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Md Habibur Rahman, Md Abdul Qader, Syed Saimul Haque, Md Abdullah Al Mamun, Golam Muin Uddin
Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS) presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Håvard Tetlie Garberg, Marianne U Huun, Lars O Baumbusch, Monica Åsegg-Atneosen, Rønnaug Solberg, Ola Didrik Saugstad
BACKGROUND: There is a lack of reliable biomarkers that can identify and grade acute hypoxic-ischemic encephalopathy in newborns. MicroRNAs (miRNA) are short, non-coding strands of RNA that are released into the circulation in response to tissue stress and injury. Some miRNAs are highly tissue specific and thus may potentially be non-invasive biomarkers of neonatal hypoxic-ischemic brain injury. OBJECTIVE: The aim of this study was to characterize the temporal expression of selected circulating miRNAs in a clinically relevant piglet model of neonatal hypoxia-ischemia (HI)...
October 18, 2016: Neonatology
Jem Ma Ahn, Chang Ha Kim, Soon Ho Um, Kyung Mee Kim, Tae Hyung Kim, Sun Young Yim, Hyuk Soon Choi, Eun Sun Kim, Bora Keum, Yeon Seok Seo, Hyung Joon Yim, Yoon Tae Jeen, Hong Sik Lee, Hoon Jai Chun, Chang Duck Kim, Ho Sang Ryu
BACKGROUND AND AIM: In a recent study, microsatellite variations (GCA tandem repeats) in the promoter region of the (kidney-type) glutaminase gene were associated with the development of hepatic encephalopathy (HE) in Spanish patients with cirrhosis. The objective of this study was to validate the relation between microsatellite variations in the glutaminase promoter region and the development of overt HE in Korean patients with liver cirrhosis. METHODS: We performed a prospective cohort study of 154 cirrhotic patients who underwent a glutaminase microsatellite study without previous overt HE history at baseline...
October 17, 2016: Journal of Gastroenterology and Hepatology
Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
Mohammad F Aljabri, Naglaa M Kamal, Moinuddin Arif, Asrar M AlQaedi, Enas Y M Santali
BACKGROUND: Biotin-thiamine-responsive basal ganglia disease (BTRBGD) is a neurometabolic autosomal recessive (AR) disorder characterized by subacute encephalopathy with confusion, convulsions, dysarthria, and dystonia. The disease is completely reversible if treated early with biotin and thiamine, and can be fatal if left untreated.We herein present our experience with in an extended family study of an index case of BTRBGD aiming to support its AR mode of inheritance, diagnose asymptomatic and missed symptomatic cases, and provide family screening with proper genetic counseling...
October 2016: Medicine (Baltimore)
Kevin D Mullen
No abstract text is available yet for this article.
October 17, 2016: Metabolic Brain Disease
Shiv Kumar Sarin, Ashok Choudhury
Acute-on-chronic liver failure (ACLF) is a distinct entity that differs from acute liver failure and decompensated cirrhosis in timing, presence of treatable acute precipitant, and course of disease, with a potential for self-recovery. The core concept is acute deterioration of existing liver function in a patient of chronic liver disease with or without cirrhosis in response to an acute insult. The insult should be a hepatic one and presentation in the form of liver failure (jaundice, encephalopathy, coagulopathy, ascites) with or without extrahepatic organ failure in a defined time frame...
December 2016: Current Gastroenterology Reports
Amr Shaaban Hanafy, Amr Talaat El Hawary
Objectives: Evaluation of the outcome and experience in 2 years of management of portal hypertensive gastropathy (PHG) by argon plasma coagulation (APC) in a cohort of Egyptian cirrhotic patients. Methods: This study was conducted over a 2-year period from January 2011 to February 2013. Upper gastrointestinal endoscopy was performed to evaluate the degree and site of PHG. APC was applied to areas with mucosal vascular lesions. Results: In total, 200 cirrhotic patients were enrolled; 12 patients were excluded due to death (n = 6) caused by hepatic encephalopathy (n = 3), hepatorenal syndrome (n = 2), or chronic lymphatic leukemia (n = 1), or did not complete the treatment sessions (n = 6), so 188 patients completed the study...
