scholarly journals Type 2 diabetes in adults with sickle cell disease: can we dive deeper? Response to Skinner et al

2019 ◽  
Vol 186 (5) ◽  
pp. 782-783
Author(s):  
Jifang Zhou ◽  
Jin Han ◽  
Edith A. Nutescu ◽  
William L. Galanter ◽  
Surrey M. Walton ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

Glycemic Monitoring in Diabetics with Sickle Cell Plus β-Thalassemia Hemoglobinopathy

2005 ◽  
Vol 39 (9) ◽  
pp. 1557-1560 ◽  
Author(s):  
Sheri M Kosecki ◽  
Philip T Rodgers ◽  
Martha B Adams
Keyword(s):  
Type 2 Diabetes ◽  
Blood Glucose ◽  
Sickle Cell Disease ◽  
Glycemic Control ◽  
Sickle Cell ◽  
Diabetes Management ◽  
Glycosylated Hemoglobin ◽  
Fasting Blood Glucose ◽  
Cell Disease

OBJECTIVE: To report a case of diabetes management in a patient with a hemoglobinopathy that caused her clinician to seek a different measure of glycemic control, fructosamine, rather than glycosylated hemoglobin (HbA1c). CASE SUMMARY: A 53-year-old African American woman presented with a past medical history of type 2 diabetes, hypertension, seizure disorder, rheumatoid arthritis, and sickle cell disease plus β-thalassemia. She reported fasting blood glucose values ranging broadly from 50 to 320 mg/dL, yet her HbA1c result remained steady in a low range of >6%. A measure of fructosamine returned elevated at 340 μmol/L (reference range 200–300%). DISCUSSION: We believe that this patient's hemoglobinopathy resulted in falsely low levels of HbA1c, and we substantiate this interpretation with the patient's self-monitored blood glucose values from home that appeared higher and inconsistent with the HbA1c results. Although few reports on using the measure of fructosamine appear in the literature, this patient's high fructosamine result supports fructosamine as the more appropriate measure of glycemic control. CONCLUSIONS: Serum fructosamine levels may be considered as an appropriate laboratory measurement when monitoring long-term glycemic control in patients with type 2 diabetes mellitus and sickle cell disease.

scholarly journals Type 2 Diabetes Mellitus in Patients with Sickle Cell Disease: A Population-Based Longitudinal Analysis of Three Cohorts

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4817-4817
Author(s):  
Jifang Zhou ◽  
Jin Han ◽  
Edith A. Nutescu ◽  
William Galanter ◽  
Surrey M. Walton ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Plan ◽  
Population Based ◽  
Cell Disease ◽  
Clinical Cohort

