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Souheil El-Chemaly, Kevin J O'Brien, Steven D Nathan, Gerald L Weinhouse, Hilary J Goldberg, Jean M Connors, Ye Cui, Todd L Astor, Philip C Camp, Ivan O Rosas, Merte Lemma, Vladislav Speransky, Melissa A Merideth, William A Gahl, Bernadette R Gochuico
Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting donor organs. The objective of this study is to determine the clinical management and outcomes of a cohort with advanced HPS pulmonary fibrosis who were evaluated for lung transplantation. Six patients with HPS-1 pulmonary fibrosis were evaluated at the National Institutes of Health Clinical Center and one of two regional lung transplant centers...
2018: PloS One
W Weinrebe, S Käbe-Frisch, I Füsgen, M Karaman, E Johannsdottir, S Rupp
OBJECTIVE: To compare the efficacy of desmopressin and placebo in independent geriatric outpatients with nocturnal polyuria (NP). METHODS: A prospective, randomized, single-center, national, double blind, placebo-controlled, fixed-dose, parallel group comparative trial was carried out. The study included 110 geriatric outpatients, 55 patients per treatment group using desmopressin acetate nasal spray (strength: 0.1 mg/ml) once daily of 10 μg/spray blast or placebo...
March 14, 2018: Zeitschrift Für Gerontologie und Geriatrie
Ana Margarida Monteiro, Olinda Marques, Sofia Martins, Ana Antunes
The authors report a case of a 15-year-old girl with hypopituitarism due to pituitary stalk interruption syndrome diagnosed in the neonatal period. The patient was admitted to the emergency room with impaired consciousness and hypoglycaemia. The day before, she increased her water intake to about 1.5 L to perform a pelvic ultrasound. In the following hours, she developed vomiting and food refusal. Blood analysis revealed hypoglycaemia, hyponatraemia, decreased serum osmolality and normal urinary density. Hyponatraemia and adrenal crisis were managed with a gradual but slow resolution of consciousness and electrolytic balance...
March 9, 2018: BMJ Case Reports
Florence Gunawan, Elizabeth George, Adam Roberts
Immune checkpoint inhibitors are the mainstay of treatment for advanced melanoma, and their use is being increasingly implicated in the development of autoimmune endocrinopathies. We present a case of a 52-year-old man with metastatic melanoma on combination nivolumab and ipilumimab therapy who developed concurrent hypophysitis, type 1 diabetes mellitus (T1DM) and diabetes insipidus. He presented prior to third cycle of combination treatment with a headache, myalgias and fatigue. Biochemistry and MRI pituitary confirmed anterior pituitary dysfunction with a TSH: 0...
2018: Endocrinology, Diabetes & Metabolism Case Reports
S Mühlstädt
No abstract text is available yet for this article.
February 28, 2018: Der Urologe. Ausg. A
Akiko Nakamichi, Kazuki Ocho, Kosuke Oka, Miho Yasuda, Kou Hasegawa, Masaya Iwamuro, Mikako Obika, Kammei Rai, Fumio Otsuka
We herein report a case of central diabetes insipidus complicated with thyroid storm. A middle-aged woman who was receiving treatment for Graves' disease suddenly complained of polydipsia, polyuria and general fatigue. Laboratory tests showed hyperthyroidism, hypernatremia, hypoosmolar urine and a decreased plasma vasopressin level. The occurrence of central diabetes insipidus with hyperthyroidism was revealed on the basis of pituitary magnetic resonance imaging, a water deprivation test and a desmopressin test...
February 28, 2018: Internal Medicine
Pier Mannuccio Mannucci
No abstract text is available yet for this article.
March 2018: Haematologica
Julia Han, Jae Hung Jung, Caitlin J Bakker, Mark H Ebell, Philipp Dahm
OBJECTIVES: To assess the effects of desmopressin as compared to other interventions in the treatment of nocturia in men. PATIENTS AND METHODS: We performed a comprehensive search using multiple databases and abstract proceedings with no restrictions on the language of publication or publication status, up until August 2017. We included randomized or quasi-randomized trials. Inclusion criteria were men with nocturia defined as one or more voids per night. Two review authors independently examined full-text reports, identified relevant studies, assessed the eligibility of studies for inclusion, extracted data and assessed risk of bias...
