1/5. 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 = 1keywords = physician (Clic here for more details about this article) |
2/5. Congenital hypopituitarism as a cause of undetectable estriol levels in the maternal triple-marker screen.We are reporting a child with congenital panhypopituitarism, in whom deficient fetal steroidogenesis was suspected prenatally because of undetectable estriol levels measured in the maternal triple-marker screen. No fetal abnormalities were detected by ultrasonography. amniocentesis demonstrated a normal 46,XX karyotype. Measurement of maternal urinary steroids failed to show elevation in the excretion of the major precursor for estriol, 16 alpha-hydroxydehydroepiandrosterone, indicating that the fetus did not have steroid sulfatase deficiency (placental sulfatase deficiency), the most common genetic cause of extremely low estriol. The steroid analysis excluded other rare single gene defects, including aromatase deficiency and 17 alpha-hydroxylase deficiency. We therefore suspected that the cause of low estriol in this fetus was adrenal insufficiency. Postnatal evaluation was consistent with panhypopituitarism, characterized by deficiency of all anterior pituitary hormones. Because this screen is now offered to more than half the pregnant women in the united states, reports of low estriol levels have become increasingly common. Therefore, it is essential that physicians be familiar with the various etiologies, perform the appropriate antenatal evaluation to determine the specific cause, and closely monitor both mother and child ante- and postnatally.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/5. Bacterial pituitary abscess: an unusual cause of panhypopituitarism.OBJECTIVE: To describe a case of primary bacterial pituitary abscess manifesting as hypopituitarism. methods: We present the case history, hormonal and bacteriologic data, and findings on imaging studies in a 34-year-old man. RESULTS: The patient had an 8-month history of intermittent fever, headache, nausea, vomiting, and weight loss. Because a computed tomographic scan of the head showed a cystic sellar mass with ring enhancement, he was referred to our medical center. On physical examination, he showed signs of meningeal irritation and had mild hypotension. Hormonal evaluation revealed evidence of hypocortisolism, hypothyroidism, and hypogonadism. Three weeks after treatment with antibiotics and hormonal replacement, he underwent transsphenoidal surgical exploration and evacuation of purulent material from the sella. On culture, this specimen grew coagulase-negative staphylococci and propionibacterium granulosum. Nine months later, dynamic testing showed persistent central hypocortisolism, hypothyroidism, and hypogonadism. CONCLUSION: Bacterial pituitary abscess is rare but manifests similar to other pituitary masses with headaches, visual field defects, and hormonal disturbances. For the correct preoperative diagnosis of this condition, the physician must have a high index of suspicion, and the characteristic ring enhancement must be present on imaging studies.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/5. empty sella syndrome, panhypopituitarism, and diabetes insipidus.We present an 18-month-old girl with short stature, obesity, panhypopituitarism, diabetes insipidus, and visual defects. Postmortem examination revealed brain atrophy due to a diffuse encephalopathy, numerous calcified neurons in cerebral cortex, deep telencephalic and diencephalic nuclei, diffuse neuronal necrosis in hypothalamic nuclei, moderate atrophy of optic nerves, very thin hypophyseal stalk, and empty sella with the hypophysis compressed to the dorsal aspect of the concavity. Our hypothesis is that the presence of an empty sella in a child with hypophyseal-hypothalamic abnormalities should alert physicians to the existence of hypothalamic lesions secondary to a perinatal insult. We discuss the possible pathogenesis of these findings as well as lines of evidence available in the literature.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/5. Hypothalamic defects in two adult patients with septo-optic dysplasia.Two adult patients with unilateral hypoplastic optic nerves, absent septa pellucida and hypopituitarism are described. Patient 1, aged 20, presented with diabetes insipidus due to partial vasopressin deficiency. patients 2, aged 29, presented with focal epilepsy. Both had short stature. They showed absent growth hormone (GH) response to insulin-hypoglycaemia or glucagon, but responded to 100 micrograms growth hormone releasing factor (GRF-44) with a rise in circulating GH, suggesting a hypothalamic defect in GH release though a co-existing pituitary defect cannot be excluded. Other hypothalamic-pituitary functions were normal. These two patients probably represent the milder form of the clinical spectrum of septo-optic dysplasia which, with the extensive use of CT brain scans, will be increasingly encountered by physicians attending adult patients.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |