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Anaemia Hypoplastica Congenita (Anaemia Typus Josephs‐Diamond‐Blackfan). Report of a Case Treated with Adrenocorticotropin with Effect
Author(s) -
KÅSS ARNE,
SUNDAL ALFRED
Publication year - 1953
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1953.tb05590.x
Subject(s) - medicine , pediatrics , girl , blood count , reticulocyte , endocrinology , psychology , developmental psychology , messenger rna , biochemistry , chemistry , gene
Summary A case of anaemia typus Josephs‐Diamond‐Blackfan in a 4 year old girl has been described. She has been dependent on regular blood transfusions with short intervals, most frequently of 4–6 weeks. The clinical features were typical, with a marked reticulocytopenia as the most striking haematological finding. After two courses with ACTH treatment was a marked increase in the reticulocyte count, both times occurring on the 9th day after starting the treatment, with a duration of several weeks, accompanied by a spontaneous remission of the blood values, and thus the intervals between the transfusions have been prolonged for 15 weeks.

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