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Zhen Huo, Tao Lu, Zhiyong Liang, Fan Ping, Jie Shen, Jingjing Lu, Wenbing Ma, Dachun Zhao, Dingrong Zhong
BACKGROUND: Isolated hypothalamic-pituitary Langerhans cell histiocytosis (HPLCH) is very rare. We investigated the clinicopathological characteristics, endocrine function changes, BRAF(V600E) mutations and treatments of isolated HPLCH. METHODS: We identified seven patients with isolated HPLCH by reviewing the clinical and pathological files in our hospital from 2007 to 2015. The clinical characteristics of the seven patients were retrospectively reviewed, especially the endocrine function changes...
October 19, 2016: Diagnostic Pathology
Julie Ann Smith
Hemophilia will be encountered in the oral and maxillofacial surgeon's office. A thorough understanding of hemophilia is necessary to safely care for these patients. One must understand the severity of the patient's hemophilia as well as whether or not inhibitors are present. The patient's surgical management will be influenced by these two factors. In addition to the possible need to transfuse factors or desmopressin, special care must be taken perioperatively to avoid bleeding complications. This article reviews the overall management of hemophilia A and B as well as the specific perioperative management of these patients...
November 2016: Oral and Maxillofacial Surgery Clinics of North America
Jehan Alsharnoubi, Adly A Sabbour, Ahmed I Shoukry, Amany M Abdelazeem
Nocturnal enuresis (NE) is intermittent involuntary voiding during sleep in a child aged 5 years or more. The study was conducted to compare the effect of using laser acupuncture and medication for the treatment of children with nocturnal enuresis (NE) and evaluation of urodynamic parameter after treatment. A randomized study included 45 children ranged from 5 to 15 years presenting with NE. They were randomized into three equal groups-group A, managed with desmopressin acetate; group B, managed with laser acupuncture; and group C, managed with a combination of laser acupuncture and desmopressin-all groups received behavioral therapy...
October 15, 2016: Lasers in Medical Science
Michael A Bohl, James Forseth, Peter Nakaji
BACKGROUND: Arginine vasopressin (AVP) is a common second-line or third-line vasopressor used in critically ill neurosurgical patients. Neurosurgical indications include hyperdynamic therapy for vasospasm, maintenance of cerebral perfusion pressure in patients with intracranial hypertension, and prevention of hypotension in patients with sepsis. CASE DESCRIPTION: A series of six neurosurgical patients receiving AVP infusions developed severe but transient diabetes insipidus (tDI) after cessation of AVP...
October 11, 2016: World Neurosurgery
H H Thakker, R Desouza, R Bhadare
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Benjamin Derman, Milli Jain, Elizabeth McAninch, Casey Gashti
A 59-year-old man presented with polyuria and polydipsia immediately following his sixth cycle of rituximab and bendamustine for chronic lymphocytic leukemia. He initially compensated by increasing his oral fluid intake at home, but later developed septic shock and was admitted with orders to be kept nil per os (NPO). This prompted an episode of acute hypernatremia during which he exhibited continued polyuria with inappropriately dilute urine. Desmopressin challenge yielded no response in the urine osmolality, indicating a nephrogenic source of his diabetes insipidus (DI)...
October 10, 2016: Clinical Nephrology
Pietro Ferrara, Annamaria Sbordone, Costanza Cutrona, Francesca Ianniello, Chiara Guadagno, Giacomo Perrone, Antonio Chiaretti, Alberto Verrotti, Vincenzo Di Lazzaro
PURPOSE: This study aims to evaluate and compare the efficacy of exogenous melatonin associated with desmopressin (dDAVP) and dietary recommendations. METHODS: A total of 189 patients were enrolled from the Service of Pediatrics, Campus Bio-Medico University Hospital of Rome, from January 2013 to June 2015. Of the 189 original patients, 153 children, aged between 5 and 14 years (mean age, 8.7 years) were included in the study. After clinical evaluation and a 3-month period of observation without treatment, children were assigned to receive treatment in one of 3 groups: group 1, dDAVP at a dose of 120 mcg a day (Minirin); group 2, dDAVP at a dose of 120 mcg and dietary recommendations; or group 3, dDAVP at a dose of 120 mcg, dietary recommendations, and melatonin at a dose of 1 mg a day (Melamil plus)...
