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Case Reports Indian Pediatrics 2002; 39:186-189 |
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Fulminant Hepatitis A in Children with Sickle Cell Disease |
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Ramesh L. Renge V.S. Dani S.D. Chitambar* V.A. Arankalle*
Infection with hepatitis A virus (HAV) is most common cause of viral hepatitis among children in India(1). The disease occurs mostly in a self-limited form. Nevertheless, it remains the major cause of hepatitis, which may result into severe disease as a single infection or in combination with other hepatitis viruses(2). It is also reported to be one of the causes of fulminant hepatic failure (FHF)(3,4). This report presents three pediatric cases of sickle cell disease with hepatitis A infection resulting in FHF. Case Reports The first case, a homozygous sickle cell patient presented with fever and jaundice. He had hepatosplenomegaly. He progressed to stage III encephalopathy but recovered in about 5 days. He never had jaundice in the past. The second case was a homozygous sickle cell patient who presented with fever, headache, convulsions and died within 72 hours of hospitalization. The third case, a heterozygous sickle cell child presented with fever, signs of vaso-occlusive crisis and jaundice. She progressed to stage II encephalopathy and recovered over a period of 6 days. Viral markers for hepatitis A (anti-HAV-IgM), hepatitis B (HBsAg) and hepatitis C, hepatitis E (anti-HEV-IgM) were tested by Microelisa. Tables I and II describe clinical features and details of laboratory investigations of the three patients. X-ray chest was normal and Widal test, urine and blood cultures were negative in all three cases. All patients revealed hepato-splenomegaly on ultrasound examination and prolonged prothrombin time. Grading of encephalopathy and management was done as per standard recommendations(5). None of the case was on regular transfusion regime. They neither had received hydroxy urea nor penicillin prophylaxis. Only case II was vaccinated against pneumococci. Case I was detected to have sickle cell disease for the first time whereas case II and III were being followed up in sickle cell clinic since the last 2 years. Discussion The clinical course and outcome of hepatitis A with sickle cell disease have been described earlier in hospitalized children suffering from prolonged jaundice and firm hepatomegaly(6). Fulminant hepatic failure (FHF) is defined as onset of encephalopathy within 8 weeks of onset of jaundice in a previously normal liver(4). This report deals with 3 cases of sickle cell disease (two with sickle cell anemia and one with sickle cell trait) who presented with fulminant hepatitis A. All the 3 cases were from Nagpur and were belonging to New Buddhist (Originally Mahar community). The gene frequency rate of sickle cell desease (SCD) in this community in this area is 22.22%(7). This raises the question whether SCD is a risk factor in development of FHF due to HAV infection. SCD patients have various abnormalities. The increased susceptibility to infections is probably related at least in part of absence of splenic function and in some cases to an abnormality of the properdin opsonization pathway(8). The functional hyposplenism is probably due to abnormality of splenic circulation in the form of vascular occlusion of sinusoidal vessels diverting blood through intrasplenic shunt and escaping phagocytic reticuloendothelial part of system(9). Plasma opsonising proteins are lacking and alternate pathway for activation of complement is defective in SCD patients(10,11). Certain bacterial infections like salmonella and pneumococcus are very common in SCD patients(12,14). Whether there is poor protection against viral infection is not clear. SCD patients are known to suffer from hepatocellular dysfunction(8). The damage to liver and its clinical manifestations are thought to be due to variety of causes such as intra-hepatic infarction, cholelithiasis, drug-induced hepatitis and hemosiderosis (15,16). Intra-hepatic infarcts may be complicated by abscess formation and a viral etiology may be overlooked(17,18).
* Anti HAV IgM positive.
Episodes of hyperbilirubinemia and intermittent elevation of serum liver enzymes in SCD may be seen in benign, self-limited course and rarely progressing rapidly to liver failure(8,19). However, if patient is acutely ill and has deep jaundice, the possibility of viral hepatitis can not be ruled out as the presence of immunological abnormalities and hepatic damage can render SCD patients at risk of developing FHF. Though over 90% hepatitis A infections in children remain subclinical, fulminant hepatitis A either singly or in combination with other hepatotropic viruses is being recognized in India(3,4). In our study, out of 25 cases of fulminant hepatic failure, 3(12%) children had hepatitis A along with SCD. SCD as one of the risk factors for development of fulminant hepatitis A, needs to be carefully evaluated. Non immune SCD patients may be considered an important group for active immunization against hepatitis A for which a potent vaccine is presently available. Contributors: VSD guided and supervised the work. She also helped in drafting the paper and will act as the guarantor for the manuscript. RLR worked up the cases, drafted the paper and performed the literature search. SDC and VAA did viral marker study and helped in drafting the paper. Funding: None. Competing interest: None stated.
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