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https://www.readbyqxmd.com/read/28923789/assessing-the-efficacy-and-safety-of-an-11%C3%AE-hydroxysteroid-dehydrogenase-type-1-inhibitor-azd4017-in-the-idiopathic-intracranial-hypertension-drug-trial-iih-dt-clinical-methods-and-design-for-a-phase-ii-randomized-controlled-trial
#1
Keira Annie Markey, Ryan Ottridge, James L Mitchell, Caroline Rick, Rebecca Woolley, Natalie Ives, Peter Nightingale, Alexandra J Sinclair
BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition with few effective management options. So far, there have been no randomized controlled trials evaluating new treatments in IIH. OBJECTIVES: The purpose of this paper is to outline the trial design for the Idiopathic Intracranial Hypertension Drug Trial (IIH:DT), assessing an innovative medical treatment in IIH and the rationale for the chosen trial methodology. METHODS: IIH:DT is a phase II double-blind randomized placebo-controlled trial recruiting 30 female participants with active IIH (intracranial pressure >25cm H2 O and papilledema)...
September 18, 2017: JMIR Research Protocols
https://www.readbyqxmd.com/read/28914984/hypercalcemic-disorders-in-children
#2
REVIEW
Victoria J Stokes, Morten F Nielsen, Fadil M Hannan, Rajesh V Thakker
Hypercalcemia is defined as a serum calcium concentration that is greater than 2 standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms...
September 15, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28914813/lactoferrin-a-natural-glycoprotein-involved-in-iron-and-inflammatory-homeostasis
#3
REVIEW
Luigi Rosa, Antimo Cutone, Maria Stefania Lepanto, Rosalba Paesano, Piera Valenti
Human lactoferrin (hLf), an iron-binding multifunctional cationic glycoprotein secreted by exocrine glands and by neutrophils, is a key element of host defenses. HLf and bovine Lf (bLf), possessing high sequence homology and identical functions, inhibit bacterial growth and biofilm dependently from iron binding ability while, independently, bacterial adhesion to and the entry into cells. In infected/inflamed host cells, bLf exerts an anti-inflammatory activity against interleukin-6 (IL-6), thus up-regulating ferroportin (Fpn) and transferrin receptor 1 (TfR1) and down-regulating ferritin (Ftn), pivotal actors of iron and inflammatory homeostasis (IIH)...
September 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28904452/long-term-response-of-cerebrospinal-fluid-pressure-in-patients-with-idiopathic-intracranial-hypertension-a-prospective-observational-study
#4
V Abdul Gafoor, B Smita, James Jose
BACKGROUND: Idiopathic intracranial hypertension (IIH) is increased intracranial pressure (ICP) with normal cerebrospinal fluid (CSF) contents, in the absence of an intracranial mass, hydrocephalus, or other identifiable causes. The current knowledge of the treatment outcome of IIH is limited, and the data on the natural history of this entity are scant. OBJECTIVE: The objective of the study is to study the treatment response of IIH by serially measuring the CSF opening pressure and to delineate the factors influencing the same...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28902838/the-cryo-electron-microscopy-structure-of-human-transcription-factor-iih
#5
Basil J Greber, Thi Hoang Duong Nguyen, Jie Fang, Pavel V Afonine, Paul D Adams, Eva Nogales
Human transcription factor IIH (TFIIH) is part of the general transcriptional machinery required by RNA polymerase II for the initiation of eukaryotic gene transcription. Composed of ten subunits that add up to a molecular mass of about 500 kDa, TFIIH is also essential for nucleotide excision repair. The seven-subunit TFIIH core complex formed by XPB, XPD, p62, p52, p44, p34, and p8 is competent for DNA repair, while the CDK-activating kinase subcomplex, which includes the kinase activity of CDK7 as well as the cyclin H and MAT1 subunits, is additionally required for transcription initiation...