September 2016: International Neurourology Journal
Christin Boldt, Tom Roeschel, Nina Himmerkus, Allein Plain, Markus Bleich, Robert Labes, Maximilian Blum, Hans Krause, Ahmed Magheli, Torsten Giesecke, Kerim Mutig, Michael Rothe, Steven M Weldon, Duska Dragun, Wolf-Hagen Schunck, Sebastian Bachmann, Alexander Paliege
Activation of the thick ascending limb (TAL) Na+-K+-2Cl--cotransporter (NKCC2) by the antidiuretic hormone arginine-vasopressin (AVP) is an essential mechanism of renal urine concentration and contributes to extracellular fluid and electrolyte homeostasis. AVP effects in the kidney are modulated by locally and/or by systemically produced epoxyeicosatrienoic acid derivates (EET). The relation between AVP and EET metabolism has not been determined. Here we show that chronic treatment of AVP-deficient Brattleboro rats with the AVP V2 receptor analog desmopressin (dDAVP; 5ng/h, 3d) significantly lowered renal EET levels (-56 ± 3% for 5,6-EET, -50 ± 3...
September 28, 2016: American Journal of Physiology. Renal Physiology
Amit Batla, Natalie Tayim, Mahreen Pakzad, Jalesh N Panicker
Urogenital dysfunction is commonly reported in Parkinson's disease (PD), and history taking and a bladder diary form the cornerstone of evaluation. The assessment of lower urinary tract (LUT) symptoms include urinalysis, ultrasonography, and urodynamic studies and help to evaluate concomitant urological pathologies such as benign prostate enlargement. Antimuscarinic medications are the first line treatment for overactive bladder (OAB) symptoms and solifenacin has been specifically studied in PD. Antimuscarininc drugs may exacerbate PD-related constipation and xerostomia, and caution is advised when using these medications in individuals where cognitive impairment is suspected...
October 2016: Current Treatment Options in Neurology
Peter Sidaway
No abstract text is available yet for this article.
September 27, 2016: Nature Reviews. Urology
D A Vassiliadi, M Balomenaki, A Asimakopoulou, E Botoula, M Tzanela, S Tsagarakis
CONTEXT: Cushing's disease (CD) has a significant relapse rate after successful transsphenoidal surgery (TSS). Many CD patients respond aberrantly to desmopressin testing (DT). Disappearance of this response post-surgery may suggest complete removal of abnormal corticotrophs and lower possibility of recurrence. OBJECTIVE: The utility of post-operative DT to predict long-term outcome compared to the widely used post-operative cortisol (Cort) level. DESIGN: Retrospective analysis...
September 23, 2016: Journal of Clinical Endocrinology and Metabolism
Vansh Kapila, Ayush K Kapila, Thomas Tailly, Bernard Rappe, Kristian Vinter Juul, Karel Everaert
Urolithiasis is a frequent problem causing a significant clinical, psychological and socio-economic burden. Analgesia remains the most important element in the medical treatment of renal colic. Nonetheless, both NSAIDs and opiates have a side effect profile which can cause further complications. As such, the use of desmopressin for renal colic has received increased attention in the last two decades. This paper provides an overview of current evidence on the use of desmopressin as an analgesic strategy in renal colic...
September 23, 2016: Acta Clinica Belgica
Vedha Sanghi, Aanchal Kapoor
Hypernatremia is a frequent cause of intensive care unit admission. The patient presented in this article had hypernatremia refractory to D5W (dextrose 5% water) therapy, which led to a complex investigation. Workup revealed central diabetes insipidus most likely secondary to flare up of neurosarcoidosis. The challenge in terms of diagnosis was a presentation with low urine output in the setting of hypernatremia resistant to treatment with desmopressin. This case unfolded the role of hypothyroidism causing secondary renal dysfunction and hence needed continued treatment with thyroxine in addition to treatment for hypernatremia...
July 2016: Journal of Investigative Medicine High Impact Case Reports
Michael J R Desborough, Peter A Smethurst, Lise J Estcourt, Simon J Stanworth
Allogeneic platelet transfusions are widely used for the prevention and treatment of bleeding in thrombocytopenia. Recent evidence suggests platelet transfusions have limited efficacy and are associated with uncertain immunomodulatory risks and concerns about viral or bacterial transmission. Alternatives to transfusion are a well-recognised tenet of Patient Blood Management, but there has been less focus on different strategies to reduce bleeding risk by comparison to platelet transfusion. Direct alternatives to platelet transfusion include agents to stimulate endogenous platelet production (thrombopoietin mimetics), optimising platelet adhesion to endothelium by treating anaemia or increasing von Willebrand factor levels (desmopressin), increasing formation of cross-linked fibrinogen (activated recombinant factor VII, fibrinogen concentrate or recombinant factor XIII), decreasing fibrinolysis (tranexamic acid or epsilon aminocaproic acid) or using artificial or modified platelets (cryopreserved platelets, lyophilised platelets, haemostatic particles, liposomes, engineered nanoparticles or infusible platelet membranes)...
