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Topic: Hyperammonemia

Related Topics

Methylmalonic acidemia

Propionic acidemia

Metabolism

Enzyme

Protein

Gene

Autosomal recessive

Lethargy

	Hyperammonemia - Wikipedia, the free encyclopedia
		Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood.
		Secondary hyperammonemia is caused by inborn errors of intermediary metabolism characterised by reduced activity in enzymes that are not part of the urea cycle (e.g.
		Hyperammonemia is one of the metabolic derangements that contribute to the encephalopathy associated with hepatic failure.
	en.wikipedia.org /wiki/Hyperammonemia (277 words)

	eMedicine - Hyperammonemia : Article by Karl S Roth, MD (Site not responding. Last check: 2007-10-18)
		The pathophysiology of hyperammonemia is that of a CNS toxin that causes irritability, somnolence, vomiting, cerebral edema, and coma that leads to death.
		Hyperammonemia is a medical emergency because of the neurotoxicity, which is a direct effect of ammonia on the CNS.
		Tofteng F, Hauerberg J, Hansen BA, et al: Persistent arterial hyperammonemia increases the concentration of glutamine and alanine in the brain and correlates with intracranial pressure in patients with fulminant hepatic failure.
	www.emedicine.com /ped/topic1057.htm (2816 words)

	HYPERAMMONEMIA IN A 5 YEAR OLD FEMALE CHILD PEDIATRIC ONCALL
		In neonates, hyperammonemia presents as refusal to feed, vomiting, tachypnea and lethargy progressing to coma.
		Hyperammonemia is seen in conditions such as urea cycle defects, organic acidemias like propionic acidemia, Methyl malonic acidemia, isovaleric acidemia, ketothiolase deficiency, multiple carboxylase deficiency, glutaric acidemia Type II and hyperornithinemia - hyperammonemia – homocitrullinemia syndrome.
		Recurrent episodes of hyperammonemia (manifested by vomiting, ataxia, mental confusion) with periods of well-being and normal mental development are seen in heterozygote females and in some affected males with ornithine transcarbamylase (OTC) deficiency.
	www.pediatriconcall.com /FORDOCTOR/CaseReports/hyper.asp (741 words)

	eMedicine - Hyperammonemia : Article by Kazi Imran Majeed, MD, (Site not responding. Last check: 2007-10-18)
		The mechanism for hyperammonemia is the deficiency of ornithine and arginine.
		The clinical presentation of hyperammonemia in the neonatal period is nonspecific and merely indicates that the infant is in distress; therefore, disorders such as sepsis, intracranial hemorrhage, cardiac disease, and gastrointestinal obstruction should be ruled out with appropriate laboratory and imaging studies.
		Hyperammonemia with respiratory alkalosis is caused by a urea cycle defect or transient hyperammonemia of the newborn.
	www.emedicine.com /neuro/topic162.htm (4734 words)

	Proteins & Amino Acids - Arginine
		Subtle impairments of the liver's function can often lead to subclinical symptoms of hyperammonemia, which is the condition of poor excretion of urea.
		Symptoms of hyperammonemia may include chronic fatigue, headache, irritability, occasional diarrhea or nausea, lack of concentration, mental confusion, and intolerance to various foods, particularly high-protein foods.
		Arginine is useful in some individuals as a supplement to help treat the symptoms of hyperammonemia if they have a block in the proper metabolism or synthesis of arginine in the liver.
	www.springboard4health.com /notebook/proteins_arginine.html (908 words)

	Indian Pediatrics - Editorial
		Two cases of hyperammonemia with elevated citrulline are reported, one resulting from a deficiency of pyruvate carboxylase and the other from a partial deficiency of argininosuccinate synthetase.
		Hyperammonemia accompanied by citrullinemia can occur either from a disruption of energy metabolism due to an absence of pyruvate carboxylase or from a defect of the urea cycle.
		Hyperammonemia with critically elevated levels of citrulline indicates a deficiency of argininosuccinate synthetase (EC 6.3.4.5) in the urea cycle, the classical citrullinemia type II (CTLN2; McKusick 603471).
	www.indianpediatrics.net /aug2004/aug-842-844.htm (1314 words)

