Fatal Acute Splenic Sequestration at 4 Months of Age

PEDIATRICS ◽  
1984 ◽  
Vol 73 (4) ◽  
pp. 507-508
Author(s):  
Juan N. Walterspiel ◽  
Joe C. Rutledge ◽  
Bryan L. Bartlett
Keyword(s):  
Cord Blood ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Screening Program ◽  
Splenic Sequestration ◽  
Blood Screening

A patient with homozygous sickle cell anemia is the youngest known to have died from acute splenic sequestration crisis. A cord blood screening program might have prevented this infant's death.

Screening Cord Bloods for Detection of Sickle Cell Disease in Jamaica

1974 ◽  
Vol 20 (6) ◽  
pp. 666-669 ◽  
Author(s):  
Beryl E Serjeant ◽  
Miriam Forbes ◽  
Leslie L Williams ◽  
Graham R Serjeant
Keyword(s):  
Sickle Cell Disease ◽  
Cord Blood ◽  
Sickle Cell ◽  
Screening Program ◽  
Blood Collection ◽  
Cell Disease ◽  
Agar Gel ◽  
Large Populations ◽  
Cord Blood Collection ◽  
Blood Screening

Abstract A cord-blood screening program, designed primarily for detecting sickle cell disease, has been in operation for seven months (8000 samples) at a large maternity unit in Kingston, Jamaica. We describe techniques of cord-blood collection and electrophoretic investigation on both cellulose acetate and agar gel. These methods appear to give rapid, valid results at minimal expense and are well adapted to screening large populations.

scholarly journals THE USE OF HPLC AS A TOOL FOR NEONATAL CORD BLOOD SCREENING OF HAEMOGLOBINOPATHY - A VALIDATION STUDY

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
A. Al-Madhani ◽  
Anil Pathare ◽  
Salam Alkindi
Keyword(s):  
Cord Blood ◽  
Sickle Cell ◽  
Neonatal Screening ◽  
Dna Analysis ◽  
Complete Blood Count ◽  
Screening Program ◽  
Carrier Status ◽  
Original Cohort ◽  
Hb Bart's ◽  
Blood Screening

Background: Newborn cord blood screening identifies infants with underlying haemoglobinopathies before they develop the characteristic symptoms or sequelae. Aims: This study was performed to validate the interpretation high-performance chromatography (HPLC) along with complete blood count (CBC) results as a tool for universal neonatal screening of hemoglobin disorders in Oman. Methods: HPLC and CBC data on subjects who participated in the National Neonatal screening program at birth were obtained from archival records. The results recorded at birth were compared with a second study performed on the same subjects, after approval from the local medical research and ethics committee.Results: Only 290 subjects from amongst the original cohort of 3740 newborns could be recalled between April 2010 to March 2011, to repeat HPLC and CBC, as well as perform confirmatory DNA studies, wherever necessary. All these subjects had been documented to show an initial abnormal result. 31 cases who had no HbA at birth on HPLC were confirmed as either homozygous β-thalassaemia major (n=5 subjects) or homozygous sickle cell anemia (n=26 subjects) by appropriate DNA analysis. Additionally, amongst 151 subjects, 72 subjects were studied in the initial study by Hb Bart’s quantitation using aalpha thalassaemia short program at birth. In this cohort, 42 subjects with Hb Bart’s >1% at birth could be confirmed as having either deletional or non-deletional thalassaemia by GAP PCR studies. No case of HbH was detected in this cohort. Further, carrier status for structural hemoglobin variants (HbS, HbC, HbD, HbE) (n=67) and beta thalassaemia allele with low HbA at birth (n=29 out of 41) were confirmed by relevant molecular studies.Conclusions: The study validated the earlier observation by 100% concordance with results of CBC and HPLC. Presence of Hb Bart’s at birth does not always mean the presence of alpha thalassemia, as subjects with Hb Bart’s was below 1% by quantitation, were shown to be normal by molecular studies. Key Words: Neonatal, screening, HPLC validation, haemoglobinopathy, sickle cell disease, thalassaemia 

Overview of the World Congress on cord blood and innovative approaches to the treatment of sickle-cell anemia in Monaco on 24-27th october 2013

2014 ◽  
Vol 2 (1) ◽  
pp. 95-98
Author(s):  
V. Kyryk
Keyword(s):  
Cord Blood ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
World Congress ◽  
The World ◽  
Innovative Approaches

Overview of the World Congress on cord blood and innovative approaches to the treatment of sickle-cell anemia in Monaco on 24-27th october 2013

scholarly journals Cord blood screening for hemoglobin abnormalities by thin layer isoelectric focusing

Blood ◽  
1980 ◽  
Vol 56 (6) ◽  
pp. 1068-1071 ◽  
Author(s):  
F Galacteros ◽  
K Kleman ◽  
J Caburi-Martin ◽  
Y Beuzard ◽  
J Rosa ◽  
...  
Keyword(s):  
Thin Layer ◽  
Cord Blood ◽  
Sickle Cell ◽  
Isoelectric Focusing ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Abnormal Hemoglobin ◽  
Excellent Method ◽  
Hemoglobin Variants ◽  
Blood Screening