Abstract Introduction The prevalence and incidence of type 2 diabetes mellitus (T2DM) in the United States (U.S.) is increasing with more than 100 million adults living with diabetes or pre-diabetes. Population-based evidence on the prevalence and risks for T2DM in patients with sickle cell disease (SCD) is limited. This study measured the prevalence of T2DM in patients with SCD and clinical characteristics associated with its incidence in a large commercially insured adult SCD cohort and also an academic institution-based clinical cohort. Methods We performed a population-based cohort study of commercially-insured health plan enrollees using the Truven MarketScan® Research Databases. Patients with SCD (1 inpatient or 2 outpatient claims that are at least 30 days apart) were identified and sampled each calendar year between 2009 and 2014. Prevalence in each closed cohort of continuously enrolled patients was determined per calendar year. Incidence rates of T2DM were estimated and compared with adult non-Hispanic Black respondents to the National Health and Nutrition Examination Survey (NHANES) over the same study period (2009-2014). Among SCD patients, multivariable Cox proportional hazard models were used to identify factors associated with incident T2DM, adjusting for relevant patient characteristics. Finally, prevalence of T2DM was measured in a cohort of patients with SCD aged ≥20 years at first medical encounter at the University of Illinois at Chicago (UIC) from January 2008 to December 2017. Prevalent T2DM was identified through a combination of diagnosis codes, self-reporting, anti-diabetic medications excluding insulin and glucose tests in outpatient settings. Results Among 7,070 health plan enrollees with SCD, the median age (mean) was 37.0 (38.9) years and 60.8% were female. Compared to SCD patients without T2DM, more SCD patients with T2DM had nephropathy (28.0% vs. 9.5%; p<0.001), neuropathy (17.7% vs. 5.2%; p<0.001), and history of stroke (24.1% vs. 9.2%; p<0.001). The standardized prevalence of T2DM among patients with SCD showed a modest increase from 15.7% to 16.5% from 2009 to 2014 (p trend=0.0259), and SCD patients had comparable prevalence of T2DM compared to the NHANES subjects (18.2%). [Figure A] Over 17,024 person-years, we observed a crude incidence rate for T2DM of 25.4 per 1,000 person-years. Risk of developing T2DM in patients with SCD increased with age, and incident T2DM was associated with comorbid hypertension (HR=1.45, 95%CI 1.14-1.83) and dyslipidemia (HR=1.43, 95%CI 1.04-1.96). [Figure B] Of the 672 adults in the UIC cohort of patients with SCD, 61.1% were female, the median (mean) age was 30.0 [32.9] years, and 478 (71.1%) had homozygous HbS disease (HbSS). A total of 76 (11.3%) patients had T2DM, with the highest prevalence among SCD patients ages ≥ 40 years (50/190, 26.3%). [Figure C] Abnormal glucose test results (≥200 mg/dl) were documented in 41 patients with mean (SD) of 294 (94) mg/dl. Among 31 patients with abnormal fructosamine tests (>285 µmol/L), the mean (SD) fructoasmine value was 392 (90) µmol/L. Conclusion We present evidence describing the prevalence of T2DM in patients with SCD both in a commercially-insured population and from an institution-based clinical cohort. These findings were similar to a general African American population with an increasing trend in T2DM over recent years. These trends support the routine screening for T2DM in patients with SCD, especially those of older age and with presence of comorbid hypertension and/or dyslipidemia. Disclosures No relevant conflicts of interest to declare.

scholarly journals Type 2 diabetes in adults with sickle cell disease: can we dive deeper?

2019 ◽  
Vol 186 (5) ◽  
pp. 783-785 ◽  
Author(s):  
Sarah Skinner ◽  
Elie Nader ◽  
Philippe Connes
Keyword(s):  
Type 2 Diabetes ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

Phosphatidylserine externalization in sickle red blood cells: associations with cell age, density, and hemoglobin F

Blood ◽  
2003 ◽  
Vol 102 (1) ◽  
pp. 365-370 ◽  
Author(s):  
Zahida Yasin ◽  
Scott Witting ◽  
Mary B. Palascak ◽  
Clinton H. Joiner ◽  
Donald L. Rucknagel ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Lower Percentage ◽  
Cell Disease ◽  
Low Level ◽  
Sickle Cells ◽  
Cell Age

Abstract Phosphatidylserine (PS) is normally confined to the cytoplasmic leaflet of the red blood cell (RBC) membrane, but some sickle RBCs expose PS in the outer leaflet (PS+ cells). This study examined the relationships among PS externalization, fetal hemoglobin content, hydration state, and cell age. Sickle RBCs exhibit a wide range of PS externalization. Those with low-level exposure (type 1 PS+) include many young transferrin-receptor-positive (TfR+) cells. This is not specific for sickle cell disease because many nonsickle TfR+ cells are also PS+. RBCs with higher PS exposure (type 2 PS+) appear to be more specific for sickle cell disease. Their formation is most likely sickling dependent because type 2 PS+ dense sickle cells have a lower percentage of fetal hemoglobin (HbF) than PS- cells in the same density fraction (1.7 vs 2.9; n = 8; P &lt; .01). In vivo experiments using biotin-labeled sickle cells showed a sharp decrease in the percentage of circulating, labeled PS+ cells in the first 24 hours after reinfusion. This decrease was confined to type 1 PS+ cells and was thus consistent with the reversal of PS exposure in very young cells. As the labeled cells aged in the circulation, the percentages of type 1 and type 2 PS+ cells increased. These studies indicate that PS externalization in sickle cells may be low level, as observed in many immature cells, or high level, which is associated with dehydration and appears to be more specific for sickle RBCs. (Blood. 2003;102: 365-370)

