Juvenile Pernicious Anemia in Sisters |
Hyun Lee, Goeng Pil Chun, Ki Yung Lee, Duk Jin Yun |
Department of Pediatrics, Yonsei University College of Medicine, Seoul,Korea |
姉妹에서 본 小兒期惡性負血症 |
李炫, 全宏弼, 李基寧, 尹德鎭 |
延世大學校醫科大學小兒科學敎室 |
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Abstract |
Two cases of juvenile pernicious anemia in Korean sisters have been followed for 4 years since 7
years and 4 years of age respectively. The symptoms and signs were pale facies, general malaise, sore
tongue, anorexia, frequent febrile episodes, macrocytic anemia, thrombocytopenia, multilobulated leu-
cocytes with more than 6 lobules and megaloblastic proliferation in bone marrow aspiration. Dramatic
hematological response to the parenteral administration of vitamin B12 with a significantly increased
excretion rat Qof vitamin B12 by Schilling test as shown by an increase from 0 to 11.6% in elder sister
and from 0 to 5.6% in younger sister with the addition of commercial intrinsic factor concentrate
were demonstrated.
These findings are compatible with the characteristics of juvenile pernicious anemia resulting from
defect of Castle’s intrinsic factor.
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