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By Süleyman Utku Celik a doctor, a general surgeon, at Ankara University Medical of School, in Tukey.
Unnikrishnan Pillai, Roopkiranjot Kahlon, James Sondheimer, Pravit Cadnapaphorncai, Zeenat Bhat
Hyperammonemia is a metabolic derangement that can be potentially fatal. Primary hyperammonemia due to urea cycle enzyme deficiency is usually discovered in neonates but rarely can present in adulthood. Late-onset manifestations of urea cycle disorders can go unnoticed, until they become life threatening. The authors report a 28-year-old man who developed hyperammonemia in the hospital following parenteral nutrition (PN), leading to cerebral edema, which was fatal despite resolution of the hyperammonemia with cessation of PN and the use of continuous renal replacement therapy...
January 2013: JPEN. Journal of Parenteral and Enteral Nutrition
Dae Eun Choi, Kang Wook Lee, Young Tai Shin, Ki Ryang Na
Ornithine carbamoyltransferase (OTC) deficiency is a urea cycle disorder that causes the accumulation of ammonia, which can lead to encephalopathy. Adults presenting with hyperammonemia who are subsequently diagnosed with urea cycle disorders are rare. Herein, we report a case of a late-onset OTC deficient patient who was successfully treated with arginine, benzoate and hemodialysis. A 59-yr-old man was admitted to our hospital with progressive lethargy and confusion. Although hyperammonemia was suspected as the cause of the patient's mental changes, there was no evidence of chronic liver disease...
May 2012: Journal of Korean Medical Science
Brian Houston, Kim A Reiss, Christian Merlo
No abstract text is available yet for this article.
April 2011: American Journal of Medicine
Jamie Nicole LaBuzetta, Jay Z Yao, Daniel L Bourque, Justin Zivin
This article presents a case report of nonhepatic hyperammonemia, i.e., elevated serum ammonia secondary to a nonhepatic etiology. It then discusses the importance of broadening one's differential diagnosis to include such nonhepatic causes of elevated ammonia levels, and provides a short review of rarer causes of hyperammonemia in the adult population. Treating the underlying condition is the best way to prevent recurrence of hyperammonemia. However, symptomatic treatment should not be delayed while investigating the underlying source...
October 2010: American Journal of Medicine
Sharmila Sehli, Kenneth M Nugent, Christina A Samathanam, Raed Alalawi
No abstract text is available yet for this article.
March 2009: Chest
R H Singh
The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein-free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.
November 2007: Journal of Inherited Metabolic Disease
Richard G Boles, Megan L Stone
Arginase deficiency is an urea cycle disorder that generally presents with mental retardation and spasticity, yet uncommonly with episodes of hyperammonemia. A female adolescent with arginase deficiency developed hyperammonemic episodes temporally related to her menstrual cycle, which ceased upon adequate treatment with depot medroxy progesterone acetate. A similar case was previously reported. A catamenial trigger should be considered in adolescent female arginase-deficient patients with episodes of hyperammonemia...
December 2006: Molecular Genetics and Metabolism
Hironori Nagasaka, Tohru Yorifuji, Kei Murayama, Mitsuru Kubota, Keiji Kurokawa, Tomoko Murakami, Masaki Kanazawa, Tomozumi Takatani, Atsushi Ogawa, Emi Ogawa, Shigenori Yamamoto, Masanori Adachi, Kunihiko Kobayashi, Masaki Takayanagi
BACKGROUND: The aim of this study was to investigate the effects of arginine on nutrition, growth and urea cycle function in boys with late-onset ornithine transcarbamylase deficiency (OTCD). Seven Japanese boys with late-onset OTCD enrolled in this study resumed arginine treatment after the cessation of this therapy for a few years. Clinical presentations such as vomiting and unconsciousness, plasma amino acids and urinary orotate excretion were followed chronologically to evaluate urea cycle function and protein synthesis with and without this therapy...
September 2006: European Journal of Pediatrics
Te-I Weng, Frank Fuh-Yuan Shih, Wen-Jone Chen
Plasma ammonia measurement is a simple yet important screening in the ED for patients with unexplained stupor or delirium. Acute hyperammonemia is a medical emergency for which immediate steps must be taken to minimize permanent brain damage. Although the most common causes of hyperammonemia are severe abnormal liver function, the absence of liver disease in some cases has been observed. This brief report describes four hyperammonemia cases with normal liver function in the ED. On careful history and speculated examinations, ornithine carbamoyltransferase (OTC) deficiency, hematologic malignancy, and the side effects of valproic acid and 5-fluorouracil (5-FU) were considered...
March 2004: American Journal of Emergency Medicine
S Kapila, M Saba, C H Lin, E V Bawle
BACKGROUND: Patients with short bowel syndrome and renal dysfunction with TPN dependence are at high risk for developing hyperammonemia if the TPN does not contain sufficient quantities of arginine. Providing proper nutrition support is essential in the management of these patients. METHODS: We report on a patient with short bowel syndrome, TPN dependence, and normal renal function who developed hyperammonemic encephalopathy due to inadvertent lack of arginine in his TPN...
