PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 8413345

[Hemoglobin SD disease].

H J Laws·R Dickerhoff·J Mayer·B Köster
View original source ↗Export metadata ↓

Abstract

A 7 year old Kurdish girl presented with a cerebral infarction and a resulting hemiparesis following a blood transfusion. Examination of the blood film suggested sickle cell anaemia. However a simultaneously carried out haemoglobin electrophoresis showed haemoglobin S and haemoglobin D. The diagnosis of haemoglobin SD disease was thereby established. This kind of haemoglobin disorder usually shows a mild clinical manifestation. According to our knowledge such serious cases have not been published before. With this disease the main emphasis is on the prevention of recurrent cerebral infarctions bay a long-term transfusion programme whereas the effects of the cerebral infarction are treated in the usual way.

Explore related subjects

Anemia, Sickle CellBrainCerebral InfarctionChildDiagnosis, DifferentialFemaleGenetic Carrier ScreeningHemoglobin SC DiseaseHemoglobin, SickleHemoglobins, AbnormalHumansMagnetic Resonance ImagingThalassemiaCase ReportsJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

H J Laws, R Dickerhoff, J Mayer, B Köster. 1993. [Hemoglobin SD disease].. https://pubmed.ncbi.nlm.nih.gov/8413345/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed1998

Time-dependent changes in the density and hemoglobin F content of biotin-labeled sickle cells.

Sickle red blood cells (RBC) are subject to a number of important cellular changes and selection pressures. In this study, we validated a biotin RBC label by comparison to the standard 51Cr label, and used it to study changes that occur in sickle cells as they age. Sickle RBC had a much shorter lifespan than normal RBC, but the two labels gave equivalent results for each cell type. A variable number of sickle, but not normal, RBC disappeared from the circulation during the first few hours after reinfusion. The number of biotinylated sickle reticulocytes was decreased by 50% after 24 h and 75% after 48 h, with a gradual decrease in the amount of reticulum per cell. The labeled sickle cells exhibited major density increases during the first 4-6 d after reinfusion, with smaller changes thereafter. A small population of very light, labeled sickle RBC was essentially constant in number after the first few days. Fetal hemoglobin (HbF) content was determined in isolated biotinylated sickle RBC after reinfusion, allowing an estimate of lifespan for RBC containing HbF (F cells) and non-F cells. The lifespan of sickle biotinylated RBC lacking HbF was estimated to be approximately 2 wk, whereas F cells survived 6-8 wk.

Anemia, Sickle Cell↗