its variable course, its diagnosis difficulty and management, as gaps remain to determine the best approach to treat this condition. Conclusions To conclude, pituitary apoplexy is a challenging condition due to its variable course, its diagnosis difficulty and management, as gaps remain to determine the best approach to treat this condition. Further studies are thus needed.

A 55-year-old patient was admitted to our department for the management of a repetitive alteration of consciousness. Biological investigation results were consistent with endogenous hyperinsulinemic hypoglycemia. Insulinoma was therefore suspected. Abdominal computed tomography and endoscopic ultrasound showed no obvious pancreatic mass. Somatostatin receptor scintigraphy showed abnormal radioactive uptake in both the pancreatic tail and the uncinate process. Contrariwise, abdominal magnetic resonance imaging showed a unique lesion in the pancreas tail. The patient was then proposed for pancreatic surgery. Both intraoperative manual palpation and intraoperative ultrasonography of the pancreas showed a single corporal lesion of 1.5 cm. No lesion was found in the uncinate process. After a left pancreatectomy, the lesion was histopathologically confirmed to be a well-differentiated neuroendocrine tumor. The symptoms of the patient resolved almost immediately following the surgery. The follow-up is one and a half years to date.

Abstract Background Pheochromocytoma and paraganglioma are rare neuroendocrine tumors of the chromaffin tissue, which may produce catecholamines. The aim of our study was to analyze the clinical and para-clinical aspects as well as the therapeutic and evolutionary aspects of pheocromocytomas and paragangliomas based on a series of 40 cases. Methods Our retrospective population-based research study includes 40 patients. Then, a statistical analysis was carried out using the SPSS software (version21). Results Our study involves 40 patients, including 23 women (57, 5%) and 17 men (42,5%). The mean age at the time of the diagnosis was 43.8 ± 16.8 years. The circumstances of the discovery were mainly characterized by adrenal incidentaloma and hypertension. The biological diagnosis was based on the dosage of urinary metanephrines and plasma-free metanephrines in, respectively, 61.5% and 18% of cases. A computerized tomography scan and/or a magnetic resonance imaging scan could help to locate the tumor in 100% of cases. Our series includes 3 cases of bilateral pheochromocytoma, 3 cases of paragangliomas and 1 case of malignant pheochromocytoma, while a hereditary form was retained in 3 patients. In fact, thirty-two patients were operated; cure was clinically labeled in 100% and biologically in 87.5% of patients. Conclusions The main points for improvement that our study has revealed are; a patient follow-up after surgery, which was not always regular, and an insufficient screening for genetic diseases associated with pheochromocytomas and paragangliomas.

Abstract Herein we report the intriguing case of a 42‐year‐old woman presenting with grade three hypertension, severe hypokalemia and primary amenorrhea, which revealed to be the complete form of 17 alphahydroxylase deficiency. We also discuss the challenging therapeutic approach as well as the outcomes and the follow‐up of this patient.

Ectopic pheochromocytomas, also called paragangliomas, are defined as catecholamine -secreting tumors, which develop outside the adrenal medulla.Pheochromocytomas of the urinary tract represent less than 1% of all paragangliomas and are most commonly located in the bladder. Nevertheless, prostatic pheochromocytoma is an extremely rare clinical entity and only a few cases have been reported in the medical literature.Herein, we report a case of ectopic pheochromocytoma arising from the prostate, revealing with hypertensive crisis occurring immediately after ejaculation.

