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Hyperammonemia

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https://www.readbyqxmd.com/read/28425821/l-carnitine-supplementation-to-reverse-hyperammonemia-in-a-patient-undergoing-chronic-valproic-acid-treatment-a-case-report
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28408159/brain-mrs-glutamine-as-a-biomarker-to-guide-therapy-of-hyperammonemic-coma
#5
Anne H O'Donnell-Luria, Alexander P Lin, Sai K Merugumala, Frances Rohr, Susan E Waisbren, Rebecca Lynch, Vatche Tchekmedyian, Aaron D Goldberg, Andrew Bellinger, J Ricardo McFaline-Figueroa, Tracey Simon, Esteban F Gershanik, Bruce D Levy, David E Cohen, Martin A Samuels, Gerard T Berry, Natasha Y Frank
Acute idiopathic hyperammonemia in an adult patient is a life-threatening condition often resulting in a rapid progression to irreversible cerebral edema and death. While ammonia-scavenging therapies lower blood ammonia levels, in comparison, clearance of waste nitrogen from the brain may be delayed. Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, the major reservoir of ammonia, in a gastric bypass patient with hyperammonemic coma undergoing therapy with N-carbamoyl glutamate and the ammonia-scavenging agents, sodium phenylacetate and sodium benzoate...
March 10, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28403092/hyperammonemic-coma-after-craniotomy-hepatic-encephalopathy-from-upper-gastrointestinal-hemorrhage-or-valproate-side-effect-case-report-and-literature-review
#6
Xiaopeng Guo, Junji Wei, Lu Gao, Bing Xing, Zhiqin Xu
RATIONALE: Postoperative coma is not uncommon in patients after craniotomy. It generally presents as mental state changes and is usually caused by intracranial hematoma, brain edema, or swelling. Hyperammonemia can also result in postoperative coma; however, it is rarely recognized as a potential cause in coma patients. Hyperammonemic coma is determined through a complicated differential diagnosis, and although it can also be induced as a side effect of valproate (VPA), this cause is frequently unrecognized or confused with upper gastrointestinal hemorrhage (UGH)-induced hepatic encephalopathy...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28401058/a-rare-cause-of-recurrent-acute-pancreatitis-in-a-child-isovaleric-acidemia-with-novel-mutation
#7
Elif Sag, Alper Han Cebi, Gulay Kaya, Gulay Karaguzel, Murat Cakir
Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28372492/prenatally-diagnosed-congenital-portosystemic-shunts
#8
Bérengère Francois, Alain Lachaux, Fréderic Gottrand, Stéphanie De Smet
AIM: Congenital portosystemic shunts (CPSS) are rare, congenital malformations that are increasingly often discovered during the fetal period, and for which, the manifestations and evolution are poorly understood. The objective of this review is to describe the phenotype and evolution of forms diagnosed in the antenatal period. MATERIALS AND METHODS: We performed a systematic review of the literature cited in Pubmed between 1982 and 2016 for CPSS cases diagnosed during the fetal period...
April 20, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28350574/recurrent-hyperammonemia-associated-with-olanzapine
#9
Yung-Fu Wu
No abstract text is available yet for this article.
June 2017: Journal of Clinical Psychopharmacology
https://www.readbyqxmd.com/read/28319158/a-morphological-method-for-ammonia-detection-in-liver
#10
Virginia Gutiérrez-de-Juan, Sergio López de Davalillo, David Fernández-Ramos, Lucía Barbier-Torres, Imanol Zubiete-Franco, Pablo Fernández-Tussy, Jorge Simon, Fernando Lopitz-Otsoa, Javier de Las Heras, Paula Iruzubieta, María Teresa Arias-Loste, Erica Villa, Javier Crespo, Raúl Andrade, M Isabel Lucena, Marta Varela-Rey, Shelly C Lu, José M Mato, Teresa Cardoso Delgado, María-Luz Martínez-Chantar
Hyperammonemia is a metabolic condition characterized by elevated levels of ammonia and a common event in acute liver injury/failure and chronic liver disease. Even though hepatic ammonia levels are potential predictive factors of patient outcome, easy and inexpensive methods aiming at the detection of liver ammonia accumulation in the clinical setting remain unavailable. Thus, herein we have developed a morphological method, based on the utilization of Nessler´s reagent, to accurately and precisely detect the accumulation of ammonia in biological tissue...
