This program was beneficial for 75% of patients who demonstrated an improvement in their metabolic control the year following transition to adult care service. To our knowledge, this study is the first one in North Africa to report on the outcome of a structured transition program from pediatric to adult diabetes care.
Erdheim-Chester disease (ECD) is a very rare and aggressive form of non-Langerhans histiocytosis with unclear pathogenesis. Because of the heterogeneity of clinical presentation, diagnosis is often challenging and delayed. Currently, Interferon alpha is the first line treatment that is associated with a better survival. The prognosis is relatively poor, especially in case of neurological and cardiovascular involvement. Herein, we report the case of a 64-year-old Tunisian female patient presenting an aggressive form of ECD revealed by diabetes insipidus and cerebellar ataxia with a diagnosis delay of 4 years. The assessment of disease extent had also shown associated asymptomatic cardiac and bone involvement. Pegylated Interferon alpha was started at high dose allowing disease stabilization. This case illustrates that physicians should be aware of the heterogeneous manifestations of ECD in order to insure an early diagnosis and treatment. Long-term and regular follow-up is crucial because of the risk of disease progression.
Pituitary apoplexy is a rare clinical emergency due to acute ischemic infarction or hemorrhage of the pituitary gland. As this disorder most often involves a pituitary adenoma, especially nonfunctioning tumors, the syndrome should be referred to as pituitary tumor apoplexy. The precise physiopathology is not completely clear. Although in most cases it occurs spontaneously, pituitary apoplexy can be precipitated by many risk factors. The main symptom is headache of sudden onset associated with visual disturbances, signs of meningeal irritation, and/or endocrine dysfunction. Corticotropic deficiency is a potentially life-threatening disorder. Magnetic resonance imaging is the most sensitive to confirm the diagnosis by revealing a pituitary tumor with hemorrhagic and/or necrotic components. Earlier studies used to consider urgent decompression of the lesion surgically, but nowadays, more recent studies favor conservative management in selected patients (those without important visual acuity or field defects and with normal consciousness). This wait-and-see approach gives evidence of excellent outcomes in terms of oculomotor palsy, pituitary function, and subsequent tumor growth. Surgical decompression may be necessary in some cases. Once the acute phase is over, the patient should be reevaluated for hormonal deficiencies. Moreover, spontaneous remission of syndromes, such as acromegaly, may be caused by pituitary adenoma apoplexy.
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