Introduction. Meningeal melanocytoma (MM) is a very rare neuroectodermal neoplasm arising from the leptomeninges. Primary suprasellar melanocytomas are exceedingly rare, with only a handful of cases reported. The systemic spread of a nontransformed meningeal melanocytoma is an unusual occurrence. Herein, we report the first case of a primary sellar melanocytoma with cerebral and spinal meningeal seeding. Case Report. A 30-year-old male with no previous medical history presented to the endocrinology department with a loss of body hair. The endocrine workup concluded with isolated hypogonadotropic hypogonadism. The Magnetic Resonance Imaging (MRI) of the brain and sella revealed a large suprasellar mass continuous with the infundibulum of the pituitary gland. It was heterogeneously hyperintense on T1-, T2-, and FLAIR-weighted images and was enhanced with contrast, along with cerebral and spinal leptomeningeal spread. The patient was referred to the neurosurgery department, and a lumbar spine biopsy was indicated. The histopathological examination was suggestive of a grade I meningeal pigmented melanocytoma. Conclusion. Thus, primary sellar melanocytomas with leptomeningeal spread are an extremely rare phenomenon. Metastatic malignant melanoma should be ruled out. Being aware of differential diagnosis and the unusual behavior of meningeal melanocytoma will be necessary to manage the patient appropriately. Complete tumor resection is the best treatment whenever possible, and radiotherapy should be considered in case of unresectability or partial resection.

VIPoma is an unusual neuroendocrine neoplasm that autonomously secretes VIP. It is associated with secretory diarrhea and electrolyte disturbances. Herein we report a case of a male patient, who was hospitalized in the Department of Endocrinology in Hedi Chaker Hospital, Sfax, Tunisia. He presented VIPoma syndrome, with hepatic metastases at diagnosis. He had a history of chronic, watery diarrhea. He was dehydrated with many electrolytic disorders as hypokalemia, hyponatremia and metabolic acidosis. Abdominal CT scan showed a heterogeneous mass in the pancreatic head with multiple hepatic lesions. A high VIP hormone level was found. Histological study of a liver biopsy revealed hepatic metastases of neuroendocrine carcinoma. The patient received analogues of somatostatin and systemic chemotherapy, with a transient symptomatic relief. Sadly the patient was lost to follow-up.

Insulin therapy is an essential treatment for type 1 and uncontrolled type 2 diabetes mellitus (DM). Hypersensitivity reactions have been described since the first administration of insulin, the same as any other therapy. Despite being a rare situation nowadays, it requires careful intra-hospital monitoring and multidisciplinary management. Here, we present a case of a 57-year-old patient with type 2 DM, an average glycemic control, and both penicillin and insulin allergy. Heunderwent a desensitization protocol which allowed successfully dismiss him with intermediate-acting insulin.

L’insulinome pancréatique est une tumeur neuroendocrine rare, souvent bénigne, mais qui peut mettre en jeu le pronostic vital du fait des accidents hypoglycémiques qu’elle engendre. L'insuffisance surrénalienne peut aussi être responsable d'hypoglycémie. Nous rapportons le cas d'un patient de 68 ans hospitalisé pour des hypoglycémies récurrentes, les explorations ont montré un hyperinsulinisme endogène, une insuffisance corticotrope et un hypogonadisme hypergondadotrope. Le patient était substitué par hydrocortisone et devant la non amélioration de la symptomatologie le diagnostic topographique d'insulinome était retenu 5 ans plus tard. On discute dans cet article les aspects cliniques, biologiques, radiologiques et thérapeutiques de l'insulinome ainsi que son rôle dans la genèse d'insuffisance corticotrope et d'hypogonadisme périphérique.

Les études récentes menées chez des patients atteints de maladie d'Addison (MA) ont permis de révéler que cette pathologie, même traitée, reste grevée d'une morbi-mortalité non négligeable. L'objectif de notre étude était de déterminer les effets délétères de la substitution glucocorticoïde au long cours principalement sur le plan métabolique et cardiovasculaire. Il s'agit d'une étude rétrospective qui a inclu 28 patients ayant une MA traitée, évoluant depuis plus que 15 ans. L'âge moyen était de 58,53 ans avec une prédominance féminine à 65%. La durée moyenne de suivi était de 17,87 ans. La dose d'hydrocortisone était initialement à 32,5mg/j (20,52 mg/m2) et à 27,9mg/j (16,41mg/m2) au moment de l'étude. La prévalence du syndrome métabolique (SM) au cours de la MA était de 35,71% après une durée de traitement supérieur à 15 ans. On note au terme du suivi que 28,57% des patients étaient obèses. Vingt-cinq (25)% des patients avaient développé une HTA et un diabète de type 2. La prévalence de la dyslipidémie était passé de 3,57% à 42,85%. Un seul patient avait présenté un infarctus de myocarde à 25 ans de suivi. Les facteurs favorisant la survenue du SM dans notre étude étaient l'ancienneté de la maladie et la perte du poids à la découverte de la maladie. L'ajustement du traitement substitutif au cours de la maladie d'Addison reste un enjeu au vu de la morbi-mortalité liée au surdosage. Un suivi régulier, et une approche thérapeutique personnalisée sont nécessaires pour améliorer le pronostic de ses patients.