2017: PloS One
https://www.readbyqxmd.com/read/28302489/modelling-urea-cycle-disorder-citrullinemia-type-1-with-disease-specific-ipscs
#11
Elena Yukie Yoshitoshi-Uebayashi, Taro Toyoda, Katsutaro Yasuda, Maki Kotaka, Keiko Nomoto, Keisuke Okita, Kentaro Yasuchika, Shinya Okamoto, Noriyuki Takubo, Toshiya Nishikubo, Tomoyoshi Soga, Shinji Uemoto, Kenji Osafune
Citrullinemia type 1 (CTLN1) is a urea cycle disorder (UCD) caused by mutations of the ASS1 gene, which is responsible for production of the enzyme argininosuccinate synthetase (ASS), and classically presented as life-threatening hyperammonemia in newborns. Therapeutic options are limited, and neurological sequelae may persist. To understand the pathophysiology and find novel treatments, induced pluripotent stem cells (iPSCs) were generated from a CTLN1 patient and differentiated into hepatocyte-like cells (HLCs)...
March 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28298669/veno-venous-extracorporeal-membrane-oxygenation-for-continuous-renal-replacement-in-a-neonate-with-propionic-acidemia
#12
Jeffrey W Gander, Erika T Rhone, William G Wilson, John P Barcia, Melissa J Sacco
The usual indications for extra corporeal membrane oxygenation (ECMO) are for respiratory or cardiac failure. Although continuous renal replacement therapy (CRRT) is frequently used when patients are on ECMO, the need for CRRT as the primary indication for ECMO is rare. A case of a neonate placed onto veno-venous ECMO for the use of CRRT to treat hyperammonemia from propionic acidemia is presented.
March 2017: Journal of Extra-corporeal Technology
https://www.readbyqxmd.com/read/28295601/idiopathic-hyperammonemia-after-solid-organ-transplantation-primarily-a-lung-problem-a-single-center-experience-and-systematic-review
#13
Dustin Krutsinger, Alejandro Pezzulo, Amy E Blevins, Robert M Reed, Michael Voigt, Michael Eberlein
BACKGROUND: Idiopathic hyperammonemia syndrome (IHS) is an uncommon, often deadly complication of solid organ transplantation. IHS cases in solid organ transplantation seem to occur predominantly in lung transplant (LTx) recipients. However to the best of our knowledge the occurrence of IHS has not been systematically evaluated. We set out to identify all reported cases of IHS following non-liver solid organ transplantations. METHODS: Retrospective review of our institutional experience and systematic review of the literature...
March 10, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28293384/hyperammonemia-crisis-following-parturition-in-a-female-patient-with-ornithine-transcarbamylase-deficiency
#14
Jun Kido, Tatsuya Kawasaki, Hiroshi Mitsubuchi, Hidenobu Kamohara, Takashi Ohba, Shirou Matsumoto, Fumio Endo, Kimitoshi Nakamura
Ornithine transcarbamylase deficiency (OTCD) is an X-linked disorder, with an estimated prevalence of 1 per 80000 live births. Female patients with OTCD develop metabolic crises that are easily provoked by non-predictable common disorders, such as genetic (private mutations and lyonization) and external factors; however, the outcomes of these conditions may differ. We resuscitated a female patient with OTCD from hyperammonemic crisis after she gave birth. Hyperammonemia after parturition in a female patient with OTCD can be fatal, and this type of hyperammonemia persists for an extended period of time...
February 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28283328/carnosine-ameliorates-liver-fibrosis-and-hyperammonemia-in-cirrhotic-rats
#15
Akram Jamshidzadeh, Reza Heidari, Zahra Latifpour, Mohammed Mehdi Ommati, Narges Abdoli, Somayeh Mousavi, Negar Azarpira, Azita Zarei, Mahdi Zarei, Behnam Asadi, Mojgan Abasvali, Yasaman Yeganeh, Faezeh Jafari, Arastoo Saeedi, Asma Najibi, Elnaz Mardani
AIM: Chronic liver injury and cirrhosis leads to liver failure. Hyperammonemia is a deleterious consequence of liver failure. On the other hand, oxidative stress seems to play a pivotal role in the pathogenesis of liver fibrosis as well as in the cytotoxic mechanism of ammonia. There is no promising therapeutic agent against ammonia-induced complications. The present study was conducted to evaluate the role of carnosine (CA) administration on liver pathological changes, elevated plasma ammonia, and its consequent events in cirrhotic rats...
