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Hereditary Hemolytic Anemia

Hah JO

The hereditary hemolytic anemia (HHA) can be classified into three types according to the pathogenesis: RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics of these three types of HHA...
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New approaches for cholestasis in hemoglobinopathies

Dhiman P, Saxena P, Bihari C, Rastogi A, Sarin SK

No abstract available.
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Hemolytic anemia in pediatrics

Hah JO

To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to...
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Comparison of Capillary Electrophoresis with Cellulose Acetate Electrophoresis for the Screening of Hemoglobinopathies

Kim JE, Kim BR, Woo KS, Kim JM, Park JI, Han JY

BACKGROUND: beta-thalassemia is primarily found in individuals of Mediterranean and Southeast Asian ancestry. With rapid growth in the Southeast Asian segments of the Korean population, the geographic distribution of hemoglobinopathies...
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Modified classification of anemia by RDW

Hwang HK, Hyun MS, Shim BS

The author obtained index of red cell volume distribution width(RDW) and other red cell indices in 210 patients of various hematoncologic conditions and 200 healthy control group using, an automated...
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A Case of Renal Papillary Necrosis Associated with Diabetes Mellitus

Hong CD, Choi TS, Ahn DW

  • KMID: 1912889
  • Korean J Urol.
  • 1990 Dec;31(6):933-936.
Renal papillary necrosis is rare. It usually occurs in association with diabetes mellitus, pyelonephritis, urinary tract obstruction, analgesic abuse and sickle cell hemoglobinopathies. The basic pathophysiologic process in renal papillary...
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Anesthetic management of a patient with hemoglobinopathy: A case report

Lee HS, Lee JY, Kim JY, Lee HJ

  • KMID: 2169143
  • Anesth Pain Med.
  • 2012 Jan;7(1):30-33.
Hemoglobinopathy poses many challenges to the anesthesiologists. The central issue is the accuracy about interpretation of pulse oximetry saturation value during anesthesia. There are limited data with respect to the...
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Diagnostic approaches for inherited hemolytic anemia in the genetic era

Kim Y, Park J, Kim M

Inherited hemolytic anemias (IHAs) are genetic diseases that present with anemia due to the increased destruction of circulating abnormal RBCs. The RBC abnormalities are classified into the three major disorders...
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Recent progress in laboratory diagnosis of thalassemia and hemoglobinopathy: a study by the Korean Red Blood Cell Disorder Working Party of the Korean Society of Hematology

Lee YK, Kim HJ, Lee K, Park SH, Song SH, Seong MW, Kim M, Han JY

Genetic hemoglobin disorders are caused by mutations and/or deletions in the α-globin or β-globin genes. Thalassemia is caused by quantitative defects and hemoglobinopathies by structural defect of hemoglobin. The incidence...
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Effectiveness of premarital screening program for thalassemia and sickle cell disorders in Ras Al Khaimah, United Arab Emirates

Salama RA, Saleh AK

PURPOSE: Genetic disorders can be prevented by basic public health measures and activities that focus primarily on education and approaches in Primary Health Care. Premarital screening is one such approach...
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Hydroxyurea Therapy in Hemoglobin Madrid Patients

Seo YK, Lee EH, Kim TW, Yang JH, Jung HL, Shim JY, Park MS, Keum DH

  • KMID: 2252435
  • Korean J Hematol.
  • 2002 Feb;37(1):54-59.
Unstable hemoglobins (Hb) are variants of adult Hb that tend to precipitate and form insoluble inclusions (Heinz bodies) within red blood cells (RBC) and RBC precursors. More than 100 structurally...
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Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology

Park ES, Jung HL, Kim HJ, Park SS, Bae SH, Shin HY, Song SH, Koh KN, Lyu CJ, Lim YT, Han DK, Hah JO

BACKGROUND: The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from...
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A Case of Heterozygous alpha(+)-Thalassemia Diagnosed in a Korean Family by Using Multiplex Ligation-Dependent Probe Amplification

Oh AC, Lee JK, Hong YJ, Hong SI, Yang SH, Park CH, Kim HJ, Jo HS, Chang YH

  • KMID: 1426767
  • J Lab Med Qual Assur.
  • 2012 Dec;34(2):113-117.
Alpha-thalassemia (alpha-thalassemia), which is prevalent in the Mediterranean region, is caused by deficient synthesis of the alpha-globin chains. It is commonly caused by HBA1 and/or HBA2 gene deletion and is...
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The role of HbA1C testing in diagnosing diabetes

Lee H

  • KMID: 1458422
  • Korean J Med.
  • 2010 Nov;79(5):495-499.
Diabetes is often not diagnosed until complications appear, and approximately 30% of people with diabetes may be undiagnosed. Also, complications of diabetes have become a leading cause of impairment of...
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Genotypic influence of alpha-deletions on the phenotype of Indian sickle cell anemia patients

Pandey S, Pandey S, Mishra RM, Sharma M, Saxena R

BACKGROUND: Some reports have shown that co-inheritance of alpha-thalassemia and sickle cell disease improves hematological parameters and results in a relatively mild clinical picture for patients; however, the exact molecular...
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Salmonella Typhi Osteomyelitis in a Non-sickle Cell Patient: Three Cases Report

Shin KH, Youn KH, Jahng JS

Salmonella osteomyelitis is uncommon and it often develops in patients with sickle cell anemia and other hemoglobinopathy. Especially, osteomyelitis caused Salmonella typhi in a nonsickle cell patients is a very...
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Steroid - Induced Early Changes in Adult Rabbit Femoral Head: Experimental Study

Yune SH, Hwang DS, Leung G, Kim SB

Osteonecrosis has been found in association with non-traumatic pathological conditions such as alcoholism, sickle cell hemoglobinopathies, Gaucher's disease and decompression sickness. It is also a major compcation in surviving renal...
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Long-term Follow-up of Hb Madrid Patient

Koo DH, Oh S, Kim HJ, Jung HL

  • KMID: 2049293
  • Clin Pediatr Hematol Oncol.
  • 2013 Apr;20(1):55-58.
We report a long-term follow-up of unstable hemoglobin (Hb) patient. He was diagnosed as Hb Madrid [beta115(G17)Ala-->Pro] by direct DNA sequencing and restriction enzyme analysis. Hydroxyurea had been given for...
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Update on the current modalities used to screen high risk youth for prediabetes and/or type 2 diabetes mellitus

Brar PC

The modalities currently employed to screen for type 2 diabetes mellitus (T2DM)/prediabetes are HbA1(c), fasting plasma glucose (FPG), and 2-hour plasma glucose (PG) during an oral glucose tolerance test (OGTT)....
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Role of Gamma Globin Promoter Region -269~-240 in Hydroxyurea Treated Erythroid Progenitor Cells

Park JI, Kim TG, Kim DI, Kim IH, Jeong JS, Han JY

  • KMID: 1863286
  • Korean J Clin Pathol.
  • 1998 Mar;18(1):29-34.
BACKGROUND: Recently, a great deal of interest has been focused on the use of hydroxyurea and hemin that may augment Hb F levels in patients with hemoglobinopathies and thalassemia, although...
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