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Clinical and hematological profile in a newborn cohort with hemoglobin SC.

Clinical and hematological profile in a newborn cohort with hemoglobin SC.
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Rezende PV, Santos MV, Campos GF, Vieira LLM, Souza MB, Belisário AR, Silva CM, Viana MB,


Rezende PV, Santos MV, Campos GF, Vieira LLM, Souza MB, Belisário AR, Silva CM, Viana MB, (click to view)

Rezende PV, Santos MV, Campos GF, Vieira LLM, Souza MB, Belisário AR, Silva CM, Viana MB,

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Jornal de pediatria 2017 11 28() pii S0021-7557(17)30244-9
Abstract
OBJECTIVES
Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood donor center.

METHODOLOGY
This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals. Kaplan-Meier survival curves were created.

RESULTS
The median age of patients was 9.2 years; 47.5% were female. Mean values of blood tests were: hemoglobin, 10.5g/dL; reticulocytes, 3.4%; white blood cells, 11.24×109/L; platelets, 337.1×109/L; and fetal hemoglobin, 6.3%. Clinical events: acute splenic sequestration in 14.8%, blood transfusion 23.4%, overt stroke in 0.2%. The incidence of painful vaso-occlusive episodes was 51 (48.9-53.4) per 100 patient-years and that of infections, 62.2 episodes (59.8-64.8) per 100 patient-years. Transcranial Doppler ultrasonography (n=71) was normal given the current reference values for SS patients. Hydroxyurea was given to ten children, all of whom improvement of painful crises. Retinopathy was observed in 20.3% of 59 children who underwent ophthalmoscopy. Avascular necrosis was detected in seven of 12 patients evaluated, predominantly in the left femur. Echocardiogram compatible with pulmonary hypertension was recorded in 4.6% of 130 children, with an estimated average systolic pulmonary artery pressure of 33.5mmHg. The mortality rate from all causes was 4.3%.

CONCLUSIONS
Clinical severity is variable in SC hemoglobinopathy. Several children have severe manifestations similar to those with SS disease.

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