Clinical and hematological manifestations of hemoglobin CS disease in children

Tuttle, A. H.; Koch, Bernd

doi:10.1016/s0022-3476(60)80184-9

Cited by 30 publications

(4 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The Hb-SC cells consistently show low oxygen affinities which remain after the removal of q -D P G and which are lower than those in the Hb-S traits, but higher than for 'Hb-S' cells. These findings are consistent with the relatively mild form of the disease associated with Hb S + C (Tuttle & Koch, 1960;Goldberg, 1971). The fresh Hb-SF cells in the phosphate buffer form a group on their own in which the oxygen affinity change with decreasing intracellular Hb-S concentration is much greater than for the other types of cell.…”

Section: Discussionsupporting

confidence: 84%

The Concentration Dependence of the Oxygen Affinity of Haemoglobin S

May¹,

Huehns²

1975

Br J Haematol

View full text Add to dashboard Cite

The effect of the concentration of haemoglobin S (Hb S) on its oxygen-dissociation properties was studied using either reconstituted Hb-S cells of different mean corpuscular haemoglobin concentrations (MCHCs) prepared by osmotic lysis, or cells in which Hb S is diluted by the presence of another haemoglobin. Only 4% (phosphate buffer) and 21%(bis Tris) of the low oxygen affinity of fresh Hb-S cells was found to be due to their slightly elevated intracellular 2,3-DPG concentrations since when the cells were depleted of 2,3-DPG most of the low affinity remained. The low affinity showed a marked dependence upon haemoglobin concentration which was absent for 2,3-DPG-depleted Hb-A cells and, by extrapolation, the MCHC at which the oxygen affinities of the Hb-S cells became identical to that of the Hb-A cells was 14.5 g/dl in phosphate buffer and 13.1 g/dl in bis Tris. Both fresh and 2,3-DPG-depleted cells containing another haemoglobin as well as Hb S (Hb-SA, Hb-SC and Hb-SF cells) were also found to have low oxygen affinities provided that the intracellular Hb-S concentration(MC(Hb-S)C) was above a certain level. These also showed a strong dependence upon the MC(Hb-S)C. The mean MC(Hb-S)C at which the low oxygen affinities of the DPG-depleted cells were abolished were 8.3 g/dl (phosphate) and 11.2 g/dl (bis Tris). Hb F in fresh Hb-SF cells brought about a much greater increase in oxygen affinity than the same amount of either Hb A or Hb C. In 2,3-DPG depleted cells Hb A showed a greater ability to 'dilute' the Hb S than did Hb C. The conditions for the low oxygen affinity of Hb S were therefore found to be very similar to those required for the gelling of both pure Hb S, and Hb S in haemoglobin mixtures. It was concluded therefore that the low oxygen affinity of the Hb S was caused by the polymerization and that the difference between the oxygen affinities of Hb-S and Hb-A cells may be used as a measure of the polymerization process.

show abstract

Section: Discussionsupporting

confidence: 84%

The Concentration Dependence of the Oxygen Affinity of Haemoglobin S

May¹,

Huehns²

1975

Br J Haematol

View full text Add to dashboard Cite

show abstract

“…Of course these scans prove only functional, not necessarily anatomical, asplenia. It may be that splenic atrophy is the result of subclinical infarctive episodes or that symptoms of splenic infarction are attributed to "abdominal pain crises" [23] or other etiologies. Splenectomy following splenic infarction has been described in only a few patients.…”

Section: Discussionmentioning

confidence: 99%

Splenic infarction, splenic sequestration, and functional hyposplenism in hemoglobin S‐C disease

Sears

Udden

1985

American J Hematol

View full text Add to dashboard Cite

Splenic atrophy or evidence of hyposplenism occurs in as many as one third of all patients with S-C hemoglobinopathy. Yet there are few reports in the literature of clinically apparent splenic infarction in this disease. We describe four instances of acute splenic infarction in three patients with hemoglobin S-C disease which illustrate a wide spectrum of clinical manifestations and severity. Of particular interest were the observations of coincident occurrences of splenic sequestration and functional hyposplenism with splenic infarction, suggesting a close pathophysiological relationship among these syndromes.

show abstract

“…The manifestations of the disease in children were reviewed by Tuttle and Koch (1960). Painful crises were experienced by most of their patients, the average age of onset being 4 years.…”

Section: Sickle-cell Haemoglobin C Disease In Childrenmentioning

confidence: 99%

Sickle-cell haemoglobin C disease in London

Black¹,

Condon²,

Gompels³

et al. 1972

J Clin Pathol

View full text Add to dashboard Cite

The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented in London, two of which were fatal. The special hazards of the condition in relation to pregnancy, air travel, and general anaesthesia are discussed.

show abstract

Clinical and hematological manifestations of hemoglobin CS disease in children

Cited by 30 publications

References 30 publications

The Concentration Dependence of the Oxygen Affinity of Haemoglobin S

The Concentration Dependence of the Oxygen Affinity of Haemoglobin S

Splenic infarction, splenic sequestration, and functional hyposplenism in hemoglobin S‐C disease

Sickle-cell haemoglobin C disease in London

Contact Info

Product

Resources

About