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MedWorm: Diabetes Insipidus



MedWorm.com provides a medical RSS filtering service. Over 7000 RSS medical sources are combined and output via different filters. This feed contains the latest news and research in the Diabetes Insipidus category.



Last Build Date: Tue, 22 Mar 2016 08:23:10 +0100

 



Genetic forms of nephrogenic diabetes insipidus (NDI): Vasopressin receptor defect (X-linked) and aquaporin defect (autosomal recessive and dominant)

Wed, 16 Mar 2016 00:00:00 +0100

Publication date: Available online 2 March 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Daniel G. Bichet, Detlef Bockenhauer Nephrogenic diabetes insipidus (NDI), which can be inherited or acquired, is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone, arginine vasopressin (AVP). Polyuria with hyposthenuria and polydipsia are the cardinal clinical manifestations of the disease. About 90% of patients with congenital NDI are males with X-linked NDI who have mutations in the vasopressin V2 receptor (AVPR2) gene encoding the vasopressin V2 receptor. In less than 10% of the families studied, congenital NDI has an autosomal recessive or autosomal dominant mode ...

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Novel AVPR2 mutation causing partial nephrogenic diabetes insipidus in a Japanese family

Tue, 15 Mar 2016 19:08:31 +0100

Journal Name: Journal of Pediatric Endocrinology and MetabolismIssue: Ahead of print (Source: Journal of Pediatric Endocrinology and Metabolism)



Persistent severe polyuria after renal transplant

Tue, 15 Mar 2016 00:00:00 +0100

We present a 44-year-old man with a strong family history of DI who had been diagnosed with autosomal dominant nephrogenic DI from infancy. At the age of 40, he had progressed to end-stage renal failure. When he experienced unresolving severe polyuria after renal transplant, further investigations revealed that he was misdiagnosed and that he had a novel mutation causing autosomal dominant cranial DI. (Source: CKJ: Clinical Kidney Journal)



Does lower urine-specific gravity predict decline in renal function and hypernatremia in older adults exposed to psychotropic medications? An exploratory analysis

Tue, 15 Mar 2016 00:00:00 +0100

Conclusions In psychotropic-exposed older adults, there appears to be a clinically important association between low USG and developing both hypernatremia and CKD. USG may be a useful surrogate measure for NDI-related outcomes in large administrative database studies, where ideal measures such as 24-h urine volume may not be available. (Source: CKJ: Clinical Kidney Journal)



Plasma Apelin concentrations in Patients with Polyuria-Polydipsia Syndrome.

Fri, 11 Mar 2016 00:00:00 +0100

CONCLUSION: In PP, normal plasma apelin to copeptin ratio attests a normal water homeostasis. In contrast, in patients with central or nephrogenic DI the increased or decreased apelin to copeptin ratio, respectively, reflects a disturbed osmotic and body fluid homeostasis. PMID: 26967692 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)



Metformin, an AMPK activator, stimulates the phosphorylation of Aquaporin 2 and Urea Transporter A1 in Inner Medullary Collecting Ducts.

Wed, 09 Mar 2016 00:00:00 +0100

Authors: Klein JD, Wang Y, Blount MA, Molina PA, LaRocque LM, Ruiz JA, Sands JM Abstract Nephrogenic diabetes insipidus (NDI) is characterized by production of very large quantities of dilute urine due to an inability of the kidney to respond to vasopressin. Congenital NDI results from mutations in the type 2 vasopressin receptor (V2R) in approximately 90% of families. These patients do not have mutations in aquaporin-2 (AQP2) or urea transporter UT-A1 (UT-A1). We tested adenosine monophosphate kinase (AMPK) since it is known to phosphorylate another vasopressin-sensitive transporter, NKCC2 (Na-K-2Cl cotransporter). We found AMPK expressed in rat inner medulla (IM). AMPK directly phosphorylated AQP2 and UT-A1 in vitro. Metformin, an AMPK activator, increased phosphorylation of both...

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Genetic Forms of Nephrogenic Diabetes Insipidus: Vasopressin Receptor Defect (X-linked) and Aquaporin Defect (Autosomal Recessive and Dominant)

Thu, 03 Mar 2016 00:00:00 +0100

Publication date: Available online 2 March 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Daniel G. Bichet, Detlef Bockenhauer Nephrogenic diabetes insipidus (NDI), which can be inherited or acquired, is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone, arginine vasopressin (AVP). Polyuria with hyposthenuria and polydipsia are the cardinal clinical manifestations of the disease. About 90 percent of patients with congenital NDI are males with X-linked NDI who have mutations in the vasopressin V2 receptor (AVPR2) gene encoding the vasopressin V2 receptor. In less than 10 percent of the families studied, congenital NDI has an autosomal recessive or autosomal ...



Copeptin as a biomarker and a diagnostic tool in the evaluation of patients with polyuria–polydipsia and hyponatremia

Thu, 03 Mar 2016 00:00:00 +0100

Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): M. Christ-Crain, N.G. Morgenthaler, W. Fenske Copeptin is part of the 164 amino acid precursor protein preprovasopressin together with vasopressin and neurophysin II. During precursor processing, copeptin is released together with vasopressin. Copeptin concentrations respond as rapidly as vasopressin to changes in osmolality, a decrease in blood pressure or stress and there is a close correlation of vasopressin and copeptin concentrations. For these reasons, copeptin is propagated as a surrogate marker for vasopressin in the differential diagnosis of the polyuria–polydipsia syndromes and hyponatremia. Results of prospective studies show t...



Cholesteatoma in the Sellar Region Presenting as Hypopituitarism and Diabetes Insipidus

Tue, 01 Mar 2016 06:00:00 +0100

This article emphasized that cholesteatomas, although rare, should be considered part of the differential diagnosis of sellar lesions. (Source: Medicine)



Concerns about the safety of patients with diabetes insipidus admitted to hospital

Tue, 01 Mar 2016 00:00:00 +0100

(Source: Clinical Endocrinology)



Genetic Forms of Nephrogenic Diabetes Insipidus: Vasopressin Receptor Defect (X-linked) and Aquaporin Defect (Autosomal Recessive and Dominant)

Tue, 01 Mar 2016 00:00:00 +0100

Nephrogenic diabetes insipidus (NDI), which can be inherited or acquired, is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone, arginine vasopressin (AVP). Polyuria with hyposthenuria and polydipsia are the cardinal clinical manifestations of the disease. About 90 percent of patients with congenital NDI are males with X-linked NDI who have mutations in the vasopressin V2 receptor (AVPR2) gene encoding the vasopressin V2 receptor. (Source: Best Practice and Research. Clinical Endocrinology and Metabolism)

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Genetic forms of nephrogenic diabetes insipidus (NDI): Vasopressin receptor defect (X-linked) and aquaporin defect (autosomal recessive and dominant)

Tue, 01 Mar 2016 00:00:00 +0100

Nephrogenic diabetes insipidus (NDI), which can be inherited or acquired, is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone, arginine vasopressin (AVP). Polyuria with hyposthenuria and polydipsia are the cardinal clinical manifestations of the disease. About 90% of patients with congenital NDI are males with X-linked NDI who have mutations in the vasopressin V2 receptor (AVPR2) gene encoding the vasopressin V2 receptor. (Source: Best Practice and Research. Clinical Endocrinology and Metabolism)



Central Diabetes Insipidus and Cisplatin‐Induced Renal Salt Wasting Syndrome: A Challenging Combination

Mon, 29 Feb 2016 00:00:00 +0100

We describe a 2‐year‐old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin‐induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin‐induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury. (Source: Pediatric Blood and Cancer)



Diabetes Insipidus in Infants and Children

Sun, 28 Feb 2016 00:00:00 +0100

Publication date: Available online 27 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Elizabeth Dabrowski, Rachel Kadakia, Donald Zimmerman Diabetes insipidus, the inability to concentrate urine resulting in polyuria and polydipsia, can have different manifestations and management considerations in infants and children compared to adults. Central diabetes insipidus, secondary to lack of vasopressin production, is more common in children than is nephrogenic diabetes insipidus, the inability to respond appropriately to vasopressin. The goal of treatment in both forms of diabetes insipidus is to decrease urine output and thirst while allowing for appropriate fluid balance, normonatremia and ensuring an acceptable quality of lif...