	Bioline International Official Site (site up-dated regularly) (Site not responding. Last check: 2007-10-18)
		Hyperammonemia is more common in children[2] and develops within days to weeks of initiation of treatment.
		Hyperammonemia is an uncommon adverse effect in patients on valproate monotherapy.
		Hyperammonemia is a serious adverse effect of valproate therapy and may be potentially life-threatening in the presence of underlying hepatic dysfunction and enzyme deficiency disorders.
	www.bioline.org.br /request?ni05071 (1673 words)

	Welcome to THE MIDDLE EAST JOURNAL OF EMERGENCY MEDICINE (Site not responding. Last check: 2007-10-18)
		The consequential hyperammonemia is a key factor in the development of central nervous system dysfunction in these patients.
		Due to the rare nature of these disorders, their various aspects of clinical presentations and acute management of hyperammonemia in an ED setting have yet to be well defined.
		Hyperammonemia is the major metabolic derangement in these disorders resulting in brain damage, coma and death.
	www.hmc.org.qa /mejem/sept2004/Edited/oral92.htm (429 words)

	health.iafrica.com \| doc online \| ask the doctor \| genetic illness Hyperammonemia
		Hyperammonemia types I, IA and II are only three of several defects in amino acid catabolism (manufacture).
		Hyperammonemia type IA describes a defect in the enzyme N-acetylglutamate synthetase which affects the amino acid N-acetylglutamate.
		Hyperammonemia type II describes a defect in the enzyme ornithine transcarbamylase, which affects the amino-acid ornithine.
	health.iafrica.com /doconline/qa/genetic/hyperammonemia.htm (571 words)

	Final Diagnosis -- Case 335
		Causes of hyperammonemia that are not genetic include severe dehydration and liver failure.
		Hyperammonemia usually is seen late in the course of severe hepatocellular damage (1).
		Hyperammonemia in women with a mutation at the ornithine carbamoyltransferase locus.
	path.upmc.edu /cases/case335/dx.html (1531 words)

	Preserved hypocapnic pial arteriolar constriction during hyperammonemia by glutamine synthetase inhibition -- Hirata et ...
		with hyperammonemia is ameliorated by hypocapnia, thereby passively
		Hyperammonemia increased basal diameter of pial arterioles without increasing normocapnic CBF above that of the control group.
		Acute hyperammonemia in the young primate: physiologic and neuropathologic correlates.
	ajpheart.physiology.org /cgi/content/full/276/2/H456 (3668 words)

	Hyperammonemia Hyperornithinemia Homocitrullinuria Syndrome (HHH)
		The etiology is a deficiency of a mitochondrial carrier protein that normally functions to transport Ornithine into the mitochondria as part of the urea cycle.
		The ORNT 1 gene that codes for the transport protein is located on chromosome 13, and several mutations have been identified in affected patients.
		Hyperammonemia occurs postprandially and is chronically elevated on a high protein diet, but may be normal when fasting.
	www.pediatrix.com /body_screening_menu.cfm?id=1578 (632 words)

	Hyperammonemia In Septicemic Patients (Site not responding. Last check: 2007-10-18)
		In this study there was hyperammonemia with septic illness and collection of pus in the 3 cases and urea splitting organisms were cultured in first and second cases and were likely in the third case.
		Hyperammonemia causes an encephalopathy secondary to astrocyte swelling, increased permeability of the blood brain barrier, mitochondrial changes and cerebral edema.
		Comment: Thus it can be concluded that in all critically ill and potentially septic children in whom the clinical signs and symptoms of encephalopathy may not be readily apparent, the plasma ammonia must be measured and also any infected collection or obstructed viscus should be identified and drained.
	www.indegene.com /Ped/Jour/indJour_ADC_Sum_01-05-2001_4.asp (564 words)

