1/95. Adrenal and hypophyseal non-Hodgkin's lymphoma presenting with panhypopituitarism.We report a case of non-Hodgkin's lymphoma presenting as anterior pituitary failure and found to have involvement of two endocrine glands, the pituitary and the adrenals.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/95. Panhypopituitarism associated with diabetes insipidus in a girl with a suprasellar arachnoid cyst.We report on a female patient with a large suprasellar arachnoid cyst (3.5 x 2.5 cm) combined with right optic nerve hypoplasia. She developed growth hormone deficiency and hypothyroidism at the age of 8.5 years, adrenal insufficiency at the age of 11 years, diabetes insipidus and hypogonadotropic hypogonadism at the age of 15 years. When last seen at the age of 19 years she was extremely obese ( 5.9 BMI SDS). The endocrine picture suggests that arachnoid cysts might be involved in far more complex hypothalamic-pituitary disturbances than previously thought.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
3/95. myxedema coma of both primary and secondary origin, with non-classic presentation and extremely elevated creatine kinase.myxedema coma is a rare, often fatal endocrine emergency that concerns elderly patients with long-standing primary hypothyroidism; myxedema coma of central origin is exceedingly rare. Here, we report a 37-year-old woman in whom classical symptoms of hypothyroidism had been absent. Six years earlier, she had severe obstetric hemorrhage and, shortly after, two subsequent episodes of pericardial effusion. On the day of admission, pericardiocentesis was performed for the third episode of pericardial effusion. Because of the subsequent grave arrhythmias and unconsciousness, she was transferred to our ICU. Prior to the endocrine consultation, a silent myocardial infarction had been suspected, based on the extremely high serum levels of creatine kinase (CK) and isoenzyme CK-MB. However, based on thyroid sonography, pituitary computed tomography, elevated titers of antithyroid antibodies and pituitary stimulation tests, the final diagnosis was myxedema coma of dual origin: an atrophic variant of Hashimoto's thyroiditis and post-necrotic pituitary atrophy (Sheehan syndrome). Substitutive therapy caused a prompt clinical amelioration and normalization of CK levels. Our patient is the first case of myxedema coma of double etiology, and illustrates how its presentation deviates markedly from the one endocrinologists and physicians at ICU are prepared to encounter. In addition, cardiac problems as those of our patient should not discourage from substitutive treatment (using L-thyroxine and the gastrointestinal route of absorption), if the age is relatively low.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
4/95. Hyperinsulinemic hypoglycemia associated with possible hypopituitarism in a patient with acquired immunodeficiency syndrome.OBJECTIVE: To describe endocrine dysfunctions associated with human immunodeficiency virus (hiv) infection. methods: We present a case report, discuss the laboratory findings, and suggest potential contributing factors in this complex milieu. RESULTS: A 44-year-old man infected with hiv was admitted with loss of consciousness after a grand mal seizure and was found to have a prolonged course of hypoglycemia in conjunction with increased serum levels of insulin and C peptide. Iatrogenic hypoglycemia was excluded by laboratory evaluations. Hypothalamic-pituitary dysfunction was suspected because the serum growth hormone and cortisol concentrations during hypoglycemia were low. Radiologic evaluation of the brain revealed no structural abnormalities involving the hypothalamic-pituitary area. A normal cortisol response to adrenocorticotropic hormone stimulation (cosyntropin, 250 mg) was documented on two occasions. The cause of the severe hypoglycemic episode and the possible hypothalamic-pituitary-adrenal axis dysfunction in this patient is unknown. CONCLUSION: Despite the complexity of endocrine disorders in hiv-infected patients, the course of the disease in this patient underscores the possibility that some of the endocrinologic changes may be reversible.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
5/95. Panhypopituitarism associated with severe retroperitoneal fibrosis.A 43-year-old man, with a history of central diabetes insipidus diagnosed 3 years previously, complained about reduced libido. An MRI scan showed a suprasellar lesion just below the supraoptic recess of the third ventricle. A stereotactically guided biopsy revealed fibrous glia, but no other specific tissue and no inflammatory cells. Two months later the patient presented with fatigue and muscular weakness. Tertiary adrenal failure and hypothyroidism were diagnosed by endocrine function tests and therapy with levothyroxine and hydrocortisone was started. Another 2 months later the patient was admitted with giddiness, nausea, peripheral oedema and oliguria. Radiological imaging and an open transperitoneal kidney exploration showed severe fibrosis around both ureters. Histological examination confirmed the diagnosis of idiopathic retroperitoneal fibrosis. Presumably the suprasellar tumour was the first manifestation of retroperitoneal fibrosis. Once the diagnosis 'idiopathic retroperitoneal fibrosis' is confirmed, fibrotic manifestations and complications involving extra-retroperitoneal tissues including the endocrine system, should be sought.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
