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Active chronic hepatitis in infancy
982
Letters to the Editor
conclusion that five of the examined children have organic brain damage somewhat localized on the right sider In all six chiIdren EEG tracings revealed rightsided alterations; two of them suffered from paroxysmal symptoms. 4 The alterations revealed in the bioelectric activity of the brain in all six cases indicate possible organic damage to the central nervous system in those parts of the brain which have a bearing upon the derivations from the right temporal regions toward the back. Negative interview findings and the investigations reported by Marie and associates5 corroborate our conclusion that the deviations from the norm revealed by means of the intelligence tests and in the EEG tracings should be attributed to Feet's disease. Three children had skin disorders of an allergic nature. Two of them also had psoriasis. That children suffering from acrodynia are subject to various disturbances of an allergic nature has been pointed out by Fanconi and Wallgren s and, among Polish authors, by Go~cifiska and associates5 Three of the examined children showed excessive nervous excitability. The mothers of four of the children have been for many years treated for so-called vegetative dystonla. The fact that children who had once suffered from acrodynia are frequently subject to vegetative dystonia has been pointed out by Fanconi and associates,a and among Polish authors, by Klepacki and associates,s Leys and Cameron 9 have stressed that most of the mothers of their child patients treated for acrodynia were mentally unstable. Four of the children had jaw and bit e irregularities; three of them had overbite and one had bilateral crossbite. These irregularities may have been caused by the acrodynia as a result of damage to buds of permanent teeth during the acute phase of the illness. In one child scarring of the tongue was observed as a vestige of an extensive ulceration which occurred in the course of the acrodynia. Marek Barbacki Provincial Centre [or the Mother and Child, Krakdw, Poland REFERENCES
I. Dekaban, A.: Neurology of infancy, Baltimore, 1959, The Williams & Wilklns Company. 2. Ford, F. R.: Diseases of the nervous system in infancy, childhood and adolescence, Springfield, Ill., 1960, Charles C Thomas, Publisher. 3. Pstragowska, W.:~Akrodynia, Pol. Tyg. lek, I4: 1170, 1959,
The Journal of Pediatrics June 1970
4. Pawlik, M., and Barbacki, M.: Alt6rations de l'61ectro-enc6phalogramme constat6es chez six enfants une longue periode apr~s acrodynie, Ann. Pediat. 7: 146, 1970. 5. Marie, J., Bricaire, H., R6mond, A., LerlqueKoechlin, A., Nekhorocheff, I., and Job, J. C.: Les alt6rations de l'61ectro-enc6phalogramme au course de l'acrodynle infantile, Sere. Hop. Paris 73: 3786, 1953. 6. Fanconi, G., and Wallgren, A.: Lehrbuch der P~idiatrie, Basel-Stuttgart, 1961, Benno Schwabe Verlag. 7. Go~eifiska, Z., Bartoszewicz, B., and Piasecka, M.: Neurological symptomatology in cases of chronic mercury poisoning in children, Pediat. Pol. 42: 297, 1967. 8. Klepacki, W., Gerkowicz, T., and Sta~kiewicz, J.: Acrodynia--Swift's and Feer's disease, Pol. Tyg. Lek. 13: 510, 1958. 9. Leys, I)., and Cameron, K.: A psychiatric study of six cases of infantile acrodynia, Brit. Med. J. 4751: 191, 1952.