Early central diabetes insipidus: An ominous sign in post–cardiac arrest patients

Fri, 26 Feb 2016 00:00:00 +0100

Conclusions All patients diagnosed with CDI had poor neurologic outcomes, and occurrence of CDI was associated with mortality. Central diabetes insipidus patients with death or brain death had earlier occurrence of CDI and more maximum urine output. (Source: Journal of Critical Care)



Diabetes Insipidus in Infants and Children

Thu, 25 Feb 2016 00:00:00 +0100

Diabetes insipidus, the inability to concentrate urine resulting in polyuria and polydipsia, can have different manifestations and management considerations in infants and children compared to adults. Central diabetes insipidus, secondary to lack of vasopressin production, is more common in children than is nephrogenic diabetes insipidus, the inability to respond appropriately to vasopressin. The goal of treatment in both forms of diabetes insipidus is to decrease urine output and thirst while allowing for appropriate fluid balance, normonatremia and ensuring an acceptable quality of life for each patient. (Source: Best Practice and Research. Clinical Endocrinology and Metabolism)

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Rare Presentation of Supratentorial Primitive Neuroectodermal Tumors Mimicking Bifocal Germ Cell Tumors: 2 Case Reports

Fri, 19 Feb 2016 10:35:02 +0100

We report 2 patients with radiologic findings of bifocal pineal and suprasellar lesions, with a histologic diagnosis of supratentorial primitive neuroectodermal tumor. The absence of diabetes insipidus and other endocrine abnormalities was noteworthy in both cases. This observation challenges previous reports on the pathognomonic value of this clinico-radiologic entity. (Source: Journal of Pediatric Hematology Oncology)



Diabetes insipidus: Differential diagnosis and management

Fri, 19 Feb 2016 00:00:00 +0100

Publication date: Available online 18 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Gary L. Robertson Diabetes insipidus (DI) is a syndrome characterized by the excretion of abnormally large volumes of dilute urine. It can be caused by any of 4 fundamentally different defects that must be distinguished for safe and effective management. They are: (1) pituitary DI, due to inadequate production and secretion of antidiuretic hormone, arginine-vasopressin (AVP); (2) gestational DI due to degradation of AVP by an enzyme made in placenta; (3) primary polydipsia, due to suppression of AVP secretion by excessive fluid intake; and (4) nephrogenic DI due to renal insensitivity to the antidiuretic effect of AVP. This review describes s...



Genetic forms of neurohypophyseal diabetes insipidus

Fri, 19 Feb 2016 00:00:00 +0100

Publication date: Available online 18 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Jonas Rutishauser, Martin Spiess, Peter Kopp Neurohypophyseal diabetes insipidus is characterized by polyuria and polydipsia owing to partial or complete deficiency of the antidiuretic hormone, arginine vasopressin (AVP). Although in most patients non-hereditary causes underlie the disorder, genetic forms have long been recognized and studied both in vivo and in vitro. In most affected families, the disease is transmitted in an autosomal dominant manner, whereas autosomal recessive forms are much less frequent. Both phenotypes can be caused by mutations in the vasopressin-neurophysin II (AVP) gene. In transfected cells expressing dominant m...



Diabetes insipidus: Differential diagnosis and management

Thu, 18 Feb 2016 00:00:00 +0100

Diabetes insipidus (DI) is a syndrome characterized by the excretion of abnormally large volumes of dilute urine. It can be caused by any of 4 fundamentally different defects that must be distinguished for safe and effective management. They are: (1) pituitary DI, due to inadequate production and secretion of antidiuretic hormone, arginine-vasopressin (AVP); (2) gestational DI due to degradation of AVP by an enzyme made in placenta; (3) primary polydipsia, due to suppression of AVP secretion by excessive fluid intake; and (4) nephrogenic DI due to renal insensitivity to the antidiuretic effect of AVP. (Source: Best Practice and Research. Clinical Endocrinology and Metabolism)

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Copeptin as a biomarker and a diagnostic tool in the evaluation of patients with polyuria-polydipsia and hyponatremia

Wed, 17 Feb 2016 00:00:00 +0100

Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): M. Christ-Crain, N.G. Morgenthaler, P.D.W. Fenske Copeptin is part of the 164 amino acid precursor protein preprovasopressin together with vasopressin and neurophysin II. During precursor processing, copeptin is released together with vasopressin. Copeptin concentrations respond as rapidly as vasopressin to changes in osmolality, a decrease in blood pressure or stress and there is a close correlation of vasopressin and copeptin concentrations. For these reasons, copeptin is propagated as a surrogate marker for vasopressin in the differential diagnosis of the polyuria-polydipsia syndromes and hyponatremia. Results of prospective studies show...



Diabetes Insipidus During Pregnancy

Tue, 16 Feb 2016 00:00:00 +0100

Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Sonia Ananthakrishnan Diabetes insipidus (DI) in pregnancy is a heterogeneous syndrome, most classically presenting with polyuria and polydipsia that can complicate approximately 1 in 30,000 pregnancies. The presentation can involve exacerbation of central or nephrogenic DI during pregnancy, which may have been either overt or subclinical prior to pregnancy. Women without preexisting DI can also be affected by the actions of placental vasopressinase which increases in activity between the 4th and 38th weeks of gestation, leading to accelerated metabolism of AVP and causing a transient form of DI of pregnancy. This type of DI may be associated...



Congenital Nephrogenic Diabetes Insipidus Presenting in Premature Neonate

Fri, 12 Feb 2016 00:00:00 +0100

(Source: PEDIATRICS)



Etiological and clinical characteristics of central diabetes insipidus in children: a single center experience

Thu, 11 Feb 2016 00:00:00 +0100

Central diabetes insipidus (CDI) results from a number of conditions affecting the hypothalamic-neurohypophyseal system to cause vasopressin deficiency. Diagnosis of CDI is challenging, and clinical data and g... (Source: International Journal of Pediatric Endocrinology)

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Desmopressin patient safety alert

Wed, 10 Feb 2016 16:51:00 +0100

A patient safety alert has been issued by NHS England to raise awareness of the risk of severe dehydration and death caused by an omission or delay of desmopressin in patients with cranial diabetes insipidus. Following reports of patient safety incidents caused by an omission or delay in the provision of desmopressin, providers of NHS care are asked to consider if immediate action needs to be taken locally to raise awareness and reduce the risk of these incidents from occurring. The (Source: The Pharmaceutical Journal)



Lamination of the Outer Plexiform Layer in Optic Atrophy Caused by Dominant Mutations

Wed, 10 Feb 2016 00:00:00 +0100

Wolfram syndrome, or diabetes insipidus, diabetes mellitus, optic atrophy [OA], and deafness (DIDMOAD), is a neurodegenerative disorder with heterogeneous clinical manifestations caused by homozygous or compound heterozygous recessive mutations in the WFS1 gene (OMIM 606201).1 More recently, the phenotypic spectrum has expanded to include patients with dominant inheritance and limited clinical features, in particular OA in association with diabetes mellitus and/or sensorineural deafness.1 WFS1 encodes for an endoplasmic reticulum transmembrane protein, Wolframin, which is highly expressed in retinal tissues, including retinal ganglion cells, the photoreceptor inner segments, and the inner nuclear layer (INL) of the human eye, and mouse Müller cells. (Source: Ophthalmology)



Lamination of the Outer Plexiform Layer in Optic Atrophy Caused by Dominant WFS1 Mutations

Wed, 10 Feb 2016 00:00:00 +0100

Wolfram syndrome, or diabetes insipidus, diabetes mellitus, optic atrophy [OA], and deafness (DIDMOAD), is a neurodegenerative disorder with heterogeneous clinical manifestations caused by homozygous or compound heterozygous recessive mutations in the WFS1 gene (OMIM 606201).1 More recently, the phenotypic spectrum has expanded to include patients with dominant inheritance and limited clinical features, in particular OA in association with diabetes mellitus and/or sensorineural deafness.1 WFS1 encodes for an endoplasmic reticulum transmembrane protein, Wolframin, which is highly expressed in retinal tissues, including retinal ganglion cells, the photoreceptor inner segments, and the inner nuclear layer (INL) of the human eye, and mouse Müller cells. (Source: Ophthalmology)



Various clinical conditions can mimic Crimean-Congo hemorrhagic fever in pediatric patients in endemic regions

Mon, 08 Feb 2016 00:00:00 +0100

Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne disease with high mortality. Many disorders can mimic CCHF. It is important to recognize the condition and to perform differential diagnosis in endemic countries. Twenty-one children aged 18 years or less with a preliminary diagnosis of CCHF were retrospectively evaluated. Real-time PCR and a confirmatory indirect immunofluorescence assay for negative results were performed. The diagnoses determined that 9 patients had (42.9%) CCHF; 7 patients had (33.3%) viral upper respiratory tract infections (URTI); 2 patients had (9.5%) brucellosis; 1 patients had (4.7%) periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome episode; 1 patient had (4.7%) cerebral palsy, diabetes insipidus, acute gastroenteritis, and hypern...



Diabetes insipidus following neurosurgery at a university hospital in Western Saudi Arabia.

Fri, 05 Feb 2016 10:16:02 +0100

CONCLUSION: In our study group, it was recognized  that permanent DI meant that our patients needed desmopressin for more than 3 months, which documented a more significant number than other studies. PMID: 26837398 [PubMed - in process] (Source: Saudi Medical Journal)

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ER stress and basement membrane defects combine to cause glomerular and tubular renal disease resulting from Col4a1 mutations in mice [RESEARCH ARTICLE]

Tue, 02 Feb 2016 00:00:00 +0100

ABSTRACT Collagen IV is a major component of basement membranes, and mutations in COL4A1, which encodes collagen IV alpha chain 1, cause a multisystemic disease encompassing cerebrovascular, eye and kidney defects. However, COL4A1 renal disease remains poorly characterized and its pathomolecular mechanisms are unknown. We show that Col4a1 mutations in mice cause hypotension and renal disease, including proteinuria and defects in Bowman's capsule and the glomerular basement membrane, indicating a role for Col4a1 in glomerular filtration. Impaired sodium reabsorption in the loop of Henle and distal nephron despite elevated aldosterone levels indicates that tubular defects contribute to the hypotension, highlighting a novel role for the basement membrane in vascular homeostasis by modulation ...



Novel large deletion in AVPR2 gene causing copy number variation in a patient with X-linked nephrogenic diabetes insipidus.

Fri, 29 Jan 2016 00:00:00 +0100

CONCLUSION: We report a novel deletion in a Chinese patient with congenital nephrogenic DI. We suggested that patients with suspected congenital DI should undergo genetic analysis of AVPR2 and AQP2 genes. A definitive diagnosis can benefit patient by treatment of hydrochlorothiazide and amiloride and avoiding unnecessary investigations. PMID: 26828532 [PubMed - as supplied by publisher] (Source: International Journal of Clinical Chemistry)



Diagnosis of diabetes insipidus observed in Swiss Duroc boars

Fri, 29 Jan 2016 00:00:00 +0100

Diabetes insipidus (DI) is a rare disease in humans and animals, which is caused by the lack of production, malfunction or dysfunction of the distal nephron to the antidiuretic effect of the antidiuretic hormo... (Source: BMC Veterinary Research)



A novel AVPR2 splice site mutation leads to partial X-linked nephrogenic diabetes insipidus in two brothers

Thu, 21 Jan 2016 00:00:00 +0100

Conclusion: We present here a unique case of partial X-linked NDI due to an AVPR2 splice site mutation; patients with diabetes insipidus of unknown etiology may harbor splice site mutations that are initially underestimated in their pathogenicity on sequence analysis. What is Known: • X-linked nephrogenic diabetes insipidus is caused by AVPR2 mutations, and disease severity can vary depending on the functional effect of the mutation. What is New: • We demonstrate here that a splice site mutation in AVPR2 leads to partial X-linked NDI in two brothers. ...



Inflammatory Myofibroblastic Tumor Presenting with Diabetes Insipidus in an Eight-Year-Old Boy: A Case Report.

Wed, 20 Jan 2016 19:51:03 +0100

Authors: Sarı E, Ataş E, Bulut EB, Sarı S, Akın O, Saldır M, Karslıoğlu Y, Yeşilkaya E Abstract Inflammatory myofibroblastic tumors (IMT) develop as a non-neoplastic proliferation of myofibroblasts in a myxoid to collagenous stroma admixed with inflammatory cells. The symptoms depend on the specific location of the tumor, which can be anywhere, but is particularly in the respiratory system. Thus, patients with IMT can present with a variety of findings. A pediatric patient with IMT who presented with cough, breathlessness, polyuria-polydipsia, and convulsions is described in this report. PMID: 26777048 [PubMed - in process] (Source: JCRPE Journal of Clinical Research in Pediatric Endocrinology)

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Extended endoscopic endonasal transsphenoidal approach for retrochiasmatic craniopharyngioma: Surgical technique and results

Wed, 20 Jan 2016 00:00:00 +0100

Conclusions: Our early experience suggests that the extended endonasal endoscopic approach is a reasonable option for removal of the retrochiasmal craniopharyngiomas. Compared to other surgical approaches, it provides better opportunities for greater tumor removal and visual improvement without any increase in risks. (Source: Journal of Pediatric Neurosciences)



Gestational diabetes insipidus. Case Report.

