1/26. Acute myeloid leukaemia with trilineage myelodysplasia complicated by masked diabetes insipidus.We describe a rare case of acute myeloid leukaemia with trilineage myelodysplasia complicated by central diabetes insipidus. In the present case, diabetes insipidus was masked by corticosteroid deficiency due to hypopituitarism and clinical symptoms presented after administering methylprednisolone. Although the remission of leukaemia was not achieved by chemotherapy, excessive urinary output was well-controlled by nasal administration of 1-desamino-8-D-arginine vasopressin (DDAVP) during the course.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
2/26. Very delayed hyponatremia after surgery and radiotherapy for a pituitary macroadenoma.Severe hyponatremia (118 mmol/l) with natriuresis, consistent with cerebral salt wasting syndrome (CSWS), occurred 38 days after transsphenoidal surgery in a 59-year-old woman affected by a pituitary non-functioning macroadenoma. From the 35th day after surgery, she showed progressive polyuria, hypotension and hyponatremia associated with natriuresis, decreased plasma and increased urinary osmolality. The clinical examination revealed signs of dehydration and gradual decline in the level of consciousness. The anterior pituitary function was normal due to appropriate replacement of thyroid and adrenal axis. The patient was treated with saline administration until normal natremia and water balance were restored and neurological symptoms had completely disappeared. This case focuses on the unusually prolonged time of development of post-surgery hyponatremia, despite delayed symptomatic hyponatremia being reported to commonly occur 7 days after transsphenoidal surgery. Therefore, we would advise not to limit the periodic follow-up of the hydroelectrolytic balance to the first two weeks after surgery, but to prolong it until after discharge from hospital. In fact, an early diagnosis is of great importance to prevent permanent neurological damage or death. Since CSWS and syndrome of inappropriate secretion of ADH, the two disorders alternatively imputed to generate post-surgical hyponatremia, are characterized by different pathogenic mechanisms and require opposing therapeutic approaches, the occurrence of extracellular volume dilution or of increased sodium renal loss should be carefully investigated. The evidences in favor of CSWS, the possible mechanisms behind the syndrome and diagnosis and management of patients with post-transsphenoidal surgery CSWS are discussed.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
3/26. polyuria associated with an antibody to vasopressin.1. A patient with polyuria in whom diabetes insipidus had been diagnosed was treated with Pitressin. Resistance to this therapy developed after 18 months and a circulating antibody to vasopressin was then demonstrated. Withdrawal of therapy led to a fall in titre of the antibody and an increase in maximal urinary concentration. 2. The antibody to vasopressin was associated with the IgA fraction of the serum immunoglobulins and its characteristics are described.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
4/26. 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 = urinary (Clic here for more details about this article) |
5/26. Idiopathic neurohypophysial diabetes insipidus: reversibility of structural and functional renal abnormalities after treatment.OBJECTIVE: To present a case of untreated long-term idiopathic neurohypophysial diabetes insipidus (DI) with structural and functional renal abnormalities that regressed after treatment. methods: We describe the clinical course, biochemical data, and imaging findings in a man with idiopathic neurohypophysial DI in whom structural and functional abnormalities involving the urinary tract diminished after treatment. The patient underwent intravenous pyelography, retrograde pyelography, computed tomographic (CT) scanning of the kidneys and abdomen, iodohippurate renal scanning, and voiding cystourethrography. ultrasonography of the kidneys at presentation and at 8-year followup and serial determinations of serum creatinine for a period of 9 years were also done. RESULTS: A 43-year-old man had polyuria for 30 years attributable to untreated idiopathic neurohypophysial DI. He presented with bilateral flank pain and a high serum creatinine level (156 mmol/L). ultrasonography, intravenous pyelography, retrograde pyelography, and CT scan of the kidneys demonstrated severe bilateral hydronephrosis, notably dilated and tortuous ureters, a distended bladder, and atrophy of the left renal cortex. Retrograde pyelography and voiding cystourethrography confirmed the absence of mechanical obstruction or urinary reflux. A renal scan study showed bilaterally impaired function. Treatment with intranasally administered desmopressin and clean intermittent straight bladder catheterization resulted in resolution of flank pain, improvement of renal function, normalization of serum creatinine levels, and decreased hydronephrosis during 9 years of follow-up. CONCLUSION: This case provides information about renal abnormalities in the natural history of a long-term polyuric state, idiopathic neurohypophysial DI, before and after treatment. Reversibility of renal structural and functional abnormalities after treatment is documented. Recognition of renal abnormalities associated with untreated neurohypophysial DI is important for prevention and treatment of such complications.- - - - - - - - - - ranking = 2keywords = urinary (Clic here for more details about this article) |