Hemoglobin variants can be successfully identified in cord blood samples. The methods most commonly used include cellulose acetate (CAC) and citrate agar (CAG) electrophoresis. Recently thin layer isoelectric focusing (TLIF) has been shown to be an excellent method for identifying hemoglobin variants. To determine the applicability of TLIF for cord blood screening, we compared the results of 835 samples obtained by TLIF with that obtained by CAC, CAG, and the combination of both CAC and CAG. In 100 of these samples we detected an abnormal hemoglobin pattern using TLIF. In contrast, we detected only 80 abnormal samples by CAC, 70 by CAG, and 80 by using the combination of CAC and CAG. Due to the increased resolution provided by TLIF, we correctly diagnosed two sickle cell trait samples by TLIF that were incorrectly suspected to be homozygous for sickle cell disease by CAC and CAG. We identified 41 samples containing Bart's hemoglobin by TLIF in contrast to only 21 using CAC and 14 using CAG. The time and cost of TLIF was comparable to that using the combination of both methods. We, therefore, conclude that TLIF is the method of choice for cord blood screening.

scholarly journals Cord blood screening for hemoglobin abnormalities by thin layer isoelectric focusing

Blood ◽  
1980 ◽  
Vol 56 (6) ◽  
pp. 1068-1071 ◽  
Author(s):  
F Galacteros ◽  
K Kleman ◽  
J Caburi-Martin ◽  
Y Beuzard ◽  
J Rosa ◽  
...  
Keyword(s):  
Thin Layer ◽  
Cord Blood ◽  
Sickle Cell ◽  
Isoelectric Focusing ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Abnormal Hemoglobin ◽  
Excellent Method ◽  
Hemoglobin Variants ◽  
Blood Screening

Abstract Hemoglobin variants can be successfully identified in cord blood samples. The methods most commonly used include cellulose acetate (CAC) and citrate agar (CAG) electrophoresis. Recently thin layer isoelectric focusing (TLIF) has been shown to be an excellent method for identifying hemoglobin variants. To determine the applicability of TLIF for cord blood screening, we compared the results of 835 samples obtained by TLIF with that obtained by CAC, CAG, and the combination of both CAC and CAG. In 100 of these samples we detected an abnormal hemoglobin pattern using TLIF. In contrast, we detected only 80 abnormal samples by CAC, 70 by CAG, and 80 by using the combination of CAC and CAG. Due to the increased resolution provided by TLIF, we correctly diagnosed two sickle cell trait samples by TLIF that were incorrectly suspected to be homozygous for sickle cell disease by CAC and CAG. We identified 41 samples containing Bart's hemoglobin by TLIF in contrast to only 21 using CAC and 14 using CAG. The time and cost of TLIF was comparable to that using the combination of both methods. We, therefore, conclude that TLIF is the method of choice for cord blood screening.

Acute Splenic Sequestration in a 2-Month-Old Infant With Sickle Cell Anemia

PEDIATRICS ◽  
1989 ◽  
Vol 84 (3) ◽  
pp. 578-579
Author(s):  
ALBERTO PAPPO ◽  
GEORGE R. BUCHANAN
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Parental Education ◽  
Early Years ◽  
Splenic Sequestration ◽  
Screening Programs ◽  
Splenic Enlargement ◽  
Clinically Significant ◽  
Overall Mortality

Acute splenic sequestration crisis is a potentially fatal complication of sickle cell anemia. It is characterized by acute splenic enlargement, with trapping of erythrocytes, causing increasing anemia and the potential for circulatory failure. The development of newborn screening programs aimed at identifying children with clinically significant hemoglobinopathies, combined with early parental education and careful follow-up, has decreased the overall mortality due to acute splenic sequestration crisis and other complications during the early years of life. The purpose of this paper is to report the case of an 8-week-old infant with sickle cell anemia and acute splenic sequestration crisis.

Diagnosis at Birth Improves Survival of Children With Sickle Cell Anemia

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 830-833
Author(s):  
Darleen Powars
Keyword(s):  
New York ◽  
Los Angeles ◽  
Medical Care ◽  
Cord Blood ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Infectious Complications ◽  
Childhood Mortality ◽  
Surveillance Program ◽  
Accurate Identification

The increased survival of children who have sickle cell disease is primarily due to state-of-the-art improvements in general pediatric medical care with particular emphasis on the management of the infectious complications that occur. Studies reported from New York City, Los Angeles, New Haven, and Jamaica clearly demonstrate the calendar era change in survival that has occurred during the 1970s and 1980s, and the greatest improvement is found among those children who have sickle cell anemia. The accurate identification of the specific hemoglobinopathy at or near birth provided the foundation for these studies documenting infant and young child mortality. In Africa, Molineaux et al and Fleming et al reported an epidemiologic investigation subsequent to a cord blood diagnosis program initiated in Garke, Nigeria, in 1976. A total of 534 infants were screened for major hemoglobinopathies, and 11 babies with SS and 125 babies with AS were identified. Minimal medical care was available for follow-up of the children. On entry to the school program at 5 years of age, the same population from the same small rural town was restudied. Only one child of 439 was found to have sickle cell anemia but 133 were AS. The inescapable conclusion was that the African babies with SS had died during early childhood, contributing disproportionate numbers to the high infant and childhood mortality in Nigeria. Not until Dr Fleming and the investigators of the British Medical Research Council had performed a cord blood hemoglobinopathy surveillance program was the incidence of sickle cell anemia and its effect on childhood mortality in Nigeria documented.

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