Endoscopic retrograde cholangiopancreatography in infantile cholestasis

Article Abstract:

Infantile jaundice, caused by high blood levels of bilirubin, may be due to liver disease or abnormalities of the bile ducts outside the liver. Three relatively common disorders that may cause jaundice are idiopathic neonatal hepatitis (liver inflammation with no known cause), arteriohepatic dysplasia (abnormal tissue growth), and extrahepatic biliary atresia (EHBA, congenital absence of or closure of the bile ducts). Only EHBA needs immediate surgical treatment (it leads to liver damage if not corrected), while the other two conditions are usually treated successfully without surgery. Infants who do not achieve normal bile drainage require liver transplantation. Diagnosis of EHBA is achieved by tests showing lack of bile flow and ruling out all other conditions, and as a last resort, by laparotomy, which is exploratory surgery. To avoid laparotomy, which has a risk of complications, visualization of the bile ducts by endoscopic retrograde cholangiopancreatography (ERCP) using a newly designed duodenoscope (viewing instrument used in the upper intestine) has been performed. The techniques used and results from ERCP in diagnosing nine infants with possible EHBA are described. The use of ERCP allowed accurate diagnosis or exclusion of EHBA in eight of the nine patients, who otherwise would have required laparotomy. The only significant side effect was abdominal distension due to difficulty in aspiration, but this problem has since been reduced by using smaller tubes. The study indicates that ERCP is a useful procedure in the diagnosis of jaundiced infants. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Abnormalities, Diagnosis, Causes of, Infants (Newborn), Newborn infants, Cholestasis, Bile ducts, Biliary atresia, Endoscopic retrograde cholangiopancreatography

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Immune reconstitution

Article Abstract:

A study of thymus transplants to treat DiGeorge syndrome sheds light on the continuing function of the thymus gland after puberty. The thymus gland produces immune cells called T cells and patients with DiGeorge syndrome have no functioning thymus gland. Therefore, they have a T cell deficiency. In the study, five babies with DiGeorge syndrome were given a thymus gland transplant. Three babies died from other causes, but two survived and began producing T cells. Studies on AIDS patients have shown that the thymus gland can continue to operate in adulthood.

Editorial, Physiological aspects, Thymus, Thymus gland

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Correction of the hyper-IgM syndrome after liver and bone marrow transplantation

Article Abstract:

Researchers discuss the use of a liver transplant and a bone marrow transplant for an 18-year-old boy with the hyper-IgM syndrome. This syndrome is an immunodeficiency syndrome that causes low blood levels of most antibodies, except IgM, which is either normal or elevated. It can damage the liver and increase the risk of cancer. In addition, low antiboy levels predispose affected patients to chronic infections. The boy had end-stage liver disease and was successfully treated with liver and bone marrow transplants.

Liver, Bone marrow, Liver transplantation, Bone marrow transplantation

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