September 13, 2017: Nature
https://www.readbyqxmd.com/read/28884026/idiopathic-intracranial-hypertension-prognostic-factors-and-multidisciplinary-management
#6
Claire Chagot, Marie Blonski, Jean-Loup Machu, Serge Bracard, Jean-Christophe Lacour, Sébastien Richard
BACKGROUND: Idiopathic intracranial hypertension (IIH) mostly affects young obese women and can lead to permanent visual impairment. However, prognostic factors and therapeutic strategy remain unclear. METHODS: We retrospectively collected data from all patients diagnosed and managed for IIH in our university center from January 2001 to December 2016. RESULTS: Seventy-nine patients were diagnosed with IIH. Bilateral transverse sinus stenosis (TSS) was found in 74% of the population...
2017: Journal of Obesity
https://www.readbyqxmd.com/read/28875121/spontaneous-cerebrospinal-fluid-rhinorrhea-association-with-body-weight-and-imaging-data
#7
Raphaële Quatre, Arnaud Attye, Christian Adrien Righini, Emile Reyt, Joris Giai, Sébastien Schmerber, Alexandre Karkas
Objective  Spontaneous cerebrospinal fluid rhinorrhea (SCSFR) might be the only clinical manifestation of idiopathic intracranial hypertension (IIH), which has been historically related to overweight. Our goal was to search for an association between SCSFR and increased body weight on the one hand and SCSFR and imaging findings suggestive of IIH on the other hand. Materials and Methods  We retrospectively collected clinical and radiological data of patients operated on endoscopically for SCSFR in our institution from 1993 to 2013...
October 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28871612/a-short-cut-diagnostic-tool-in-cystinosis-bone-marrow-aspiration
#8
Serra Sürmeli Döven, Ali Delibaş, Uğur Raşit Karacan, Selma Ünal
BACKGROUND: Cystinosis is a rare metabolic genetic disorder caused by a mutation in the cystinosin lysosomal cystine transporter (CTNS) gene. The diagnosis of nephropathic cystinosis (NC) is made by observing corneal cystine crystals and/or measuring the cystine content of leukocytes. Analysis by the CTNS gene mutation confirms the diagnosis of cystinosis. But leukocyte cystine level measurement and CTNS gene analysis haven't been widely available and cystine crystals in the cornea may not be apparent in the first months of life...
September 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28868187/tumor-cerebri-metastatic-renal-cell-carcinoma-with-dural-venous-sinus-compression-leading-to-intracranial-hypertension-a-case-report
#9
Eric Marvin, Jordan Synkowski, Michael Benko
BACKGROUND: Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a condition associated with increased intracranial pressure (ICP) in the absence of radiographic findings such as mass lesions or cerebral edema. CASE DESCRIPTION: We describe a case of progressive headache and visual disturbances attributed to PTC that resulted from subacute superior sagittal sinus (SSS) stenosis by a metastatic tumor. CONCLUSIONS: Venous outflow obstruction often presents with an acute symptomatology including infarcts, hemorrhages, and seizures, but only rarely does it cause the progressive development of raised ICP...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28852857/efficacy-of-laparoscopic-herniorrhaphy-for-treating-incarcerated-pediatric-inguinal-hernia
#10
S R Lee
PURPOSE: Inguinal hernia repair is one of the most common elective surgeries. Most patients present with reducible inguinal bulging; however, in cases of incarcerated inguinal hernia (IIH), an emergency surgery is required. Here, we report the surgical outcomes of a laparoscopic approach for IIH. METHODS: Laparoscopic herniorrhaphy was performed in 4782 pediatric patients from September 2012 to December 2016 at Damsoyu Hospital, Seoul, Korea. Among them, the surgical outcomes of 164 IIH patients were retrospectively analyzed...
