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| SHORT COMMUNICATION |
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| Year : 2013 | Volume
: 4
| Issue : 2 | Page : 111-112 |
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Hepatic encephalopathy: An enigma
Rajamanickom Saranya, K Shreedhara Avabratha, Aby Dany Varghese, B Sanjeev Rai
Department of Pediatrics, Father Muller Medical College and Hospital, Mangalore, Karnataka, India
| Date of Web Publication | 16-Sep-2013 |
Correspondence Address:
K Shreedhara Avabratha
Department of Pediatrics, Fr Muller Medical College, Mangalore - 575 002, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-9727.118241
Hepatic encephalopathy (HE) is an important and common metabolic disturbance in children. The causes are many. Reye-like syndrome (RLS) can manifest with hypoglycemia, hyperammonemia and altered sensorium with elevated liver enzymes as in Reye syndrome, where the cause remains elusive. This disease is associated with a significant risk of mortality. This report discusses the diagnostic possibilities with a brief review of literature in a one year boy who recovered completely from acute hepatic encephalopathy. Keywords: Hepatic encephalopathy, metabolic disorders, Reye like syndrome
How to cite this article:
Saranya R, Avabratha K S, Varghese AD, Rai B S. Hepatic encephalopathy: An enigma. Muller J Med Sci Res 2013;4:111-2 |
| Introduction | |  |
Hepatic encephalopathy (HE) is an important and common metabolic disturbance in children. Causes of HE in children are varied and range from viral hepatitis to inborn errors of metabolism, as opposed to predominant alcoholic liver disease in adults. [1] Reye syndrome is an acute metabolic encephalopathy with fatty degeneration of the liver, seen commonly in children and adolescents. The Reye-like syndrome (RLS) can manifest with hypoglycemia, hyperammonemia, and altered sensorium with elevated liver enzymes, as in the Reye syndrome, where the cause remains elusive. Of late a number of metabolic errors like fatty-acid oxidation defects have been incriminated, especially in a recurrent Reye-like syndrome. This disease is associated with a significant risk of sudden death or permanent damage of the central nervous system. We report a case of a one-year-old boy who recovered completely from acute hepatic encephalopathy, along with a brief discussion on the varied possibilities and review of literature.
| Case Report | | |
A one-year-old boy presented with altered sensorium of one-day duration. He had a history of fever and cough for 10 days and black-colored stools for two days. He had received paracetamol and antibiotics at a local hospital. On examination his Glasgow Coma Scale (GCS) was 9/15 and he had hepatomegaly without clinical jaundice. The spleen was not palpable. After admission he developed convulsions and vomiting and his GCS worsened. Investigations showed Hb: 8.6g%, RBS-50 mg/dl, CRP:24.4 mg/l, prolonged PT, aPTT, SGPT-6898IU/l,SGOT-17541IU/l, S.album  in-2.9 gm/dl, and conjugated hyperbilirubinemia (Total/Direct-3.3/2.5 mg/dl). The plasma ammonia (153 μmol/L) was elevated. Hepatitis A virus (HAV), hepatitis B surface antigen (HBSAg), hepatitis C virus (HCV), and blood culture tests were negative. Ultrasound abdomen showed features of acute hepatitis and mild ascites. Computed tomography (CT) of the brain was normal. Ophthalmological examination did not reveal the Kayser - Fleischer ring and the serum ceruloplasmin level was normal. A working diagnosis of hepatic encephalopathy/Reye-like syndrome was made. The child was treated with anticonvulsants, broad spectrum antibiotics, vitamin K, and blood components. The child was started on lactulose, carnitine, L-Ornithine, and L-aspartate, and co-enzyme Q supplements and ursodeoxycholic acid. The level of consciousness gradually improved and the GCS was 14/15 by day six. The liver size normalized by two weeks. The child was started on a liquid diet by day eight, which was gradually increased and he was on normal diet by day 12. The child was followed up regularly. His parameters gradually improved and he needed plasma transfusion to normalize the PT and aPTT at the first followup. The supplements were continued for six months. One year after the incident there is no further recurrence and his development is normal.
| Discussion | | |
Hepatic encephalopathy is an important metabolic disturbance in children and can have varied etiologies. The Reye syndrome (RS) is a life-threatening state with encephalopathy and fatty degeneration of the liver, which appeared suddenly in 1963. [2] In the 1970s and 1980s RS was the epitome of pediatric intensive care. The origin of the illness is unknown, but failure of the mitochondria function is thought to be the underlying cause for the disorder. [3],[4],[5] A number of epidemiological case - control studies demonstrate an association with viral infection and ingestion of aspirin. [6] Later, its occurrence began to decrease significantly. There is a considerable variation, however, in the incidence, presentation, and severity of the disease. Moreover, its etiology, particularly in infants, is unclear.
