INFANTILE GENETIC AGRANULOCYTOSIS
ABSTRACT: Kostmann, R. R. O. (Department of Paediatrics, University Hospital, Uppsala, Sweden). Infantile genetic agranulocytosis. Acta Paediatr Stand, 64:362, 1975.–A review of the literature on the subject since 1956 is made in connection with a presentation of ten new cases from northern Sweden....
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crwiley:10.1111/j.1651-2227.1975.tb03847.x 2024-06-23T07:55:36+00:00 INFANTILE GENETIC AGRANULOCYTOSIS KOSTMANN, ROLF 1975 http://dx.doi.org/10.1111/j.1651-2227.1975.tb03847.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1651-2227.1975.tb03847.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1651-2227.1975.tb03847.x en eng Wiley http://onlinelibrary.wiley.com/termsAndConditions#vor Acta Paediatrica volume 64, issue 2, page 362-368 ISSN 0803-5253 1651-2227 journal-article 1975 crwiley https://doi.org/10.1111/j.1651-2227.1975.tb03847.x 2024-05-31T08:13:53Z ABSTRACT: Kostmann, R. R. O. (Department of Paediatrics, University Hospital, Uppsala, Sweden). Infantile genetic agranulocytosis. Acta Paediatr Stand, 64:362, 1975.–A review of the literature on the subject since 1956 is made in connection with a presentation of ten new cases from northern Sweden. Nine of these are related to the main pedigree published in 1956. Consanguinity between the parents has been established in two of the new families. The clinical course was identical to that described in 1956. A few additional details are presented. The granulocytopenia is present on the first day of life and the granulocyte count subsequently rapidly decreases during the first week. The existence of a diaplacental factor is regarded highly probable. It is assumed that the maturation defect in the granulocyte precursors may be due to deficiency of a serum factor. The fact that many cases of infantile genetic agranulocytosis occur sporadically is finally explained. Article in Journal/Newspaper Northern Sweden Wiley Online Library Acta Paediatrica 64 2 362 368 |
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Wiley Online Library |
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crwiley |
language |
English |
description |
ABSTRACT: Kostmann, R. R. O. (Department of Paediatrics, University Hospital, Uppsala, Sweden). Infantile genetic agranulocytosis. Acta Paediatr Stand, 64:362, 1975.–A review of the literature on the subject since 1956 is made in connection with a presentation of ten new cases from northern Sweden. Nine of these are related to the main pedigree published in 1956. Consanguinity between the parents has been established in two of the new families. The clinical course was identical to that described in 1956. A few additional details are presented. The granulocytopenia is present on the first day of life and the granulocyte count subsequently rapidly decreases during the first week. The existence of a diaplacental factor is regarded highly probable. It is assumed that the maturation defect in the granulocyte precursors may be due to deficiency of a serum factor. The fact that many cases of infantile genetic agranulocytosis occur sporadically is finally explained. |
format |
Article in Journal/Newspaper |
author |
KOSTMANN, ROLF |
spellingShingle |
KOSTMANN, ROLF INFANTILE GENETIC AGRANULOCYTOSIS |
author_facet |
KOSTMANN, ROLF |
author_sort |
KOSTMANN, ROLF |
title |
INFANTILE GENETIC AGRANULOCYTOSIS |
title_short |
INFANTILE GENETIC AGRANULOCYTOSIS |
title_full |
INFANTILE GENETIC AGRANULOCYTOSIS |
title_fullStr |
INFANTILE GENETIC AGRANULOCYTOSIS |
title_full_unstemmed |
INFANTILE GENETIC AGRANULOCYTOSIS |
title_sort |
infantile genetic agranulocytosis |
publisher |
Wiley |
publishDate |
1975 |
url |
http://dx.doi.org/10.1111/j.1651-2227.1975.tb03847.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1651-2227.1975.tb03847.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1651-2227.1975.tb03847.x |
genre |
Northern Sweden |
genre_facet |
Northern Sweden |
op_source |
Acta Paediatrica volume 64, issue 2, page 362-368 ISSN 0803-5253 1651-2227 |
op_rights |
http://onlinelibrary.wiley.com/termsAndConditions#vor |
op_doi |
https://doi.org/10.1111/j.1651-2227.1975.tb03847.x |
container_title |
Acta Paediatrica |
container_volume |
64 |
container_issue |
2 |
container_start_page |
362 |
op_container_end_page |
368 |
_version_ |
1802648263365492736 |