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Congenital Heinz Body Hemolytic Anemia

Unstable Hemoglobin Disease


Presentation

  • Over 20 NEW test entries present the latest tests and procedures, with a strong focus on affordable, clinically relevant genetic tests.[books.google.com]
  • For easy reference, information is provided by clinical presentation and abnormalities, and includes hundreds of tables, boxes, key points, and algorithms.[books.google.com]
  • The classic clinical presentation of hemolytic anemia is jaundice, pallor, and splenomegaly.[obgynkey.com]
  • Other beta chain structural defects may also present under such circumstances.[oncologynurseadvisor.com]
  • Splenomegaly and Heinz bodies may also be present. Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate.[mayomedicallaboratories.com]
Anemia
  • Abstract The mechanisms of hemoglobin precipitation into Heinz bodies and hemolytic anemia that characterize congenital Heinz body hemolytic anemia (CHBHA) were studied in patients with the unstable hemoglobins, Köln (beta-98 valine -- methionine) and[ncbi.nlm.nih.gov]
  • Stay current on the developments and research surrounding neonatal anemia from the entirely new chapter on Blood and Hematopoietic System highlights.[books.google.com]
Physician
  • This volume is recommended for students, researchers, teachers, and physicians aiming to acquire knowledge of the red blood cell.[books.google.com]
  • […] detected by any of the above tests except globin sequencing Do not repeat hemoglobin electrophoresis in patients with a prior result who do not require therapeutic intervention or monitoring of hemoglobin variant levels ( Choosing Wisely: 20 Things Physicians[arupconsult.com]
  • The following DoveMed website link is a useful resource for additional information: Please visit our Laboratory Procedures Center for more physician-approved health information: References and Information Sources used for the Article: Kee, J.[dovemed.com]
  • American Family Physician [On-line journal]. Available online at . Cutler, C. (2003 September 14, Updated).Glucose-6-phosphate dehydrogenase deficiency. MedlinePlus Medical Encyclopedia [On-line information]. Available online at .[labtestsonline.it]
  • Journal of intensive care medicine. 29(6):327-33. 2014. [ pubmed ] About Dr Chris Nickson An oslerphile emergency physician and intensivist suffering from a bad case of knowledge dipsosis.[lifeinthefastlane.com]
Pathologist
  • Confidently prepare for the upcoming recertification exams for clinical pathologists set to begin in 2016.[books.google.com]
  • […] medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists[emedicine.medscape.com]
Plethora
  • […] appropriately selecting and interpreting laboratory tests, Small Animal Clinical Diagnosis by Laboratory Methods, 5th Edition helps you utilize your in-house lab or your specialty reference lab to efficiently make accurate diagnoses without running a plethora[books.google.com]
Epilepsy
  • […] reflux disease Gastrointestinal bleeding Gastrointestinal tract Gene expression and transcription General bacteriology General endocrinology General histology General mycology General oncology General principles of fractures General virology Generalized epilepsy[amboss.com]
Heart Failure
  • In physiologic response to hypoxia as in congenital heart disease and congestive heart failure. Basophilic stippling: These are the inclusion in the cytoplasm of RBC e.g. Lead poisoning. Reticulocytosis.[labpedia.net]
  • The severe anemia causes congestive heart failure and subsequent massive total body edema, termed " hydrops fetalis ."[web2.iadfw.net]
  • Severe anemia due to bleeding in a neonate or a live hydropic baby (alpha thalassemia major, Diamond Blackfan anemia, congenital parvovirus) is a pediatric emergency that requires rapid evaluation and treatment to prevent hypoxia, congestive heart failure[oncologynurseadvisor.com]
  • failure Conjunctivitis Connective tissue Connective tissue diseases Conservative treatment of fractures Constipation Coronary artery bypass grafting Cough Counseling patients Coxsackie virus infections Cranial nerve palsies Craniosynostosis Craniovertebral[amboss.com]
  • Congestive heart failure and metabolic disorders should be considered.[emedicine.medscape.com]
Myopathy
  • Immunosuppressants Impetigo Inborn errors of carbohydrate metabolism Inborn errors of metabolism Incomplete spinal cord syndromes Infection prevention and control Infectious mononucleosis Infective endocarditis Infertility Inflammation of the eyelids Inflammatory myopathies[amboss.com]
Suggestibility
  • Concomitantly, heme avidity to beta-92 lessens, suggesting that degradation of the resulting excessively freed heme may produce the pigmented dipyrroluria of this syndrome.[ncbi.nlm.nih.gov]
  • Intravascular hemolysis is also suggested by urinary hemosiderin.[merckmanuals.com]
  • Anemia does not appear to be present in these infants, and this suggest that liver dysfunction plays a greater role.[g6pd.org]
  • Useful For Suggests clinical disorders or settings where the test may be helpful Work-up of congenital hemolytic anemias Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Unstable[mayomedicallaboratories.com]
  • The heme units are identical in each hemoglobin molecule; changes in amino acid sequence in the globin […] Laboratory Tests in Hemolytic Anemias Certain laboratory tests are extremely helpful in suggesting or demonstrating the presence of hemolytic anemia[med-life.net]
Stroke
  • […] all the latest approaches in clinical laboratory medicine with new and updated coverage of: the chemical basis for analyte assays and common interferences; lipids and dyslipoproteinemia; markers in the blood for cardiac injury evaluation and related stroke[books.google.com]
  • Stroke. Children with SCD, particularly types SS and S β-thalassemia, may develop hemorrhagic or infarctive strokes.[obgynkey.com]
  • Sickle Cell Crisis: Sickle cell crisis results in pain, anemia, edema, organ problems or failure, infections, and can also lead to stroke due to clogging up of the blood vessels with the sickled cells.[hematologylearning.weebly.com]
  • […] ataxia Spleen Splenic rupture Splenomegaly Spondylolisthesis Sports injuries Staphylococcal diseases Staphylococcal scalded skin syndrome Statins Statistical analysis of data Stevens-Johnson syndrome Stiff person syndrome Stomach Stress incontinence Stroke[amboss.com]
Forgetful
  • Forget, Douglas R. Higgs, Ronald L. Nagel Cambridge University Press, 2001 - 1268 من الصفحات Disorders of Hemoglobin is the first comprehensive reference on the genetic and acquired disorders of hemoglobin in over a decade.[books.google.com]
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Encephalopathy
  • Virtual histology slide box Virtual histopathology slide box Vitamin B12 deficiency Vitamin K deficiency bleeding of the newborn Vitamins Vitiligo Volvulus and intestinal malrotation Vulvar and vaginal cancer Vulvovaginitis Wallenberg syndrome Wernicke encephalopathy[amboss.com]