February 28, 2018: BJU International
Pietro Ferrara, Giulia Franceschini, Serena Mercurio, Ester Del Vescovo, Francesca Ianniello, Tommasangelo Petitti
Objective: The aim of this study was to evaluate adverse effects of oral desmopressin lyophilisate (MELT) in enuretic children. Material and methods: We enrolled 260 children with nocturnal enuresis (NE) referred to the Pediatric Service, 'Campus Bio-Medico' University of Rome, from April 2014 to April 2017 in the study, of these 23 were excluded. The study was characterized by 2 phases. During Phase 1 a careful patient's medical history was obtained and physical examination was performed...
January 2018: Turkish Journal of Urology
Ricardo A Macau, Tiago Nunes da Silva, Joana Rego Silva, Ana Gonçalves Ferreira, Pedro Bravo
Lithium-induced nephrogenic diabetes insipidus (Li-NDI) is a rare and difficult-to-treat condition. A study in mice and two recent papers describe the use of acetazolamide in Li-NDI in 7 patients (a case report and a 6 patient series). We describe the case of a 63-year-old woman with bipolar disorder treated with lithium and no previous history of diabetes insipidus. She was hospitalized due to a bowel obstruction and developed severe dehydration after surgery when she was water deprived. After desmopressin administration and unsuccessful thiazide and amiloride treatment, acetazolamide was administrated to control polyuria and hydroelectrolytic disorders without significant side effects...
2018: Endocrinology, Diabetes & Metabolism Case Reports
Dimitra Argyro Vassiliadi, Stylianos Tsagarakis
Desmopressin is a vasopressin analogue selective for type 2 vasopressin receptors that mediate renal water retention. In contrast to the native hormone arginine-vasopressin, a well-known ACTH secretagogue, desmopressin exerts minimal or no activity on ACTH excretion. However, in a substantial proportion of patients with ACTH-dependent Cushing's syndrome (CS), desmopressin elicits an ACTH and cortisol response, which contrasts with the minimal responses obtained in healthy subjects. The mechanism underlying this paradoxical response involves upregulation of vasopressin type 3 and/or the aberrant expression of type 2 receptors by neoplastic ACTH-producing cells...
February 22, 2018: European Journal of Endocrinology
Julie Refardt, Mirjam Christ-Crain
Diabetes insipidus, characterized by polyuria and polydipsia, is a rare disease during pregnancy. Nevertheless, its recognition is important to avoid complications due to dehydration and hypernatremia. Its manifestation during pregnancy ranges from exacerbation of pre-existing central or nephrogenic diabetes insipidus to transient pregnancy-induced diabetes insipidus due to the increased metabolism of the antidiuretic hormone vasopressin by the placental vasopressinase. Diagnosis can be challenging, as urinary frequency is common during pregnancy and primary polydipsia also needs to be excluded...
February 19, 2018: Minerva Endocrinologica
Malcolm Prentice
Diabetes Insipidus (DI) is a name given historically to two conditions, which result in 'diabetes', which is Greek for 'syphon' or polyuria. The "Insipidus" refers to urine being insipid. 'Cranial DI' is so called because it relates to a cause within the head characterised by deficiency of vasopressin [antidiuretic hormone (ADH)] secretion from the posterior pituitary. Treatment is with desmopressin, a synthetic long-acting analogue of vasopressin. In contrast 'Nephrogenic DI' is due to resistance of the renal tubules to vasopressin action...
February 20, 2018: Clinical Endocrinology
L M Schütte, R M van Hest, S C M Stoof, F W G Leebeek, M H Cnossen, M J H A Kruip, R A A Mathôt
BACKGROUND: Nonsevere haemophilia A (HA) patients can be treated with desmopressin. Response of factor VIII activity (FVIII:C) differs between patients and is difficult to predict. OBJECTIVES: Our aims were to describe FVIII:C response after desmopressin and its reproducibility by population pharmacokinetic (PK) modelling. PATIENTS AND METHODS: Retrospective data of 128 nonsevere HA patients (age 7-75 years) receiving an intravenous or intranasal dose of desmopressin were used...
February 19, 2018: Thrombosis and Haemostasis
Christine Demers, Christine Derzko, Michele David, Joanne Douglas
OBJECTIVE: The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in adolescents presenting with menorrhagia. OPTIONS: Diagnostic tools and specific medical and, where appropriate, surgical alternatives to management are reviewed and evidence-based recommendations presented. EVIDENCE: A MEDLINE search of the English literature between January 1975 and November 2003 was performed using the following key words: menorrhagia, uterine bleeding, pregnancy, von Willebrand, congenital bleeding disorder, desmopressin/DDAVP, tranexamic acid, oral contraceptives, medroxyprogesterone, therapy, hysterectomy, anesthesia, epidural, spinal...
February 2018: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
Elke Gasthuys, An Vermeulen, Siska Croubels, Joske Millecam, Stijn Schauvliege, Thomas van Bergen, Pauline De Bruyne, Johan Vande Walle, Mathias Devreese
Desmopressin is used to treat primary nocturnal enuresis in children. Over the years, various formulations of desmopressin were commercialized of which the sublingual melt tablet is preferred in the pediatric population, despite the lack of full PK studies in this population. A full PK study was performed in growing conventional piglets to evaluate if this juvenile animal model can provide supplementary information to complement the information gap in the pediatric population. A desmopressin sublingual melt tablet (120 μg) was administered to 32 male piglets aged 8 days, 4 weeks, 7 weeks, and 6 months (each group n = 8)...
2018: Frontiers in Pharmacology
Rachel Barry, Antoinette O' Connor, Mohn Hazriq Awang, Orna O' Toole
A 58-year-old right-handed woman presented to our institution with a 1-month history of polydipsia and polyuria. She had a remote history of neurofibroma excision by dermatology and, on examination, was noted to meet the clinical diagnostic criteria for neurofibromatosis type 1. Laboratory investigations revealed hypernatraemia and elevated serum osmolality, accompanied by reduced urinary osmolality. A subsequent water deprivation test confirmed central diabetes insipidus, which responded to treatment with desmopressin...
February 11, 2018: BMJ Case Reports
Ayako Ito, Aya Nozaki, Ichiro Horie, Takao Ando, Atsushi Kawakami
BACKGOUND: Patients with central diabetes insipidus (CDI) are known to lose weight because their polydipsia interferes with their nutritional intake. We retrospectively examined weight changes in CDI patients when they switched from nasal to oral desmopressin. METHODS: Twenty-three patients with CDI were included. Weight change was defined as an increase or decrease of more than 3 kg or 3% body weight. As factors contributing to the weight change, we studied the patients' clinical characteristics and quality of life (QOL) scores as determined by our original questionnaire...
February 8, 2018: Minerva Endocrinologica
Varun R Kshettry, Gurston Nyquist, James J Evans
Surgery for craniopharyngiomas can be challenging due to the involvement of multiple critical neurovascular structures. The expanded endoscopic endonasal approach can provide superior access to suprasellar craniopharyngiomas, particularly with retrochiasmatic extension and significant hypothalamic involvement. We describe the surgical technique used to treat a 30-year-old patient who presented with 4 weeks of worsening vision, fatigue, and memory loss. His vision was counting fingers at 1 feet on the right and 20/800 on the left with a temporal hemianopsia...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
Shiva Kumar Gudlawar, Nageswara Rao Pilli, Sridhar Siddiraju, Jaya Dwivedi
An analytical method based on ultra-performance liquid chromatography with positive ion electrospray ionization (ESI) coupled with tandem mass spectrometry detection (UPLC-MS/MS) was developed and validated for the determination of therapeutic peptide desmopressin in human plasma. A desmopressin stable labeled isotope (desmopressin d8) was used as an internal standard. Analyte and the internal standard were extracted from 200 µL of human plasma via solid-phase extraction technique using Oasis WCX cartridges...
June 2017: Journal of Pharmaceutical Analysis
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