1/37. Rathke's cleft cyst as a cause of growth hormone deficiency and micropenis.Rathke's cleft cyst has rarely been reported in pediatric patients, and such cysts are usually found by chance, in 2-33% of routine necropsies, as they have not interfered with pituitary function. In general, they are intrasellar with a single layer of ciliated cuboidal or columnar epithelium containing mucoid material. The age range in which symptomatic Rathke's cleft cysts occur is between 30 and 60 years. This paper reports an 8.1-year-old boy presenting with growth hormone deficiency and micropenis attributable to hypogonadotropic hypogonadism (HH), implying altered pituitary function since intrauterine life. At this age (before puberty) the diagnosis of HH can be made by means of the LHRH agonist stimulation test, since conventional LHRH is not able to discriminate HH from a normal prepubertal child. To our knowledge, this is the first case of micropenis caused by Rathke's cleft cyst interfering with gonadotropin and growth hormone secretion since intrauterine life.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/37. Lymphocytic hypophysitis associated with dacryoadenitis: an autoimmunologically mediated syndrome.We report a rare case of lymphocytic hypophysitis followed by dacryoadenitis. Lymphocytic hypophysitis is a rare disease that can easily be mistaken for neoplastic proliferation. Because combination with rheumatoid arthritis, thyroiditis, or pernicious anemia is frequent, an immunological pathogenesis is likely.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
3/37. endocrinology teaching rounds: primary amenorrhea in a 17-year-old woman.This endocrinology teaching rounds documents the evaluation of a 17.7-year-old woman with primary amenorrhea. The normal timing of developmental milestones for young women from adrenarche and pubarche through pubertal maturation and growth is reviewed, as are etiologies for amenorrhea. The differential diagnosis of the problem is developed from the patient's initial visit to diagnosis and treatment. The highlights of the management of this young woman after a diagnosis of craniopharyngioma are also discussed. This clinical case emphasizes the importance of understanding normal developmental landmarks and of detecting aberrant physiology-associated amenorrhea even when screening laboratory tests appear normal so that defects in normal development can be addressed earlier in life.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
4/37. hypopituitarism following closed head injury.We describe four young patients (age 19-34 years) with hypopituitarism following closed head injury. The diagnosis was made by demonstration of low basal pituitary hormone levels and dynamic tests showing low pituitary reserve. The time interval between the injury and diagnosis of hypopituitarism was between three weeks and two months demonstrating the difficulty and complexity of making this diagnosis. Three of our patients (all patients suffering from anterior pituitary hormone deficiency) had ACTH deficiency, a condition which may be life threatening if left undiagnosed; these patients also demonstrated central hypothyroidism. Hypogonadotrophic hypogonadism occurred in three of the patients and was treated with hormonal replacement. diabetes insipidus was the only insult in one of our patients, accompanied other hormonal deficits in two, and did not appear at all in another patient. Information about skull damage was available for three of the patients, and included skull base and facial bone fractures, probably reflecting the extent of injury necessary to cause hypopituitarism. All patients regained normal lives with adequate hormonal replacement therapy.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
5/37. A case of torsade de pointes associated with hypopituitarism due to hemorrhagic fever with renal syndrome.We describe a 51-yr-old man presenting with syncope due to torsade de pointes. The torsade de pointes was refractory to conventional medical therapy, including infusion of isoproterenol, MgSO4, potassium, lidocaine, and amiodarone. His past history, physical findings, and hormone study confirmed that QT prolongation was caused by anterior hypopituitarism that developed as a sequela of hemorrhagic fever with renal syndrome. The long QT interval with deep inverted T wave was completely normalized 4 weeks after starting steroid and thyroid hormone replacement. Hormonal disorders should be considered as a cause of torsade de pointes, because this life-threatening arrhythmia can be treated by replacing the missing hormone.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
6/37. hypopituitarism after surgical clipping of a ruptured cerebral aneurysm.OBJECTIVE: The causes of hypopituitarism in adult life are most frequently cerebral tumors, pituitary infarction, head trauma, pituitary surgery, or irradiation. We report a case of hypopituitarism after surgical clipping of a ruptured cerebral aneurysm. Two previous cases after the rupture of a cerebral aneurysm have been reported. DESIGN: Case report. patients: One 42-yr-old man. MEASUREMENTS AND MAIN RESULTS: A 42-yr-old man was admitted as an emergency for unconsciousness. The computed tomography showed a massive subarachnoid hemorrhage, and specific angiography showed an aneurysm in the internal carotid. The aneurysm was successfully clipped through craniotomy. The patient's hospital course was marked by a few episodes of pulmonary infection, and a tracheotomy was performed. The patient was transferred to the