September 2001: JPEN. Journal of Parenteral and Enteral Nutrition
J C Lee, H S Lai, S M Huang, C J Chang, S T Wang, W J Chen
Hyperammonemic encephalopathy has occasionally been reported in uremic patients receiving hyperalimentation with essential amino acid (EAA) as a source of nitrogen as one of the remaining treatment options when the enteric routes were prohibited. We encountered this complication in a patient with normal renal function. A rat animal model was designed to elucidate the mechanism of hyperammonemia resulting from hyperalimentation with EAA as a source of nitrogen. Sixty-four male Long-Evan rats were divided into eight groups receiving feeds ad libitum or different formula of hyperalimentation...
June 1994: Journal of the Formosan Medical Association, Taiwan Yi Zhi
D M Felig, S W Brusilow, J L Boyer
Ornithine transcarbamylase deficiency is an X-linked disorder of the urea cycle that can cause hyperammonemic encephalopathy in hemizygous males and heterozygous females. Affected females typically limit protein intake in their diet. This case report describes a 36-year-old woman with ulcerative colitis who went into hyperammonemic coma after administration of total parenteral nutrition. A similar episode of coma had occurred 7 years earlier after she delivered a normal boy. Heterozygous ornithine transcarbamylase deficiency was diagnosed based on a positive allopurinol tolerance test result after elevated levels of plasma glutamine and low plasma citrulline were detected...
July 1995: Gastroenterology
J H Seashore, M R Seashore, C Riely
Serial blood ammonia (NH3) determinations in 19 low birth weight (LBW) infants, 14 term neonates and 12 children receiving total parenteral nutrition (TPN) have shown that 73% of patients had one or more elevated NH3 values (greater than 150 micrograms/dl). The mean blood NH3 was 220 +/- 13 micrograms/dl in LBW infants, 180 +/- 9 micrograms/dl in 10 infants, and 140 +/- 7 micrograms/dl in children. All of these values are significantly higher than normal (p less than 0.001). There was no difference in incidence or mean blood ammonia concentration between patients receiving casein hydrolysate and those receiving a crystalline amino acid solution...
March 1982: JPEN. Journal of Parenteral and Enteral Nutrition
R E Grazer, J M Sutton, S Friedstrom, F D McBarron
Hyperammonemic associated encephalopathy developed in an adult receiving essential amino acids. Evidence that her encephalopathy was related to her hyperammonemia included (1) elevated CSF glutamine and serum ammonia levels, (2) the absence of any other drug or metabolic cause of encephalopathy, and (3) resolution of her encephalopathy and abnormal ammonia levels with discontinuation of the hyperalimentation. The serum ammonia levels of patients receiving essential amino acid fluid should be monitored. If the levels remain elevated or if toxicity develops, consideration should be given to switching to an alternate fluid...
November 1984: Archives of Internal Medicine
A Benque, G Bommelaer, G Rozental, P Cales, L Cathelineau, D Pham Dinh, A Ribet
We report a case of a 56 year old woman who presented with a long history of chronic attacks of vomiting. On admission to hospital she was cachectic, and attempted parenteral nutrition induced coma. The illness was found to be due to citrullinaemia, a metabolic disorder of the urea cycle. Our patient is the oldest with this disorder so far described in the literature. The main points of the case and its investigation are outlined: hyperammonaemia, amino acid chromatogram, measurement of enzyme activity in skin and liver biopsy material...
May 1984: Gut
A Morali, J F Duhamel, L Cathelineau, C Ricour
Twenty-one children presenting with severe malnutrition were fed with total parenteral nutrition using 2 different aminoacids solutes containing 19% and 42% branched chain aminoacids respectively. With the 42% branched chain aminoacids solute a dysregulation of ammonia metabolism and changes in protein synthesis were observed. Several factors probably accounted for these changes. An excessive intake of branched chain aminoacids in children with malnutrition, under parenteral nutrition, is therefore iatrogenic and inappropriate...
February 1983: Archives Françaises de Pédiatrie
W C Heird, J F Nicholson, J M Driscoll, J N Schullinger, R W Winters
No abstract text is available yet for this article.
July 1972: Journal of Pediatrics
J D Johnson, W L Albritton, P Sunshine
No abstract text is available yet for this article.
July 1972: Journal of Pediatrics
V D Larkin
No abstract text is available yet for this article.
March 1973: Pediatrics
J M Hackl, W Hengl, E Rumpl, F Geir, P Rosmanith, E Jarosch
Metabolic features of parenteral feeding with conventional amino acid solutions were examined in 47 patients over a long period. 30 patients were kept alive by artificial respiration. The metabolic parameters ammonium, blood urea nitrogen, GOT, alkaline phosphatase were carried out, in 6 patients the pattern of amino acids was analysed. All patients showed a significant increase of ammonium during the course of parenteral feeding. The amino acids demonstrated pattern of imbalance. The other other parameters were not changed significantly...
August 1977: Infusionstherapie und Klinische Ernährung
2014-03-21 21:33:37
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