6/95. Autoimmune hypopituitarism in patients with coeliac disease: symptoms confusingly similar.Coeliac disease does not always respond properly to a gluten-free diet, and treatment may be complicated by an underlying autoimmune endocrine disorder. We report three cases of hypopituitarism in patients with coeliac disease who seemed to have incomplete dietary response. The first patient had diabetes and suffered from hypoglygaemic events; the second had muscular atrophy of unknown origin while the third had growth failure. None had a pituitary mass, suggesting that hypopituitarism was of autoimmune origin. overall condition improved only after replacement therapy for the underlying hormone deficiency; this association should thus be recognized.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
7/95. arthrogryposis multiplex congenita and pituitary ectopia. A case report.We report a patient with a form of arthrogryposis multiplex congenita who developed seizures at 4 months of age that proved to be hypoglycemic. Further evaluation of the etiology of hypoglycemia led to the discovery of partial anterior hypopituitarism, with normal posterior pituitary function. neuroimaging revealed an ectopic neurohypophysis with very small anterior pituitary, the presumed anatomic basis for his endocrine dysfunction. A chance association between the pituitary ectopia and the arthrogryposis cannot be ruled out. However, it is more likely that in the present patient a common genetic mechanism may be the basis for both the arthrogryposis and the pituitary dysfunction.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
8/95. hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.central nervous system (CNS) involvement and, in particular, hypothalamic-pituitary involvement are well described features of Langerhans cell histiocytosis (LCH). The actual incidence of CNS-LCH disease is unknown and the natural history is poorly understood. diabetes insipidus (DI) is reported to be the most common and well described manifestation of hypothalamic-pituitary involvement (up to 50%). Anterior pituitary dysfunction has been reported in up to 20% of patients with LCH, and occurs almost exclusively concurrently with DI. In the current paper we describe our experience with 7 patients (6 females and 1 male) in whom hypothalamicpituitary involvement was a major feature of LCH. diagnosis was made in 4 patients during childhood or adolescence, and 3 patients were over 18 years old at the time of diagnosis. Our series exemplifies the wide spectrum of LCH-induced hypopituitarism, and demonstrates some unique features, including a higher incidence of CRH/ACTH deficiency compared to other reports (4/7 patients), and massive obesity in 2 of our patients. Endocrine function was not improved in any of our patients following medical treatment of LCH with chemotherapy and glucocorticoids. We conclude that pituitary-hypothalamic dysfunction is a common feature of LCH, and therefore all LCH patients should undergo a thorough endocrine evaluation periodically.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
9/95. Two tumors detected by thyroid assessment in two children.OBJECTIVE: To describe the early detection of two tumors in two children by recognition of unusual features in initial thyroid assessments. methods: We present the clinical findings and results of laboratory studies in two children. In addition, we describe RET proto-oncogene studies in one of them. RESULTS: A 14.5-year-old boy was referred for assessment because of short stature in conjunction with lack of physical growth and development. His physical examination was remarkable for height at the 50th percentile (height age, 11.5 years), weight at the 50th percentile (weight age, 13 years), and prepubertal male status. Pertinent laboratory findings were a normal thyroid-stimulating hormone (TSH) level but low free thyroxine (FT4) index. These findings prompted an immediate magnetic resonance imaging study of the head. A parasellar tumor was detected and removed; histopathologic examination revealed that it was a craniopharyngioma. The patient requires lifelong multihormonal therapy for his panhypopituitarism and has responded with physical growth. Our second patient, a 7.5-year-old girl, was referred because of a painless left thyroid nodule of 4 months' duration. Her physical examination was remarkable for (1) upper lip swelling, (2) intermittent rash, and (3) a goiter with painless mobile left and right nodules. Normal levels of TSH and FT4, serum calcitonin of 6,192 pg/mL, and a fine-needle biopsy specimen that stained strongly for calcitonin were obtained at her first clinic visit. A total thyroidectomy was performed and confirmed the presence of medullary thyroid carcinoma. Genetic studies showed that she was positive for the RET multiple endocrine neoplasia IIB mutation. After 4 years of follow-up, the patient had serum calcitonin levels that remained low (<2.2 pg/mL). CONCLUSION: attention to thyroid physical findings and laboratory studies can promptly lead to correct diagnoses and management of some rare and life-threatening tumors in children.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
10/95. Symptomatic Rathke's cleft cyst with amyloid stroma.A patient with panhypopituitarism and visual field defects due to a Rathke's cleft cyst is presented. These cysts are commonly found in random pituitaries examined at necropsy, but rarely produce symptoms. Subtle endocrine deficiencies, however, may now be uncovered more frequently with modern diagnostic techniques. An unusual and unreported feature of this cyst was an apudamyloid stroma. This would imply that cells derived from the neural crest participate in the formation of the cyst. Recognition of these cysts at the time of operation is important in avoiding confusion with pituitary adenomas and unnecessarily aggressive treatment.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
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