-
Hormone Research in Paediatrics 2012Central diabetes insipidus (CDI) is the end result of a number of conditions that affect the hypothalamic-neurohypophyseal system. The known causes include... (Review)
Review
Central diabetes insipidus (CDI) is the end result of a number of conditions that affect the hypothalamic-neurohypophyseal system. The known causes include germinoma/craniopharyngioma, Langerhans cell histiocytosis (LCH), local inflammatory, autoimmune or vascular diseases, trauma resulting from surgery or an accident, sarcoidosis, metastases and midline cerebral and cranial malformations. In rare cases, the underlying cause can be genetic defects in vasopressin synthesis that are inherited as autosomal dominant, autosomal recessive or X-linked recessive traits. The diagnosis of the underlying condition is challenging and raises several concerns for patients and parents as it requires long-term follow-up. Proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress to more sophisticated tools. Specifically, MRI identification of pituitary hyperintensity in the posterior part of the sella, now considered a clear marker of neurohypophyseal functional integrity, together with the careful analysis of pituitary stalk shape and size, have provided the most striking findings contributing to the diagnosis and understanding of some forms of 'idiopathic' CDI. MRI STIR (short-inversion-time inversion recovery sequencing) is a promising technology for the early identification of LCH-dependent CDI.
Topics: Adult; Age Factors; Animals; Antidiuretic Agents; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Nephrogenic; Diabetes Insipidus, Neurogenic; Drug Monitoring; Fluid Therapy; Humans; Infant
PubMed: 22433947
DOI: 10.1159/000336333 -
Pediatrics in Review Feb 2020
Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans
PubMed: 32005690
DOI: 10.1542/pir.2018-0337 -
American Journal of Kidney Diseases :... Jun 2022Hypertonic saline has been used for the treatment of hyponatremia for nearly a century. There is now general consensus that hypertonic saline should be used in patients...
Hypertonic saline has been used for the treatment of hyponatremia for nearly a century. There is now general consensus that hypertonic saline should be used in patients with hyponatremia associated with moderate or severe symptoms to prevent neurological complications. However, much less agreement exists among experts regarding other aspects of its use. Should hypertonic saline be administered as a bolus injection or continuous infusion? What is the appropriate dose? Is a central venous line necessary? Should desmopressin be used concomitantly and for how long? This article considers these important questions, briefly explores the historical origins of hypertonic saline use for hyponatremia, and reviews recent evidence behind its indications, dosing, administration modality and route, combined use with desmopressin to prevent rapid correction of serum sodium, and other considerations such as the need and degree for fluid restriction. The authors conclude by offering some practical recommendations for the use of hypertonic saline.
Topics: Deamino Arginine Vasopressin; Goals; Humans; Hyponatremia; Saline Solution, Hypertonic
PubMed: 34508830
DOI: 10.1053/j.ajkd.2021.07.020 -
Hong Kong Medical Journal = Xianggang... Aug 2019Enuresis is a common complaint in children, with a prevalence of around 15% at age 6 years. Evidence suggests that enuresis could affect neuropsychiatric development.... (Review)
Review
Enuresis is a common complaint in children, with a prevalence of around 15% at age 6 years. Evidence suggests that enuresis could affect neuropsychiatric development. The condition may represent an entire spectrum of underlying urological conditions. It is important to understand the difference between monosymptomatic and non-monosymptomatic enuresis. Primary monosymptomatic enuresis can be managed efficaciously with care in different settings, like primary care, specialist nursing, or paediatric specialists, while non-monosymptomatic enuresis requires more complex evaluation and treatment. The diagnosis, investigation, and management of the two types of enuresis are discussed in this review.
Topics: Antidiuretic Agents; Behavior Therapy; Child; Child, Preschool; Deamino Arginine Vasopressin; Humans; Nocturnal Enuresis; Physical Examination
PubMed: 31395789
DOI: 10.12809/hkmj197916 -
The New England Journal of Medicine Aug 2018The indirect water-deprivation test is the current reference standard for the diagnosis of diabetes insipidus. However, it is technically cumbersome to administer, and...
BACKGROUND
The indirect water-deprivation test is the current reference standard for the diagnosis of diabetes insipidus. However, it is technically cumbersome to administer, and the results are often inaccurate. The current study compared the indirect water-deprivation test with direct detection of plasma copeptin, a precursor-derived surrogate of arginine vasopressin.
METHODS
From 2013 to 2017, we recruited 156 patients with hypotonic polyuria at 11 medical centers to undergo both water-deprivation and hypertonic saline infusion tests. In the latter test, plasma copeptin was measured when the plasma sodium level had increased to at least 150 mmol per liter after infusion of hypertonic saline. The primary outcome was the overall diagnostic accuracy of each test as compared with the final reference diagnosis, which was determined on the basis of medical history, test results, and treatment response, with copeptin levels masked.
RESULTS
A total of 144 patients underwent both tests. The final diagnosis was primary polydipsia in 82 patients (57%), central diabetes insipidus in 59 (41%), and nephrogenic diabetes insipidus in 3 (2%). Overall, among the 141 patients included in the analysis, the indirect water-deprivation test determined the correct diagnosis in 108 patients (diagnostic accuracy, 76.6%; 95% confidence interval [CI], 68.9 to 83.2), and the hypertonic saline infusion test (with a copeptin cutoff level of >4.9 pmol per liter) determined the correct diagnosis in 136 patients (96.5%; 95% CI, 92.1 to 98.6; P<0.001). The indirect water-deprivation test correctly distinguished primary polydipsia from partial central diabetes insipidus in 77 of 105 patients (73.3%; 95% CI, 63.9 to 81.2), and the hypertonic saline infusion test distinguished between the two conditions in 99 of 104 patients (95.2%; 95% CI, 89.4 to 98.1; adjusted P<0.001). One serious adverse event (desmopressin-induced hyponatremia that resulted in hospitalization) occurred during the water-deprivation test.
CONCLUSIONS
The direct measurement of hypertonic saline-stimulated plasma copeptin had greater diagnostic accuracy than the water-deprivation test in patients with hypotonic polyuria. (Funded by the Swiss National Foundation and others; ClinicalTrials.gov number, NCT01940614 .).
Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Diagnosis, Differential; Female; Glycopeptides; Humans; Hyponatremia; Male; Middle Aged; Osmolar Concentration; Polydipsia; Polyuria; ROC Curve; Saline Solution, Hypertonic; Sensitivity and Specificity; Urine; Water Deprivation
PubMed: 30067922
DOI: 10.1056/NEJMoa1803760 -
The Lancet. Diabetes & Endocrinology Oct 2022Central diabetes insipidus is a rare neuroendocrine condition. Data on treatment-associated side-effects, psychological comorbidities, and incorrect management are...
Central diabetes insipidus from a patient's perspective: management, psychological co-morbidities, and renaming of the condition: results from an international web-based survey.
BACKGROUND
Central diabetes insipidus is a rare neuroendocrine condition. Data on treatment-associated side-effects, psychological comorbidities, and incorrect management are scarce. The aim of this study was to investigate patients' perspectives on their disease.
METHODS
This study used a cross-sectional, web-based, anonymous survey, developed by endocrinologists and patient representatives, to collect the opinions of patients with central diabetes insipidus on management and complications of their disease, psychological comorbidities, degree of knowledge and awareness of the condition among health-care professionals, and renaming the disease to avoid confusion with diabetes mellitus (diabetes).
FINDINGS
Between Aug 23, 2021, and Feb 7, 2022, 1034 patients with central diabetes insipidus participated in the survey. 91 (9%) participants were children and adolescents (37 [41%] girls and 54 [59%] boys; median age 10 years [IQR 6-15]) and 943 (91%) were adults (757 [80%] women and 186 [20%] men]; median age 44 years [34-54]). 488 (47%) participants had isolated posterior pituitary dysfunction and 546 (53%) had combined anterior and posterior pituitary dysfunction. Main aetiologies were idiopathic (315 [30%] of 1034 participants) and tumours and cysts (pre-surgical 217 [21%]; post-surgical 254 [25%]). 260 (26%; 95% CI [0·23-0·29]) of 994 patients on desmopressin therapy had hyponatraemia leading to hospitalisation. Patients who routinely omitted or delayed desmopressin to allow intermittent aquaresis had a significantly lower prevalence of hyponatraemia compared with those not aware of this approach (odds ratio 0·55 [95% CI 0·39-0·77]; p=0·0006). Of patients who had to be hospitalised for any medical reason, 71 (13%; 95% CI 0·10-0·16) of 535 patients did not receive desmopressin while in a fasting state (nil by mouth) without intravenous fluid replacement and reported symptoms of dehydration. 660 (64%; 0·61-0·67) participants reported lower quality of life, and 369 (36%; 0·33-0·39) had psychological changes subjectively associated with their central diabetes insipidus. 823 (80%; 0·77-0·82) participants encountered a situation where central diabetes insipidus was confused with diabetes mellitus (diabetes) by health-care professionals. 884 (85%; 0·83-0·88) participants supported renaming the disease; the most favoured alternative names were vasopressin deficiency and arginine vasopressin deficiency.
INTERPRETATION
This is the largest survey of patients with central diabetes insipidus, reporting a high prevalence of treatment-associated side-effects, mismanagement during hospitalisation, psychological comorbidities, and a clear support for renaming the disease. Our data are the first to indicate the value of routinely omitting or delaying desmopressin.
FUNDING
Swiss National Science Foundation, Swiss Academy of Medical Sciences, and G&J Bangerter-Rhyner-Foundation.
Topics: Adolescent; Adult; Arginine; Child; Cross-Sectional Studies; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Female; Humans; Hyponatremia; Internet; Male; Middle Aged; Morbidity; Quality of Life
PubMed: 36007536
DOI: 10.1016/S2213-8587(22)00219-4 -
American Family Physician Apr 2003Nocturnal enuresis is a common problem that can be troubling for children and their families. Recent studies indicate that nocturnal enuresis is best regarded as a group... (Review)
Review
Nocturnal enuresis is a common problem that can be troubling for children and their families. Recent studies indicate that nocturnal enuresis is best regarded as a group of conditions with different etiologies. A genetic component is likely in many affected children. Research also indicates the possibility of two subtypes of patients with nocturnal enuresis: those with a functional bladder disorder and those with a maturational delay in nocturnal arginine vasopressin secretion. The evaluation of nocturnal enuresis requires a thorough history, a complete physical examination, and urinalysis. Treatment options include nonpharmacologic and pharmacologic measures. Continence training should be incorporated into the treatment regimen. Use of a bed-wetting alarm has the highest cure rate and the lowest relapse rate; however, some families may have difficulty with this treatment approach. Desmopressin and imipramine are the primary medications used to treat nocturnal enuresis, but both are associated with relatively high relapse rates.
Topics: Adolescent; Adrenergic Uptake Inhibitors; Behavior Therapy; Child; Deamino Arginine Vasopressin; Enuresis; Humans; Imipramine; Renal Agents; Sleep
PubMed: 12722850
DOI: No ID Found -
Blood Sep 2022
Topics: Adolescent; Deamino Arginine Vasopressin; Exercise; Factor VIII; Hemophilia A; Humans; Male; von Willebrand Diseases
PubMed: 36074536
DOI: 10.1182/blood.2022017652 -
PLoS Medicine Oct 2019Desmopressin was approved by the Food and Drug Administration (FDA) in 1978 for use in diabetes insipidus and bleeding disorders, but it is also prescribed off-label for...
BACKGROUND
Desmopressin was approved by the Food and Drug Administration (FDA) in 1978 for use in diabetes insipidus and bleeding disorders, but it is also prescribed off-label for patients with nocturia. Quantifying the potential risks facing adult patients taking desmopressin has taken on added importance because a new intranasal formulation of desmopressin was approved by the FDA in 2017. Like the old formulation, the main active ingredient is desmopressin acetate, but the new formulation also contains an excipient designed to enhance absorption. Our objective was to quantify the rate of hyponatremia in routine clinical care for patients prescribed the older formulation of desmopressin.
METHODS AND FINDINGS
We conducted a population-based new-user cohort study from 1 February 2006 to 1 February 2017 using a nationwide commercial health plan database. Patients newly prescribed the older formulation of desmopressin were propensity-score (PS)-matched to patients newly prescribed oxybutynin. As a sensitivity analysis, tamsulosin was used as the comparator rather than oxybutynin. The primary outcome was a primary position diagnosis of hyponatremia. Proportional hazard models after 1:1 PS matching were used to estimate hazard ratios (HRs) and 95% confidence intervals (CI). We identified 3,137 adults who were newly prescribed desmopressin and matched them to 3,137 adults who were newly prescribed oxybutynin. Mean age was 70, 55% were male, 13% filled a prescription for a diuretic during the baseline time period, and the mean baseline sodium prior to receiving either study drug was 140 mmol/L (normal: 135-145). The rate of hyponatremia was 146 per 1,000 person-years for adults prescribed desmopressin compared to 11 per 1,000 person-years for adults prescribed oxybutynin, corresponding to a 13-fold higher rate (HR 13.19; 95% CI 6.69, 26.01; p < 0.01). When follow-up was truncated at 30 days, a similar increased rate was observed (HR 19.41; 95% CI 7.11, 52.99; p < 0.01). A higher rate of hyponatremia was also observed with desmopressin when tamsulosin was the comparator (HR 12.10; 95% CI 6.54, 22.37; p < 0.01). Important limitations of our study include unmeasured confounding (for example, over-the-counter medication use, dietary intake), missing data (i.e., only 20% of patients had a baseline serum sodium), and a lack of data on the newer formulation of desmopressin.
CONCLUSIONS
Use of an older formulation of desmopressin was associated with a marked increased rate of subsequent hyponatremia compared to use of other medications indicated for lower urinary tract symptoms. Such risks should be clearly communicated to patients prescribed this formulation of desmopressin.
Topics: Administration, Intranasal; Aged; Aged, 80 and over; Cohort Studies; Deamino Arginine Vasopressin; Female; Hemostatics; Humans; Hyponatremia; Male; Mandelic Acids; Middle Aged; Nocturia; Propensity Score; Proportional Hazards Models; Risk Factors; Tamsulosin; Treatment Outcome
PubMed: 31634354
DOI: 10.1371/journal.pmed.1002930 -
Blood Advances Sep 2022Patients with type 1 and type 2 von Willebrand disease (VWD) can be treated with desmopressin. Although a previous study has shown that the location of the causative VWF...
Patients with type 1 and type 2 von Willebrand disease (VWD) can be treated with desmopressin. Although a previous study has shown that the location of the causative VWF gene variant is associated with desmopressin response in type 1 VWD, the association between variants in the VWF gene and desmopressin response is not yet fully understood. Our primary aim was to compare desmopressin response in type 1 VWD patients with and without a VWF gene variant. Secondly, we investigated whether desmopressin response depends on specific VWF gene variants in type 1 and type 2 VWD. We included 250 patients from the Willebrand in the Netherlands study: 72 type 1 without a VWF gene variant, 108 type 1 with a variant, 45 type 2A, 16 type 2M, and 9 type 2N patients. VWF gene was analyzed with ion semiconductor sequencing and Multiplex Ligation-dependent Probe Amplification. Complete response to desmopressin was observed in all type 1 VWD patients without a variant, 64.3% of type 1 patients with a variant, and 31.3% of type 2 patients (P < .001). Despite a large interindividual variability in desmopressin response, patients with the same variant had comparable desmopressin responses. For instance, in 6 type 1 patients with exon 4 to 5 deletion, mean VWF activity at 1 hour after desmopressin was 0.81 IU/mL, with a coefficient of variation of 22.9%. In conclusion, all type 1 VWD patients without a VWF gene variant respond to desmopressin. In type 1 and type 2 VWD patients with a VWF variant, desmopressin response highly depends on the VWF gene variants.
Topics: Deamino Arginine Vasopressin; Exons; Humans; von Willebrand Disease, Type 2; von Willebrand Diseases; von Willebrand Factor
PubMed: 35446929
DOI: 10.1182/bloodadvances.2021006757