Objective: To investigate fertility in a sample of Tunisian patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Design: Tunisian bicentric prospective study. Setting: Endocrinology department, Hedi Chaker Hospital, Sfax, Tunisia and Department of Endocrinology and Internal Medicine, Tahar Sfar Hospital, Mahdia, Tunisia. Materials and methods: Twenty-six patients (11 M; 15 F), aged 16.5–48 years, were enrolled. Clinical, biological, hormonal and ultrasound examinations were performed to assess fertility. Results: Eighteen had the classical form and eight the non classic. One patient had palpable testicular nodule. Inhibin B level was decreased in four male patients. Semen analysis showed abnormalities in four of 10 patients. Testicular adrenal rest tumors (TARTs) were detected in 6/11 patients. Menstrual disorders and hirsutism were noted in four and nine female patients, respectively. Six patients showed polycystic ovary syndrome. Anti-Mullerian hormone level was reduced in four female patients. Among four female patients who wished to get pregnant, two of them achieved one successful pregnancy, miscarriage occurred in one patient and the remaining patient was sterile. Fertility issues in our patients appeared to be related to poor hormonal control and a result of noncompliance with medication schedules. Conclusion: Fertility in male and female patients with CAH is reduced. Early and adequate glucocorticoid therapy along with good compliance, careful monitoring of androgen levels and continuous psychological management could contribute to improved fertility rates in this population, even among those with the severe variant.

Une femme enceinte âgée de 27 ans a été admise dans le service de réanimation pour une décompensation acidocétosique sévère spontanée inaugurale d'un diabète type 1. La patiente a été réanimée et insulinée avec une bonne évolution clinique et biologique. Au 4ème jour, la patiente a présentée un tableau de polyradiculonévrite aigue d'installation brutale. Les examens complémentaires faites en urgences étaient négatives. Une cytoponction lombaire a montré une dissociation albuminocytologique. L'électromyogramme a confirmé le diagnostic de syndrome de Guillain Barré (SGB). La patiente a été mise sous veinoglobuline avec rééducation physique. Une amélioration spectaculaire des signes neurologiques a été notée. Concernant sa grossesse, la patiente a avorté au bout d'une semaine de diagnostic de SGB. L'association de SGB avec une décompensation cétosique est rare. En effet, quelques cas ont été rapportés dans la littérature. Cette association au cours d'une grossesse n'est jamais décrite d'où l'originalité de notre cas.

Background: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis. The enzymes most commonly affected are 21-hydroxylase. Past reports suggested brain magnetic resonance imaging (MRI) abnormalities in CAH patients, affecting white matter signal, temporal lobe and amygdala structure and function. Aims: In the present study, we aimed to investigate the frequency of white matter changes and temporal lobes structures dysgenesis in a population of patients having CAH due to 21-hydroxylase deficiency. Materials and Methods: Neurological examination and brain MRI were performed in 26 patients. Results: Neurological examination revealed mental retardation in three patients, tremor in two patients, tendon reflexes asymmetry in one patient, and cerebellar syndrome in one patient. Eleven patients (42.3%) showed MRI abnormalities: Eight of them had white matter hyperintensities, one patient had moderate atrophy in the right temporal, and hippocampal dysgenesis was found in the remaining two patients. Conclusions: Brain MRI abnormalities in CAH patients include white matter hyperintensities and temporal lobe structures dysgenesis. The mechanisms involved seem related to hormonal imbalances during brain development and exposure to excess exogenous glucocorticoids. Clinical implications of such lesions remain unclear. More extensive studies are required to define better the relationships between brain involvement and different CAH phenotypes and treatment regimens.

