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Pediatric encephalopathy

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https://www.readbyqxmd.com/read/28062149/prediction-of-neonatal-seizures-in-hypoxic-ischemic-encephalopathy-using-electroencephalograph-power-analyses
#1
Siddharth V Jain, Amit Mathur, Preethi Srinivasakumar, Michael Wallendorf, Joseph P Culver, John M Zempel
BACKGROUND: The severity of the initial encephalopathy in neonatal hypoxic-ischemic encephalopathy correlates with seizure burden. Early electroencephalograph (EEG) background activity reflects the severity of encephalopathy. Thus, we hypothesized that early EEG background would be predictive of subsequent seizures in neonatal hypoxic-ischemic encephalopathy. METHODS: This study included infants undergoing therapeutic hypothermia at St. Louis Children's Hospital between January 2009 and April 2013...
November 11, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/28060109/posterior-reversible-encephalopathy-syndrome-and-cerebral-sinus-thrombosis-in-a-case-of-pediatric-b-cell-all
#2
Ellen Fraint, Robin Miller, Andrew Walter
Posterior reversible encephalopathy syndrome (PRES) and cerebral sinus thrombosis are 2 known complications of acute lymphoblastic leukemia and its treatment. We describe a patient with acute lymphoblastic leukemia whose course was complicated by both of these conditions. This case is novel both for the fact that PRES developed before the initiation of therapy and that PRES was followed shortly by the development of cerebral sinus thrombosis. Our patient's story raises questions about our current understanding of the pathophysiology of PRES, and it suggests that PRES may actually be a predisposing risk factor for cerebral sinus thrombosis...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28042689/early-and-late-neurological-complications-of-liver-transplantation-in-pediatric-patients
#3
Serdal Gungor, Betul Kilic, Mujgan Arslan, M Ayse Selimoglu, Hamza Karabiber, Sezai Yilmaz
NCs occur commonly after solid organ transplantation and affect 15%-30% of liver transplant recipients. The aim of this retrospective study was to evaluate the type and incidence of neurologic events in pediatric patients following LT. Between May 2006 and June 2015, 242 patients (118 females, 124 males) requiring LT for different etiologies at the İnönü University Liver Transplantation Institute were included. The incidence, types, and risk factors of NCs that occurred following LT were evaluated retrospectively...
January 1, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28031453/acute-liver-failure-meets-soph-syndrome-a-case-report-on-an-intermediate-phenotype
#4
Fanny Kortüm, Iris Marquardt, Malik Alawi, Georg Christoph Korenke, Stephanie Spranger, Peter Meinecke, Kerstin Kutsche
Acute liver failure (ALF) is a life-threatening condition in the absence of preexisting liver disease in children. The main clinical presentation comprises hepatic dysfunction, elevated liver biochemical values, and coagulopathy. The etiology of ALF remains unclear in most affected children; however, the recent identification of mutations in the neuroblastoma amplified sequence (NBAS) gene in autosomal recessively inherited ALF has shed light on the cause of a subgroup of fever-triggered pediatric ALF episodes...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/28009765/autoimmune-encephalitides-a-broadening-field-of-treatable-conditions
#5
Bernadette Kalman
BACKGROUND: Neurology has been continuously transforming by the refinement of molecular diagnostics and the development of disease-modifying treatments. The discovery of new antibody markers has elucidated the pathogenesis, provided the means of diagnostics, and offered cure or treatment for several immune-mediated neurological and neuropsychiatric disorders. The identification of pathogenic and marker autoantibodies has also facilitated defining the associated phenotypic spectra and the overlap among the phenotypes linked to individual immune markers...
January 2017: Neurologist
https://www.readbyqxmd.com/read/28006936/pathophysiology-of-perinatal-hypoxic-ischemic-encephalopathy-biomarkers-animal-models-and-treatment-perspectives
#6
V Riljak, J Kraf, A Daryanani, P Jiruška, J Otáhal
Hypoxic-ischemic encephalopathy (HIE) is one of the leading pediatric neurological conditions causing long-term disabilities and socio-economical burdens. Nearly 20-50 % of asphyxiated newborns with HIE die within the newborn period and another third will develop severe health consequences and permanent handicaps. HIE is the result of severe systemic oxygen deprivation and reduced cerebral blood flow, commonly occurring in full-term infants. Hypoxic-ischemic changes trigger several molecular and cellular processes leading to cell death and inflammation...
December 22, 2016: Physiological Research
https://www.readbyqxmd.com/read/28005690/low-bone-mineral-density-in-vertically-hiv-infected-children-and-adolescents-risk-factors-and-the-role-of-t-cell-activation-and-senescence
#7
Beatriz Jiménez, Talía Sainz, Laura Díaz, María José Mellado, María Luisa Navarro, Pablo Rojo, María Isabel González-Tomé, Luis Prieto, Jorge Martínez, María Isabel de José, José Tomás Ramos, María Ángeles Muñoz-Fernandez
BACKGROUND: Our aim was to determine the prevalence and risk factors associated with low bone mineral density (BMD) in vertically HIV-infected patients; and to investigate whether low BMD is related to immune activation and senescence induced by HIV infection. METHODS: A cross-sectional study was performed in 98 vertically HIV-infected patients. BMD was measured by dual-energy X-ray absorptiometry at lumbar spine. Height adjustment of BMD Z-score was performed using height-for-age Z-score...