March 7, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28281899/targeting-cps1-in-the-treatment-of-carbamoyl-phosphate-synthetase-1-cps1-deficiency-a-urea-cycle-disorder
#16
Carmen Diez-Fernandez, Johannes Häberle
Carbamoyl phosphate synthetase 1 (CPS1) deficiency (CPS1D) is a rare autosomal recessive urea cycle disorder (UCD), which can lead to life-threatening hyperammonemia. Unless promptly treated, it can result in encephalopathy, coma and death, or intellectual disability in surviving patients. Over recent decades, therapies for CPS1D have barely improved leaving the management of these patients largely unchanged. Additionally, in many cases, current management (protein-restriction and supplementation with citrulline and/or arginine and ammonia scavengers) is insufficient for achieving metabolic stability, highlighting the importance of developing alternative therapeutic approaches...
April 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28275973/normal-neurological-development-during-infancy-despite-massive-hyperammonemia-in-early-treated-nags-deficiency
#17
Hallvard Reigstad, Berit Woldseth, Johannes Häberle
A girl born at term was admitted to the neonatal intensive care unit because of mild respiratory distress after a complicated delivery. She recovered, but was readmitted at 58 h of life with mild respiratory distress and increased muscle tone. Neonatal abstinence syndrome because of maternal use of lithium, clomipramine, and quetiapine during pregnancy was suspected, but at 115 h of life she became unresponsive, and an immediate work-up for coma was initiated. An ammonia of 2,235 μmol/l was found, and treatment with sodium benzoate, sodium phenylacetate, arginine, glucose, and N-carbamylglutamate (NCG, Carbaglu(®)) was started...
March 9, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28270654/a-proposed-physiopathological-pathway-to-hyperammonemic-encephalopathy-in-a-non-cirrhotic-patient-with-fibrolamellar-hepatocellular-carcinoma-without-ornithine-transcarbamylase-otc-mutation
#18
Rodrigo C Surjan, Elizabeth S Dos Santos, Tiago Basseres, Fabio F Makdissi, Marcel A Machado
BACKGROUND Hyperammonemic encephalopathy is a potentially fatal condition that may progress to irreversible neuronal damage and is usually associated with liver failure or portosystemic shunting. However, other less common conditions can lead to hyperammonemia in adults, such as fibrolamellar hepatocellular carcinoma. Clinical awareness of hyperammonemic encephalopathy in patients with normal liver function is paramount to timely diagnosis, but understanding the underlying physiopathology is decisive to initiate adequate treatment for complete recovery...
March 8, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28266016/clinical-and-mutation-analysis-of-24-chinese-patients-with-ornithine-transcarbamylase-deficiency
#19
Y Shao, M Jiang, Y Lin, H Mei, W Zhang, Y Cai, X Su, H Hu, X Li, L Liu
The principal aim of this study was to examine the clinical manifestations, biochemical features, and molecular genetic characteristics of Chinese patients with ornithine transcarbamylase deficiency (OTCD) at a single medical center. We retrospectively analyzed 24 patients (17 males and 7 females) diagnosed with OTCD between 2006 and 2015. Five male patients had a neonatal presentation; 12 male patients had late onset disease and 7 female patients presented as symptomatic. Patients with a neonatal presentation had the highest peak plasma ammonia and glutamine levels at diagnosis with a high mortality (80% vs 16% in late onset disease)...
March 7, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28265840/characterization-of-the-ca1-pyramidal-neurons-in-rat-model-of-hepatic-cirrhosis-insights-into-their-electrophysiological-properties
#20
Mahshid Tahamtan, Iraj Aghaei, Vahid Pooladvand, Vahid Sheibani, Mohammad Khaksari, Mohammad Shabani
Although the key contributors of altering neurological function in hepatic encephalopathy are relatively well known, the electrophysiological mechanism of CA1 damage, a key vulnerable area during hyperammonemia, have not yet been defined. Therefore, here we focus on the electrophysiological mechanisms of cognitive impairments following bile duct ligation (BDL). We performed patch-clamp recordings from the CA1 pyramidal neurons in hippocampus of male Wistar rats, which underwent sham or BDL surgery. A striking electrophysiological change of hippocampal neurons in experimental model of BDL was observed in the present study...
March 7, 2017: Metabolic Brain Disease
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