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Hyperammonemia

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https://www.readbyqxmd.com/read/28634175/interrelations-between-3-hydroxypropionate-and-propionate-metabolism-in-rat-liver-relevance-to-disorders-of-propionyl-coa-metabolism
#1
Kirkland A Wilson, Yong Han, Miaoqi Zhang, Jeremy Hess, Kimberly A Chapman, Gary W Cline, Gregory P Tochtrop, Henri Brunengraber, Guo-Fang Zhang
Propionate, 3-hydroxypropionate (3HP), methylcitrate, related compounds and ammonium accumulate in body fluids of patients with disorders of propionyl-CoA metabolism, such as propionic acidemia. Although liver transplantation alleviates hyperammonemia, high concentrations of propionate, 3HP and methylcitrate persist in body fluids. We hypothesized that conserved metabolic perturbations occurring in transplanted patients result from the simultaneous presence of propionate and 3HP in body fluids. We investigated the interrelations of propionate and 3HP metabolism in perfused livers from normal rats using metabolomic and stable isotopic technologies...
June 20, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28627027/elevated-serum-myostatin-level-is-associated-with-worse-survival-in-patients-with-liver-cirrhosis
#2
Hiroki Nishikawa, Hirayuki Enomoto, Akio Ishii, Yoshinori Iwata, Yuho Miyamoto, Noriko Ishii, Yukihisa Yuri, Kunihiro Hasegawa, Chikage Nakano, Takashi Nishimura, Kazunori Yoh, Nobuhiro Aizawa, Yoshiyuki Sakai, Naoto Ikeda, Tomoyuki Takashima, Ryo Takata, Hiroko Iijima, Shuhei Nishiguchi
BACKGROUND: We aimed to elucidate the relationship between serum myostatin levels and other markers including skeletal muscle mass and to investigate the influence of serum myostatin levels on survival for patients with liver cirrhosis (LC). METHODS: A total of 198 LC subjects were analysed in this study. Myostatin levels were measured using stored sera. We retrospectively investigated the relationship between myostatin level and other markers, and the influence of myostatin level on overall survival (OS)...
June 18, 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/28605806/hyperammonemia-after-blood-transfusion-a-case-report
#3
Zachary M Rossfeld, Nathan R Wright
No abstract text is available yet for this article.
June 13, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28597413/late-onset-ornithine-transcarbamylase-deficiency-associated-with-hyperammonemia
#4
Kana Daijo, Tomokazu Kawaoka, Takashi Nakahara, Yuko Nagaoki, Masataka Tsuge, Akira Hiramatsu, Michio Imamura, Yoshiiku Kawakami, Hiroshi Aikata, Keiichi Hara, Go Tajima, Masao Kobayashi, Kazuaki Chayama
The urea cycle converts ammonia and produces urea. One form of urea cycle abnormality is ornithine transcarbamylase (OTC) deficiency. This hereditary disorder is associated with hyperammonemia. OTC deficiency commonly appears during neonatal and early childhood life and is rare in adults. We report a 69-year-old man who presented at the local hospital with 3-day loss of appetite, early morning vomiting, and state of confusion. Blood ammonia was 293 μg/dl. At 2-3 h after admission, the patient went into a deep coma...
June 9, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/28592010/-clinical-diagnosis-and-treatment-of-three-cases-with-hyperornithinemia-hyperammonemia-homocitrullinuria-syndrome
#5
H Z Guan, Y Ding, D X Li, H Dong, J Q Song, Y Jin, Z J Zhu, L Y Sun, Y L Yang
Objective: To study the clinical characteristics, methods of diagnosis and treatment of hyperornithinemia-hyperammonemia- homocitrullinuria (HHH) syndrome. Method: From July 2011 to August 2016, 3 Chinese patients with HHH syndrome were enrolled in this study. The clinical course, biochemical features, brain MRI findings, and gene mutations were analyzed. Result: The three patients' age at onset of symptoms was 3 months to 7 years, and the age of diagonosis was 3 years and 10 months to 9 years and 10 months...