August 29, 2017: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/28841117/transient-resolution-of-venous-sinus-stenosis-after-high-volume-lumbar-puncture-in-a-patient-with-idiopathic-intracranial-hypertension
#11
Thomas J Buell, Daniel M S Raper, I Jonathan Pomeraniec, Dale Ding, Ching-Jen Chen, Davis G Taylor, Kenneth C Liu
Stenosis of the transverse sinus (TS) and sigmoid sinus (SS), with a trans-stenosis pressure gradient, has been implicated in the pathophysiology of idiopathic intracranial hypertension (IIH). MRI has shown improvement in TS and SS stenosis after high-volume lumbar puncture (HVLP) in a subset of patients with IIH. The authors present the first report of an IIH patient with immediate post-HVLP TS and SS trans-stenosis pressure gradient reduction and an attendant increase in TS and SS cross-sectional area confirmed using intravascular ultrasonography (IVUS)...
August 25, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28838819/pediatric-idiopathic-intracranial-hypertension-is-the-fixed-threshold-value-of-elevated-lp-opening-pressure-set-too-high
#12
Lucia Gerstl, Nikola Schoppe, Lucia Albers, Birgit Ertl-Wagner, Noam Alperin, Oliver Ehrt, Andreas Pomschar, Mirjam N Landgraf, Florian Heinen
BACKGROUND: Idiopathic intracranial hypertension (IIH) in children is a rare condition of unknown etiology and various clinical presentations. The primary aim of this study was to evaluate if our pediatric IIH study group fulfilled the revised diagnostic criteria for IIH published in 2013, particularly with regard to clinical presentation and threshold value of an elevated lumbar puncture opening pressure. Additionally we investigated the potential utilization of MR-based and fundoscopic methods of estimating intracranial pressure for improved diagnosis...
August 12, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28833207/semicircular-canal-dehiscence-among-idiopathic-intracranial-hypertension-patients
#13
Phoebe Kuo, Kenneth A Bagwell, Gino Mongelluzzo, Christopher A Schutt, Ajay Malhotra, Babar Khokhar, John F Kveton
OBJECTIVES/HYPOTHESIS: The cause of superior semicircular canal dehiscence (SSCD) is unknown. Because of a demonstrated association with tegmental defects and obesity, some have suggested idiopathic intracranial hypertension (IIH) could contribute by eroding the bone over the canal and resulting in SSCD. However, an association between IIH and SSCD has not previously been evaluated. Our objective was to evaluate an association between IIH and SSCD. STUDY DESIGN: Retrospective cohort...
August 22, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28815909/proximal-neuropathy-and-associated-skeletal-muscle-changes-resembling-denervation-atrophy-in-hind-limbs-of-chronic-hypoglycaemic-rats
#14
Vivi F H Jensen, Anne-Marie Molck, Henrik Soeborg, Jette Nowak, Melissa Chapman, Jens Lykkesfeldt, Ingrid B Bogh
Peripheral neuropathy is one of the most common complications of diabetic hyperglycaemia. Insulin-induced hypoglycaemia (IIH) might potentially exacerbate or contribute to neuropathy as hypoglycaemia also causes peripheral neuropathy. In rats, IIH induces neuropathy associated with skeletal muscle changes. Aims of the present study were to investigate the progression and sequence of histopathologic changes caused by chronic IIH in rat peripheral nerves and skeletal muscle, and whether such changes were reversible...
August 16, 2017: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28800199/intracranial-elastance-is-increased-in-idiopathic-intracranial-hypertension
#15
Jason T Chisholm, Padmaja Sudhakar, Abdulnasser N Alhajeri, Jonathan H Smith
BACKGROUND: To date, no pathophysiologic model has sufficiently accounted for all the findings encountered in patients with idiopathic intracranial hypertension (IIH). Intracranial elastance is an index of volume-buffering capacity known to play a role in certain disorders of CSF dynamics, which has not been previously investigated in relation to IIH patients. METHODS: Single-center retrospective cohort study from July 1, 2011 to July 1, 2016. We collected values for opening pressure (PO ), closing pressure (PC ), volume (V) of CSF removed, as well as demographic and clinical covariates...