Type-2 Phosphatidylserine (PS)-Positive Erythrocytes and Their Association with Markers of Hemolysis and Hemostatic Activation in Children with Sickle Cell Disease (SCD)

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 943-943
Author(s):  
Yamaja B. Setty ◽  
Suhita Gayennebetal ◽  
Nigel S. Key ◽  
Marie Stuart
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Red Cells ◽  
Red Cell ◽  
Cell Disease ◽  
Activation Markers ◽  
D Dimer

Abstract Introduction: Type-2 phosphatidylserine (PS)-positive red cells are a subpopulation of erythrocytes that are highly positive for PS, contain low levels of fetal hemoglobin, are specific for sickle cell disease (SCD) and have been identified in the dense red cell fraction. Studies have implicated PS-positive red cells in enhancing anemia due to phagocytosis and hemolysis. Shielding of red cell PS by diannexin, a synthetic homodimer of human annexin-V, has been demonstrated to provide protection against hemolysis and prevent activation of prothrombinase. Methods: Using flow cytometry, we measured the levels of type-1 (red cells with low PS positivity) and type-2 PS-positive red cells in 50 children with SCD (31 with HbSS and 19 with HbSC), and assessed their association with various markers of hemolysis and hemostatic activation. Markers of hemolysis evaluated included plasma lactate dehydrogenase (LDH), reticulocyte count, and hemoglobin. Whole blood tissue factor (WBTF), pro-thrombin fragment F1+2, and D-dimer were evaluated as markers of hemostatic activation. Results: We demonstrate that the levels of type-2 PS-positive red cells are significantly increased in HbSS patients (1.37 ± 0.97%, p<0.01) compared to children with HbSC disease (0.32 ± 0.21%) and age- and race-matched controls (0.15 ± 0.15%, n=19). WBTF and D-dimer showed significant associations with both type-1 and -2 red cells with no significant differences in the strength of their association. However, significantly greater correlations were noted between type-2 PS red cells and hemolytic markers compared to those noted with type-1 (Steiger's Z=3.05 to 4.59, p<0.01). In addition our in vitro studies demonstrate increased osmotic fragility of these red cells. Table 1. Association of PS-positive RBCs with markers of hemolysis and hemostatic activation Biomarker Type-1 PS-positive RBCs Type-2 PS-positive RBCs Markers of Hemolysis LDH r = 0.44, p<0.002 r = 0.63, p<0.00001 % Reticulocyte r = 0.43, p=0.002 r = 0.66, p<0.00001 Hemoglobin r =-0.35, p=0.014 r =-0.63, p<0.00001 Markers of Hemostatic Activation WBTF r = 0.41, p=0.008 r = 0.56, p<0.0002 F1+2 r = 0.26, p=0.07 r = 0.31, p<0.03 D-dimer r = 0.46, p<0.001 r = 0.56, p<0.0005 Conclusions: Type-2 PS-positive red cells are elevated in SCD and the number of these cells correlates significantly with both markers of hemolysis and hemostasis. These findings provide a patho-physiologic link between the intravascular hemolytic milieu of SCD and the hemostatic perturbations previously noted in this disease. Disclosures No relevant conflicts of interest to declare.

scholarly journals Oculocutaneous albinism type 2 (OCA2) with homozygous 2.7-kb deletion of the P gene and sickle cell disease in a Cameroonian family. Identification of a common TAG haplotype in the mutated P gene

2007 ◽  
Vol 52 (9) ◽  
pp. 771-780 ◽  
Author(s):  
Robert Aquaron ◽  
Nadem Soufir ◽  
Jean-Louis Bergé-Lefranc ◽  
Catherine Badens ◽  
Frederic Austerlitz ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Oculocutaneous Albinism ◽  
Cell Disease ◽  
P Gene
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