June 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28571812/a-hypothesis-for-mechanism-for-brain-oedema-due-to-glycine
#6
Suchita J Mehta, Andrea Roche Recinos, Subodh J Saggi, Man S Oh
Death following the use the glycine distension solution in transurethral prostatectomy (TURP) or hysteroscopic surgery has been attributed to the toxic effect of glycine on the brain through the glycine receptors and hyperammonemia, contending that glycine-associated hyponatremia is isosmotic and therefore would not cause brain oedema. Here we propose a hypothesis that the mechanism of brain oedema and death is actually osmotic brain oedema caused by selective diffusion of glycine into the brain while sodium cannot diffuse out of the brain despite favourable concentration gradient because of the absence of sodium transporter on the cerebral capillaries needed for the exit of sodium from the brain...
June 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28559637/reversible-encephalopathy-due-to-valproic-acid-induced-hyperammonemia-in-a-patient-with-bipolar-i-disorder-a-cautionary-report
#7
Neel Patel, Katherine B Landry, Rachel E Fargason, Badari Birur
Valproic acid (VPA) is an FDA-approved medication widely prescribed for seizures, migraines, and mixed or manic episodes in bipolar disorder. Hyperammonemia is a rare complication of VPA use, which can result in high morbidity and occasionally fatal encephalopathy. The scant literature on Valproate Induced Hyperammonemic Encephalopathy (VIHE) is characterized by acute onset of decreasing level of consciousness, drowsiness, lethargy which in rare instances can lead to seizures, stupor, coma, and persistent morbidity and cortical damage...
January 26, 2017: Psychopharmacology Bulletin
https://www.readbyqxmd.com/read/28557005/sarcopenia-in-alcoholic-liver-disease-clinical-and-molecular-advances
#8
Jaividhya Dasarathy, Arthur J McCullough, Srinivasan Dasarathy
Despite advances in treatment of alcohol use disorders that focus on increasing abstinence and reducing recidivism, alcoholic liver disease is projected to be the major cause of cirrhosis and its complications. Malnutrition is recognized as the most frequent complication in alcoholic liver disease (ALD) and despite the high clinical significance, there are no effective therapies to reverse malnutrition in ALD. Malnutrition is a relatively imprecise term and sarcopenia or skeletal muscle loss, the major component of malnutrition, is primarily responsible for the adverse clinical consequences in patients with liver disease...
May 29, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28526534/3-methylglutaconic-aciduria-a-frequent-but-underrecognized-finding-in-carbamoyl-phosphate-synthetase-i-deficiency
#9
Dariusz Rokicki, Magdalena Pajdowska, Joanna Trubicka, Meow-Keong Thong, Elżbieta Ciara, Dorota Piekutowska-Abramczuk, Maciej Pronicki, Roman Sikora, Rijad Haidar, Mariusz Ołtarzewski, Ewa Jabłońska, Premala Muthukumarasamy, Pavai Sthaneswar, Chin-Seng Gan, Małgorzata Krajewska-Walasek, Rosalba Carrozzo, Daniela Verrigni, Michela Semeraro, Cristiano Rizzo, Roberta Taurisano, Bader Alhaddad, Reka Kovacs-Nagy, Tobias B Haack, Carlo Dionisi-Vici, Ewa Pronicka, Saskia B Wortmann
The urea cycle disorder carbamoyl phosphate synthetase I deficiency is an important differential diagnosis in the encephalopathic neonate. This intoxication type inborn error of metabolism often leads to neonatal death or severe and irreversible damage of the central nervous system, even despite appropriate treatment. Timely diagnosis is crucial, but can be difficult on routine metabolite level. Here, we report ten neonates from eight families (finally) diagnosed with CPS1 deficiency at three tertiary metabolic centres...
May 17, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28514703/bacterial-infections-and-hepatic-encephalopathy-in-liver-cirrhosis-prophylaxis-and-treatment
#10
REVIEW
Damian Piotrowski, Anna Boroń-Kaczmarska
Infections are common among patients with liver cirrhosis. They occur more often in cirrhotic patient groups than in the general population and result in higher mortality. One reason for this phenomenon is bacterial translocation from the intestinal lumen that occurs as a consequence of intestinal bacterial overgrowth, increased permeability and decreased motility. The most common infections in cirrhotic patients are spontaneous bacterial peritonitis and urinary tract infections, followed by pneumonia, skin and soft tissue infections...