Background: The prevalence of children obesity is rising alarmingly in both developed and developing countries. Developing effective exercise programs is a strategy for decreasing this prevalence and limiting obesity-associated long-term comorbidities. Objectives: To determine whether a 16-week training program; in addition to the school physical education and without dietary intervention; could have beneficial effects on body composition and aerobic capacity of obese children. Materials and Methods: Twenty-eight obese children (16 boys, 12 girls; aged 12-14 years) were enrolled and were divided into either the exercise group (EG, n = 14) or the control group (CG, n = 14). EG participated in a 16-week aerobic exercises (four 60-min sessions per week at 70-85% of HRmax (maximum heart rate)), in addition to the school physical education. Fat-Free Mass (FFM) and Fat Mass (FM) were assessed with bioelectrical impedance equipment. To assess aerobic capacity, maximal metabolic equivalent of task (METmax) and maximal workload (Wmax) were estimated with an electronically braked cycle ergometer (type Ergoline 500® ). Results: At baseline, there were no differences between the two groups. After the training program, only the EG showed significant reduction in BMI (body mass index) and waist circumference compared with the baseline values (P < 0.001). Exercise training significantly decreased FM only in the EG. A significant increase in FFM was seen in both groups; more marked in the EG. There was a significant increase in METmax (P < 0.05) and Wmax (P = 0.02) in the EG, and no significant changes in these parameters were seen in the CG. HRmax significantly decreased only in the EG (P < 0.05). Conclusion: This training program has beneficial effects on body composition and aerobic capacity parameters in obese children. Our intervention has the advantage of providing a sustainable and reproducible school and community approach for the management of children obesity.

Diabetes mellitus has a number of long-term effects on the genitourinary system. These effects predispose to bacterial urinary tract infections (UTIs) in the patient with diabetes mellitus. Complicated UTIs are also common and potentially life-threatening conditions. They include emphysematous pyelonephritis, emphysematous pyelitis/cystitis, xanthogranulomatous pyelonephritis, renal/perirenal abscess, and renal papillary necrosis. Improved outcomes of these entities may be achieved by early diagnosis, knowledge of common predisposing factors, appropriate clinical and radiological assessment, and prompt management. Herein we review complicated UTIs associated with diabetes mellitus in terms of pathogenesis, clinical manifestations, radiological features, and current management options.

We report a 22-year-old woman who presented with asthenia, weight loss and hypotension in which extensive pituitary and adrenal investigations were diagnostic of isolated adrenocorticotropic hormone deficiency (IAD) of pituitary origin. Magnetic resonance imaging of the hypothalamus and pituitary showed a normal-sized pituitary, with no mass lesion. The diagnosis of IAD probably secondary to lymphocytic hypophysitis (LYH) was made.IAD is able to be the way of presentation of LYH, although the disease could or could not turn into a panhypopituitarism. Prompt recognition of this potentially fatal condition is important because of the availability of effective treatment. Indeed, regular endocrine and imaging follow up is important for patients with IAD and normal initial pituitary imaging results to detect early new-onset pituitary hormones deficiencies or imaging abnormalities.

Fertility in women with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) appears to be reduced, especially in women with the classic salt-wasting type. Several factors have been suggested to contribute to this subfertility such as androgen excess, adrenal progesterone hypersecretion, consequences of genital reconstructive surgery, secondary polycystic ovaries syndrome, and psychosexual factors. In contrast to this subfertility, pregnancies are commonly normal and uneventful. Adequate glucocorticoid therapy and improvement of surgical and psychological management could contribute to optimize fertility in CAH female patients, even among women with the classic variant. This review provides current information regarding the reproductive outcomes of women with CAH due to 21-OHD and the fertility and pregnancy issues in this population.