Active chronic hepatitis in infancy To the Editor: The paper by Benjamins and SunshinO once again suggests that the widely held concept that active "chronic hepatitis" occurs mainly in young women and as a well-defined entity should be abandoned. It seems to be sufficiently documented that chronic or progressive liver disease is not rare, occurs in both sexes, and does not spare any age group. Systemic manifestations, however, are observed more frequently in young females. 2 The variety of names (chronic hepatitis, lupoid hepatitis, plasma-cell hepatitis, and variants thereof) attached to chronic types of liver disease is largely a tribute to our ignorance about the etiology(ies) of these conditions. Yet there is a need to classify these disorders. An etiologic classification (like "-itis") is impractical and confusing. The descriptive term "chronic active liver disease" for these conditions is perhaps preferable. Criteria such as chronicity (continuing or recurrent disease possibly leading to cirrhosis) and activity (perpetuation of the initial injury and referring to hepatocellular necrosis) seem less controversial and more meaningful from the point of view of prognosis and management.S These 2 criteria are useful to all of the liver disorders described. Ultimately, the ideal nomenclature will bear etiologic, prognostic, or therapeutic significance,a We have, indeed, few ideas about the cause(s) leading to chronic active liver disease, and factors responsible for the initial injury may not be
Volume 76 Number 6
those responsible for its perpetuation. Regarding the latter, the possibility of continuing viral infections remains speculative in the absence of solid evidence in support of such hypothesis. Occasional coexistence of other diseases of suspected autoimmune etiology or the detection of associated autoimmune phenomena remains inconclusive with regard to etiology. Preliminary evidence suggests that an increased intrahepatic concentration of bile acids may be one factor concerned with perpetuation of liver cell injury.4 Prognosis and management are the 2 most important considerations. The former is generally not goo& Patients with chronic active liver disease constitute a rather high-risk population; death due to cirrhosis and its complications or to liver failure looms ahead. Controlled therapeutic trials 3 with either steroids or antimetabolites remain unconvincing in regard to ultimate usefulness: Tests reflecting liver function revert toward normal, but hepatocellular necrosis seems to continue. It should also be remembered that antimetabolites may be in themselves hepatotoxic.
Letters to the Editor
983
Results of controlled therapeutic trials with cholestyramine, a bile acid-sequesterlng agent, are not yet available. It is conceivable that treatment with cholestyramine may be of value in chronic active liver disease.
]. R. Poley, M.D. The University o[ Oklahoma Medical Center 800 Northeast Thirteenth St. Oklahoma City, Okla. 73104 REFERENCES
1. Benjamins, D., and Sunshine, P.: Active chronic hepatitis in infancy: Possible presence of the disease in siblings, J. PEDIAT. 75: 294, 1969. 2. Mackay, I. R., and Wood, I. J.: Lupoid hepatitis: A comparison of 22 cases with other types of chronic liver disease, Quart. J. Med. 31: 485, 1962. 3. Geall, M. G., Schoenfield, L. J., and Summerskill, W. H. J.: Classification and treatment of chronic active liver disease, Gastroenterology 55: 724, 1968. 4. Poley, J. R.: Unpublished observations.
Letters to the Editor
conclusion that five of the examined children have organic brain damage somewhat localized on the right sider In all six chiIdren EEG tracings revealed rightsided alterations; two of them suffered from paroxysmal symptoms. 4 The alterations revealed in the bioelectric activity of the brain in all six cases indicate possible organic damage to the central nervous system in those parts of the brain which have a bearing upon the derivations from the right temporal regions toward the back. Negative interview findings and the investigations reported by Marie and associates5 corroborate our conclusion that the deviations from the norm revealed by means of the intelligence tests and in the EEG tracings should be attributed to Feet's disease. Three children had skin disorders of an allergic nature. Two of them also had psoriasis. That children suffering from acrodynia are subject to various disturbances of an allergic nature has been pointed out by Fanconi and Wallgren s and, among Polish authors, by Go~cifiska and associates5 Three of the examined children showed excessive nervous excitability. The mothers of four of the children have been for many years treated for so-called vegetative dystonla. The fact that children who had once suffered from acrodynia are frequently subject to vegetative dystonia has been pointed out by Fanconi and associates,a and among Polish authors, by Klepacki and associates,s Leys and Cameron 9 have stressed that most of the mothers of their child patients treated for acrodynia were mentally unstable. Four of the children had jaw and bit e irregularities; three of them had overbite and one had bilateral crossbite. These irregularities may have been caused by the acrodynia as a result of damage to buds of permanent teeth during the acute phase of the illness. In one child scarring of the tongue was observed as a vestige of an extensive ulceration which occurred in the course of the acrodynia. Marek Barbacki Provincial Centre [or the Mother and Child, Krakdw, Poland REFERENCES
I. Dekaban, A.: Neurology of infancy, Baltimore, 1959, The Williams & Wilklns Company. 2. Ford, F. R.: Diseases of the nervous system in infancy, childhood and adolescence, Springfield, Ill., 1960, Charles C Thomas, Publisher. 3. Pstragowska, W.:~Akrodynia, Pol. Tyg. lek, I4: 1170, 1959,
The Journal of Pediatrics June 1970
4. Pawlik, M., and Barbacki, M.: Alt6rations de l'61ectro-enc6phalogramme constat6es chez six enfants une longue periode apr~s acrodynie, Ann. Pediat. 7: 146, 1970. 5. Marie, J., Bricaire, H., R6mond, A., LerlqueKoechlin, A., Nekhorocheff, I., and Job, J. C.: Les alt6rations de l'61ectro-enc6phalogramme au course de l'acrodynle infantile, Sere. Hop. Paris 73: 3786, 1953. 6. Fanconi, G., and Wallgren, A.: Lehrbuch der P~idiatrie, Basel-Stuttgart, 1961, Benno Schwabe Verlag. 7. Go~eifiska, Z., Bartoszewicz, B., and Piasecka, M.: Neurological symptomatology in cases of chronic mercury poisoning in children, Pediat. Pol. 42: 297, 1967. 8. Klepacki, W., Gerkowicz, T., and Sta~kiewicz, J.: Acrodynia--Swift's and Feer's disease, Pol. Tyg. Lek. 13: 510, 1958. 9. Leys, I)., and Cameron, K.: A psychiatric study of six cases of infantile acrodynia, Brit. Med. J. 4751: 191, 1952.