October 2016: Endoscopy International Open
Naser Amini, Nasim Vousooghi, Mansoureh Soleimani, Ali Samadikuchaksaraei, Mehdi Akbari, Hosein Safakheil, Pezhman Atafimanesh, Ali Shahbazi, Peiman Brouki Milan, Sara Ramezani, Masoud Mozafari, Mohammad Taghi Joghataei
INTRODUCTION: Hemolytic kernicterus, an indirect bilirubin-induced brain dysfunction, is associated with hyper-bilirubinemia in mammalian neonates. In this study, a new model of kernicterus has been developed using intra-peritoneal injections of phenyl hydrazine and subcutaneous injections of sulfisoxazole. These drugs can potentially induce kernicterus in neonatal through changes in hemolysis and hypo-albumin. METHODS: For this purpose, 7-day-old male Wistar rats (n=72; mean weight 11±1 g) were used...
October 13, 2016: Journal of Pharmacological and Toxicological Methods
Tomokazu Kimizu, Yukitoshi Takahashi, Taikan Oboshi, Asako Horino, Takayoshi Koike, Shinsaku Yoshitomi, Tatsuo Mori, Tokito Yamaguchi, Hiroko Ikeda, Nobuhiko Okamoto, Mitsuko Nakashima, Hirotomo Saitsu, Mitsuhiro Kato, Naomichi Matsumoto, Katsumi Imai
INTRODUCTION: Mutations of SLC35A2 that encodes Golgi-localized Uridine diphosphate (UDP)-galactose transporter at Xp11.23 lead to congenital disorders of glycosylation (CDG). Although patients with CDG generally have diverse systemic symptoms, patients with a SLC35A2 mutation manifest predominantly disorders of the central nervous system (CNS). CASE REPORT: A female infant aged 12months was referred to our center because of intractable seizures. The patient was born with birth weight of 3228g after 40weeks of unremarkable gestation...
October 12, 2016: Brain & Development
Angela C Gauthier, Joachim M Baehring
Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved...
October 12, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Minkyung Jung, Shingo Kasamatsu, Tetsuro Matsunaga, Soichiro Akashi, Katsuhiko Ono, Akira Nishimura, Masanobu Morita, Hisyam Abdul Hamid, Shigemoto Fujii, Hiroshi Kitamura, Tomohiro Sawa, Tomoaki Ida, Hozumi Motohashi, Takaaki Akaike
Reactive persulfide species such as glutathione persulfide (GSSH) are highly abundant biomolecules. Persulfide dioxygenase (also called ethylmalonic encephalopathy protein 1, ETHE1) reportedly metabolizes GSSH to GSH with simultaneous oxygen consumption. How ETHE1 activity is regulated is still unclear, however. In this study, we describe the possible role of protein polysulfidation in the catalytic activity of ETHE1. We first found that ETHE1 catalyzed the persulfide dioxygenase reaction mostly for glutathione polysulfides, GS-(S)n-H, as well as for GSSH, but not for other endogenous persulfides such as cysteine and homocysteine persulfides/polysulfides...
October 11, 2016: Biochemical and Biophysical Research Communications
Romain Lefaucheur, Axel Lebas, Emmanuel Gérardin, Lou Grangeon, Ozlem Ozkul-Wermester, Carole Aubier-Girard, Olivier Martinaud, David Maltête
A 29-year-old man was admitted for acute cognitive impairment. Three weeks earlier, he had been admitted for coma due to sniffed heroin abuse responsive to naloxone infusion. At admission, the patient presented with apraxia, severe memory impairment and anosognosia. Brain MRI revealed symmetric hyperintensities of supratentorial white matter, sparing brainstem and cerebellum, on FLAIR and B1000 sequences. Four months later, repeated neuropsychological assessment revealed dramatic improvement of global cognitive functions...
