UREA AS A SOURCE OF BLOOD AMMONIA

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.

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Diagnostic Criteria for Influenza B-Associated Reye's Syndrome: Clinical vs. Pathologic Criteria

PEDIATRICS ◽

10.1542/peds.60.5.702 ◽

1977 ◽

Vol 60 (5) ◽

pp. 702-708

Author(s):

Lawrence Corey ◽

Robert J. Rubin ◽

Dennis Bregman ◽

Michael B. Gregg

Keyword(s):

Disease Control ◽

Diagnostic Criteria ◽

Hepatic Dysfunction ◽

Elevated Serum ◽

Acute Onset ◽

Demographic Characteristics ◽

Influenza B ◽

Elevated Blood ◽

Blood Ammonia ◽

Reye's Syndrome

Between December 15, 1973, and June 30, 1974, a total of 379 cases of Reye's syndrome was reported to the Center for Disease Control. One hundred forty-seven (40%) were confirmed by either autopsy or biopsy, while 232 were diagnosed by clinical and laboratory parameters. Comparisons of the epidemiologic and demographic characteristics, the hospital course, the outcome, and the laboratory abnormalities of the clinically diagnosed and the pathologically confirmed cases revealed no significant differences. In the epidemiologic setting of influenza B outbreaks, children who have the acute onset of noninflammatory encephalopathy associated with elevated serum transaminase levels, hypoprothrombinemia, and elevated blood ammonia levels should be considered to have Reye's syndrome. Further evaluation of diagnostic criteria is needed, however, for sporadically occurring, nonepidemic cases of noninflammatory encephalopathy associated with hepatic dysfunction.

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HYPERAMMONEMIA DUE TO A MUTANT ENZYME OF ORNITHINE TRANSCARBAMYLASE

PEDIATRICS ◽

10.1542/peds.48.4.595 ◽

1971 ◽

Vol 48 (4) ◽

pp. 595-600

Author(s):

Ichiro Matsuda ◽

Shinichiro Arashima ◽

Haruo Nambu ◽

Yasuo Takekoshi ◽

Michiya Anakura

Keyword(s):

In Vitro Study ◽

Ornithine Transcarbamylase ◽

Vitro Study ◽

Mutant Enzyme ◽

Elevated Blood ◽

Blood Ammonia ◽

Carbamyl Phosphate

In a case of congenital hyperammonemia described in an 8½-month-old girl, elevated blood ammonia was shown to result from a mutant enzyme of ornithine transcarbamylase. An in vitro study indicated a decrease in affinity of the enzyme for carbamyl phosphate but not for ornithine.

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Mitochondrial Dysfunction in Early Diagnosed Autism Spectrum Disorder Children

Journal of Dhaka Medical College ◽

10.3329/jdmc.v26i1.34000 ◽

2017 ◽

Vol 26 (1) ◽

pp. 43-47 ◽

Cited By ~ 3

Author(s):

Shorifa Shahjadi ◽

Arif Salam Khan ◽

Mesbah Uddin Ahmed

Keyword(s):

Autism Spectrum Disorder ◽

Mitochondrial Dysfunction ◽

Venous Blood ◽

Autism Spectrum ◽

Serum Lactate ◽

Spectrum Disorder ◽

Control Group ◽

P Value ◽

Blood Ammonia ◽

Normal Children

Background: Mitochondrial dysfunction and abnormal brain bioenergetics can cause autism.Cellular function impairment due to mitochondrial dysfunction may cause cognitive impairment, language deficits and abnormal energy metabolism in autism.Objective: The aim of this study was to evaluate biochemical evidence of the mitochondrial dysfunction by measuring blood ammonia, serum lactate, alanine aminotransferase (ALT), aspartate aminotransferase (AST) and creatinine kinase (CK) in autism spectrum disorder children.Methods: This observational type of analytical study with case-control design was conducted in the Department of Physiology of Bangabandhu Sheikh Mujib Medical University (BSMMU), Shahbag, Dhaka. For this study, a total number of 20 Subjects were randomly selected, among which 10 were apparently healthy subjects (control group-A) for comparison and 10 were diagnosed children with autism spectrum disorder (study group-B). 5ml venous blood was collected from both groups for analysis serum CK, AST, ALT, lactate and blood ammonia. Blood ammonia, serum lactate, AST, ALT and CK level were estimated in all children by standard laboratory method. Independent samplet test was used for statistical analysis. P value <0.05 was accepted as significant. The mean of all the measured biochemical variables in normal children were within normal ranges.Result: Blood ammonia, serum lactate, AST, CK were found significantly higher in autism spectrum disorder children in comparison to control Conclusion: From the result of this study it may be concluded that mitochondrial dysfunction occur in autistic spectrum disorder children .J Dhaka Medical College, Vol. 26, No.1, April, 2017, Page 43-47

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Significant Improvement of Survival by Intrasplenic Hepatocyte Transplantation in Totally Hepatectomized Rats

Cell Transplantation ◽

10.1177/096368979600500303 ◽

1996 ◽

Vol 5 (3) ◽

pp. 369-378 ◽

Cited By ~ 14

Author(s):

Birgit A.P.M. Vogels ◽

Martinus A.W. Maas ◽

Anne Bosma ◽

Robert A.F.M. Chamuleau

Keyword(s):

Liver Failure ◽

Acute Liver Failure ◽

Survival Time ◽

Histological Examination ◽

Hepatocyte Transplantation ◽

Shunt Operation ◽

Blood Ammonia ◽

Liver Atrophy ◽

Arterial Blood ◽

Clinical Grading

The effect of intrasplenic hepatocyte transplantation (HTX) was studied in an experimental model of acute liver failure in rats with chronic liver atrophy. Rats underwent a portacaval shunt operation on Day -14 to induce liver atrophy, and underwent total hepatectomy on Day 0 as a start of acute liver failure. Intrasplenic hepatocyte or sham transplantation was performed on Day -7, -3, or -1 (n = 4 to 6 per group). During the period following hepatectomy, mean arterial blood pressure was maintained above 80 mm Hg and hypoglycaemia was prevented. Severity of hepatic encephalopathy was assessed by clinical grading and EEG spectral analysis, together with determination of blood ammonia and plasma amino acid concentrations, and “survival” time. Histological examination of the spleen and lungs was performed after sacrifice. Intrasplenic hepatocyte transplantation resulted in a significant improvement in clinical grading in all transplanted groups (p < 0.05), whereas a significant improvement in EEG left index was seen only in the group with transplantation on Day -1 (p < 0.05). In contrast to hepatocyte transplantation 1 day before total hepatectomy, rats with hepatocyte transplantation 3 and 7 days before total hepatectomy showed a significant 3- and 2-fold increase in “survival” time compared to sham transplanted controls: HTX at Day -1: 7.5 ± 0.3 h vs. 5.9 ± 0.6 h (p > 0.05), HTX at Day -3:19.7 ± 3.7 h vs. 6.5 ± 0.3 h (p < 0.05), and HTX at Day -7: 13.8 ± 3.2 h vs. 6.3 ± 0.3 h (p < 0.05). Furthermore, rats with hepatocyte transplantation on Day -3 and -7 showed significantly lower blood ammonia concentrations after total hepatectomy (p < 0.0001). Histological examination of the spleens after sacrifice showed clusters of hepatocytes in the red pulp. Hepatocytes present in the spleen for 3 and 7 days showed bile accumulation and spots of beginning necrosis. The present data show that in a hard model of complete liver failure in portacaval shunted rats, intrasplenic hepatocyte transplantation is able to prolong “survival” time significantly 2- to 3-fold. The relevance of this observation for human application is discussed.

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