1/8. Thickened pituitary stalk with central diabetes insipidus: report of three cases.diabetes insipidus of central origin usually results from lesions in the hypothalamic neurohypophyseal system. Lymphocytic infundibuloneurohypophysitis is an uncommon cause. Cases of lymphocytic infundibuloneurohypophysitis with thickening of the pituitary stalk and enlargement of the neurohypophysis with no hyperintense signal in the posterior pituitary have been reported. Reported cases presenting with isolated thickening of the pituitary stalk are very rare. We report three such cases, one in a nulliparous woman and the other two in men. Magnetic resonance (MR) imaging in these patients revealed isolated thickening of the pituitary stalk, loss of the hyperintense signal of the posterior pituitary, and an adenohypophysis of normal size. All cases had abnormal nodular infundibular enlargement. One male patient had hypogonadism; the other patients showed no sign of adenohypophyseal deficiency on stimulation test. Serial follow-up MR imaging revealed that all three patients had persistent thickening of the pituitary stalk. diabetes insipidus was controlled by the administration of desmopressin acetate in all patients.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/8. Transient lymphocytic panhypophysitis associated with SIADH leading to diabetes insipidus after glucocorticoid replacement.A 52-year-old man presented with vomiting, general fatigue and hyponatremia. His symptoms and signs were consistent with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Endocrine studies revealed hypopituitarism and administration of hydrocortisone resulted in a marked polyuria. The patient was diagnosed as masked diabetes insipidus. The lymphocytic hypophysitis was also diagnosed on the basis of MRI findings and anti-pituitary antibody. Six months later, these abnormalities disappeared. diabetes insipidus may exist in a case of hyponatremia due to contrastive SIADH. Such patients may recover spontaneously and careful follow-up is required, avoiding a long-term treatment by monotonous continuation of hormonal replacement.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
3/8. A rare case of central post-gravid diabetes insipidus.Central diabetes insipidus (CDI) arising in the puerperal period has been attributed mainly to Sheehan's syndrome or to lymphocytic infundibulo-neurohypophysitis. We report the case of a 24-year-old woman who came to our observation for the appearance, 3 weeks after a normal delivery, of a polyuric-polydipsic syndrome. Measurements of urinary volumes, plasma osmolality and urinary osmolality, in conditions of free water intake, water deprivation and a water deprivation-vasopressin administration test, demonstrated CDI. brain magnetic resonance imaging showed a normal morphology of the adenohypophysis and total absence of the neurohypophysis. Assays of the pituitary hormones were found to be within normal limits. These results, incompatible with a diagnosis of Sheehan's syndrome and lymphocytic infundibulo-neurohypophysis, excluded all the other known causes of acquired CDI. Our diagnosis was therefore of post-gravid idiopathic CDI. Thus, it is possible that in the puerperal period other diseases of the posterior hypophysis may develop, of unknown etiopathogenesis but equally responsible for CDI.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
4/8. A novel mutation in the preprovasopressin gene identified in a kindred with autosomal dominant neurohypophyseal diabetes insipidus.Autosomal dominant neurohypophyseal diabetes insipidus (ADNDI) is a defect in free water conservation caused by mutations in the single gene that encodes both vasopressin (VP) and its binding protein, neurophysin II (NP II). Most of the human mutations in this gene have been in the portion encoding the NP molecule; the resultant abnormal gene products are believed to cause cellular toxicity as improperly folded precursor molecules accumulate in the endoplasmic reticulum. We identified a new American kindred with ADNDI and found a novel mutation in the VP molecule. A 78-yr-old man was noted to have hypotonic polyuria and plasma hyperosmolarity; the urinary concentration defect was reversed by administration of VP. His symptomatology dated to childhood, and his family history was consistent with autosomal transmission of the polyuric syndrome, with affected members in three generations, including several females. Affected individuals were found to be heterozygous for a 3-bp deletion in exon 1 of arginine VP (AVP)-NP II, predicting a deletion of phenylalanine 3 (known to be critical for receptor binding) in the VP nonapeptide. Neuro 2A cells stably transfected with the mutant AVP-NP construct showed increased rates of apoptosis as assessed by flow cytometric methods. These observations support the concept that cellular toxicity of abnormal AVP-NP gene products underlies the development of ADNDI, and the data further demonstrate that mutations affecting the AVP moiety can result in initiation of these pathological processes.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