Fri, 15 Jan 2016 07:45:03 +0100

In this study, a case of 34-year-old female patient with diabetes insipidus associated with pregnancy was reported. We discussed process of diagnosis and treatment with particular emphasis on the monitoring of water-electrolyte imbalance during labor. PMID: 26707038 [PubMed - as supplied by publisher] (Source: Neuroendocrinology Letters)



Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview

Tue, 12 Jan 2016 18:16:03 +0100

A young male presented with panhypopituitarism (including diabetes insipidus) and temporal lobe epilepsy. A histology specimen of cutaneous papules was diagnostic of non-Langerhans histiocytosis. The diagnosis of xanthoma granulomata was considered based on the clinical and brain MRI findings. Brain lesions significantly worsened over time despite radiotherapy until anakinra induced a complete clinical and radiological remission of all active lesions. Although a single case, the outcome of this patient with xanthoma disseminatum treated with an interleukin-1 receptor antagonist opens and strengthens new and recent physiopathogenic and treatment perspectives for the otherwise difficult-to-treat non-Langerhans cell histiocytosis. Similar results with anakinra have been observed in patients w...



Acute myeloid leukemia with t(3;8)(q26;q24) complicated by diabetes insipidus

Mon, 11 Jan 2016 00:00:00 +0100

(Source: Annals of Hematology)



Wolfram Syndrome: Diagnosis, Management, and Treatment

Thu, 07 Jan 2016 00:00:00 +0100

Abstract Wolfram syndrome is a rare genetic disorder characterized by juvenile-onset diabetes mellitus, diabetes insipidus, optic nerve atrophy, hearing loss, and neurodegeneration. Although there are currently no effective treatments that can delay or reverse the progression of Wolfram syndrome, the use of careful clinical monitoring and supportive care can help relieve the suffering of patients and improve their quality of life. The prognosis of this syndrome is currently poor, and many patients die prematurely with severe neurological disabilities, raising the urgency for developing novel treatments for Wolfram syndrome. In this article, we describe natural history and etiology, provide recommendations for diagnosis and clinical management, and introduce new treatments for Wol...

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Binostril versus mononostril approaches in endoscopic transsphenoidal pituitary surgery: clinical evaluation and cadaver study.

Fri, 01 Jan 2016 00:00:00 +0100

CONCLUSIONS Because of maneuverability of instruments and a wider view in the sphenoid sinus, the binostril technique is superior for resection of large tumors with parasellar and suprasellar expansion and tumors requiring extended approaches. The mononostril technique is preferable for tumors with limited extension in the intra- and suprasellar area. PMID: 26722858 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)



Clinical management of teratoma, a rare hypothalamic-pituitary neoplasia

Wed, 23 Dec 2015 00:00:00 +0100

Abstract Intracranial teratomas are rare and comprise about 0.5 % of all intracranial tumours. Actually, a total of 15 cases of sellar-suprasellar teratoma have been described in the last 24 years. Although rare, hypothalamic-pituitary teratomas should be taken into account in the differential diagnosis of hypothalamic-pituitary region tumours. The current review focuses on the clinical and therapeutic management of pituitary region teratomas. Teratomas occur more frequently in children and young adults than in the older population and in males as compared to females. Symptoms at diagnosis are usually neurological defects, diabetes insipidus and hypopituitarism. Teratoma diagnosis can be suggested though neuroimaging findings. Magnetic resonance imaging remains the preferred...



Delayed Hyponatremia Is the Most Common Cause of 30-Day Unplanned Readmission After Transsphenoidal Surgery for Pituitary Tumors

Tue, 22 Dec 2015 10:46:46 +0100

CONCLUSION: This study establishes a quality benchmark for readmission rates after transsphenoidal surgery for pituitary lesions and identifies delayed hyponatremia as the primary cause. Implementation of an outpatient care pathway for managing hyponatremia may improve readmission rates. ABBREVIATIONS: CMS, Centers for Medicare and Medicaid Services DI, diabetes insipidus SIADH, syndrome of inappropriate antidiuretic hormone (Source: Neurosurgery)



The confounding effect of the development of idiopathic orthostatic edema and thyrotoxcosis on weight fluctuation related to effects on free water clearance in a woman with long-standing surgically induced panhypopituitarism and diabetes insipidus.

Sun, 20 Dec 2015 22:56:44 +0100

CONCLUSIONS: Hypopituitarism and diabetes insipidus controlled with hormone replacement do not protect against fluid retention from idiopathic edema. PMID: 26152015 [PubMed - indexed for MEDLINE] (Source: Clinical and Experimental Obstetrics and Gynecology)



[Lithium treatment and potential long-term side effects: a systematic review of the literature].

Sun, 20 Dec 2015 14:40:07 +0100

CONCLUSIONS: Lithium remains a fundamental tool for the treatment of BD. Clinicians should know potential side effects (renal, endocrine and dermatological) associated with long-term treatment with lithium, for a correct management of the patient. A specialist referral is often necessary; the question is how to deal with long-term side effects more than whether or not withdrawing lithium. This decision should remain a psychiatrist's competence. PMID: 24572579 [PubMed - indexed for MEDLINE] (Source: Rivista di Psichiatria)

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Therapeutic Dose Monitoring for Linezolid in a Patient with MRSA Pneumonia with Bacteremia in Diabetes Insipidus

Sat, 19 Dec 2015 00:00:00 +0100

Conclusion In spite of the low level of LZD AUC24/MIC caused by the high CrCl with DI, MRSA pneumonia with concomitant bacteremia was successfully treated with LZD. (Source: Infectious Diseases and Therapy)



Outcomes in pituitary surgery in Nelson's syndrome - therapeutic pitfalls.

Fri, 18 Dec 2015 02:48:03 +0100

CONCLUSIONS: Transsphenoidal microsurgical removal of pituitary adenomas is a safe and effective treatment of Nelson' syndrome. (Endokrynol Pol 2015; 66 (6): 504-513). PMID: 26662649 [PubMed - in process] (Source: Endokrynologia Polska)



A case of syndrome of inappropriate scretion of anti-diuretic hormone associated with sodium valproate.

Fri, 11 Dec 2015 22:31:07 +0100

We report a rare case of the concurrent manifestation of central diabetes insipidus (CDI) and type 2 diabetes mellitus (DM). A 56 year-old man was diagnosed as a type 2 DM on the basis of hyperglycemia with polyuria and polydipsia at a local clinic two months ago and started an oral hypoglycemic medication, but resulted in no symptomatic improvement at all. Upon admission to the university hospital, the patient's initial fasting blood sugar level was 140 mg/dL, and he showed polydipsic and polyuric conditions more than 8 L urine/day. Despite the hyperglycemia controlled with metformin and diet, his symptoms persisted. Further investigations including water deprivation test confirmed the coexisting CDI of unknown origin, and the patient's symptoms including an intense thirst were markedly i...



Polyuria with the Concurrent manifestation of Central Diabetes Insipidus (CDI) & Type 2 Diabetes Mellitus (DM).

