Ammonia ; blood ; Humans ; Liver Failure ; blood ; pathology ; therapy ; Liver, Artificial ; Urea ; blood

OBJECTIVETo study the role of carbamyl phosphate I (CPS-I)and ornithine transcarbamoylase (OCT) levels in cirrhosis patients with and without hepatic encephalopathy, and to analyze the correlations between CPS-Iand OCT with the development of hepatic encephalopathy.

METHODSCPS-I, OCT, plasma ammonia and liver function of 95 cirrhosis patients with hepatic encephalopathy and 25 cirrhosis patients without hepatic encephalopathy in our hospital from January 2008 to December 2009 were analyzed. 60 healthy controls were recruited in the control group. The differences of serum CPS-I, OCT levels among the cirrhosis patients with and without hepatic encephalopathy and the healthy controls were analyzed; the correlations of CPS-I, OCT levels with plasma ammonia and total protein in cirrhosis patients,and the correlations of CPS-I, OCT levels with Child-Pugh classification of cirrhosis symptom severity in cirrhosis were analyzed. the clinical characteristics between patients who had HE and no HE with chi-square tests were compared. Comparisons of CPS-I, OCT levels across patients based on the Child-Pugh classification were performed with One-Way ANOVA and Student-Newman-Keuls, correlation of CPS-I, OCT with other indicators were performed with Pearson correlation analysis.

RESULTSSerum CPS-I and OCT levels in cirrhosis patients with hepatic encephalopathy were (143.3+/-48.5) U/L, (297.0+/-102.6) is multiplied by 10 U/L, which were lower than that in cirrhosis patients without hepatic encephalopathy (180.3+/-51.5) U/L, (351.8+/-109.0) is multiplied by 10 U/L (t = 2.53, t = 2.78, P < 0.01). Compared with healthy controls, serum CPS-I and OCT levels in cirrhosis patients with and without hepatic encephalopathy were all lower (t = 3.21, t = 4.16, t = 2.12, t = 3.15, P < 0.05). CPS-I was correlated with OCT, (r = 0.946, P < 0.05); CPS-I and OCT were negatively correlated with ALT and AST (r = -0.284, r = -0.239, r = -0.303, r = -0.322, P < 0.05). Additionally, CPS-I and OCT levels were negatively correlated with the Child-Pugh classification in Cirrhosis (F = 10.13, F = 20.28, P < 0.01).

CONCLUSIONThe serum CPS-I and COT levels were important factors affecting plasma ammonia in patients with cirrhosis and played an important role in the development of hepatic encephalopathy.

Adult ; Ammonia ; blood ; Carbamoyl-Phosphate Synthase (Ammonia) ; metabolism ; Case-Control Studies ; Female ; Hepatic Encephalopathy ; blood ; enzymology ; Humans ; Male ; Middle Aged ; Ornithine Carbamoyltransferase ; metabolism

This study aimed to investigate the clinical effect of transplantation of CD133⁺ peripheral blood stem cells or umbilical cord mesenchymal stem cells via the hepatic artery in children with type II hyperammonemia and its possible action mechanism. Umbilical cord mesenchymal stem cells were obtained by collecting cord blood (100-150 mL) from healthy fetuses and separating stem cell suspension (5 mL) from the cord blood by hydroxyethyl starch sedimentation. CD133⁺ peripheral blood stem cells were obtained by mobilizing peripheral blood from the fathers of sick children using recombinant human granulocyte colony-stimulating factor for 5 days, collecting mononuclear cells (120 mL), and separating out CD133⁺ cells by sorting. With catheterization and percutaneous puncture, the obtained stem cells were slowly injected into the liver of sick children via the hepatic artery. The changes in clinical symptoms and laboratory indices such as blood ammonia, liver function, and arginine and citrulline concentrations were observed. After stem cell transplantation via the hepatic artery, the 6 children showed significantly decreased blood ammonia levels, and their blood ammonia levels slowly increased 1 to 2 weeks later, but remained below 100 μmol/L, and changes in glutamic-pyruvic transaminase levels were similar to blood ammonia. Plasma citrulline and arginine concentrations increased significantly after transplantation and the increase in citrulline level exceeded the increase in arginine level. An 8 months follow-up visit for one typical patient showed that the weight and height increased after transplantation and sleep was improved without night crying. The child could actively gaze at interesting objects instead of responding indifferently and started to say simple words. With regard to fine motor skills, the child could pinch things with the thumb and middle finger instead of displaying a lack of hand-eye coordination and progress was also made in gross motor skills. Gesell test showed that the child made progress for an average of 3.82 months in all areas. It was concluded that after stem cell transplantation, children with type II hyperammonemia have decreased blood ammonia levels, stable and improved liver function and steadily increased plasma citrulline and arginine concentrations. They display a progressive trend in such aspects as movement, language and environmental adaptability. It is hypothesized that stem cell transplantation via the hepatic artery partially or totally activates, or provides supplementary ornithine carbamoyl transferase, so that plasma citrulline and arginine concentrations increase and urea cycle disorder can be corrected to some extent.
AC133 Antigen ; Ammonia ; blood ; Antigens, CD ; analysis ; Arginine ; blood ; Citrulline ; blood ; Female ; Glycoproteins ; analysis ; Hepatic Artery ; Humans ; Hyperammonemia ; blood ; surgery ; Infant ; Male ; Peptides ; analysis ; Stem Cell Transplantation