	Hyperammonemia -
		Primary hyperammonemia is caused by several inborn errors of metabolism hyperammonemia that are characterised by reduced activity of any of the enzymes in the urea cycle.
		Secondary hyperammonemia is caused by inborn errors of intermediary metabolism characterised by reduced activity in enzymes that are not part of the urea cycle (e.g.Propionic acidemia, Methylmalonic acidemia) or dysfunction of cells that make major contributions to metabolism (eg hepatic failure).
		A disease is any abnormal condition of the body or mind that causes discomfort, dysfunction, or distress to the person affected or those in contact with the person.
	www.medicalgeo.com /Med-Diseases-Ho---Hy/Hyperammonemia.html (242 words)

	Glutamine-dependent inhibition of pial arteriolar dilation to acetylcholine with and without hyperammonemia in the rat ...
		glutamine in the absence of hyperammonemia decreases hypercapnic
		in the absence of hyperammonemia attenuated dilation to ACh
		Primary dilation of the cerebral resistance vessels as a cause of increased intracranial pressure.
	ajpregu.physiology.org /cgi/content/full/288/6/R1612 (4129 words)

	Inborn Errors of Metabolism in Infancy: A Guide to Diagnosis -- Burton 102 (6): 69 -- Pediatrics (Site not responding. Last check: 2007-10-18)
		A flowchart for the differentiation of conditions producing significant hyperammonemia in the newborn is presented in Fig 1.
		Not all infants with life-threatening metabolic disease have metabolic acidosis or hyperammonemia.
		Hudak ML, Jones MD Jr, Brusilow SW Differentiation of transient hyperammonemia of the newborn and urea cycle defects by clinical presentation.
	pediatrics.aappublications.org /cgi/content/full/102/6/e69 (4597 words)

	UCDC for Patients - Learn More
		This causes hyperammonemia (an increase of ammonia in the blood).
		Arginase deficiency is not typically characterized by rapid-onset hyperammonemia (high ammonia levels in the blood).
		Diagnosis is made by the elevated levels of arginine in the blood and by analysis of enzymatic activity in red blood cells.
	rarediseasesnetwork.epi.usf.edu /ucdc/learnmore/definitions.htm (1478 words)

	Ornithine Transcarbamylase Deficiency
		OTC is one of six enzymes that play a role in the break down and removal of nitrogen the body, a process known as the urea cycle.
		The estimated frequency of OTC deficiency is one in 80,000.
		People with these disorders also have hyperammonemia and experience symptoms that are similar to those of urea cycle enzyme disorders.
	hw.healthdialog.com /kbase/nord/nord309.htm (2784 words)

	Ammonium-Induced Impairment of Axonal Growth Is Prevented through Glial Creatine -- Braissant et al. 22 (22): 9810 -- ...
		Hyperammonemia in neonates and infants affects brain development and causes mental retardation.
		Braissant O, Honegger P, Loup M, Iwase K, Takiguchi M, Bachmann C (1999b) Hyperammonemia: regulation of argininosuccinate synthetase and argininosuccinate lyase genes in aggregating cell cultures of fetal rat brain.
		Ratnakumari L, Qureshi IA, Butterworth RF (1992) Effects of congenital hyperammonemia on the cerebral and hepatic levels of the intermediates of energy metabolism in spf mice.
	www.jneurosci.org /cgi/content/full/22/22/9810 (5656 words)

	Other Diseases (Site not responding. Last check: 2007-10-18)
		Background: The cause of severe acquired hyperammonemia, an uncommon but often fatal complication of organ transplantation and chemotherapy for cancer, is obscure.
		The activity of hepatic glutamine synthetase was markedly reduced (in patient 1, 12% of the mean value in controls; in patient 2, 28% of the mean value in controls), and a concomitant reduction in the amount of glutamine synthetase protein was observed.
		Conclusion: Hyperammonemia after transplantation was associated with hepatic glutamine synthetase deficiency in two patients, but the causal relation between these two conditions must be further studied.
	www.altcorp.com /AffinityLaboratory/gsotherdisease.htm (229 words)