Fri, 11 Dec 2015 22:31:07 +0100

We report a rare case of the concurrent manifestation of central diabetes insipidus (CDI) and type 2 diabetes mellitus (DM). A 56 year-old man was diagnosed as a type 2 DM on the basis of hyperglycemia with polyuria and polydipsia at a local clinic two months ago and started an oral hypoglycemic medication, but resulted in no symptomatic improvement at all. Upon admission to the university hospital, the patient's initial fasting blood sugar level was 140 mg/dL, and he showed polydipsic and polyuric conditions more than 8 L urine/day. Despite the hyperglycemia controlled with metformin and diet, his symptoms persisted. Further investigations including water deprivation test confirmed the coexisting CDI of unknown origin, and the patient's symptoms including an intense thirst were markedly i...



Renal effects of prostaglandins and cyclooxygenase-2 inhibitors.

Fri, 11 Dec 2015 22:31:07 +0100

Authors: Kim GH Abstract Prostaglandins (PGs) with best-defined renal functions are PGE2 and prostacyclin (PGI2). These vasodilatory PGs increase renal blood flow and glomerular filtration rate under conditions associated with decreased actual or effective circulating volume, resulting in greater tubular flow and secretion of potassium. Under conditions of decreased renal perfusion, the production of renal PGs serves as an important compensatory mechanism. PGI2 (and possibly PGE2) increases potassium secretion mainly by stimulating secretion of renin and activating the renin-angiotensin system, which leads to increased secretion of aldosterone. In addition, PGE2 is involved in the regulation of sodium and water reabsorption and acts as a counterregulatory factor under conditions of...

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Dysregulation of Renal Cyclooxygenase-2 in Rats with Lithium-induced Nephrogenic Diabetes Insipidus.

Fri, 11 Dec 2015 22:31:07 +0100

In conclusion, there is a differential regulation of COX-2 between cortex and inner medulla in Li-NDI and urinary PGE2 excretion is increased in Li-NDI, possibly due to an increased renal production. This may suggest that increased renal production of PGE2 could play a role in modulating water reabsorption in the renal collecting duct in Li-NDI. PMID: 24459504 [PubMed] (Source: Electrolyte and Blood Pressure)



A Case of Nephrogenic Diabetes Insipidus During Transsphenoidal Pituitary Adenomectomy

Fri, 11 Dec 2015 06:12:30 +0100

No abstract available (Source: Journal of Neurosurgical Anesthesiology)



Early central diabetes insipidus; an ominous sign in post-cardiac arrest patients

Thu, 10 Dec 2015 00:00:00 +0100

Central diabetes insipidus (CDI) after cardiac arrest is not well described. Thus, we aim to study the occurrences, outcomes and risk factors of central diabetes insipidus (CDI) of survivors after out-of-hospital cardiac arrest (OHCA). (Source: Journal of Critical Care)



Early central diabetes insipidus: An ominous sign in post–cardiac arrest patients

Thu, 10 Dec 2015 00:00:00 +0100

Central diabetes insipidus (CDI) after cardiac arrest is not well described. Thus, we aim to study the occurrences, outcomes, and risk factors of CDI of survivors after out-of-hospital cardiac arrest (OHCA). (Source: Journal of Critical Care)



Antihypertensive therapy in patients on chronic lithium treatment for bipolar disorders

Thu, 03 Dec 2015 08:27:59 +0100

Bipolar disorders are chronic conditions treated with lithium, which exerts deleterious effects on the kidney, among which nephrogenic diabetes insipidus, tubular acidosis and ultimately chronic kidney disease. Conversely, drugs that alter renal function can modify its serum levels and lead to the potentially fatal lithium intoxication. A search in the main library databases from 1975 to 2015 to identify interactions between antihypertensive drugs and lithium using the Population Intervention Comparison Outcome strategy provided only 30 reports of lithium intoxication. A regression analysis showed that the severity of lithium intoxication was significantly predicted by female, age, and use of certain classes of antihypertensive agents. A model including certain albeit not all diuretics and...

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Response to low dose indomethacin in two children with nephrogenic diabetes insipidus.

Wed, 02 Dec 2015 11:22:04 +0100

Authors: Dayal D, Verma Attri S, Kumar Bhalla A, Kumar R Abstract Two children with nephrogenic diabetes insipidus (NDI) were treated with oral indomethacin (0.75-1.2 mg/kg/day) three times a day for a mean duration of 3 yrs. Remission occurred in both patients in terms of achieving a normal fluid balance and body growth and the drug was withdrawn in one patient after 2 yrs. The treatment was well tolerated and no side effects were noted. The mean duration of follow-up was 6.5 yrs. These long-term observations of a favourable response to low dose indomethacin in 2 children with NDI need to be tested on larger number of patients. PMID: 26615585 [PubMed - in process] (Source: Diabetes Metab)



Reversible diabetes insipidus in a patient with multiple myeloma.

Mon, 30 Nov 2015 21:52:01 +0100

Authors: Shand JA, McGrath N PMID: 26149905 [PubMed - indexed for MEDLINE] (Source: New Zealand Medical Journal)



Vasopressin regulates renal calcium excretion in humans

Mon, 30 Nov 2015 00:00:00 +0100

The objective of this study was to assess whether AVP modulates renal calcium excretion in humans. We analyzed calcium, potassium, and sodium fractional excretion in eight patients affected by insipidus diabetes (nephrogenic or central) under acute vasopressin receptor agonist action and in 10 patients undergoing oral water load test affected or not by inappropriate antidiuretic hormone secretion (SIADH). Synthetic V2 receptor agonist (dDAVP) reduced significantly calcium fractional excretion from 1.71% to 0.58% (P < 0.05) in patients with central diabetes insipidus. In patients with nephrogenic diabetes insipidus (resistant to AVP), calcium fractional excretion did not change significantly after injection (0.48–0.68%, P = NS). In normal subjects undergoing oral water load test, c...



P2Y12 Receptor Localizes in the Renal Collecting Duct and Its Blockade Augments Arginine Vasopressin Action and Alleviates Nephrogenic Diabetes Insipidus

Mon, 30 Nov 2015 00:00:00 +0100

In conclusion, pharmacologic blockade of renal P2Y12-R increases urinary concentrating ability by augmenting the effect of AVP on the kidney and ameliorates lithium-induced NDI by potentiating the action of AVP on the CD. This strategy may offer a novel and effective therapy for lithium-induced NDI. (Source: Journal of the American Society of Nephrology : JASN)



Gamma knife radiosurgery of craniopharyngioma: results of 30 cases treated at Nagoya Radiosurgery Center.

Sat, 21 Nov 2015 07:47:12 +0100

Authors: Kobayashi T, Tsugawa T, Hatano M, Hashizume C, Mori Y, Shibamoto Y Abstract Evaluation of 30 cases of craniopharyngioma treated by Gamma Knife at Nagoya Radiosurgery Center (NRC), Nagoya Kyoritsu Hospital since July, 2004 has been made. The mean volume of the tumor was 2.64 ml, which was treated with the marginal dose of 11.7 Gy. Mean follow-up period was 79.9 months. The effects were evaluated by MRI findings, neuro-endocrine and hypothalamic signs and symptoms, complications and KPS every 3~6 months. As the results, complete remission was obtained in 8, partial remission in 12, no change in 6, progression in 3, in which two died by hypothalamic invasion. Tumor response rate was 68.9% and control rate 87.9%. Actuarial survival was 96% at 5 and 86% at 10 years. However, pr...