Active chronic hepatitis in infancy To the Editor: The paper by Benjamins and SunshinO once again suggests that the widely held concept that active "chronic hepatitis" occurs mainly in young women and as a well-defined entity should be abandoned. It seems to be sufficiently documented that chronic or progressive liver disease is not rare, occurs in both sexes, and does not spare any age group. Systemic manifestations, however, are observed more frequently in young females. 2 The variety of names (chronic hepatitis, lupoid hepatitis, plasma-cell hepatitis, and variants thereof) attached to chronic types of liver disease is largely a tribute to our ignorance about the etiology(ies) of these conditions. Yet there is a need to classify these disorders. An etiologic classification (like "-itis") is impractical and confusing. The descriptive term "chronic active liver disease" for these conditions is perhaps preferable. Criteria such as chronicity (continuing or recurrent disease possibly leading to cirrhosis) and activity (perpetuation of the initial injury and referring to hepatocellular necrosis) seem less controversial and more meaningful from the point of view of prognosis and management.S These 2 criteria are useful to all of the liver disorders described. Ultimately, the ideal nomenclature will bear etiologic, prognostic, or therapeutic significance,a We have, indeed, few ideas about the cause(s) leading to chronic active liver disease, and factors responsible for the initial injury may not be
Volume 76 Number 6
those responsible for its perpetuation. Regarding the latter, the possibility of continuing viral infections remains speculative in the absence of solid evidence in support of such hypothesis. Occasional coexistence of other diseases of suspected autoimmune etiology or the detection of associated autoimmune phenomena remains inconclusive with regard to etiology. Preliminary evidence suggests that an increased intrahepatic concentration of bile acids may be one factor concerned with perpetuation of liver cell injury.4 Prognosis and management are the 2 most important considerations. The former is generally not goo& Patients with chronic active liver disease constitute a rather high-risk population; death due to cirrhosis and its complications or to liver failure looms ahead. Controlled therapeutic trials 3 with either steroids or antimetabolites remain unconvincing in regard to ultimate usefulness: Tests reflecting liver function revert toward normal, but hepatocellular necrosis seems to continue. It should also be remembered that antimetabolites may be in themselves hepatotoxic.
Letters to the Editor
983
Results of controlled therapeutic trials with cholestyramine, a bile acid-sequesterlng agent, are not yet available. It is conceivable that treatment with cholestyramine may be of value in chronic active liver disease.
]. R. Poley, M.D. The University o[ Oklahoma Medical Center 800 Northeast Thirteenth St. Oklahoma City, Okla. 73104 REFERENCES
1. Benjamins, D., and Sunshine, P.: Active chronic hepatitis in infancy: Possible presence of the disease in siblings, J. PEDIAT. 75: 294, 1969. 2. Mackay, I. R., and Wood, I. J.: Lupoid hepatitis: A comparison of 22 cases with other types of chronic liver disease, Quart. J. Med. 31: 485, 1962. 3. Geall, M. G., Schoenfield, L. J., and Summerskill, W. H. J.: Classification and treatment of chronic active liver disease, Gastroenterology 55: 724, 1968. 4. Poley, J. R.: Unpublished observations.