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diabetes insipidus [keywords]
- Comparison of incidence of hyponatremia between intranasal and oral desmopressin in patients with central diabetes insipidus. [JOURNAL ARTICLE]
- Endocr J 2014 Nov 7.
Central diabetes insipidus (CDI), which is characterized by polyuria and polydipsia, is caused by a deficiency of the antidiuretic hormone arginine vasopressin (AVP). While CDI is treated with desmopressin, an analogue of AVP, the intranasal formulation is inconvenient and CDI patients reportedly prefer the oral formulation to the intranasal one. In Japan, intranasal desmopressin had been the only formulation for the treatment of CDI until 2012, when the desmopressin orally disintegrating tablet (ODT) was approved for treatment. In this study we analyzed 26 patients with CDI in whom intranasal desmopressin was switched to desmopressin ODT. The mean daily dose of intranasal desmopressin was 10 ± 8 μg/day, and that of desmopressin ODT was 142 ± 59 μg/day. The mean serum sodium levels were 140 ± 5 mmol/l and 140 ± 3 mmol/l with intranasal desmopressin and desmopressin ODT, respectively, and there were no significant differences between these values. The frequency of hyponatremia (<135 mmol/l) with intranasal desmopressin was 11.7% and that with desmopressin ODT was 7.6%, while the frequency of hyponatremia (<130 mmol/l) with intranasal desmopressin was 4.2% and that with desmopressin ODT was 1.3%. Statistical analyses revealed that incidence of hyponatremia was significantly decreased after the switch to desmopressin ODT. Thus, it is suggested that water balance is better controlled with desmopressin ODT than with intranasal desmopressin in patients with CDI.
- Water and Electrolyte Disorders at Long-Term Post-Treatment Follow-Up in Paediatric Patients with Suprasellar Tumours Include Unexpected Persistent Cerebral Salt-Wasting Syndrome. [JOURNAL ARTICLE]
- Horm Res Paediatr 2014 Nov 1.
Background: Patients with brain tumours have a high risk of water and electrolyte disorders (WED). Postsurgery diabetes insipidus (DI) may be transient or permanent, the syndrome of inappropriate antidiuretic hormone secretion (SIADH) and cerebral salt-wasting syndrome (CSWS) are usually transient. Methods: Retrospective study, including patients with suprasellar tumours, treated at Hôpital Necker, Institut Gustave-Roussy or Institut Curie, in Île-de-France, between 2007 and 2011. WED were noted if they persisted >1 month after surgery. Results: 159 patients were included, 54.1% girls, 43.9% boys. Tumour types were: glioma (43.4%), craniopharyngioma (43.4%), germinoma (11.3%), others (1.9%). Age at diagnosis was 7.1 ± 4.6 years. The median time from end of treatment was 1.9 (0-7.8) years. DI was the most frequent disorder after tumour treatment (50.3%) and was significantly associated with surgery (p < 0.001). Persistent CSWS was present in 3.6%, persistent SIADH in 1.3%. Two cases of hypernatraemia were due to adipsia. Thyrotropin deficiency after treatment was noted in 68.9% of patients tested, adrenocorticotropin deficiency in 66.2%. Conclusions: Patients with suprasellar tumours have a high incidence of long-term WED, mainly DI. Assessment of thyrotroph and corticotroph function, and thirst sensation, is necessary to diagnose and manage these disorders correctly. CSWS may be persistent in few patients and requires special attention to prescribe the appropriate care. © 2014 S. Karger AG, Basel.
- Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood-onset - a population-based study in Sweden. [JOURNAL ARTICLE]
- J Clin Endocrinol Metab 2014 Nov 6.:jc20143525.
Context: Craniopharyngiomas in adults have been associated with excess mortality. Objective: The aim of the study was to investigate mortality and morbidity in childhood-onset and adult-onset craniopharyngioma patients. Methods: Craniopharyngioma patients were identified and followed in Swedish national health registries, 1987-2011. The inclusion criteria for the craniopharyngioma diagnosis were internally validated in 28% of the study population against patient records. Settings: Nationwide population-based study. Patients: 307 patients (151 men; 156 women) were identified and included (mean follow-up: 9 years, range 0-25). The inclusion criteria had a positive predictive value of 97% and a sensitivity of 92%. Intervention: None. Main Outcome Measures: Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as reference. Results: During the study 54 patients died compared with the expected number of 14.1, resulting in an SMR of 3.2 (2.2-4.7) for men and 4.9 (3.2-7.2) for women. Childhood-onset (n=106) and adult-onset craniopharyngioma (n=201) had SMRs of 17 (6.3-37) and 3.5 (2.6-4.6), respectively. Patients with hypopituitarism (n=250), diabetes insipidus (n=110), and neither of these (n=54) had SMRs of 4.3 (3.1-5.8), 6.1 (3.5-9.7), and 2.7 (1.4-4.6), respectively. SMR due to cerebrovascular diseases was 5.1 (1.7-12). SIRs were 5.6 (3.8-8.0) for type 2 diabetes mellitus (T2DM), 7.1 (5.0-9.9) for cerebral infarction, 0.7 (0.2-1.7) for myocardial infarction, 2.1 (1.4-3.0) for fracture, and 5.9 (3.4-9.4) for severe infection. The SIR for all malignant tumors was 1.3 (0.8-2.1). Conclusions: This first nationwide population-based study of craniopharyngioma patients demonstrated an excess mortality that was especially marked in patients with childhood-onset disease and among women. Death due to cerebrovascular diseases was increased five-fold. Hypopituitarism and diabetes insipidus were negative prognostic factors for mortality and morbidity. Craniopharyngioma patients suffered from an increased disease burden related to T2DM, cerebral infarction, fracture, and severe infection.
- A novel deletion mutation of the arginine vasopressin receptor 2 gene in a Japanese infant with nephrogenic diabetes insipidus. [Journal Article]
- Clin Pediatr Endocrinol 2014 Oct; 23(4):115-7.
- Diabetes Insipidus in Pediatric Patients. [JOURNAL ARTICLE]
- Indian J Pediatr 2014 Nov 6.
- Enuresis in children: a case based approach. [Journal Article]
- Am Fam Physician 2014 Oct 15; 90(8):560-8.
Enuresis is defined as intermittent urinary incontinence during sleep in a child at least five years of age. Approximately 5% to 10% of all seven-year-olds have enuresis, and an estimated 5 to 7 million children in the United States have enuresis. The pathophysiology of primary nocturnal enuresis involves the inability to awaken from sleep in response to a full bladder, coupled with excessive nighttime urine production or a decreased functional capacity of the bladder. Initial evaluation should include a history, physical examination, and urinalysis. Several conditions, such as constipation, obstructive sleep apnea, diabetes mellitus, diabetes insipidus, chronic kidney disease, and psychiatric disorders, are associated with enuresis. If identified, these conditions should be evaluated and treated. Treatment of primary monosymptomatic enuresis (i.e., the only symptom is nocturnal bed-wetting in a child who has never been dry) begins with counseling the child and parents on effective behavioral modifications. First-line treatments for enuresis include bed alarm therapy and desmopressin. The choice of therapy is based on the child's age and nighttime voiding patterns, and the desires of the child and family. Referral to a pediatric urologist is indicated for children with primary enuresis refractory to standard and combination therapies, and for children with some secondary causes of enuresis, including urinary tract malformations, recurrent urinary tract infections, or neurologic disorders.
- Hypoparathyroidism and central diabetes insipidus: in search of the link. [JOURNAL ARTICLE]
- Eur J Pediatr 2014 Nov 4.
Two siblings (a 15-year-old boy and an 11-year-old girl) who presented with hypocalcemic seizure at the age of 2 years and 2 months (boy) and 2 years and 4 months (girl) were diagnosed with hypoparathyroidism. At the age of 3 years, the girl developed central diabetes insipidus with good response to desmopressin acetate treatment. The family history was unremarkable, and there was no consanguinity between the parents. The father is of Iraqi/Egyptian Jewish origin and the mother is of Iranian/Romanian Jewish origin. Sequence analysis of the candidate genes for isolated hypoparathyroidism encoding calcium-sensing receptor, parathyroid hormone, and glial cells missing homolog B did not reveal any mutations. Whole-exome sequencing identified a homozygous mutation in the autoimmune regulatory gene (AIRE), c.374A>G;p.Y85C, characteristic for Jewish Iranians with autoimmune polyendocrine syndrome type 1 (APS1), which was confirmed by the Sanger sequencing. Antibodies against the adrenal, pancreatic islet cell, ovary, thyroid, pituitary, celiac, and parietal cell were negative in both siblings, while anti-diuretic hormone antibodies were positive only in the girl. No other symptoms or signs of APS1 developed during all the years of follow-up. Conclusion: APS1 should be part of the differential diagnosis in children presenting with isolated hypoparathyroidism or hypoparathyroidism with central diabetes insipidus (CDI). These cases show that the AIRE mutation characteristic of Iranian Jews can also be found in non-Iranian Jews.
- Endocrinological disorders affecting neurosurgical patients: An intensivists perspective. [Journal Article, Review]
- Indian J Endocrinol Metab 2014 Nov; 18(6):778-83.
Management of critically ill neurosurgical patients is often complicated by the presence or development of endocrinological ailments which complicate the clinical scenario and adversely affect the prognosis of these patients. The anatomical proximity to the vital centers regulating the endocrinological physiology and alteration in the neurotransmitter release causes disturbances in the hormonal homeostasis. This paves the way for development of diverse disorders where single or multiple hormones may be involved which can have deleterious effect on the different organ system. Understanding and awareness of these disorders is important for the treating intensivist to recognize these changes early in their course, so that appropriate and timely therapeutic measures can be initiated along with the treatment of the primary malady.
- National treatment trends, complications, and predictors of in-hospital charges for the surgical management of craniopharyngiomas in adults from 2007 to 2011. [Journal Article]
- Neurosurg Focus 2014 Nov; 37(5):E6.
Object Treatment of craniopharyngiomas is one of the most demanding and controversial neurosurgical procedures performed. The authors sought to determine the factors associated with hospital charges and fees for craniopharyngioma treatment to identify possible opportunities for improving the health care economics of inpatient care. Methods The authors analyzed the hospital discharge database of the Nationwide Inpatient Sample (NIS) covering the period from 2007 through 2011 to examine national treatment trends for adults (that is, those older than 18 years) who had undergone surgery for craniopharyngioma. To predict the drivers of in-hospital charges, a multistep regression model was developed that accounted for patient demographics, acuity measures, comorbidities, hospital characteristics, and complications. Results The analysis included 606 patients who underwent resection of craniopharyngioma; 353 resections involved a transsphenoidal approach (58%) and 253 a transfrontal approach (42%). The mean age (± SD) of patients was 47.7 ± 16.3 years. The average hospital length of stay (LOS) was 7.6 ± 9 days. The mean hospital charge (± SD) was $92,300 ± $83,356. In total, 48% of the patients experienced postoperative diabetes insipidus or an electrolyte abnormality. A multivariate regression model demonstrated that LOS, hospital volume for the selected procedure, the surgical approach, postoperative complications, comorbidities, and year of surgery were all significant predictors of in-hospital charges. The statistical model accounted for 54% of the variance in in-hospital charge. Conclusions This analysis of inpatient hospital charges in patients undergoing craniopharyngioma surgery identified key drivers of charges in the perioperative period. Prospective studies designed to evaluate the long-term resource utilization in this complex patient population would be a useful future direction.
- Pretreatment Diagnosis of Suprasellar Papillary Craniopharyngioma and Germ Cell Tumors of Adult Patients. [JOURNAL ARTICLE]
- AJNR Am J Neuroradiol 2014 Oct 22.
Suprasellar papillary craniopharyngiomas and germ cell tumors in adults share some clinical and imaging similarities but have different therapeutic strategies and outcomes. This study aimed to evaluate the pretreatment diagnosis of these 2 tumors to improve the therapeutic outcome.We retrospectively enrolled 18 adults with papillary craniopharyngiomas and 17 with germ cell tumors. The MR imaging findings were evaluated, including signal change and anatomic extension. The medical records were reviewed to collect clinical findings, management, and outcomes.The clinical findings of papillary craniopharyngiomas versus germ cell tumors were as follows: age: 46 ± 13.9 years versus 23 ± 7.1 years (P < .0001); diabetes insipidus: 2/18 (11%) versus 11/17 (65%) (P = .001); recurrence 13/16 (81%) versus 4/17 (24%) (P = .0031). The MR imaging findings of papillary craniopharyngiomas versus germ cell tumors were as follows-pituitary stalk thickening: 1.6 ± 0.4 mm versus 5.4 ± 4.2 mm (P < .0001); vertical infundibular extension: 1/18 (6%) versus 16/17 (94%) (P < .0001); sagittal spheric shape: 17/18 (94%) versus 1/17 (6%) (P < .0001); diffusion restriction: 1/17 (6%) versus 8/12 (67%) (P = .0009).Younger age, diabetes insipidus, MR imaging characteristics of restricted diffusion, and vertical infundibular extension favor the diagnosis of germ cell tumors. Spheric shape without infundibular infiltration provides clues to papillary craniopharyngiomas, which originate from the pars tuberalis and are located outside the third ventricle. We suggest that suprasellar germ cell tumor is possibly an intraventricular lesion. Appropriate treatment planning can be initiated according to the diagnosis and anatomic location.