By the late 1980s, a number of inborn errors of metabolism were discovered that could mimic RS clinically, biochemically, and pathologically. The term 'RS' was replaced by the term 'Reye-like syndrome (RLS)'. The list of diseases that could mimic RS became quite extensive. [7] The most important of these mimickers of RS were the metabolic disorders, especially the fatty acid β-oxidation defects. The present consensus seems to be that the Reye syndrome is very rare, and that any child suspected of manifesting this disorder should undergo investigations for inborn errors of metabolism. In many patients the accurate cause of hepatic encephalopathy remains an enigma. In a Malaysian study, [8] causes of the Reye-like syndrome/ encephalo-hepatopathies included fulminant hepatitis, Japanese B encephalitis, dengue, septicemia, and complex febrile fits. It was not possible to differentiate clinical Reye's syndrome from the other encephalo-hepatopathies by either the clinical features (except for jaundice) or biochemical parameters. Liver biopsy is considered necessary for a definitive diagnosis of Reye's syndrome in Malaysia, because of the high prevalence of Reye-like diseases.
Thus, the diagnosis of this disorder has been based on clinical and laboratory symptoms of the Reye-like syndrome (mental confusion, increased values of aminotransferases, hypoketotic hypoglycemia, decreased value of free carnitine), the detection of increased urinary excretion of dicarboxylic acid and other metabolites by use of GC-MS (Gas Chromatography-Mass spectrometry), provocation tests, measurement of enzymatic activities in cultivated fibroblasts and lymphocytes. In recent years tremendous progress has been made with respect to the enzymology of the mitochondrial fatty acid β-oxidation machinery and defect therein. A number of new mitochondrial β-oxidation enzymes have been identified and introduction of tandem mass spectrometry for the analysis of plasma acylcarnitines has greatly facilitated the identification of patients with a defect in fatty acid oxidation. [9] There is no effective therapy for most patients with mitochondrial hepatopathies. Neurological involvement often precludes liver transplantation.
Several drug mixtures that include antioxidants, vitamins, cofactors, and electron acceptors have been proposed, but none have been conclusively proved. Therefore, the current treatment strategies are supportive. [10]
In this case further workup was withheld initially due to severity of the illness and later due to gradual recovery and no recurrence. Although clinically there was no icterus, his bilirubin levels were mildly elevated, probably making other differential diagnosis of RLS more likely. Diagnosis was made with the clinical and available laboratory evidence and treatment for metabolic disorder of a Reye-like syndrome was started, with which the child showed improvement and recovered successfully. In conclusion, hepatic encephalopathy has a varied etiology and needs aggressive and optimistic treatment. However, every effort should be made to arrive at the right diagnosis.
| References | | |
| 1. | Arya R, Gulati S, Deopujari S. Management of hepatic encephalopathy in children. Postgrad Med J 2010;86:34-41. 
[PUBMED] |
| 2. | Reye RD, Morgan G, Baral J. Encephalopathy and fatty degeneration of the viscera a disease entity in childhood. Lancet 1963;2:749-52.
[PUBMED] |
| 3. | Pugliese A, Beltramo T, Torre D. Reyes and Reyes-like syndromes. Cell Biochem Funct 2008;26:741-6.
[PUBMED] |
| 4. | Glasgow JF, Middleton B. Reye syndrome-insights on causation and prognosis. Arch Dis Child 2001;85:351-3.
[PUBMED] |
| 5. | Duerksen DR, Jewell LD, Mason AL, Bain VG. Co-existence of hepatitis A and adult Reyes syndrome. Gut 1997;41:121-4.
[PUBMED] |
| 6. | Waldman RJ, Hall WN, McGee H, van Amburg G. Aspirin as a risk factor in Reye's syndrome. JAMA 1982;247:3089-94.
[PUBMED] |
| 7. | Orlowski JP. Whatever happened to Reye's syndrome? Did it ever really exist? Crit Care Med 1999;27:1582-7.
[PUBMED] |
| 8. | Sinniah D, Sinniah R, Yap YF, Singh M, George R, Lim NL, et al. Reye and Reye-like syndromes: Results of a pilot study in Peninsular Malaya, 1986. Acta Paediatr Jpn 1990;32:385-90.
[PUBMED] |
| 9. | Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, Ijlst L. Disorders of mitochondrial fatty acyl-CoA beta-oxidation. J Inherit Metab Dis 1999;22:442-87.
[PUBMED] |
| 10. | Carey RG, Balistreri WF. Mitochondrial hepatopathies. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE, editors. Nelson Textbook of Pediatrics. Philadelphia: Saunders; 2011. p. 1405-8.
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