Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032617 (polyuria)
3,056 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

We present the eldest case ever reported of central diabetes insipidus (DI) associated with infundibulo-neurohypophysitis. A 77-year old woman, who complained of recent development of excessive thirst, polyuria and polydipsia, was referred to our hospital. The daily urine volume was markedly increased to 6 L. DDAVP administration effectively reduced urine volume and increased urine osmolality. The loading test using high-osmolar sodium chloride showed impaired excretion of vasopressin discordant with plasma osmolar changes. The anterior pituitary function was normal. Pituitary magnetic resonance imaging (MRI) showed thickening of the pituitary stalk and a lack of high-intensity signal of the neurohypophysis on T1-weighted images, suggestive of lymphocytic infundibulo-neurohypophysitis. The thickness of pituitary stalk on MRI improved 6 months later. DI was controlled with DDAVP for 40 days. This was followed by stabilization of the daily urine volume to less than 2.5 L without DDAVP. Our case is the eldest case of central DI associated with infundibulo-neurohypophysitis. The rapid remission of pituitary changes on MRI provides an insight that spontaneously partial remission of central DI may occur, resulting in transient polyuria and polydipsia.
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PMID:An elderly patient with transient diabetes insipidus associated with lymphocytic infundibulo-neurohypophysitis. 1067 Jul 63

Aquaporin (AQP) water channel AQP3 has been proposed to be the major glycerol and non-AQP1 water transporter in erythrocytes. AQP1 and AQP3 are also expressed in the kidney where their deletion in mice produces distinct forms of nephrogenic diabetes insipidus. Here AQP1/AQP3 double knockout mice were generated and analyzed to investigate the functional role of AQP3 in erythrocytes and kidneys. 53 double knockout mice were born out of 756 pups from breeding double heterozygous mice. The double knockout mice had reduced survival and impaired growth compared with the single knockout mice. Erythrocyte water permeability was 7-fold reduced by AQP1 deletion but not further reduced in AQP1/AQP3 null mice. AQP3 deletion did not affect erythrocyte glycerol permeability or its inhibition by phloretin. Daily urine output in AQP1/AQP3 double knockout mice (15 ml) was 9-fold greater than in wild-type mice, and urine osmolality (194 mosm) was 8.4-fold reduced. The mice remained polyuric after DDAVP administration or water deprivation. The renal medulla in most AQP1/AQP3 null mice by age 4 weeks was atrophic and fluid-filled due to the severe polyuria and hydronephrosis. Our data provide direct evidence that AQP3 is not functionally important in erythrocyte water or glycerol permeability. The renal function studies indicate independent roles of AQP1 and AQP3 in countercurrent exchange and collecting duct osmotic equilibration, respectively.
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PMID:Erythrocyte water permeability and renal function in double knockout mice lacking aquaporin-1 and aquaporin-3. 1103 42

A 59-year-old woman whose first clinical manifestations were polyuria and polydipsia was admitted to our hospital. Brain MRI showed multiple mass lesions and a thickened pituitary stalk. Chest CT showed hilar and mediastinal lymphadenopathy and a small nodule measuring about 1.5 cm in the apex of the right lung. Histopathological examination revealed adenocarcinoma of the lung, and primary lung cancer with diabetes insipidus secondary to pituitary stalk metastasis was diagnosed. She received systemic chemotherapy and whole-brain irradiation concurrent with intranasal desmopressin (DDAVP) treatment. Although the size of the tumor was reduced, her symptoms did not improve and the same dose of hormone replacement therapy was required. We present this rare case and review the twenty cases of metastatic pituitary lesions arising from lung cancer reported in the literature.
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PMID:[Pituitary stalk metastasis from lung cancer, preceding diabetes insipidus--case report and clinical review of the literature]. 1269 6

A 45-year-old male was diagnosed as having myelodysplastic syndrome (RAEB) in March 2001. He was admitted to our hospital because of cellulitis in his left lower limb in May. The blood cell count showed pancytopenia and immature myeloid cells were seen in the peripheral blood. Bone marrow aspiration showed the proliferation of myeloblasts (11.2%) and complex karyotypic abnormalities were detected including the long arm deletion of chromosome 7. The patient developed polyuria and polydipsia after admission and was diagnosed as having central diabetes insipidus. He was treated with DDAVP and the polyuria disappeared. In November, he underwent unrelated allogeneic bone marrow transplantation after conditioning with total body irradiation and cytarabine. After transplantation DDAVP was no longer required. Central diabetes insipidus has been reported as a rare complication of leukemia or myelodysplastic syndrome, but the underlying mechanism remains unclear. The complete remission of diabetes insipidus after bone marrow transplantation suggests that the infiltration of leukemic cells into the pituitary gland caused the diabetes insipidus in this case.
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PMID:[Improvement of diabetes insipidus after allogeneic bone marrow transplantation in a patient with myelodysplastic syndrome]. 1282 6