PURPOSE: In patients with altered mentality caused by drugs or unknown causes, ammonia is checked to facilitate differential diagnosis or diagnose hepatic coma. This helps early prevention and treatment of brain damage due to hyperammonemia. This study was conducted to evaluate clinical characteristics of intoxicated adult patients with hyperammonemia. METHODS: We evaluated 95 patients with hyperammonemia among intoxicated patients above the age of 15 who visited our ED from January 2013 to December 2015. We analyzed the demographic characteristics and type of poisoning substance, reason for ingestion, toxicological characteristics such as elapsed time from ingestion to hospital visit, lab, clinical progression and complications. Data were evaluated using the student's t test or Mann-Whitney U test for continuous variables, and Chi-square test and Fisher's exact test for frequency analysis of categorical variables. RESULTS: When compared to healthy individuals, patients with hyperammonemia showed statistical significance on their SOFA score (p=0.016) and poison severity score (p<0.001). Additionally, patients with hyperammonemia showed significantly different initial serum AST level (p=0.012) and maximum serum AST level during the hospital stay (p=0.026) when compared to healthy individuals. Moreover, individuals with sustained hyperammonemia compared to transient hyperammonemia showed clinically significant SOFA scores (p<0.001), poison severity scores (p=0.007), mortality rates in the ICU (p=0.021), as well as different duration of hospital stay (p=0.037), serum creatinine level (p=0.002), erythrocyte sedimentation rate (p=0.025), and serum myoglobin (p=0.015). CONCLUSION: Most poisoning-induced hyperammonemia cases were transient and recovered without special treatment. Therefore, hyperammonemia is almost non-specific among poisoning patients.
Adult ; Ammonia ; Blood Sedimentation ; Brain ; Creatinine ; Diagnosis, Differential ; Eating ; Hepatic Encephalopathy ; Humans ; Hyperammonemia* ; Length of Stay ; Mortality ; Myoglobin ; Poisoning

PURPOSE: The association between Helicobacter pylori (H. pylori) and blood ammonia levels in cirrhotic patients is controversial. We aimed to clarify this controvercy by performing a meta-analysis of published studies. MATERIALS AND METHODS: We searched PubMed, EMBASE and Cochrane library for studies which explored the association between H. pylori and blood ammonia levels in cirrhotic patients before May 2012. Six cohort studies involved in 632 H. pylori positive and 396 H. pylori negative cirrhotic patients were eligible for our analysis. The summary estimates were presented as standard means differences (SMD) and 95% confidence intervals (CI) from individual studies. RESULTS: Overall, there was significant association between H. pylori infection and the elevated blood ammonia levels in cirrhotic patients (SMD=0.34, 95% CI=0.21-0.47, I2=42.1%). Sensitivity analysis further confirmed this association. Subgroup analysis showed that the association was found only in Asian ethnicity, but not in Caucasian ethnicity. CONCLUSION: H. pylori infection is associated with elevated blood ammonia levels in cirrhotic patients, and more large scale studies and stratify analysis are warranted in order to further evaluate this association.
Ammonia/blood ; Asian Continental Ancestry Group ; European Continental Ancestry Group ; Helicobacter Infections/*blood ; Helicobacter pylori/pathogenicity ; Humans ; Liver Cirrhosis/*blood/*microbiology ; Publication Bias ; Regression Analysis

OBJECTIVETo analyze the relationship between hepatorenal syndrome (HRS) and plasma ammonia.