December 21, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#8
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28002390/therapeutic-hypothermia-modulates-complement-factor-c3a-and-c5a-levels-in-a-rat-model-of-hypoxic-ischemic-encephalopathy
#9
Tushar A Shah, Jasmine E Nejad, Haree K Pallera, Frank A Lattanzio, Rawad Farhat, Parvathi S Kumar, Pamela S Hair, W Thomas Bass, Neel K Krishna
BACKGROUND: Therapeutic hypothermia (HT) is the only intervention that improves outcomes in neonatal hypoxic-ischemic encephalopathy (HIE). However, the multifactorial mechanisms by which HT impacts HIE are incompletely understood. The complement system plays a major role in the pathogenesis of ischemia-reperfusion injuries such as HIE. We have previously demonstrated that HT modulates complement activity in vitro. METHODS: Term equivalent rat pups were subjected to unilateral carotid ligation followed by hypoxia (8% O2) for 45 minutes to simulate HIE...
December 21, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27975112/acute-liver-failure
#10
Catherine D Newland
Pediatric acute liver failure (ALF) is a complex and rapidly progressive syndrome that results from a variety of age-dependent etiologies. It is defined by the acute onset of liver disease with no evidence of chronic liver disease. There must be biochemical or clinical evidence of severe liver dysfunction as defined by an international normalized ratio (INR) ≥2. If hepatic encephalopathy is present, INR should be ≥1.5. Unfortunately, due to the rarity of ALF in pediatric patients, there is a paucity of diagnostic and management algorithms and each patient must have an individualized approach...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27940680/wernicke-encephalopathy-in-adolescents-after-bariatric-surgery-case-report-and-review
#11
Amy Armstrong-Javors, Janey Pratt, Sigmund Kharasch
Roughly 1% of all weight loss surgery is performed in adolescents. There is strong evidence demonstrating significant postsurgical weight loss, improvement in quality of life, and reduction in comorbidities such as hypertension and diabetes. Reports of postoperative complications in adolescents are few because of the small sample size in most series. Despite vitamin supplementation, nutritional deficiencies requiring hospitalization occur occasionally after Roux-en-Y gastric bypass. Wernicke encephalopathy, a triad of ophthalmoplegia, ataxia, and altered mental status, is a serious consequence of thiamine (vitamin B1) deficiency...
December 2016: Pediatrics
https://www.readbyqxmd.com/read/27940505/a-previously-healthy-adolescent-with-acute-encephalopathy-and-decorticate-posturing
#12
Yu Kawai, Andrea G DeMonbrun, Rebecca S Chambers, Danielle A Nolan, Bram A Dolcourt, Nasuh M Malas, Michael W Quasney
A 14-year-old previously healthy female was transferred from a local emergency department after being found unresponsive at home. Parental questioning revealed she had fever and pharyngitis 2 weeks before presentation. Past mental health history was negative, including concern for past or present suicidal ideation/attempts, suspected substance use, or toxic ingestion. In the emergency department, she was orotracheally intubated due to a Glasgow Coma Scale of 3. She was hemodynamically stable and euglycemic...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/27938315/epilepsy-after-heart-transplant-a-single-center-experience
#13
Ruhsen Öcal, Taner Sezer, Seda Kibaroğlu, Eda Derle, Sibel Benli, Atilla Sezgin, Sait Aşlamacı
OBJECTIVES: Cardiac transplant is the best treatment for patients with end-stage heart failure. Neurologic complications occur at a rate of 30% to 80% in patients undergoing cardiac transplant. Seizures occur at a rate of 2% to 20%. The main causative factors include immunosuppressant drug toxicity, infections, brain lesions, and metabolic disorders. Here, our aim was to determine seizure types and associated conditions in patients undergoing cardiac transplant and to report our treatment experience at our institution...
December 12, 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27916450/dramatic-response-after-functional-hemispherectomy-in-a-patient-with-epileptic-encephalopathy-carrying-a-de-novo-col4a1-mutation
#14
Naomi Hino-Fukuyo, Atsuo Kikuchi, Masaki Iwasaki, Yuko Sato, Yuki Kubota, Tomoko Kobayashi, Tojo Nakayama, Kazuhiro Haginoya, Natsuko Arai-Ichinoi, Tetsuya Niihori, Ryo Sato, Tasuku Suzuki, Hiroki Kudo, Ryo Funayama, Keiko Nakayama, Yoko Aoki, Shigeo Kure
We describe the first case of a successful functional hemispherectomy in a patient with epileptic encephalopathy and a de novo collagen type IV alpha 1 (COL4A1) mutation. A 4-year-old girl was COL4A1 mutation-positive and suffered from drug-resistant epilepsy, hemiplegia, and developmental delay. Magnetic resonance imaging detected no porencephaly, and she had no cataract or renal abnormality. Following a presurgical evaluation for epilepsy, she underwent a functional hemispherectomy. She has been seizure free with no intracranial hemorrhage or other perioperative complications...