Gestational diabetes insipidus is a rare disorder characterized by polyuria and polydipsia due to the inability of the kidneys to concentrate urine. We report two cases of transient gestational diabetes insipidus in which patients responded to intranasal DDAVP (1-deamino-8-D-arginine vasopressin) with greater than 50% increase in urine osmolality and marked reduction in urine output. Intranasal DDAVP was discontinued after their discharge and both patients maintained normal urine output and appropriate urine osmolality. In determining whether diabetes insipidus is present in a patient who is polyuric and hypernatremic, a urine osmolality below that of the plasma suggests the presence of diabetes insipidus. Understanding of the pathophysiology may soon lead to improved methods of prevention, diagnosis and treatment.
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PMID:Transient gestational diabetes insipidus: report of two cases and review of pathophysiology and treatment. 1498 11

Hydroelectrolytic disorders often complicate surgery of intra/parasellar tumors in children and adolescents. Eighteen patients undergoing microneurosurgical procedures for intra-supra-sellar craniopharyngioma (10 patients), hypothalamic germinomas (3 patients), hypothalamic-chiasmatic astrocytomas (3 patients), pituitary adenomas (2 patients) were studied. The hydroelectrolytic balance was assessed from 8 hours before surgery to 1 week after with a specific protocol in which water metabolism alterations were treated with standard procedure. Diabetes insipidus (DI) was observed in 10/18 patients before surgery and in 15/18 patients after surgery; during surgery it was effectively treated with synthetic desmopressin (DDAVP) and hydroelectrolytic solutions. Hyponatremia, isolated or associated (with diuresis contraction or polyuria), seen during surgery and in the following 24 hours, was treated with variation of the infusion rate. We show that close monitoring and treatment of hydroelectrolytic disorders in patients submitted to neurosurgery for intra/ parasellar tumors may significantly reduce their morbidity and mortality rate.
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PMID:Endocrine function and water metabolism in children and adolescents with surgically treated intra/parasellar tumors. 1557 Sep 84

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.
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PMID:Novel mutant vasopressin-neurophysin II gene associated with familial neurohypophyseal diabetes insipidus. 1564 73

A 9-year-old boy was admitted to our hospital with daytime urinary incontinence for the past one year. MRI showed craniopharyngioma occupying the third ventricle. The tumor was excised by interhemispheric approach. Because hyponatremia and polyuria with high renal loss of sodium were observed on postoperative day 3, hydrocortisone and DDAVP were replaced. On postoperative day 24, successive general convulsions and hyponatremia recurred, and MRI FLAIR imaging showed marked brain edema in the bilateral parieto-occipital lobes. This finding disappeared late in the course of treatment, and the case was diagnosed as posterior reversible encephalopathy syndrome. The pathophysiology of cerebral salt wasting and posterior reversible encephalopathy syndrome in a craniopharyngioma patient are also discussed in the article.
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PMID:[Prolonged cerebral salt wasting following craniopharyngioma surgery and posterior reversible encephalopathy syndrome: a case report]. 1578 2

Thrombotic thrombocytopenic purpura (TTP) is a rare and often fatal disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, and renal dysfunction. Central diabetes insipidus (CDI) is a rare disease due to anatomic lesions of the hypothalamicpituitary system associated with various underlying diseases, or trauma. We present an unusual case of TTP and CDI in a 47 year-old African American female who was admitted to our hospital with crampy abdominal pain and nausea. The patient had tachycardia, fever and hypotension. The patient subsequently became confused, developed seizures, and her renal function deteriorated. Bone marrow analysis showed adequate megakaryocytes while a peripheral smear revealed severe thrombocytopenia, polychromasia and schistocytes. The diagnosis of thrombotic thrombocytopenic purpura (TTP) was made and plasmapharesis initiated. Over the next few days, the patient developed severe polyuria with a rise in serum sodium. Central diabetes insipidus was diagnosed and DDAVP (desmopressin acetate, 1-deamino-8-D-arginine vasopressin) was given. However, DDAVP was stopped several times due to worsening thrombocytopenia. Renal function worsened and the patient expired. A review of the literature revealed only one case of report of TTP and central diabetes insipidus. Our case was the only one reporting the use of DDAVP in such a setting.
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PMID:Unique case of thrombotic thrombocytopenic purpura and diabetes insipidus. 1641 83


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