5/8. Novel mutant vasopressin-neurophysin II gene associated with familial neurohypophyseal diabetes insipidus.We describe a novel missense mutant of arginine vasopressin (AVP)-dependent neurohypophyseal diabetes insipidus in an autosomal dominant family. A 54-year-old woman was admitted to our hospital because of thyroidectomy for thyroid cancer. After thyroidectomy she was found to have hypernatremia and polyuria and polydipsia both of which had been present from childhood. She had no obstructive hydronephrosis. Her father, father's younger sister and her third son also had polyuria and polydipsia. Basal plasma AVP concentration at normal plasma osmolality was normal but did not respond to increased plasma osmolality despite hyperosmolality during infusion of hypertonic saline infusion, indicating that plasma AVP secretion was impaired. sodium concentration in urine and urine osmolality were low and increased after nasal administration of DDAVP. There was a diminished but bright signal of pituitary posterior gland on magnetic resonance T1 weighted image. Molecular genetic analysis demonstrated that the patient and her son had a single heterozygous missense mutation (G-->A) at nucleotide 1829 in 1 AVP allele, yielding an abnormal AVP precursor with lacking Glu-47 in its neurophysin II moiety. The abnormal AVP precursor may be related to the impaired AVP secretion.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
6/8. Pituitary infarction resulting from intranasal cocaine abuse.OBJECTIVE: To report a case of pituitary infarction attributable to long-term intranasal cocaine use. methods: We present the clinical findings, laboratory results, and imaging studies in a woman with hypopituitarism, diabetes insipidus, and a palsy of cranial nerve VI, associated with cocaine-induced destruction of her nasal and paranasal structures and the anterior base of her skull. RESULTS: A 55-year-old woman, a long-term cocaine abuser, presented with delirium, nausea, vomiting, hypoglycemia, hypercalcemia, and hypotension as manifestations of adrenal insufficiency (cortisol levels <1 mg/dL before and after administration of cosyntropin). She was found to have a deficiency of adrenocorticotropic hormone (<1 pg/mL), gonadotropin deficiency (estradiol <20 pg/mL, luteinizing hormone 1.7 mIU/mL, and follicle-stimulating hormone 4.9 mIU/mL), and diabetes insipidus. She also had a palsy of right cranial nerve VI. Computed tomography and magnetic resonance imaging of the head demonstrated extensive destruction of the paranasal sinuses, extending into the base of the skull. Serial magnetic resonance images showed reduction of pituitary volume. Nasal biopsy specimens disclosed findings consistent with cocaine-induced ischemic necrosis. CONCLUSION: To our knowledge, this is the first case of hypopituitarism and diabetes insipidus due to prolonged intranasal cocaine abuse.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
7/8. hypocalcemia due to tubular dysfunction in a patient with holoprosencephaly.This is the first report of a case of hypocalcemia in a female infant with holoprosencephaly. hypocalcemia developed 60 days after birth, secondary to decreased serum 1,25-dihydroxyvitamin D, as a result of renal tubular dysfunction which may have been induced by prerenal acute renal failure, the administration of anticonvulsants, and hypothyroidism. However, there was no evidence of rickets, and her serum 25-hydroxyvitamin D value was normal. She was treated with high-dose (0.5 microg/kg) 1alpha-hydroxyvitamin D3 and calcium lactate, and her calcium and 1,25-dihydroxyvitamin D values were consequently, normalized. However, she died at 268 days after birth.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
8/8. A case of reversed pituitary dysfunction with intrasellar mass.hypopituitarism can be caused by tumor, inflammation, granuloma and injuries. Once pituitary function is disturbed, hormone replacement therapy is necessary for the remaining life span in most cases. We have experienced a rare case of a unique intrasellar mass associated with pituitary dysfunction in which both spontaneously reversed. A 61-yr-old woman developed hypoadrenalism and central diabetes insipidus (cDI). Magnetic resonance (MR) imaging revealed a lobular, strong hypointense lesion with spotty signal in the middle of the hypophysis. This spotty lesion showed isointensity on T1- and high-intensity on T2-weighted MR images. The spotty signal as well as the normal pituitary lobe were enhanced by the administration of gadolinium. As replacement therapies for hypoadrenalism and cDI, 10 mg of hydrocortisone and 2.5 microg of desmopressin acetate were prescribed. Three months later, slight shrinkage of intrasellar mass and spontaneous improvement of pituitary functions were found. hydrocortisone was then discontinued. Furthermore, because polyuria and polydipsia were improved nine months later, desmopressin acetate was stopped. Currently, the intrasellar mass continues to shrink, and the patient shows no symptoms without medication. Based upon the unique features of MR images, we suspect that the origin of the mass is an intrasellar hemangioma.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |