Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01185 (vasopressin)
 23,126 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The posterior pituitary lobe and stalk were studied by magnetic resonance imaging in 20 children with diabetes insipidus of different origins: primary familial autosomal dominant (n = 2) or idiopathic (n = 2), and secondary to craniopharyngioma (n = 6, resected in 5), to Langerhans cell histiocytosis (n = 5), to excessive water intake (dipsogenic; n = 3), to renal vasopressin insensitivity (n = 1), and to osmoreceptor dysfunction (n = 1). Of the four children with primary diabetes insipidus, the posterior bright signal was recognizable in two with the familial autosomal dominant form and one with the idiopathic form; in the latter, the pituitary stalk was thin, while it was normal in the first two patients; no posterior hyperintense signal with enlarged and gadolinium-enhanced pituitary stalk was observed in the fourth. The posterior hyperintense signal was absent without evidence of ectopic posterior pituitary tissue regeneration in five children with surgically removed craniopharyngioma and was doubtful in the child with unresected craniopharyngioma; the stalk was unrecognizable in all patients. In the five children with Langherans cell histiocytosis, the posterior bright signal was absent, while the stalk was normal in two and unexpectedly enlarged in three (uniformly in two and mainly at the level of median eminence and hypothalamus in one). All five patients with dipsogenic or nephrogenic diabetes insipidus or osmoreceptor dysfunction had normal images of posterior pituitary lobe and stalk. Normal posterior pituitary bright signal and stalk were found in all 25 healthy control children. Plasma vasopressin was undetectable in all patients except in nephrogenic one, in the child with osmoreceptor dysfunction, and in two of three dipsogenic children, the third mimicking partial neurogenic diabetes insipidus.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus. 154 43

Twelve patients, aged 16-66 years, affected by cranial diabetes insipidus either idiopathic or secondary (head injuries, histiocytosis X, metastatic tumors, pituitary or hypothalamic surgery) were studied. Diagnosis was made on the basis of both dehydration test and sensitivity to exogenous vasopressin. The relationship between plasma and urine osmolality at the end of the dehydration test was evaluated (and its usefulness for diagnostic purpose confirmed). According to this test two different conditions, namely partial and complete, of cranial diabetes insipidus were defined. All the patients underwent the following diagnostic procedures: skull x-ray, evaluation of visual fields, encephalic CT-scan. Idiopathic central diabetes insipidus incidence was found to be 33.3% of all cases, with a sharp prevalence for male sex; the median age of onset was 12.5 years. In our experience the frequency of idiopathic condition was similar to that reported in most recent literature confirming a decreasing incidence, most likely due to the use of more sophisticated diagnostic procedures.
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PMID:[Vasopressin-sensitive diabetes insipidus. Case histories]. 157 92

Hypothalamic-pituitary radiation therapy has been the standard treatment for the diabetes insipidus of Langerhans cell histiocytosis. The goal of this study was to assess the role of radiation therapy in Langerhans cell histiocytosis-associated diabetes insipidus and to compare the results with nonirradiated controls. Forty-seven patients with pathologically confirmed Langerhans cell histiocytosis were diagnosed with diabetes insipidus between 1950 and 1989 and were treated at the Mayo Clinic. These patients were divided into two groups on the basis of treatment for the diabetes insipidus: The first group (radiation group) included 30 patients (28 of whom were evaluable for response) who received hypothalamic-pituitary radiation therapy, and the second group (control group) included 17 patients who did not. A partial response to treatment was defined as a reduction in vasopressin dosage or improvement in computed tomography (CT) or magnetic resonance imaging (MRI). A complete response was defined as no further need for vasopressin therapy or normalization of CT or MRI. End points analyzed included treatment response, patient characteristics, morbidity, dose-response relationship, and survival. Patient characteristics of the two groups were similar except for age and lung involvement, both of which were significantly less in the radiation group. Thirty-six percent of patients (10 of 28) in the radiation group responded to hypothalamic-pituitary radiation therapy (22% complete response and 14% partial response), whereas none in the control group responded. Five of the six complete responders were irradiated within 14 days of the diagnosis of diabetes insipidus. The mean dose used in the responding and nonresponding patients was 11.2 and 10 Gy, respectively. Three of five patients (60%) treated with more than 15 Gy responded compared to seven of 23 (30%) treated with less than 15 Gy. Eight of the 10 responders (80%), compared to 16 of 35 nonresponders (46%), were female. Only one in 20 patients with concomitant lung histiocytosis responded. Complications of therapy may include insufficiency in other hypothalamic-pituitary axes in the treated patients. Actuarial survivals at 5, 10, 20, and 40 years for the entire group were 80%, 78%, 75%, and 65%, respectively, with a median follow-up in living patients of 14.7 years.
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PMID:Radiation therapy for diabetes insipidus caused by Langerhans cell histiocytosis. 161 71

Diabetes insipidus is a clinical syndrome characterized by the excretion of copious volumes of dilute urine combined with persistent intake of abnormally large quantities of fluid. There are two general forms of the disease, central (vasopressin deficient) and nephrogenic (vasopressin resistant). Diabetes insipidus of central origin most often results from lesions in the hypothalamic-neurohypophyseal axis. Twenty-six cases of central diabetes insipidus were evaluated with the use of high-field-strength MR imaging. A wide variety of precipitating conditions were found, including Langerhans cell histiocytosis, neoplasia, trauma, and infection. A thickened pituitary infundibulum was seen in most patients, and an absence of high intensity signal in the posterior pituitary lobe on T1-weighted images was seen in every case. Analysis of stalk morphology; associated brain findings; and correlation with the patient's age, sex, history, and radiographs of other body parts improved diagnostic specificity. When combined with clinical information, MR imaging is able to provide a specific diagnosis in almost all cases of central diabetes insipidus.
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PMID:MR imaging of the brain in patients with diabetes insipidus. 827 42

The brains of eight patient with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus. An orbital extraconal mass was noted in one child with exopthalmus. This decreased in a follow-up study after Vinblastine therapy. The cerebellum was abnormal in two patients; the area of the dentate nuclei exhibited abnormal signal in one asymptomatic child and the MRI of another boy with a ten year history of progressive cerebellar dysfunction showed cerebral and cerebellar atrophy. Three of the five patients with deficiency of antidiuretic hormone (ADH) were studied both before and after the administration of intravenous Gadolinium-DTPA. In all three, after administration of the contrast agent, there was enhancement of involved areas.
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PMID:Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA. 239 58

Thirty-nine patients with idiopathic cranial diabetes insipidus (DI) and 81 secondary to hypothalamic lesions were investigated for the presence both of associated autoimmune diseases and autoantibodies. Eleven (28%) of the idiopathic but none of the secondary DI cases had an overt autoimmune disease. A further two patients with idiopathic DI had associated organ-specific autoantibodies. Autoantibodies to vasopressin (AVP)-secreting hypothalamic cells were detected in 12 patients with idiopathic DI (31%). Seven out of 13 cases of DI secondary to histiocytosis X (HX) were also positive (54%), whereas only two (3%) of the other 68 sera from patients with secondary DI reacted with AVP cels. Of the 13 patients with DI associated with frank organ-specific autoimmune diseases or autoantibodies alone, eight (62%) were positive for AVP-cell antibodies. The finding of associated autoimmune diseases in a patient with idiopathic DI is therefore suggestive of an autoimmune origin of DI, and this can be supported by the detection in the serum of AVP cell antibodies. In cases of HX, the new finding of the presence of AVP-cell antibodies reflects hypothalamic infiltration by HX cells, and suggests that DR + 'Langerhans-like' cells play more than a passive role in the hypothalamic lesion.
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PMID:Autoimmune cranial diabetes insipidus: its association with other endocrine diseases and with histiocytosis X. 349 38

Langerhans' cell histiocytosis (LCH) is a proliferative disease of Langerhans' cells that has multiple clinical manifestations including diabetes insipidus (DI). This study reviews the effectiveness of hypothalamic-pituitary radiation therapy (HPRT) as a treatment of LCH-induced DI in the modern era. A retrospective review was done of 116 pediatric patients with LCH seen from 1975 to 1992. Seventeen of the 116 patients (15%) were diagnosed with complete or partial diabetes insipidus. Diagnosis was made either by water deprivation test or on clinical grounds. Fourteen patients received hypothalamic-pituitary irradiation as treatment for DI. The median interval from the onset of DI symptoms (polyuria and polydipsia) to treatment was 30 days. The median interval from the onset of diagnosis to treatment was 4 days. With a mean follow-up of 7.3 years (range, 2.4-14.3), only two patients had a complete response to therapy, as defined as no need for antidiuretic hormone (ADH) replacement therapy. No patient had a partial response, defined as a decrease in the dose of ADH replacement. Of the two responders, neither had a complete ADH deficiency, suggesting "early" disease. In addition, both received RT within 3 days. We feel that the standard treatment of RT to all patients with LCH-induced DI is no longer justified. Our series has shown no benefit in treating patients with a long history of DI. Rather, an improved rationale would be rapid initiation of hypothalamic-pituitary irradiation in patients with new symptoms of DI and an abnormal water deprivation test.
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PMID:Diabetes insipidus secondary to Langerhans' cell histiocytosis: is radiation therapy indicated? 914 4

Nephrogenic diabetes insipidus is a rare, mostly X-linked recessive disorder characterised by renal tubular resistance to the antidiuretic effect of arginine vasopressin. The gene responsible for the X-linked nephrogenic diabetes insipidus, the G-protein-coupled vasopressin V2-receptor, has been localised on the Xq28 region. In this study four patients were investigated with molecular genetic methods. Diagnosis was based on clinical symptoms and lack of increase of urinary osmolality after administration of the arginine vasopressin, or the synthetic vasopressin analogue DDAVP. Three different mutations (C112R, N317K, W323S) were found in three patients, while no mutation was detected in the fourth patient. Since earlier histiocytosis X has been diagnosed in this patient, this patient has probably central diabetes insipidus. Although the main symptoms of the disease can be found in all patients, there are significant differences in the seriousness of the symptoms as well as in some other symptoms. The explanations might be the different mutations in the V2-receptor gene and the various other genetic and environmental factors; these findings provide further evidence that X-linked nephrogen diabetes insipidus results from defects in the V2-receptor gene.
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PMID:[Molecular biological studies on patients with nephrogenic diabetes insipidus]. 957 1

The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in Taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. Skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.
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PMID:Diabetes insipidus in Langerhans' cell histiocytosis: report of a case. 1037 74

Xanthoma disseminatum is a rare non-Langerhans' cell histiocytosis, characterized by papular cutaneous eruption, possible mucosal involvement, and frequent association with vasopressin-sensitive diabetes insipidus. Herein we report a case of xanthoma disseminatum with pharyngolaryngeal involvement. In this patient, mucosal xanthomas involving the arytenoid cartilages and the interarytenoid area resulted in laryngeal stenosis and severe impairment of both cricoarytenoid joints' motility. Endoscopic CO2 laser medial arytenoidectomy, according to the technique described by Crumley (1993), and vaporization of interarytenoid xanthomas were successfully performed, thus reestablishing bilateral cordal motility and the laryngeal airway. Four years later, a CO2 laser revision was necessary because of recurrence of xanthomas in the posterior larynx. Two years after the latter operation, the patient has no signs of laryngeal obstruction and has a normal voice quality. This case report suggests that endoscopic medial arytenoidectomy may be successfully used in the treatment of bilateral laryngeal pseudoparalysis secondary to xanthoma disseminatum.
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PMID:Bilateral laryngeal pseudoparalysis in xanthoma disseminatum treated by endoscopic laser medial arytenoidectomy. 1126 72


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