September 21, 2016: British Journal of Haematology
Emily Reynen, Paula James
von Willebrand disease (VWD) is a common, inherited bleeding disorder. There are three main types of VWD, which result in a quantitative or qualitative deficiency in von Willebrand factor (VWF) and in severe cases, also Factor VIII (FVIII). The severity of bleeding depends on the underlying pathophysiology. Type 1 VWD is usually mild, while types 2 or 3 VWD can be associated with moderate or significant bleeding. Managing pregnant women with VWD requires a multidisciplinary approach. Such patients are at increased risk of postpartum hemorrhage...
September 20, 2016: Seminars in Thrombosis and Hemostasis
Kevin J O'Brien, Jay Lozier, Andrew R Cullinane, Brigitte Osorio, Khanh Nghiem, Vladislav Speransky, Wadih M Zein, James C Mullikin, Anne T Neff, Karen L Simon, May Christine V Malicdan, William A Gahl, Lisa R Young, Bernadette R Gochuico
PURPOSE: Hemophilia B, an X-linked disease, manifests with recurrent soft tissue bleeding episodes. Hermansky-Pudlak syndrome, a rare autosomal recessive disorder, is characterized by oculocutaneous albinism and an increased tendency to bleed due to a platelet storage pool defect. We report a novel mutation in HPS6 in a Caucasian man with hemophilia B and oculocutaneous albinism. RESULTS: The patient was diagnosed with hemophilia B at age 4months due to recurrent soft tissue bleeding episodes, and he was also diagnosed with Hermansky-Pudlak syndrome at 32years of age due to unexplained oculocutaneous albinism...
September 3, 2016: Molecular Genetics and Metabolism
Wolfgang Miesbach, Erik Berntorp
Von Willebrand disease (VWD) is the most common genetic bleeding disorder. VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilises coagulation factor VIII (FVIII) in the blood. Prophylaxis of surgical bleeding in patients with VWD requires consideration of the individual situation, including the type of procedure and the bleeding rate, before decisions about treatment type, dose, duration and adjunctive therapy with antifibrinolytics or antithrombotic prophylaxis can be made...
September 13, 2016: European Journal of Haematology
Joon Chul Kim, Kang Jun Cho, Jeong Gu Lee, Ju Tae Seo, Duk Yoon Kim, Seung-June Oh, Kyu-Sung Lee, Myung-Soo Choo, Jeong Zoo Lee
PURPOSE: We investigated the efficacy and safety of desmopressin add-on therapy in men with persistent nocturia on alpha-blocker for lower urinary tract symptoms (LUTS) with placebo-controlled study. MATERIALS AND METHODS: The study included men aged 40-65 years with LUTS and persistent nocturia despite alpha-blocker for at least 8 weeks. The patients were randomized to once-daily placebo or desmopressin 0.2 mg for 8 weeks. The primary end point was to assess changes in the mean number of nocturia episodes from the baseline to the final assessment...
September 10, 2016: Journal of Urology
Elzbieta Skowronska-Jozwiak, Stanislaw Sporny, Joanna Szymanska-Duda, Dominika Baranowska, Andrzej Lewinski
Langerhans cell histiocytosis (LCH) in adults is a rare disorder of unknown etiology characterized by monoclonal proliferation of Langerhans cells. It belongs to dendritic cell disorders and occurs in 1-2 adults per million. The most common endocrine manifestation of classical LCH is associated with the posterior pituitary, with clinical symptoms of diabetes insipidus. Less than 80 reported cases of LCH involving the thyroid gland have been published so far. We present the case of a 39 years old woman with 10 years history of diabetes insipidus and secondary amenorrhoea, which appeared after second delivery...
July 12, 2016: Neuro Endocrinology Letters
Klaus Hahnenkamp, Klaus Böhler, Heiner Wolters, Karsten Wiebe, Dietmar Schneider, Hartmut H-J Schmidt
BACKGROUND: The ascertainment of brain death (the irreversible, total loss of brain function) gives the physician the opportunity to limit or stop further treatment. Alternatively, if the brain-dead individual is an organ donor, the mode of treatment can be changed from patient-centered to donationcentered. Consensus-derived recommendations for the organ-protective treatment of brain-dead organ donors are not yet available in Germany. METHODS: This review is based on pertinent publications retrieved by a selective search in PubMed, and on the authors' clinical experience...
August 22, 2016: Deutsches Ärzteblatt International
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