October 11, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Regan E Giesinger, Liane J Bailey, Poorva Deshpande, Patrick J McNamara
No abstract text is available yet for this article.
October 11, 2016: Journal of Pediatrics
María Sol Pacha, Lucas Orellana, Emanuel Silva, Glenda Ernst, Fatima Pantiu, Julieta Quiroga Narvaez, Ricardo Reisin, Oscar Martinez
Status epilepticus (SE) is a severe neurological condition with significant morbidity and mortality. A reliable tool for prognosis is needed to take decision regarding treatment strategies. We compared 2 available prognostic scores of outcome: the Status Epilepticus Severity Score (STESS) and the Epidemiology-based Mortality score in SE (EMSE). We included 46 patients with SE evaluated out the last 5years in our hospital. We excluded patients with postanoxic encephalopathy or incomplete data. Among the 46 patients with SE, in-hospital mortality was 28%...
October 10, 2016: Epilepsy & Behavior: E&B
Maurizio Koch
Microbes are mostly important for the digestion of food, the absorption of some micronutrients, and the production of vitamins. The microbiota stimulates lymphoid structures in the gastrointestinal mucosa and decrease pathogens by competing for nutrients and space. Bacterial translocation is defined as the escape of gut bacteria and their products through the intestinal mucosa to the outside of the intestine as portovenous or systemic circulation. This is induced by a leaky gut barrier. There is evidence for a role of intestinal permeability in the pathogenesis of nonalcoholic fatty liver disease...
November 2016: Journal of Clinical Gastroenterology
Omar D Borjas-Almaguer, Carlos A Cortez-Hernández, Emmanuel I González-Moreno, Francisco J Bosques-Padilla, José A González-González, Aldo A Garza, Juan A Martínez-Segura, Diego García-Compean, Juan V Alejandre-Loya, Jesús García-García, Guillermo Delgado-García, Héctor J Maldonado-Garza
:  Background & Aims. It is unclear whether portal vein thrombosis (PVT) unrelated to malignancy is associated with reduced survival or it is an epiphenomenon of advanced cirrhosis. The objective of this study was to assess clinical outcome in cirrhotic patients with PVT not associated with malignancy and determine its prevalence. MATERIAL AND METHODS: Retrospective search in one center from June 2011 to December 2014. RESULTS: 169 patients, 55 women and 114 men, median age 54 (19-90) years...
November 2016: Annals of Hepatology
Stefan Mogk, Christian M Boßelmann, Celestin N Mudogo, Jasmin Stein, Hartwig Wolburg, Michael Duszenko
African trypanosomes induce sleeping sickness. The parasites are transmitted during the blood meal of a tsetse fly and appear primarily in blood and lymph vessels, before they enter the central nervous system. During the latter stage, trypanosomes induce a deregulation of sleep-wake cycles and some additional neurological disorders. Historically, it was assumed that trypanosomes cross the blood-brain barrier and settle somewhere between the brain cells. The brain, however, is a strictly controlled and immune-privileged area that is completely surrounded by a dense barrier that covers the blood vessels: this is the blood-brain barrier...
October 14, 2016: Biological Reviews of the Cambridge Philosophical Society
Ruby Upadhyay, Thomas P Bleck, Katharina M Busl
Purpose. A 66-year-old man who presented with coma was found to have isolated severe hyperammonemia and diagnosed with a late-onset urea-cycle disorder. He was treated successfully and had full recovery. Methods. We report a novel case of noncirrhotic hyperammonemia and review the literature on this topic. Selected literature for review included English-language articles concerning hyperammonemia using the search terms "hyperammonemic encephalopathy", "non-cirrhotic encephalopathy", "hepatic encephalopathy", "urea-cycle disorders", "ornithine transcarbamylase (OTC) deficiency", and "fulminant hepatic failure"...
2016: Case Reports in Medicine
Fatih Aygün, Pınar Özge Avar Aydın, Şenol Emre, Seval Şimşek Uzunoğlu, Sema Saltık, Halit Çam
No abstract text is available yet for this article.
September 2016: Türk Pediatri Arşivi
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