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Dysnatraemia is frequently a poor prognostic indicator in patients with acute subarachnoid hemorrhage having targeted sodium management.

Fri, 20 Nov 2015 12:45:54 +0100

CONCLUSIONS: Our 10-year targeted sodium management regimen in acute SAH patients showed that dysnatraemias were frequent, predominantly hyponatraemia of which the more usual causes were CSW and not SIADH. Hypernatraemia was shown to be an independent risk factor for inpatient mortality and poor outcome. PMID: 26496416 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgical Sciences)



Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature.

Fri, 20 Nov 2015 12:44:21 +0100

CONCLUSION: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH. PMID: 26539311 [PubMed] (Source: Surgical Neurology International)



Central diabetes insipidus in infancy with or without hypothalamic adipsic hypernatremia syndrome: early identification and outcome.

Fri, 20 Nov 2015 00:00:00 +0100

CONCLUSION: Neonatal CDI may be transient or permanent. These vulnerable patients have high rates of comorbidity and require careful monitoring. PMID: 26588450 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)



Early and late endocrine effects in pediatric central nervous system diseases.

Thu, 19 Nov 2015 03:17:14 +0100

Authors: Aslan IR, Cheung CC Abstract Endocrinopathies are frequently linked to central nervous system disease, both as early effects prior to the disease diagnosis and/or late effects after the disease has been treated. In particular, tumors and infiltrative diseases of the brain and pituitary, such as craniopharyngioma, optic pathway and hypothalamic gliomas, intracranial germ cell tumor, and Langerhans cell histiocytosis, can present with abnormal endocrine manifestations that precede the development of neurological symptoms. Early endocrine effects include diabetes insipidus, growth failure, obesity, and precocious or delayed puberty. With improving prognosis and treatment of childhood brain tumors, many survivors experience late endocrine effects related to medical and surgica...



Late onset of familial neurogenic diabetes insipidus in monozygotic twins.

Wed, 18 Nov 2015 17:29:17 +0100

CONCLUSIONS: The present paper supports the notion that molecular genetic evaluation should be performed in all patients with familial occurrence of DI regardless of the clinical results. PMID: 24156710 [PubMed - indexed for MEDLINE] (Source: Endocrine Regulations)

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Diabetes insipidus in children

Wed, 18 Nov 2015 17:28:19 +0100

Journal Name: Journal of Pediatric Endocrinology and MetabolismIssue: Ahead of print (Source: Journal of Pediatric Endocrinology and Metabolism)



An unusual case of hereditary nephrogenic diabetes insipidus (HNDI) affecting mother and daughter

Wed, 18 Nov 2015 17:28:19 +0100

Journal Name: Journal of Pediatric Endocrinology and MetabolismIssue: Ahead of print (Source: Journal of Pediatric Endocrinology and Metabolism)



Simvastatin increases AQP2 urinary excretion in hypercholesterolemic patients: A pleiotropic effect of interest for patients with impaired AQP2 trafficking.

Tue, 17 Nov 2015 00:00:00 +0100

In conclusions, this study strongly suggests that statins may effectively enhance the efficacy of current pharmacological treatment of patients with urine concentrating defects caused by defective AQP2 plasma membrane trafficking, like X-NDI. This article is protected by copyright. All rights reserved. PMID: 26575415 [PubMed - as supplied by publisher] (Source: Clinical Pharmacology and Therapeutics)



Early-Onset Central Diabetes Insipidus due to Compound Heterozygosity for AVP Mutations

Sat, 14 Nov 2015 18:29:09 +0100

Conclusions: The early polyuria in recessive central diabetes insipidus contrasts with the delayed presentation in patients with monoallelic AVP mutations. This diagnosis needs to be considered in infants with very early onset of polyuria-polydipsia and no brain malformation, even if there is no consanguinity and regardless of whether the posterior pituitary is visible or not on imaging. In addition to informing family counseling, making a molecular diagnosis eliminates the need for repeated imaging studies.Horm Res Paediatr (Source: Hormone Research in Paediatrics)



Endocrine manifestations in a monocentric cohort of 64 patients with Erdheim-Chester disease.

Fri, 13 Nov 2015 00:00:00 +0100

CONCLUSION: Endocrine involvement is very frequent in ECD and should carefully be evaluated at diagnosis and during follow-up. PMID: 26565949 [PubMed - as supplied by publisher] (Source: Diabetes Metab)

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A Case of Nephrogenic Diabetes Insipidus Associated with Anidulafungin Treatment

Thu, 12 Nov 2015 00:00:00 +0100

Publication date: December 2015 Source:Canadian Journal of Diabetes, Volume 39, Issue 6 Author(s): Jasmine Bahrami, Leslie Martin, Natalia McInnes (Source: Canadian Journal of Diabetes)



Chinese Erdheim-Chester disease: clinical-pathology-PET/CT updates.

Wed, 04 Nov 2015 20:34:03 +0100

We reported a case of ECD and reviewed the clinical features of 13 cases of ECD reported so far in China. A 53-year-old male was diagnosed with central diabetes insipidus in March 2014, followed by fever, splenomegaly and anemia in July 2014. His initial pituitary magnetic resonance imaging (MRI) revealed the absence of high signal at T1-weighted image in posterior pituitary without any lesion. A further positron emission tomography/computer tomography (PET/CT) images showed elevated metabolic activity of (18)F-2-fluro-D-deoxy-glucose (FDG) and low (13)N-NH3 uptake in the posterior pituitary, and multi-organ involvement. Biopsy at right femur lesion revealed that granulomatous infiltration of foamy histiocytes and Touton giant cells surrounded by fibrosis tissues. Immunohistochemistry stai...



Transient diabetes insipidus in pregnancy.

Wed, 04 Nov 2015 20:34:03 +0100

Authors: Marques P, Gunawardana K, Grossman A Abstract UNLABELLED: Gestational diabetes insipidus (DI) is a rare complication of pregnancy, usually developing in the third trimester and remitting spontaneously 4-6 weeks post-partum. It is mainly caused by excessive vasopressinase activity, an enzyme expressed by placental trophoblasts which metabolises arginine vasopressin (AVP). Its diagnosis is challenging, and the treatment requires desmopressin. A 38-year-old Chinese woman was referred in the 37th week of her first single-gestation due to polyuria, nocturia and polydipsia. She was known to have gestational diabetes mellitus diagnosed in the second trimester, well-controlled with diet. Her medical history was unremarkable. Physical examination demonstrated decreased skin turgor;...



A Unilateral Subfrontal Basal Interhemispheric Approach to Large Craniopharyngiomas

Sun, 01 Nov 2015 05:00:00 +0100

Conclusions: The present results suggest that the unilateral subfrontal basal interhemispheric approach provides direct and wide exposure of large craniopharyngiomas. Anatomic preservation of the pituitary stalk, hypothalamic structure, perforating vessels, anterior communicating complex, the visual pathway, and the olfactory nerves is often possible. (Source: Neurosurgery Quarterly)



A case of idiopathic diabetes insipidus presented with bilateral hydroureteronephrosis and neurogenic bladder: A pediatric case report and literature review.