METHODSPlasma ammonia, liver and renal function of 465 patients with liver cirrhosis in our hospital, from June 2007 to March 2009, were analyzed. 80 renal dysfunction patients and 80 healthy controls were recruited in the control group. In addition, 40 patients with HRS were followed up.

RESULTSUsing urea as the diagnosis standard of HRS, the morbidity rate of HRS was 39.6%, which was higher than that using creatinine as the diagnosis standard of HRS (Chi-square test = 97.33, P less than 0.01). using urea and creatinine as the diagnosis standard of HRS, the ammonia level of HRS groups was (57.39+/-48.83)mumol/L, (64.80+/-47.25)mumol/L, which were higher than that in the non-HRS groups (t = -3.07, t = -3.67, P less than 0.01). The ammonia level of patients with renal dysfunction was (26.59+/-14.34)mumol/L, which was lower than that in HRS group, non-HRS group (P less than 0.01), but there was no statistical significance between the patients with renal dysfunction and the healthy peoples [(22.36+/-8.72)mumol/L] (t = 1.52, P more than 0.05). The followed-up analysis of 40 patients with HRS indicated that plasma ammonia level was positively correlated with urea and creatinine, and correlation coefficients were 0.874 and 0.834 (P less than 0.05).

CONCLUSIONHepatic encephalopathy is liver-kidney-intestine-brain syndrome. HRS plays an important role in the development of hepatic encephalopathy.

Adult ; Aged ; Ammonia ; blood ; Biomarkers ; blood ; Blood Urea Nitrogen ; Case-Control Studies ; Creatinine ; blood ; Female ; Hepatic Encephalopathy ; etiology ; prevention & control ; Hepatorenal Syndrome ; blood ; diagnosis ; epidemiology ; Humans ; Liver Cirrhosis ; blood ; complications ; Liver Function Tests ; Male ; Middle Aged ; Retrospective Studies

We experienced an unusual case of acute encephalopathy in a 4 month-old boy He was admitted to our hospital because of lethargy and seizures after preceding symptoms of upper respiratory tract infection a few days before admission. On admission, he was in semicomatous mental state with decorticated rigidity. Laboratory tests showed normal blood sugar, ammonia, and transaminase levels. CSF was acellular and sterile. Brain MRI in both 71 and 72 weighted-image showed high signal density in both thalami and caudate nucleus head. After recovery, neurologic sequales of developmental delay, mental retardation, right hemiplegia and seizure remained. We report a case of acute encephalopathy that have clinical course similar to Reye syndrome but have specific brain image.
Ammonia ; Blood Glucose ; Brain ; Caudate Nucleus ; Head ; Hemiplegia ; Humans ; Infant ; Intellectual Disability ; Lethargy ; Magnetic Resonance Imaging ; Male ; Respiratory Tract Infections ; Reye Syndrome ; Seizures

BACKGROUND/AIMS: Helicobacter pylori (H. pylori) can survive in the acid milieu of stomach by producing urease, which generates acid neutralizing ammonia by splitting gastric urea and creates a satisfactory environment for H. pylori. Thus the patients with chronic renal failure (CRF) with increased diffusion of blood urea to gastric lumen may be theoretically more susceptible to colonization with H. pylori. To investigate the infection rate of H. pylori in CRF and its relation to gastrointestinal symptoms, we performed prospective controlled study. METHODS: We performed gastroscopy in forty-two patients with CRF. Rapid urease test and histologic examination for H. pylori infection were performed. Histological gastritis was graded by updated Sydney classification. Gastrointestinal symptoms were assessed in all CRF patients and serum blood urea nitrogen and creatinine levels were also measured. RESULTS: Twenty-one (50.0%) demonstrated H. pylori infection in patients with CRF. H. pylori infection and major endoscopic findings were not related to the gastrointestinal symptoms in patients with CRF. In H. pylori-positive CRF patients, density of H. pylori and grade of histological gastritis were not related to the severity of gastrointestinal symptoms. CONCLUSIONS: The infection rate of H. pylori was 50% in patients with CRF. Gastrointestinal symptoms in CRF were related to factors other than H. pylori infection.
Ammonia ; Blood Urea Nitrogen ; Classification ; Colon ; Creatinine ; Diffusion ; Gastritis ; Gastroscopy ; Helicobacter pylori* ; Helicobacter* ; Humans ; Kidney Failure, Chronic* ; Prospective Studies ; Stomach ; Urea ; Urease

