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Neonatal Hepatitis Syndrome and Alpha-1-Antitrypsin Deficiency: an Epidemiological Study in South-East England K

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Neonatal Hepatitis Syndrome and Alpha-1-Antitrypsin Deficiency: an Epidemiological Study in South-East England K Postgrad Med J: first published as 10.1136/pgmj.50.584.376 on 1 June 1974. Downloaded from Postgraduate Medical Journal (June 1974) 50, 376-380. Neonatal hepatitis syndrome and alpha-1-antitrypsin deficiency: An epidemiological study in south-east England K. COTTRALL P. J. L. COOK B.Sc., M.R.C.P., D.C.H. M.B., B.Chir. A. P. MOWAT M.B.Ch.B., M.R.C.P., D.C.H. The Department of Child Health, King's College Hospital Medical School, London, S.E.5, and M.R.C. Human Biochemical Genetics Unit, University College, London, W.C.1 Summary this area have also kindly allowed us to include their A prospective epidemiological study of the Neonatal patients in the survey, and from these three patients Hepatitis Syndrome in S.E. England showed Alpha-1- will be included in whom the diagnosis of alpha-1- Antitrypsin Deficiency (Pi ZZ) to be present in seven antitrypsin deficiency was made long after neonatal was out offifty-twopatients. Data are considered from these nepatitis diagnosed. by copyright. seven patients, and from a further six cases from out- For the purpose of this study we have defined the side this area. The nature of acute illness, pathological Neonatal Hepatitis Syndrome as a disorder starting changes on early liver biopsy, and short-term prog- in the first 4 months of life and characterized by the nosis show considerable variability, but in general the presence of conjugated hyperbilirubinaemia with hepatitis is more severe in patients with Alpha-1-A.T. features of hepatocellular damage, such as raised deficiency than in those in whom no aetiological factor serum transaminases, and often bilirubinuria, pale was found. stools and hepatosplenomegaly. By appropriate tests all the known causes of the syndrome were sys- THE following clinical features were observed in tematically sought. Details were also obtained of thirteen children who presented with the Neonatal the pregnancy, delivery and perinatal period, ashttp://pmj.bmj.com/ well Hepatitis Syndrome and a genetic deficiency of as of illness in the parents, siblings, and other family alpha-1-antitrypsin. The relative frequency of the members. deficiency in infants with the Neonatal Hepatitis Table 1 refers to patients from the South-East Syndrome will be indicated and the variable clinical Metropolitan Hospital Board area alone, during the and pathological course of hepatitis, particularly in period of January 1971 to September 1973. Fifty-two the first year of life will be observed. Some aspects cases have been observed so far, seven of these of these cases will be with who showed biliary atresia, and seven showed alpha-1- compared patients on September 26, 2021 by guest. Protected did not show the deficiency. antitrypsin deficiency. The estimated frequency of The data were obtained as a result of a prospective neonatal hepatitis syndrome in this population is ongoing survey started at King's College Hospital in therefore about 1 in 2000 live births, while that of January 1971 to try and determine the aetiological importance of infections, genetic and other possible TABLE 1. Neonatal hepatitis survey: South-East Metro- harmful environmental factors. All cases are recorded politan Regional Hospital Board area, January 1971- in a central register and long-term follow up is September 1973 proposed. Total population 2-5 m The study was limited initially to a circumscribed Births per annum (estimated) 40,000 geographical area of the South-East Metropolitan Total no. of cases of N.H. syndrome 52 which extends from Central London Alpha-1-antitrypsin deficiency 7 Hospital Board, Biliary atresia 7 to the South-East coast of England. With the help Estimated incidence of neonatal of all the paediatricians in the area we have been hepatitis syndrome 1 in 2000 live births able to study all cases of the Neonatal Hepatitis Frequency of alpha-1-antitrypsin Syndrome in the region. Paediatricians from outside deficiency and N.H. syndrome 1 in 14,000 live births Postgrad Med J: first published as 10.1136/pgmj.50.584.376 on 1 June 1974. Downloaded from Neonatal hepatitis syndrome 377 alpha-1-antitrypsin deficiency and neonatal hepatitis hepatitis was associated with eosinophilia and raised is about 1 in 14,000 live births. We do not, unfortun- antimitochondrial antibodies after 3 exposures to ately, have data on the frequency of alpha-l-anti- halothane; this suggested a halothane injury at the trypsin deficiency in this population, but would time. However, the finding of a gallstone in the same surmise that it may be near the figure of 1 in 3460 as infant at the age of 18 months, made this conclusion