Several disorders of coagulation and fibrinolysis have been widely reported in patients with hyperthyroidism. Most reports have focused on only the venous thromboembolism risk, and few of them have studied specifically the association between hyperthyroidism and pulmonary embolism (PE). We report two cases of Graves′ disease complicated by PE. The first patient is a 32 year-old man, and the second patient is a 23-year-old female. PE was suspected on the basis of pulmonary hypertension in patient one, and clinical presentation in the other patient. The first patient had also right heart failure. PE was confirmed in both patients by a lung perfusion-ventilation scan test. Thrombophilia screen revealed normal findings in the first patient and an elevation in coagulation factor VIII in the second one. Both patients received heparin, followed by oral anticoagulant therapy. In addition, they were treated with radioactive iodine resulting in partial recovery from hyperthyroidismforpatient oneand clinical euthyroidism for patient two.The former died of acute heart failure secondary to a chest infection, while the later was lost to follow-up. In conclusion, hyperthyroidism is associated with increased risk of venous thromboembolism, including PE. Potential mechanisms involved in this association include endothelial dysfunction, decreased fibrinolytic activity, and increased coagulation factors levels. Thyroid evaluation is recommended in patients with unprovoked venous thromboembolic events. Conversely, the diagnosis of venous thromboembolism should be considered in patients with hyperthyroidism, particularly if additional prothrombotic risk factors are present.

Background: In congenital adrenal hyperplasia (CAH), long-term glucocorticoid treatment coupled with increased androgens may lead to undesirable metabolic effects. The aim of our report was to determine the prevalence of metabolic abnormalities and cardiovascular risk factors in a population of adult patients with CAH due to 21 hydroxylase deficiency. Materials and Methods: Twenty-six patients (11 males and 15 females, mean age ± SD=27.4±8.2 years) were recruited. Anthropometry, body composition, metabolic parameters and cardiovascular risk factors were studied. Results: Obesity (overweight included) was noted in 16 patients (61.5%), with android distribution in all cases. Bioelectrical impedance showed increased body fat mass in 12 patients (46.1%). Lipid profile alterations and carbohydrate metabolism disorders were detected in seven (26.9%) and five (19.2%) patients respectively. Moderate hepatic cytolysis, associated with hepatic steatosis, was found in one patient. Seven patients (27%) had insulin resistance. Ambulatory blood pressure monitoring showed abnormalities in six patients (23%). Increased carotid intima media thickness was found in 14 patients (53.8%). Conclusion: Adult CAH patients tend to have altered metabolic parameters and a higher prevalence of cardiovascular risk factors. Lifelong follow-up, lifestyle modifications, and attempts to adjust and reduce the glucocorticoid doses seem important.

INTRODUCTION: le but de notre étude était d'évaluer la prévalence du surpoids et de l'obésité chez des adolescents scolarisés dans la ville de Sfax mais aussi, d'étudier son association avec le temps de sédentarité et l'activité physique (AP). METHODES: la population étudiée était composée de 1695 adolescents âgés de 15-18 ans. Tous les participants avait rempli un questionnaire porté sur leurs activités physiques et temps de sédentarité, donné lors d'un entretien direct. Le niveau d'AP était évalué avec l'International Physical Activity Questionnaire (IPAQ) version courte. RESULTATS: notre étude comportait 43,7% de garçons et 56,3% de filles. L'âge moyen était de 16,78 , 1, 1 an. Dans notre échantillon, 23,4% des adolescents étaient en surpoids ou obèses. Le score de l'IPAQ nous a montré que le niveau d'AP de nos participants était faible dans 6,4%, modéré dans 65,4% et élevé dans 28,2% des cas. Nos résultats avaient démontré que l'augmentation du temps de sédentarité (plus de 2 h / jour) est associée à une augmentation significative de l'indice de masse corporelle (IMC) et du tour de taille (TT) (P =0,001). Alors qu'un niveau d'AP élevé et/ou la participation aux séances d'AP structurées dans le cadre scolaire et hors scolaire est accompagnée par une diminution significative de l'IMC et du TT (P =0,001). CONCLUSION: nos résultats apportent une preuve supplémentaire sur la nécessité de promouvoir la vie active chez les jeunes Tunisiens.