December 1, 2016: Brain & Development
https://www.readbyqxmd.com/read/27903293/personalized-medicine-approach-confirms-a-milder-case-of-abat-deficiency
#15
A Besse, A K Petersen, J V Hunter, V Appadurai, S R Lalani, P E Bonnen
ABAT deficiency (OMIM 613163) is a rare inborn error of metabolism caused by recessive variants in the gene 4-aminobutyric acid transaminase (ABAT), which is responsible for both the catalysis of GABA and maintenance of nucleoside pools in the mitochondria. To date, only a few patients have been reported worldwide. Their clinical presentation has been remarkably consistent with primary features of severe psychomotor retardation, encephalopathy, hypotonia, and infantile-onset refractory epilepsy. We report a new case of ABAT deficiency that marks an important departure from previous clinical findings...
December 1, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27895948/pediatric-balint-s-syndrome-variant-a-possible-diagnosis-in-children
#16
Swetha Sara Philip, Sunithi Elizabeth Mani, Gordon N Dutton
Balint's syndrome is well described in adults, but not in children. It is caused by bilateral posterior parietal lobe damage and comprises a triad of simultanagnosia (inability to simultaneously see more than a small number of items), optic ataxia (impaired visual guidance of movement of the limbs and body), and apraxia of gaze (inability to volitionally direct gaze despite the requisite motor substrate) often associated with homonymous lower visual field loss. We, here, describe five children (four males, one female; mean age 7...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27887787/vascular-endothelial-growth-factor-vegf-and-platelet-derived-growth-factor-pdgf-levels-in-the-cerebrospinal-fluid-of-children-with-influenza-associated-encephalopathy
#17
Shinichiro Morichi, Natsumi Morishita, Mika Takeshita, Yu Ishida, Shingo Oana, Gaku Yamanaka, Yasuyo Kashiwagi, Hisashi Kawashima
INTRODUCTION: To search for an index of neurologic prognosis of children with influenza-associated encephalopathy (IAE), involvement of angiogenesis-related growth factors in the pathology was investigated. PATIENTS AND METHODS: The subjects were 11 IAE patients, 6 patients with bacterial meningitis (BM), and 24 patients with non-central nervous system infection as a control group admitted to our hospital. The correlation between the vascular endothelial growth factor (VEGF) and platelet-derived growth factor (PDGF) levels in cerebrospinal fluid and the relationship with an index of inflammatory marker, interleukin (IL)-6, were investigated...
February 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/27878780/a-pilot-study-using-lactulose-in-management-of-minimal-hepatic-encephalopathy-in-children-with-extrahepatic-portal-vein-obstruction
#18
Hanaa Mostafa El-Karaksy, Omneya Afifi, Azza Bakry, Ann Abdel Kader, Noha Saber
BACKGROUND: Minimal hepatic encephalopathy (MHE) is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurophysiologic tests. We aimed to diagnose MHE in children with extrahepatic portal vein obstruction (EHPVO) and to evaluate the effect of lactulose on MHE. METHODS: A prospective study was carried out on 30 patients with EHPVO (21 males; mean age 10±2.5 years). The study was carried out in the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Cairo, Egypt, between 2011 and 2013...
February 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27836006/mycoplasma-pneumoniae-associated-mild-encephalitis-encephalopathy-with-a-reversible-splenial-lesion-report-of-two-pediatric-cases-and-a-comprehensive-literature-review
#19
Norishi Ueda, Satoshi Minami, Manabu Akimoto
BACKGROUND: No literature review exists on Mycoplasma pneumoniae-associated mild encephalitis/encepharopathy with a reversible splenial lesion (MERS). METHODS: M.pneumoniae-associated MERS cases were searched till August 2016 using PubMed/Google for English/other-language publications and Ichushi ( http://www.jamas.or.jp/ ) for Japanese-language publications. Inclusion criteria were children fulfilling definition for encephalitis, M.pneumoniae infection, and neuroimaging showing hyperintensity in the splenium of the corpus callosum (SCC) alone (type I) or SCC/other brain areas (type II)...
November 11, 2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27831626/-non-fatal-case-of-h1n1-influenza-encephalopathy-in-a-pediatric-patient
#20
María Del Carmen Valdivia-Tapia, Nilton Yhuri Carreazo
A 2-year-old girl presented with fever, catarrhal symptoms, and focal right hemispheric seizures that persisted and led to signs of intracranial hypertension. An influenza A H1N1 infection was confirmed via polymerase chain reaction analysis of a nasopharyngeal swab. The patient, who was not treated with oseltamavir, has responded favorably to supportive measures.
July 2016: Revista Peruana de Medicina Experimental y Salud Pública
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