reported by Dr P. J. L. Cook. If we assume this as much less certain. In the genetic group, one child had being the true frequency of the Pi ZZ phenotype, it galactosaemia, two were found to have cystic becomes necessary to explain why only some of these fibrosis, but thirteen showed alpha-1-antitrypsin infants develop hepatitis in the newborn period. deficiency, these children constitute the rest of this Our early experience caused us to infer that paper. hepatitis B antigen could be such a trigger factor All of our thirteen patients presented with jaun- (Porter et al., 1972); as three out of the original five dice, pale stools and dark urine, starting within the cases described had the antigen in their sera, while first 4 weeks of life, except for one child whose the parents of these children also showed a high in- jaundice was not noticed until the sixtieth day. The cidence of either antigen or antibody in their sera. In maximum recorded bilirubins ranged from 2 mg% these children the hepatitis B antigen was found only to 16 mg% and in all these patients jaundice cleared transiently, although the antigen or antibody or both during a period ranging from 2 weeks to 6 months, have persisted in some of the parents. In the sub- and was accompanied by raised serum aspartate sequent eight cases, however, only one child was transaminases and alkaline phosphatase. Apart from found to have the antigen, and that by radio- the findings of hepatitis B antigen already mentioned, immunoassay only. Not all cases were investigated no other similar aetiological factor was detectable in the acute phase however, and it would appear during the acute period of the illness. therefore, that the hepatitis B antigen, whilst being Earlier reports in the literature have stated that the and a possibly important factor, is not an invariable one outlook for children with hepatitis alpha-1- by copyright. in this series of patients. antitrypsin deficiency is uniformly bleak with Table 2 shows the possible aetiological or associ- features of cirrhosis appearing in the first and second ated factors that have been observed by us in a total decade of life (Sharp et al., 1969; Sharp, 1971). of 100 cases of Neonatal Hepatitis Syndrome by Since these reports have been based on studies in September 1973. Twenty-one cases not shown in this patients who already had cirrhosis, we wondered if table had biliary atresia at laparotomy. Note that in this was necessarily so in patients who had only a nearly 50% of these cases, no aetiological or associ- mild illness in the neonatal period. ated factor could be identified, in spite of the fact Of the thirteen patients in our series, four have and all developed cirrhosis and its complications by the age that this was a prospective study diagnostic http://pmj.bmj.com/ tests were performed during the acute illness in most of 1 year, and two of these have died. The remaining instances. The clinical features associated with each nine, however, are clinically well and thriving. of these factors will not be described. Although two The range of maximum recorded abnormality of neonates were exposed to hepatotoxins, namely, liver function tests in twelve of the thirteen patients halothane and lincomycin, their role as aetiological shown in Fig. 1 reflects the variability of the acute agents is by no means clear. Indeed, the difficulty in hepatitis among these patients. It is noteworthy that ascribing the disease to any of these causes is well the maximal abnormal results were found in the illustrated by the history ofone ofthese infants whose four infants who have developed cirrhosis and its on September 26, 2021 by guest. Protected complications by the age of 1 year. For the sake of the affected four will TABLE 2. Possible aetiological factors in convenience, severely patients neonatal hepatitis syndrome be referred to as group A, and the remaining nine as group B. Type of abnormality No. of cases Are the complications of cirrhosis inevitable in Genetic patients in group B? As the ages of all our patients a- -antitrypsin deficiency 13 ranged from 7 months to 61 years (mean=27-8 Cystic fibrosis 2 short to Galactosaemia 1 months), the period of follow-up is too Chromosomal abnormality 3 answer this important question. We felt that it was Blood group incompatibility 5 of interest to look at the course of the acute illness Infection and the pathological changes in the liver in the first HB Ag/Ab 14 Viral 6 6 months oflife to see ifa different pattern ofresponse Toxoplasma 1 was evident. Bacterial 1 The variable nature of the course of the disease is ? Hepatotoxins 2 illustrated in Table 3, where it is seen that the age at 'Unclassified' 49 onset ofjaundice was less (range 2-10 days) and the Postgrad Med J: first published as 10.1136/pgmj.50.584.376 on 1 June 1974. Downloaded from 378 K. Cottrall, P. J. L. Cook and A.
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