Sun, 01 Nov 2015 00:00:00 +0100

We report a case of an 8-year-old male patient with CDI with severe bilateral non-obstructive hydronephrosis and megaureter. Dramatic clinical and radiological responses to dDAVP treatment were achieved and therapy reduced urine volume and led to marked radiological improvement in hydronephrosis. PMID: 26600892 [PubMed - as supplied by publisher] (Source: Canadian Urological Association Journal)

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Surgery induced hypopituitarism in acromegalic patients: a systematic review and meta-analysis of the results

Fri, 30 Oct 2015 10:16:29 +0100

Conclusion Our study provides new data on the incidence rates of hypopituitarism, specific pituitary axis deficiencies and diabetes insipidus after surgical treatment of acromegaly. Somatotroph function appears to be more prone to deficit than the other axes. However, there is a high heterogeneity between studies and several factors may influence the incidence of hypopituitarism. (Source: Pituitary)



AVP - NPII gene mutations and clinical characteristics of the patients with autosomal dominant familial central diabetes insipidus

Fri, 30 Oct 2015 10:16:24 +0100

Conclusion Familial central DI is a progressive disease, and age of onset of symptoms can differ depending on the mutation. Bright spot on pituitary MRI might be present at onset, but become invisible over time. Genetic testing and appropriate counseling should be given in familial cases of central DI to ensure adequate treatment, and to avoid chronic water deprivation that might result in growth retardation in childhood. (Source: Pituitary)



Diabetes insipidus: An unusual presentation of adenocarcinoma of the lung in a patient with no identifiable lung mass

Fri, 30 Oct 2015 00:00:00 +0100

Conclusion: Clinicians should be aware of malignancies that are well known to metastasize to the posterior pituitary. Conversely, since not every patient presents with symptoms of metastasis, there is a need to recognize the clinical syndromes (e. g., diabetes insipidus-like symptoms or more subtle symptoms like cranial nerve palsies) associated with potential metastasis to the pituitary. (Source: North American Journal of Medical Sciences)



Endocrine Dysfunction in Diamond–Blackfan Anemia (DBA): A Report from the DBA Registry (DBAR)

Sat, 24 Oct 2015 13:07:13 +0100

ConclusionsEndocrine dysfunction is common in DBA, as early as the teenage years. Although prevalence is highest in transfused patients, patients taking glucocorticoids or in remission also have endocrine dysfunction. Longitudinal studies are needed to better understand the etiology and true prevalence of these disorders. (Source: Pediatric Blood and Cancer)



Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome After Traumatic Brain Injury

Sun, 18 Oct 2015 00:00:00 +0100

Combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury (TBI) is rare, is characterized by massive polyuria leading to severe water and electrolyte disturbances, and usually is associated with very high mortality mainly as a result of delayed diagnosis and improper management. (Source: World Neurosurgery)

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Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome following Traumatic Brain Injury

Sat, 17 Oct 2015 00:00:00 +0100

Conclusions For combined DI and CSW after TBI, massive polyuria is a major typical presentation; and intensive monitoring of fluid and sodium status is key for timely diagnosis as are proper sodium chloride supplementation and cortisone acetate and vasopressin co-administration for management to achieve favorable outcome. (Source: World Neurosurgery)



Central diabetes insipidus following cardiopulmonary arrest in a dog

Fri, 16 Oct 2015 00:00:00 +0100

Abstract ObjectiveTo describe a clinical case of transient central diabetes insipidus (CDI) occurring post cardiopulmonary arrest (CPA) in a dog. Case SummaryAn 8‐week‐old dog presented for intensive care after successful resuscitation following CPA. The patient exhibited neurologic deficits at initial presentation and over the following days developed marked polyuria, isosthenuria, and low urine osmolality. Treatment with synthetic vasopressin resulted in a reduction in urine output, increase in urine specific gravity (>50%), and increase in urine osmolality, suggesting a diagnosis of partial CDI. Clinical signs resolved over the following weeks and treatment was discontinued. New or Unique Information ProvidedCPA has been described as a cause of ischemic injury to the pituitary gl...



Evaluation of electrocardiographic parameters in patients with diabetes insipidus.

Wed, 14 Oct 2015 00:00:00 +0100

CONCLUSIONS: DI patients without any cardiovascular disease or risk factors displayed significantly shorter QRS duration and increased p wave dispersion compared with controls. PMID: 26466837 [PubMed - as supplied by publisher] (Source: Wiener Klinische Wochenschrift)



Polyuria and cerebral vasospasm after aneurysmal subarachnoid hemorrhage

Tue, 13 Oct 2015 00:00:00 +0100

Background: Natriuresis with polyuria is common after aneurysmal subarachnoid hemorrhage (aSAH). Previous studies have shown an increased risk of symptomatic cerebral vasospasm or delayed cerebral ischemia (DCI) in patients with hyponatremia and/or the cerebral salt wasting syndrome (CSW). However, natriuresis may occur in the absence of hyponatremia or hypovolemia and it is not known whether the increase in DCI in patients with CSW is secondary to a concomitant hypovolemia or because the physiology that predisposes to natriuretic peptide release also predisposes to cerebral vasospasm. Therefore, we investigated whether polyuria per se was associated with vasospasm and whether a temporal relationship existed. Methods: A retrospective review of patients with aSAH was performed. Exclusion cr...



Postoperative diabetes insipidus in craniopharyngiomas: Effective management by adherence to a strict protocol

Tue, 06 Oct 2015 00:00:00 +0100

Kiyoshi SaitoNeurology India 2015 63(5):659-660 (Source: Neurology India)

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Management of diabetes insipidus in craniopharyngiomas

Tue, 06 Oct 2015 00:00:00 +0100

Deepu BanerjiNeurology India 2015 63(5):661-662 (Source: Neurology India)



Evaluation of a protocol-based treatment strategy for postoperative diabetes insipidus in craniopharyngioma

Tue, 06 Oct 2015 00:00:00 +0100

Conclusion: A strict protocol based management results in better control of postoperative DI in CP. (Source: Neurology India)



Cardiac abnormalities and Wolfram (DIDMOAD) syndrome: a case report

Thu, 01 Oct 2015 00:00:00 +0100

We report a child who presented with cyanotic heart disease and was operated twice for cardiac lesion. At the age of 6 years, the case developed osmotic symptoms and, during evaluation, diabetes insipidus, optic atrophy and deafness were reported. DIDMOAD syndrome with atrial septal defect, severe pulmonary stenosis and hypoplastic right ventricle is not previously reported. In addition to our case, in a literature survey on all reported cases of DIDMOAD syndrome (more than 700), only 11 cases with cardiac abnormalities have been stated. Common abnormalities found are pulmonary stenosis, followed by tetralogy of Fallot and ventricular septal defects. Impact of DIDMOAD syndrome on cardiovascular development needs to be elucidated. (Source: International Journal of Diabetes in Developing Co...