May 14, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28506823/hyperammonemia-compromises-glutamate-metabolism-and-reduces-bdnf-in-the-rat-hippocampus
#11
Fabiana Galland, Elisa Negri, Carollina Da Ré, Fernanda Fróes, Liliane Strapazzon, Maria Cristina Guerra, Lucas Silva Tortorelli, Carlos-Alberto Gonçalves, Marina Concli Leite
Ammonia is putatively the major toxin associated with hepatic encephalopathy (HE), a neuropsychiatric manifestation that results in cognitive impairment, poor concentration and psychomotor alterations. The hippocampus, a brain region involved in cognitive impairment and depressive behavior, has been studied less than neocortical regions. Herein, we investigated hippocampal astrocyte parameters in a hyperammonemic model without hepatic lesion and in acute hippocampal slices exposed to ammonia. We also measured hippocampal BDNF, a neurotrophin commonly related to synaptic plasticity and cognitive deficit, and peripheral S100B protein, used as a marker for brain damage...
May 12, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28505498/ameliorative-effects-of-rutin-on-hepatic-encephalopathy-induced-by-thioacetamide-or-gamma-irradiation
#12
Somaya Z Mansour, Seham M El-Marakby, Fatma S M Moawed
Hepatic encephalopathy (HE) is a syndrome resulting from acute or chronic liver failure. This study was designed to evaluate the effect of rutin on thioacetamide (TAA) or γ-radiation-induced HE model. Animals were received with TAA (200mg/kg, i.p.) twice weekly for four weeks or exposed to 6Gy of γ-radiation to induce HE then groups orally treated with rutin (50mg/kg b.wt.) for four weeks. At the end of experiment, blood, liver and brain samples were collected to assess biochemical and biophysical markers as well histopathological investigations...
May 8, 2017: Journal of Photochemistry and Photobiology. B, Biology
https://www.readbyqxmd.com/read/28491179/transhepatic-embolization-of-a-congenital-intrahepatic-portosystemic-shunt-for-the-treatment-of-hepatic-encephalopathy-in-a-noncirrhotic-patient-using-amplatzer-vascular-plug-device
#13
Rachel Ann Brader, Kyung Rae Kim
A 73-year-old male with no history of liver disease was hospitalized for weakness, confusion, ataxia, and new onset hepatic encephalopathy with hyperammonemia. After management with lactulose and rifaximin, his symptoms persisted, and he underwent transjugular liver biopsy. Biopsy showed normal liver, but a portosystemic shunt was incidentally identified on postbiopsy venogram. The patient underwent occlusion of the shunt with two Amplatzer vascular plugs and four Nester coils. Following embolization, the patient's symptoms resolved completely...
June 2017: Radiology case reports
https://www.readbyqxmd.com/read/28469525/recommendation-of-repeated-ammonia-tests-for-intrahepatic-portal-systemic-shunt-without-cirrhosis-in-elderly-patients-with-psychiatric-symptoms
#14
Michiaki Abe, Temma Soga, Nobuya Obana, Kazumasa Seiji, Masao Tabata, Natsumi Saito, Ryutaro Arita, Takehiro Numata, Junichi Tanaka, Hitoshi Kuroda, Shin Takayama, Yutaka Kagaya, Tadashi Ishii
We report an elderly male patient with hyperammonemia induced by intrahepatic portal-systemic shunt without cirrhosis (IPSSwoC). The occasional emergence of his erratic behaviors was misdiagnosed as a psychiatric disorder. Regardless of his uneven symptoms, IPSSwoC was suspected due to his hyperammonemia. The contrast computed tomography of the abdomen revealed a congenital type of IPSSwoC. As blood ammonia levels are inconstant, repeated blood tests are recommended when this disease is suspected in elderly patients with psychiatric symptoms...
2017: Japanese Clinical Medicine
https://www.readbyqxmd.com/read/28444906/carbamoyl-phosphate-synthetase-1-deficiency-diagnosed-by-whole-exome-sequencing
#15
Guoqing Zhang, Yulin Chen, Huiqun Ju, Fei Bei, Jing Li, Jian Wang, Jianhua Sun, Jun Bu
BACKGROUND: Carbamoyl Phosphate Synthetase 1 deficiency (CPS1D) is a rare autosomal recessive inborn metabolic disease characterized mainly by hyperammonemia. The fatal nature of CPS1D and its similar symptoms with other urea cycle disorders (UCDs) make its diagnosis difficult, and the molecular diagnosis is hindered due to the large size of the causative gene CPS1. Therefore, the objective of the present study was to investigate the clinical applicability of exome sequencing in molecular diagnosis of CPS1D in Chinese population...
