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Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya

Severe Combined Immunodeficiency (SCID) involves the lymphocyte lineage and mimics Human Immunodeficiency Virus (HIV) disease common in our region, making it difficult to diagnose and manage effectively. SCID in East Africa stands underdiagnosed because of lack of awareness and diagnostic resources....

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Autores principales: Patil, Rohini Kalagouda, Irungu, Anne, Kabera, Beatrice, Mutua, Doreen Karimi, Manguyu, Wangui, Lagat, David Kiptum, Mutua, Kaumbulu Cleopas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8363958/
https://www.ncbi.nlm.nih.gov/pubmed/34422179
http://dx.doi.org/10.11604/pamj.2021.39.56.26419
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author Patil, Rohini Kalagouda
Irungu, Anne
Kabera, Beatrice
Mutua, Doreen Karimi
Manguyu, Wangui
Lagat, David Kiptum
Mutua, Kaumbulu Cleopas
author_facet Patil, Rohini Kalagouda
Irungu, Anne
Kabera, Beatrice
Mutua, Doreen Karimi
Manguyu, Wangui
Lagat, David Kiptum
Mutua, Kaumbulu Cleopas
author_sort Patil, Rohini Kalagouda
collection PubMed
description Severe Combined Immunodeficiency (SCID) involves the lymphocyte lineage and mimics Human Immunodeficiency Virus (HIV) disease common in our region, making it difficult to diagnose and manage effectively. SCID in East Africa stands underdiagnosed because of lack of awareness and diagnostic resources. A case series of three SCID patients admitted to a Tertiary Paediatric Centre in Kenya between 2016 and 2019. The clinical presentations, laboratory findings, management and outcome for each were studied. Three cases were diagnosed between the ages of 4 to 15 months. Two of them were male and one was a female. All had a history of previous sibling death. There was no parental consanguinity. All presented with pneumonia. One of them had vaccine acquired Rotavirus infection and a persistent generalised maculopapular rash. The T, B cell profile was T- B- in two and T- B+ in one case and the immunoglobulins were reduced in all. All the cases were fatal. Thus, Primary immunodeficiency disorders are prevalent in East Africa. A proper clinical history, examination and laboratory tests like a haemogram, peripheral blood film can aid to suspect and diagnose SCID even with limited resources.
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spelling pubmed-83639582021-08-20 Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya Patil, Rohini Kalagouda Irungu, Anne Kabera, Beatrice Mutua, Doreen Karimi Manguyu, Wangui Lagat, David Kiptum Mutua, Kaumbulu Cleopas Pan Afr Med J Case Series Severe Combined Immunodeficiency (SCID) involves the lymphocyte lineage and mimics Human Immunodeficiency Virus (HIV) disease common in our region, making it difficult to diagnose and manage effectively. SCID in East Africa stands underdiagnosed because of lack of awareness and diagnostic resources. A case series of three SCID patients admitted to a Tertiary Paediatric Centre in Kenya between 2016 and 2019. The clinical presentations, laboratory findings, management and outcome for each were studied. Three cases were diagnosed between the ages of 4 to 15 months. Two of them were male and one was a female. All had a history of previous sibling death. There was no parental consanguinity. All presented with pneumonia. One of them had vaccine acquired Rotavirus infection and a persistent generalised maculopapular rash. The T, B cell profile was T- B- in two and T- B+ in one case and the immunoglobulins were reduced in all. All the cases were fatal. Thus, Primary immunodeficiency disorders are prevalent in East Africa. A proper clinical history, examination and laboratory tests like a haemogram, peripheral blood film can aid to suspect and diagnose SCID even with limited resources. The African Field Epidemiology Network 2021-05-20 /pmc/articles/PMC8363958/ /pubmed/34422179 http://dx.doi.org/10.11604/pamj.2021.39.56.26419 Text en Copyright: Rohini Kalagouda Patil et al. https://creativecommons.org/licenses/by/4.0/The Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Series
Patil, Rohini Kalagouda
Irungu, Anne
Kabera, Beatrice
Mutua, Doreen Karimi
Manguyu, Wangui
Lagat, David Kiptum
Mutua, Kaumbulu Cleopas
Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title_full Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title_fullStr Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title_full_unstemmed Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title_short Severe combined immunodeficiency: a case series from a paediatric hospital in Kenya
title_sort severe combined immunodeficiency: a case series from a paediatric hospital in kenya
topic Case Series
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8363958/
https://www.ncbi.nlm.nih.gov/pubmed/34422179
http://dx.doi.org/10.11604/pamj.2021.39.56.26419
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