August 11, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28799016/intraparenchymal-intracranial-pressure-monitoring-for-hydrocephalus-and-cerebrospinal-fluid-disorders
#16
Aswin Chari, Debayan Dasgupta, Alexander Smedley, Claudia Craven, Edward Dyson, Samir Matloob, Simon Thompson, Lewis Thorne, Ahmed K Toma, Laurence Watkins
BACKGROUND: Elective intraparenchymal intracranial pressure (ICP) monitoring is useful for the diagnosis and treatment of hydrocephalus and cerebrospinal fluid (CSF) disorders. This retrospective study analyzes median ICP and pulse amplitude (PA) recordings in neurosurgically naïve patients undergoing elective ICP monitoring for suspected CSF disorders. METHODS: Retrospective review of prospectively collated database of neurosurgically naïve patients undergoing elective ICP monitoring for suspected hydrocephalus and CSF disorders...
August 10, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28768582/blind-overnight-a-case-of-fulminant-idiopathic-intracranial-hypertension
#17
Janpreet Singh Bhandohal, Taimur Mirza
Idiopathic Intracranial Hypertension (IIH) is a syndrome seen predominantly in obese women of reproductive age group, characterized by signs and symptoms of increased intracranial pressure due to an unknown cause. Some individuals have a more malignant form of disease called fulminant idiopathic intracranial hypertension with rapid worsening of symptoms over days. We report a case of 33year-old obese female (BMI 36.9) who presented with a severe headache and blurred vision for one week, found to have idiopathic intracranial hypertension with rapid worsening of symptoms suggestive of a fulminant course of disease...
July 5, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28758206/the-idiopathic-intracranial-hypertension-treatment-trial-a-review-of-the-outcomes
#18
Stacy V Smith, Deborah I Friedman
OBJECTIVE: The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) was the first large, randomized study on the use of acetazolamide and weight loss for treatment of idiopathic intracranial hypertension-associated vision loss. The multicenter trial also examined a number of secondary outcomes. This review summarizes all available results of the study published in the literature since 2014. BACKGROUND: Prior to the IIHTT, clinicians managed idiopathic intracranial hypertension based on data from small, unmasked trials, expert opinion, and clinical experience...
July 30, 2017: Headache
https://www.readbyqxmd.com/read/28752894/headache-in-idiopathic-intracranial-hypertension-findings-from-the-idiopathic-intracranial-hypertension-treatment-trial
#19
Deborah I Friedman, Peter A Quiros, Prem S Subramanian, Luis J Mejico, Shan Gao, Michael McDermott, Michael Wall
OBJECTIVE: To characterize the phenotype, headache-related disability, medical co-morbidities, use of symptomatic headache medications, and headache response to study interventions in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). METHODS: Patients with untreated IIH and mild vision loss enrolled in the IIHTT and randomized to acetazolamide (ACZ) and weight loss or placebo (PLB) and weight loss had prospective assessment of headache disability using the Headache Impact Test-6 (HIT-6) questionnaire...
September 2017: Headache
https://www.readbyqxmd.com/read/28751968/lumboperitoneal-shunt-insertion-without-fluoroscopy-guidance-accuracy-of-placement-in-a-series-of-107-procedures
#20
Sabah Al-Rashed, Haider Kareem, Neeraj Kalra, Linda D'Antona, Mouness Obeidat, Bhavesh Patel, Ahmed Toma
Background: Lumboperitoneal (LP) shunts were the mainstay of cerebrospinal fluid diversion therapy for idiopathic intracranial hypertension (IIH). The traditionally cited advantage of LP shunts over ventriculoperitoneal (VP) shunts is the ease of insertion in IIH. This needs to be placed at the level of L3/4 to be below the level of the spinal cord. The objective of this study was to analyse the position of LP shunts inserted without portable fluoroscopy guidance.  Methods: A retrospective analysis of radiology was performed for patients who underwent lumboperitoneal shunts between 2006 and 2016 at the National Hospital for Neurology and Neurosurgery...
2017: F1000Research
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