Ectopic germinoma involving multiple midline and paramedian structures outside the pineal gland or hypophyseal region of the brain prior to tumor development

Sun, 27 Sep 2015 00:00:00 +0100

A previously healthy 29-year-old Japanese male presented with a two-month history of hypersomnia, which had gradually developed and deteriorated and he could not continue his job as a system engineer. On admission, no other neurological deficts were observed. There were no symptoms indicative of diabetes insipidus, such as polyuria/nocturia or polydipsia. Brain MRI showed multiple T1-iso- or -hypo-intensities, and T2-hyperintensity lesions without contrast enhancement in the dorsal medulla (Fig. (Source: Journal of the Neurological Sciences)



Novel mutations associated with nephrogenic diabetes insipidus. A clinical-genetic study

Sat, 19 Sep 2015 00:27:53 +0100

Conclusion: Genetic analysis presumably confirmed the diagnosis of nephrogenic diabetes insipidus in every patient of the studied cohort. We emphasize that we detected a high presence (50 %) of heterozygous females with clinical NDI symptoms. What is known: • In most cases (90 %), inherited nephrogenic diabetes insipidus (NDI) is an X-linked disease, caused by mutations in the AVPR2 gene. • In rare occasions (10 %), it is caused by mutations in the AQP2 gene. What is new: • In this ...

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Adipsia in a Diabetes Insipidus Patient

Fri, 11 Sep 2015 12:28:02 +0100

We describe here a very infrequent oncological case of dangerous adipsic diabetes insipidus in a young girl who survived.Case Rep Oncol 2015;8:385-388 (Source: Case Reports in Oncology)



Case report of severe Cushing’s syndrome in medullary thyroid cancer complicated by functional diabetes insipidus, aortic dissection, jejunal intussusception, and paraneoplastic dysautonomia: remission with sorafenib without reduction in cortisol concentration

Wed, 09 Sep 2015 00:00:00 +0100

Conclusion: Extreme cortisol concentration may have contributed to aortic dissection and suppressed antidiuretic hormone secretion; which combined with hypokalemia due cortisol activation of mineralocorticoid receptors, manifested as diabetes insipidus. This is the first report of paraneoplastic dysautonomia and jejunal intussusception in medullary thyroid cancer, they may be related to medullary thyroid cancer’s neuroendocrine origin and metastasis, respectively. Remission of Cushing’s syndrome without measurable reduction in cortisol concentration suggests a novel cortisol-independent mechanism of action or assay cross-reactivity. Normalization of dehydroepiandrostenedione sulfate and elevation of progesterone suggest inhibition of 17-hydroxylase and 21-hydroxylase activities by sora...



Desmopressin as an adjuvant to opioids or NSAIDs in treatment of renal colic: a nationwide register‐based study

Wed, 09 Sep 2015 00:00:00 +0100

ConclusionIn summary, these real‐life prescription data provide exact epidemiological measures on desmopressin utilisation in renal colic. Copyright © 2015 John Wiley & Sons, Ltd. (Source: Pharmacoepidemiology and Drug Safety)



Pediatric Longitudinal Clivus Fracture: Survival With Minimal Morbidity

Tue, 08 Sep 2015 00:00:00 +0100

We report a case of longitudinal clivus fracture in a teenager with survival and complete neurological recovery. Our case is the first pediatric case of longitudinal clivus fracture caused by frontal impact and the first described pediatric case associated with transient diabetes insipidus (DI). (Source: Journal of Intensive Care Medicine)



Teaching NeuroImages: Erdheim-Chester disease (polyostotic sclerosing histiocytosis)

Mon, 07 Sep 2015 00:00:00 +0100

A 34-year-old man presented with progressive visual loss, diplopia, headache, intermittent fever, and significant weight loss for 7 months. Physical examination revealed bilateral visual loss and exophthalmos with complete ophthalmoplegia. Imaging demonstrated pachymeningeal enhancement, soft tissue infiltration in the retro-orbital spaces and multiple organs including kidneys and abdominal aorta, and mixed osteosclerotic and osteolytic lesions in bilateral femoral heads1 (figure). Pathologic study of the retro-orbital lesion revealed foamy histiocytes negative for CD1a and S100,2 consistent with Erdheim-Chester disease (ECD). The patient was treated with IV methylprednisolone followed by cyclosporine with minimal benefit. Four months later, he died due to sudden unexplained death during s...

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Diabetes insipidus in children

Thu, 03 Sep 2015 00:00:00 +0100

Journal Name: Journal of Pediatric Endocrinology and MetabolismVolume: 29Issue: 1Pages: 39-45 (Source: Journal of Pediatric Endocrinology and Metabolism)



Pure Endoscopic Endonasal Transsphenoidal Approach for Nonfunctioning Pituitary Adenomas in the Elderly: Surgical Outcomes and Complications in 158 Patients

Wed, 02 Sep 2015 00:00:00 +0100

Conclusion NFPAs in the elderly can be resected via a pure endoscopic technique with low morbidity and mortality. (Source: World Neurosurgery)



Endonasal Endoscopic Transsphenoidal Approach to Lesions of the Sellar Region in Pediatric Patients

Tue, 01 Sep 2015 05:00:00 +0100

Conclusions: Endoscopic endonasal (transnasal) transsphenoidal approach (EETA) provides a safe and effective surgical option with low morbidity and mortality in pediatric patients. (Source: Journal of Craniofacial Surgery)



Comparison of Pituitary Adenomas in Elderly and Younger Adults: Clinical Characteristics, Surgical Outcomes, and Prognosis

Tue, 01 Sep 2015 00:00:00 +0100

ConclusionAvoiding misdiagnosis and emergency surgery is critical for frail elderly adults with multiple comorbidities. With early active management, sufficient preoperative preparation, and multidisciplinary collaboration, the long‐term outcomes and prognosis of elderly adults with PAs are comparable with those of younger adults. (Source: Journal of the American Geriatrics Society)



Cyclophosphamide-induced vasopressin-independent activation of aquaporin-2 in the rat kidney

Tue, 01 Sep 2015 00:00:00 +0100

Because cyclophosphamide-induced hyponatremia was reported to occur without changes in plasma vasopressin in a patient with central diabetes insipidus, we hypothesized that cyclophosphamide or its active metabolite, 4-hydroperoxycyclophosphamide (4-HC), may directly dysregulate the expression of water channels or sodium transporters in the kidney. To investigate whether intrarenal mechanisms for urinary concentration are activated in vivo and in vitro by treatment with cyclophosphamide and 4-HC, respectively, we used water-loaded male Sprague-Dawley rats, primary cultured inner medullary collecting duct (IMCD) cells, and IMCD suspensions prepared from male Sprague-Dawley rats. In cyclophosphamide-treated rats, significant increases in renal expression of aquaporin-2 (AQP2) and Na-K-2Cl cot...

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Identification of a Novel Deletion in AVP-NPII Gene in a Patient with Central Diabetes Insipidus.

Tue, 01 Sep 2015 00:00:00 +0100

Authors: Deniz F, Acar C, Saglar E, Erdem B, Karaduman T, Yonem A, Cagiltay E, Ay SA, Mergen H Abstract Central Diabetes Insipidus (CDI) is caused by a deficiency of antidiuretic hormone and characterized by polyuria, polydipsia and inability to concentrate urine. Our objective was to present the results of the molecular analyses of AVP-neurophysin II (AVP-NPII) gene in a large familial neurohypophyseal (central) DI pedigree. A male patient and his family members were analyzed and the prospective clinical data were collected. The proband applied to hospital for eligibility to be a recruit in Armed Forces. The patient had severe polyuria (20 L/day), polydipsia (20.5 L/day), fatique, and deep thirstiness. CDI was confirmed with the water deprivation-desmopressin test according to an ...