Neonatal Screening and the Clinical Outcome in Children with Sickle Cell Disease in Central India

PloS One
Dipti S UpadhyeRoshan Colah

Abstract

Sickle cell disease (SCD) is a major health burden in India. The objective of the study was to establish a neonatal screening program and to understand the clinical course of children with SCD in central India. Pregnant mothers were screened for sickle hemoglobin using the solubility test. Babies were screened by high performance liquid chromatography if the mother was positive for sickle hemoglobin. The diagnosis was confirmed by molecular analysis. They received early prophylactic treatment and vaccination. Of 2134 newborns screened, 104 were sickle homozygous (SS), seven had sickle β-thalassemia (S-β thal) and 978 were sickle heterozygous (AS). The other hemoglobin abnormalities detected included HbS-δβ thalassemia-1, HbSD disease-2, HbE traits-5, β-thalassemia traits-4, alpha chain variants-3 and HbH disease-1.These babies were followed up regularly for hematological and clinical evaluation. Pain, severe anemia requiring blood transfusions and acute febrile illness were the major complications with 59.7, 45.1 and 42.6 cases per 100 person years. Fetal hemoglobin (HbF) levels were inversely associated with vaso-oclussive crisis (VOC) and severe anemia while presence of alpha thalassemia increased the rate of painful events a...Continue Reading

References

Dec 1, 1979·American Journal of Diseases of Children·S SarnaikJ Lusher
Sep 1, 1978·The American Journal of Medicine·D PowarsJ Allen
Apr 1, 1992·Archives of Disease in Childhood·K BaileyG R Serjeant
Mar 1, 1992·The Journal of Pediatrics·B BalkaranG R Serjeant
Jul 4, 1991·The New England Journal of Medicine·O S PlattT R Kinney
Jan 1, 1989·Annals of the New York Academy of Sciences·A L Hurtig, K B Park
Jun 19, 1986·The New England Journal of Medicine·M H GastonJ S Lobel
Jan 1, 1988·The American Journal of Pediatric Hematology/oncology·R B Scott
Mar 1, 1980·The Journal of Clinical Investigation·D R PowarsS P Azen
Aug 28, 1982·British Medical Journal·A N ThomasG R Serjeant
May 1, 1981·Clinical Pediatrics·M C StevensG R Serjeant
Jun 9, 1994·The New England Journal of Medicine·O S PlattP P Klug
Jun 1, 1997·American Journal of Hematology·M B MukherjeeR Krishnamoorthy
Jan 13, 2000·The New England Journal of Medicine·S T MillerT R Kinney
Nov 13, 2001·Clinical and Laboratory Haematology·A DonaldsonG R Serjeant
Feb 7, 2004·Blood·Charles T QuinnGeorge R Buchanan
Jan 20, 2006·Paediatric and Perinatal Epidemiology·Lydia FoucanUNKNOWN Paediatric Cohort of Guadeloupe
Oct 11, 2007·Journal of Medical Screening·L KingM Reid
Dec 24, 2008·Journal of Clinical Pathology·B GulbisF Vertongen
Jan 27, 2012·Indian Journal of Pediatrics·Sumanta PanigrahiPrafulla Kumar Khodiar
Jun 26, 2012·Indian Pediatrics·Dipty JainRoshan Colah
Jul 3, 2013·Cold Spring Harbor Perspectives in Medicine·Graham R Serjeant
Apr 10, 2014·Current Opinion in Hematology·Roshan ColahKanjaksha Ghosh
Aug 20, 2014·British Journal of Haematology·Khushnooma ItaliaRoshan B Colah

❮ Previous
Next ❯

Citations

Feb 22, 2018·Current Opinion in Hematology·Dipty Jain, Dipika Mohanty
May 29, 2020·The Indian Journal of Medical Research·Dipty JainGraham Roger Serjeant
Oct 5, 2019·Tropical Medicine and Infectious Disease·Kapilkumar DaveGayatri Desai
Nov 13, 2018·International Journal of Neonatal Screening·Roshan B ColahMalay B Mukherjee
Jun 3, 2021·Journal of the Neurological Sciences·Pallab BhattacharyaOfelia Alvarez
Nov 4, 2021·Orphanet Journal of Rare Diseases·Ahmar U ZaidiDave Nellesen

❮ Previous
Next ❯

Methods Mentioned

BETA
genotyping
PCR

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.