April 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28436001/gait-instability-in-valproate-treated-patients-call-to-measure-ammonia-levels
#16
S Kipervasser, C E Elger, A D Korczyn, R D Nass, C M Quesada, M Y Neufeld
OBJECTIVE: Hyperammonemia induced by valproate (VPA) treatment may lead to several neurological and systemic symptoms as well as to seizure exacerbation. Gait instability and recurrent falls are rarely mentioned as symptoms, especially not as predominant ones. METHODS: We report five adult patients with frontal lobe epilepsy (FLE) who were treated with VPA and in whom a primary adverse effect was unstable gait and falls. RESULTS: There were four males and one female patients with FLE, 25-42-year-old, three following epilepsy surgery...
April 23, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28425821/l-carnitine-supplementation-to-reverse-hyperammonemia-in-a-patient-undergoing-chronic-valproic-acid-treatment-a-case-report
#17
Cecilia Maldonado, Natalia Guevara, Alicia Silveira, Pietro Fagiolino, Marta Vázquez
Valproic acid is a broad-spectrum anticonvulsant that has also gained attention in the psychiatric setting. With respect to safety, valproic acid may induce a seemingly rare condition, hyperammonemia, which can induce a wide variety of symptoms ranging from irritability to coma. The proposed mechanism of hyperammonemia involves depletion of carnitine and overproduction of a toxic metabolite, 4-en-valproic acid, both of which impair the urea cycle and thus ammonia elimination. Carnitine is a commonly used antidote for acute intoxication of valproic acid, but is not a therapeutic option for management of chronic adults with adverse effects related to valproic acid...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28425418/recurrent-hyperammonemia-after-abernethy-malformation-type-2-closure-a-case-report
#18
Hui Li, Zhi Ma, Ying Xie, Feng Tian
The Abernethy malformation is a rare congenital malformation defined by the presence of an extrahepatic portosystemic shunt. Although most patients are asymptomatic, clinical encephalopathy is present in 15% of cases. We present a patient with type 2 Abernethy malformation, hyperammonemia, and encephalopathy. Shunt closure was performed successfully using interventional angiography; however, hyperammonemia recurred 3 months later. The diagnosis of Abernethy malformation can be made easily, but the ideal patient management strategy has not yet been established...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28422839/unexpected-side-effect-in-mcrc-a-care-compliant-case-report-of-regorafenib-induced-hyperammonemic-encephalopathy
#19
Michela Quirino, Sabrina Rossi, Giovanni Schinzari, Michele Basso, Antonia Strippoli, Alessandra Cassano, Carlo Barone
RATIONALE: Regorafenib represents a treatment option in heavily pretreated patients affected by metastatic colorectal cancer (mCRC). Its safety profile is typical of small-molecule tyrosine-kinase inhibitors (TKIs) and most adverse events are manageable. PATIENT CONCERNS: A 56 years-old Caucasian man affected by mCRC with normal hepatic reserve was treated with regorafenib as second-line treatment. After only 2 days of therapy, the patient presented to the emergency department due to impairment of both spatial and temporal orientation and motor function with bradylalia...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28411969/valproate-induced-hyperammonemic-encephalopathy-in-general-hospital-patients-with-one-or-more-psychiatric-disorders
#20
Chandani Lewis, George E Tesar, Roman Dale
BACKGROUND: Divalproex sodium/valproic acid (VPA) is an antiepileptic drug approved for use in epilepsy and bipolar disorder. Valproate-induced hyperammonemia occurs in up to 50% of VPA-treated patients, some of whom may become encephalopathic. Valproate-induced hyperammonemic encephalopathy (VHE) is thought to be rare, and for a variety of reasons, the diagnosis requires a high index of suspicion. OBJECTIVE: The study's goals are to determine how common VHE is, and the quality of treatment provided when diagnosed...
February 24, 2017: Psychosomatics
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