rehabilitation unit; he received a rating of 9 on the glasgow coma scale. Seven months after rupture of the aneurysm, the patient was readmitted to the intensive care unit for septic shock, with pulmonary infection associated with vomiting and diarrhea. Despite standard therapy and inotropic support, there was no improvement of his clinical condition. Adrenal failure was then suspected. Treatment was started immediately with hydrocortisone (50 mg) four times a day. Within hours, his clinical condition improved. The following month, the patient was weaned off his tracheotomy and had nearly recovered. Endocrine tests confirmed the cortisol insufficiency but also hypothyroidism and hypogonadotropic hypogonadism secondary to hypopituitarism. CONCLUSION: Our case is the first one reported of hypopituitarism after surgical clipping of a ruptured cerebral aneurysm.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
7/37. 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 = life (Clic here for more details about this article) |
8/37. Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies.The association of hypoglycemia and microphallus in the male neonate is presumptive evidence of congenital hypopituitarism. This was observed in four male infants with normal birth weight and length, optic discs, and intelligence, and without gross central nervous system malformations. plasma and urinary cortisol values were low. Stimulation with metyrapone and insulin hypoglycemia failed to elicit a rise in plasma corticoids, but multiple doses of ACTH evoked a response. growth hormone responses to arginine, insulin, sleep, L-dopa, and glucagon were uniformly less than 2.5 ng/ml. In three patients, however, length remained within 2 SD of the mean until two years of age; in one, there was a sharp decrease in growth by three months. Two patients had low plasma TSH and thyroxine concentrations within the first month of life. In the other two patients, whose thyroxine levels were measurable, intravenous administration of thyrotropin-releasing factor evoked a normal rise in plasma TSH; serum thyroxine decreased into the hypothyroid range in one after GH therapy was initiated. plasma prolactin was normal in the first two patients receiving thyroxine replacement therapy. The other two patients had elevated baseline prolactin levels and had an augmented rise in plasma prolactin after administration of TRF. Human chorionic gonadotropin induced a 10- to 15-fold rise in plasma testosterone in the two patients tested. The changes in plasma FSH and LH after luteinizing hormone-releasing factor were either low or in the prepubertal range. In three patients, treated with testosterone enanthate intramuscularly, phallic growth occurred. In addition, all three had a transient increase in height but no acceleration of skeletal maturation. The data suggest a deficiency of hypothalamic hypophysiotropic hormones rather than a primary pituitary defect. Early recognition of this syndrome complex is critical for prompt treatment of the life-threatening cortisol deficiency. The diagnosis is more difficult in affected females because their external genitals are normal. The microphallus is a remediable manifestation of hypopituitarism.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/37. A relapsed non-Hodgkin lymphoma presenting as panhypopituitarism successfully treated by chemotherapy.We report a case of relapsed large B-cell non-Hodgkin lymphoma (NHL) affecting the anterior pituitary. The NHL relapsed after three years in complete remission. The patient was a 72-year-old woman who presented fever, weakness, hyponatremia, and hypotension. The levels of thyroid-stimulating hormone and gonadotropins were very low and magnetic resonance imaging showed infiltration of the pituitary gland and stalk. After controlling the hormonal deficiencies with substitution using hydroxycortisone and levothyroxin, the patient was treated with combination chemotherapy using cyclophosphamide, vincristine, mitoxantrone, etoposide, and bleomycin (VNCOP-B regimen), achieving a complete regression of the pituitary mass and partial recovery of the endocrine function. lymphoproliferative disorders affecting the anterior pituitary are exceedingly rare, with only six cases in immunocompetent adults reported in the literature. To our knowledge this is the first report of a relapsed NHL presenting by hypopituitarism.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
10/37. Panhypopituitarism in a child with common variable immunodeficiency.BACKGROUND: common variable immunodeficiency (CVID) represents a group of heterogeneous, still undifferentiated, syndromes that are all characterized by defective antibody formation. It is often associated with autoimmune disease. methods: An African-American girl was diagnosed with CVID at age 3 years. She was seen during an adrenal crisis precipitated by pneumonia at the age of 8 years and 10 months. The diagnosis of panhypopituitarism was established soon after. RESULTS: Panhypopituitarism in this patient was believed to be the result of the autoimmune process known as lymphocytic hypophysitis. This hypothesis was suggested by the results of magnetic resonance imaging. CONCLUSIONS: awareness of the possibility of this process in children or adults with CVID may lead to earlier diagnosis of panhypopituitarism. These patients also have failure to thrive, and earlier diagnosis may avoid a life-threatening event.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
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