6/26. Congenital nephrogenic diabetes insipidus presented with bilateral hydronephrosis: genetic analysis of V2R gene mutations.Most cases of hydronephrosis are caused by urinary tract obstruction. However, excessive polyuric syndrome rarely gives rise to non-obstructive hydronephrosis, megaureter, and a distended bladder. The authors report here on two cases of congenital nephrogenic diabetes insipidus (NDI) with severe bilateral hydronephrosis and megaureter. It is Interesting that the patients were symptomless except for their polyuria, and they both presented with bilateral hydronephrosis. Fluid deprivation testing revealed the presence of AVP resistant NDI. Gene analysis for these patients showed the AVP receptor 2 (V2R) missense mutations (Q225X and S126F), which have previously been reported on in other studies. We made the diagnosis of NDI by using a physiologic test, and we confirmed it by mutation analysis of the V2R gene.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
7/26. Dipsogenic diabetes insipidus: report of a novel treatment strategy and literature review.Dipsogenic diabetes insipidus is a syndrome of disordered thirst, in patients without psychiatric disease, which may be confused with partial central diabetes insipidus. Distinguishing these entities involves monitored water testing. Therapy with antidiuretic hormone in patients with dipsogenic diabetes insipidus is thought to be contraindicated for fear of inducing water intoxication. We report a case of a 26-year-old woman without psychiatric illness referred for longstanding polyuria and polydipsia. Otherwise healthy, she complained of near-constant thirst and frequent urination, causing severe disruption of her personal and professional life. She had been consistently eunatremic and polyuric, with low urine osmolality. Results of extensive water testing revealed intact urinary concentrating and diluting capacity, physiologic though blunted antidiuretic hormone (ADH) release, and an abnormally low thirst threshold, consistent with the diagnosis of dipsogenic diabetes insipidus. To control her polyuria we initiated treatment with intermittent, low-dose, intranasal desmopressin and strict water restriction during drug dosing. In follow-up she reported excellent control of polyuria and significant functional improvement. The reviewed literature demonstrates a limited number of reports about dipsogenic diabetes insipidus, and no prior report of a similar treatment strategy. Dipsogenic diabetes insipidus is an uncommonly (and not universally) recognized disorder, requiring monitored testing in order to distinguish it from incomplete forms of central diabetes insipidus. Though therapy with desmopressin cannot be recommended based on the results of a single case, the outcome presented here is intriguing and suggests that larger studies in such patients is warranted to assess the broader application of such an intervention.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
8/26. Malignant hemangiopericytoma of the pelvis. Report of a case with urological implications and immunohistochemical analysis.A case of a malignant hemangiopericytoma located in the pelvic fossa of a 50-year old man is reported. The tumor displaced the urinary bladder and caused bilateral hydronephrosis. light microscopic diagnosis was followed by immunohistochemistry. After surgery the patient was successfully treated by chemotherapy and irradiation. This paper reviews clinicopathological features in respect of prognosis and a possible treatment.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
9/26. Nonobstructive urinary tract dilatation in nephrogenic diabetes insipidus.The follow-up study has been performed in 4 male patients with nephrogenic diabetes insipidus lasting for 4 to 15 years. hydronephrosis or hydroureter appeared to be caused by polyuria with or without urinary tract obstruction. The efficacy of drainage operation is emphasized when polyuria surpasses the transporting ability of urine in the urinary tract.- - - - - - - - - - ranking = 6keywords = urinary (Clic here for more details about this article) |
10/26. plasma atrial natriuretic polypeptide and polyuria during paroxysmal tachycardia in wolff-parkinson-white syndrome patients.The human atrial natriuretic polypeptide (hANP) concentration in plasma was measured during paroxysmal supraventricular tachycardia provoked in 2 patients with wolff-parkinson-white syndrome. A 10- to 20-fold increase in plasma hANP concentration was observed during the tachycardia: from 12 to 291 pg/ml in 1 case and from 14 to 174 pg/ml in the other. Although polyuria was associated with the tachycardia, urinary sodium excretion as well as urinary osmolality were decreased. The urinary arginine vasopressin was appreciably decreased during the tachycardia. These results suggest that hANP released by paroxysmal tachycardia might not act as a natriuretic factor in this range of plasma concentration. polyuria during paroxysmal tachycardia was attributed mainly to the inhibition of arginine vasopressin release.- - - - - - - - - - ranking = 3keywords = urinary (Clic here for more details about this article) |
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