Le syndrome de Fahr (SF) est caractérisé par la présence de calcifications intracérébrales, bilatérales et symétriques, des noyaux gris centraux. Il est souvent associé à des troubles du métabolisme phosphocalcique, et principalement à une hypoparathyroïdie. Il constitue une complication rare de la radiothérapie probablement lié à une vascularite nécrotique. Nous rapportons l'observation d'un homme âgé de 27 ans qui était hospitalisé pour syndrome dysmorphique acrofacial. Le diagnostic d'une acromégalie secondaire à un macroadénome hypophysaire a été retenu. Une première exérèse fut réalisée, suivie de d'une réintervention pour récidive et d'une radiothérapie complémentaire de 56 grays répartis sur 12 séances. L'évolution était marquée par la survenue d'une insuffisance antéhypophysaire. Cinq ans plus tard, le patient présentait des céphalées frontales. Un scanner cérébral objectivait un aspect stable de la tumeur, par ailleurs, elle révélait une calcification quasitotale des noyaux gris centraux, du tronc cérébral et du cervelet, de la substance blanche et de la jonction cortico-souscorticale absentes sur les examens neuroradiologiques précédents. Le diagnostic d'un syndrome de Fahr a été porté. Le bilan biologique ne révélait pas de troubles du métabolisme calcique, ni d'hypoparathyroidie. L'hypothroidie et l'hypogonadisme ont été incriminés mais le caractère étendu des macrocalcifications n'était pas en faveur. Ainsi, l'origine post radique était retenue. Une surveillance clinique rapprochée était préconisée afin de déceler d'éventuelles complications neurologiques.

INTRODUCTION: L'objectif de ce travail etait d'etudier la prévalence du surpoids et de l'obésité chez un groupe d'enfants d'âge scolaire, habitant la ville de Sfax en Tunisie, et identifier les facteurs favorisant la prise pondérale. METHODES: enquête descriptive transversale était réalisée en 2011 sur un échantillon représentatif d'élèves recrutés dans 11 écoles primaires publiques. Des informations concernant les caractéristiques sociodémographiques, les habitudes alimentaires et le comportement sédentaire pour chaque élève ont été précisées au moyen d'un questionnaire. RESULTATS: Nous avons colligé 1529 élèves, âgés entre 9 et 12 ans et se répartissant en 787 garçons (51,14%) et 747 filles (48,86%). Selon les seuils de référence de l'IOTF, la fréquence de l'obésité était de 2,4% et celle du surpoids était de 6,3%. L'obésité était significativement associée à l'obésité parentale, un niveau socioéconomique élevé, la prise de plus de deux goûters par jour et à l'activité sédentaire. CONCLUSION: L'identification des facteurs de risque du surpoids et de l'obésité infantile permettrait de dépister les enfants à risques afin de leur proposer des mesures de prévention adaptées. Ces mesures de prévention devraient inclure non seulement des approches individuelles, mais aussi l'environnement social et physique de l'enfant.

Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (LCTs) are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.

Gardner's syndrome (GS) is a dysplasia characterized by neoformations of the intestine, soft tissue, and osseous tissue. Endocrine neoplasms have occasionally been reported in association with GS. Adrenal masses in GS are rare, and few have displayed clinical manifestations. In the current paper, The authors report a 37-year-old male patient with GS including familial adenomatous polyposis (FAP) and mandible osteoma who presented with an incidental adrenal mass. Computerized tomography adrenal scan identified bilateral masses. Functional analyses showed a hormonal secretion pattern consistent with pre-Cushing's syndrome. Other extraintestinal manifestations were hypertrophy of the pigmented layer of the retina and histiocytofibroma in the right leg. This paper describes a rare association of adrenocortical secreting mass in an old male patient with Gardner syndrome.

Abstract Introduction Sheehan's syndrome is defined by varying degrees of anterior pituitary deficiency due to postpartum ischemic necrosis of the pituitary gland after massive bleeding. It is a rare disorder in western countries and even in Tunisia. Hematologic abnormalities such as normochromic anemia have been reported in these patients. However, pancytopenia is rarely observed. Case presentation We describe the case of a 48-year-old Tunisian woman with features of hypopituitarism. Laboratory tests showed pancytopenia that was completely reversed after adequate hormone replacement. Conclusion Clinicians should consider the possibility of hypopituitarism as a cause of pancytopenia. This is an original case report that is of interest to hematologists, who should be aware of Sheehan's syndrome as a treatable etiology of pancytopenia for women.