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Fortuitous detection of composite heterozygous S/C sickle cell disease.

Abstract Composite S/C sickle cell disease accounts for 20%-30% of major sickle cell syndromes. We report a case of fortuitous detection of composite heterozygous S/C sickle cell disease in the context of retinal detachment. The patient had been hospitalized in the Department of Ophthalmology for treatment-resistant decreased visual acuity detected 06 months before. The patient's clinical history was marked by total hip replacement (THR) twelve years before. Our study highlights the wide clinical variability of sickle cell disease underlying the importance of early screening and adapted clinical monitoring of patients at-risk, in order to avoid its evolution toward irreversible organic sequelae such as sickle cell retinopathy.
PMID
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Authors

Mayor MeshTerms
Keywords

Retinopathy

composite S/C sickle cell disease

hemoglobinopathy

Journal Title the pan african medical journal
Publication Year Start




PMID- 28819514
OWN - NLM
STAT- MEDLINE
DA  - 20170818
DCOM- 20170904
LR  - 20170904
IS  - 1937-8688 (Electronic)
VI  - 27
DP  - 2017
TI  - [Fortuitous detection of composite heterozygous S/C sickle cell disease].
PG  - 93
LID - 10.11604/pamj.2017.27.93.12724 [doi]
AB  - Composite S/C sickle cell disease accounts for 20%-30% of major sickle cell
      syndromes. We report a case of fortuitous detection of composite heterozygous S/C
      sickle cell disease in the context of retinal detachment. The patient had been
      hospitalized in the Department of Ophthalmology for treatment-resistant decreased
      visual acuity detected 06 months before. The patient's clinical history was
      marked by total hip replacement (THR) twelve years before. Our study highlights
      the wide clinical variability of sickle cell disease underlying the importance of
      early screening and adapted clinical monitoring of patients at-risk, in order to 
      avoid its evolution toward irreversible organic sequelae such as sickle cell
      retinopathy.
FAU - Biaz, Asmaa
AU  - Biaz A
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
FAU - Neji, Maroua
AU  - Neji M
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
FAU - Ajhoun, Yousra
AU  - Ajhoun Y
AD  - Service d'Ophtalmologie Hopital Militaire d'Instruction Mohammed V, Rabat, Maroc.
FAU - Idrissi, Samira El Machtani
AU  - Idrissi SEM
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
AD  - Faculte de Medecine et de Pharmacie de Rabat, Universite Mohamed V Souissi,
      Rabat, Maroc.
FAU - Dami, Abdellah
AU  - Dami A
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
AD  - Faculte de Medecine et de Pharmacie de Rabat, Universite Mohamed V Souissi,
      Rabat, Maroc.
FAU - Reda, Karim
AU  - Reda K
AD  - Service d'Ophtalmologie Hopital Militaire d'Instruction Mohammed V, Rabat, Maroc.
AD  - Faculte de Medecine et de Pharmacie de Rabat, Universite Mohamed V Souissi,
      Rabat, Maroc.
FAU - Ouzzif, Zohra
AU  - Ouzzif Z
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
AD  - Faculte de Medecine et de Pharmacie de Rabat, Universite Mohamed V Souissi,
      Rabat, Maroc.
FAU - Bouhsain, Sanae
AU  - Bouhsain S
AD  - Service de Biochimie-Toxicologie Hopital Militaire d'Instruction Mohammed V,
      Rabat, Maroc.
AD  - Faculte de Medecine et de Pharmacie de Rabat, Universite Mohamed V Souissi,
      Rabat, Maroc.
LA  - fre
PT  - Case Reports
PT  - Journal Article
TT  - Decouverte fortuite d'une drepanocytose heterozygote composite S/C.
DEP - 20170607
PL  - Uganda
TA  - Pan Afr Med J
JT  - The Pan African medical journal
JID - 101517926
SB  - IM
MH  - Anemia, Sickle Cell/complications/*diagnosis
MH  - Female
MH  - Heterozygote
MH  - Humans
MH  - Mass Screening/methods
MH  - Middle Aged
MH  - Retinal Detachment/*etiology
MH  - Retinal Diseases/*diagnosis/etiology
MH  - Time Factors
MH  - Visual Acuity
PMC - PMC5554668
OTO - NOTNLM
OT  - Retinopathy
OT  - composite S/C sickle cell disease
OT  - hemoglobinopathy
EDAT- 2017/08/19 06:00
MHDA- 2017/09/05 06:00
CRDT- 2017/08/19 06:00
PHST- 2017/05/11 [received]
PHST- 2017/05/16 [accepted]
AID - 10.11604/pamj.2017.27.93.12724 [doi]
AID - PAMJ-27-93 [pii]
PST - epublish
SO  - Pan Afr Med J. 2017 Jun 7;27:93. doi: 10.11604/pamj.2017.27.93.12724. eCollection
      2017.