	Specialty Laboratories ::: we help doctors help patients
		The first, carbamylphosphate synthetase, transfers ammonia nitrogen to bicarbonate to form carbamylphosphate; the last enzyme, arginase, hydrolyzes arginine to form ornithine and urea; waste nitrogen is excreted in the urine via the latter compound.
		Other inborn errors of metabolism causing hyperammonemia include the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, lysinuric protein intolerance and the organic acidemias and carnitine deficiencies (in which the urea cycle is poisoned as a secondary effect).
		Acquired hyperammonemias can result from overload or bypass of the urea-generating system, as in chemotherapy and ureterosigmoidoscopy.
	www.specialtylabs.com /books/display.asp?id=193 (498 words)

	Ammonul
		Ammonul is indicated as adjunctive therapy for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.
		In acute neonatal hyperammonemic coma, in moderate to severe episodes of hyperammonemic encephalopathy, and in episodes of hyperammonemia which fail to respond to an initial course of Ammonul therapy, hemodialysis is the most rapid and effective technique for removing ammonia.
		Acute symptomatic hyperammonemia should be treated as life-threatening.
	ammonul.com (370 words)

	ScienceDaily: After A Lung Transplant, Patients May Suffer Dangerous Blood Ammonia Levels (Site not responding. Last check: 2007-10-18)
		Of those six patients with hyperammonemia, four, or 67 percent, died within 30 days of the surgery, compared to deaths in 24 (or 17 percent) of the 139 patients with normal levels of blood ammonia.
		The only lung transplant patient with hyperammonemia who survived had her condition recognized early and received hemodialysis and medications to lower her blood ammonia level.
		Among the lung transplant recipients in the study sample who developed hyperammonemia, the researchers identified certain risk factors: major gastrointestinal complications (such as intestinal bleeding, intestinal perforation or infection), the need for feeding through an intravenous line (total parenteral nutrition), and high pressure in the arteries leading to the lungs (primary pulmonary hypertension).
	www.sciencedaily.com /releases/2000/02/000214112147.htm (1841 words)

	[No title]
		Manufacturer Medicis Pharmaceutical Corp., Scottsdale, AZ 85258 Indication(s)1 Ammonul is indicated as adjunctive therapy for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.
		The efficacy of sodium phenylacetate and sodium benzoate as adjunctive therapy for the treatment of acute hyperammonemia has been demonstrated in an analysis of data from 316 patients who were hospitalized with acute hyperammonemia (1,045 episodes) over a 22-year period of time.
		Uncontrolled hyperammonemia can rapidly result in brain damage or death, and prompt use of all therapies necessary to reduce ammonia levels is essential.
	www.heritage-info.com /mocaidrx/june2005drugs/Ammonulmon.doc (1971 words)

	Comparison of the effects of ammonia on brain mitochondrial function in rats and gulf toadfish -- Veauvy et al. 283 ...
		We compared the effect of hyperammonemia on NADH levels in brain slices and on the rate of oxygen consumption from isolated
		Opsanus beta, the gulf toadfish, is a potentially important animal model for ammonia toxicity studies (29).
		Hyperammonemia in brain slices from toadfish did not affect NADH
	ajpregu.physiology.org /cgi/content/full/283/3/R598 (3984 words)

	Low myo-inositol and high glutamine levels in brain are associated with neuropsychological deterioration after induced ...
		Low myo-inositol and high glutamine levels in brain are associated with neuropsychological deterioration after induced hyperammonemia -- Shawcross et al.
		hyperammonemia may be determined not by the degree of hyperammonemia
		the susceptibility of the brain to the effects of induced hyperammonemia.
	ajpgi.physiology.org /cgi/content/full/287/3/G503 (4300 words)

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