Hyperinsulinism/Hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism (CHI) caused by a mutation in the GLUD1 gene. It is characterized by hyperinsulinemic hypoglycemia accompanying hyperammonemia. A 6-month-old male infant presented with seizure caused by fasting-induced hypoglycemia. At the time of seizure, the serum glucose and ammonia levels were 17 mg/dL and 203 micron mol/L, respectively. Even though he was fed as usual, his blood glucose level reduced to below 50 mg/dL with an increased plasma insulin level. He was thought to have hyperinsulinemic hypoglycemia associated with hyperammonemia. Analysis of the GLUD1 gene revealed a heterozygous c.1337G>A (p.Gly446Asp) mutation. He was administered diazoxide, following which his blood glucose levels were maintained within the normal range. Because HI/HA syndrome is a diazoxide-responsive form of CHI, early detection and appropriate management are important to prevent brain injury. Since patients with HI/HA syndrome may have neurological complications such as developmental delay, and cognitive impairment, careful and repeated neurologic evaluation is needed.
Ammonia ; Blood Glucose ; Brain Injuries ; Congenital Hyperinsulinism ; Diazoxide ; Glucose ; Glutamate Dehydrogenase ; Glutamic Acid ; Humans ; Hyperammonemia ; Hyperinsulinism ; Hypoglycemia ; Infant ; Insulin ; Male ; Plasma ; Reference Values ; Seizures

OBJECTIVETo investigate the incidences of urea cycle defects (UCDs) in the patients with hyperammonemia and study their etiology, clinical and laboratory features.

METHODSIn the past 7 years, 26 cases (10.2%) of UCDs were detected from 254 patients with hyperammonemia. The etiological diagnoses were made by blood amino acids analysis, urinary organic acid analysis and blood acylcarnitine profile analysis. Three patients with citrullinemia type II were further confirmed by liver pathological analysis and gene diagnosis.

RESULTSAmong 26 cases with UCDs, 15 had ornithine transcarbamylase (OTC) deficiency, 5 had citrullinemia type I, 3 had citrullinemia type II and 3 patients had arginemia. The age of onset of the patients ranged from 3 days to 13 years. Three cases (11.5%) developed hyperammonemic encephalopathy during neonatal period. Thirteen (50.0%), 7 (26.9%) and 3 (11.5%) cases developed clinical symptoms at the age of 1 to 12 months, 1 to 3 years and 6 to 13 years, respectively. Positive family history was found in 11 cases (42.3%). Among 26 patients with UCDs, 9 (34.6%) were hospitalized with the complains of seizures, psychomotor retardation, vomiting and unconsciousness, 8 (30.8%) with recurrent vomiting, headache and coma, 6 due to liver dysfunction. Intrahepatic cholestatic jaundice was found in 3 patients with citrullinemia type II. Blood ammonia ranged from 58 to 259 micromol/L on their first visit to our hospital. Twenty cases (76.9%) had liver dysfunction, 4 patients (15.4%) were diagnosed postmortem. Twenty-one patients got treatment and were followed up. Among them, 7 cases died of hyperammonemic encephalopathy or upper alimentary tract bleeding. Clinical improvement was observed in 14 cases. A boy with OTC deficiency who received a partial liver transplant from his mother showed normal general condition for two years.

CONCLUSIONSUCDs are the most frequent causes of congenital hyperammonemia. In this study, 26 patients (10.2%) with UCDs were identified from 254 patients with hyperammonemia resulting in encephalopathy and liver dysfunction. Early diagnosis and treatment can contribute a lot to improve the prognosis of the patients. Blood ammonia assay and further etiological analysis should be considered in the differential diagnosis of neurological and hepatic abnormality.

Adolescent ; Ammonia ; blood ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Hyperammonemia ; congenital ; diagnosis ; genetics ; Infant ; Infant, Newborn ; Male ; Urea ; metabolism