Workup

  • A convenient way to organize and think through the workup of an anemic infant would be: 1.[oncologynurseadvisor.com]
  • An array of laboratory tests are available for detecting hemolysis, and specialized tests may be indicated to diagnose the cause for hemolysis (see Workup ).[emedicine.medscape.com]
Heinz Bodies
  • Abstract The mechanisms of hemoglobin precipitation into Heinz bodies and hemolytic anemia that characterize congenital Heinz body hemolytic anemia (CHBHA) were studied in patients with the unstable hemoglobins, Köln (beta-98 valine -- methionine) and[ncbi.nlm.nih.gov]
  • Denatured hemoglobin forms Heinz bodies, which damage the erythrocyte membrane and promote premature hemolysis.[medical-dictionary.thefreedictionary.com]
  • (Equivalent Terms) Heinz-Ehrlich Bodies Blood Test What is Heinz Bodies Blood Test? (Background Information) Heinz bodies are irregularly-shaped masses of nonfunctional hemoglobin.[dovemed.com]
Heinz Bodies
  • Abstract The mechanisms of hemoglobin precipitation into Heinz bodies and hemolytic anemia that characterize congenital Heinz body hemolytic anemia (CHBHA) were studied in patients with the unstable hemoglobins, Köln (beta-98 valine -- methionine) and[ncbi.nlm.nih.gov]
  • Denatured hemoglobin forms Heinz bodies, which damage the erythrocyte membrane and promote premature hemolysis.[medical-dictionary.thefreedictionary.com]
  • (Equivalent Terms) Heinz-Ehrlich Bodies Blood Test What is Heinz Bodies Blood Test? (Background Information) Heinz bodies are irregularly-shaped masses of nonfunctional hemoglobin.[dovemed.com]

Treatment

  • The Red Blood Cell, Second Edition, Volume II provides a comprehensive treatment and review of basic biomedical knowledge about the circulating, adult red blood cell.[books.google.com]
  • This text is divided into eight distinct sections, and includes coverage of the molecular and genetic basis of hemoglobinopathies and thalassemias, their epidemiology and genetic selection, and the diagnosis and special treatments of D*b and D*a thalassemias[books.google.com]
  • Walsh, along with a multi-disciplinary team of contributors guide you through the sweeping developments in diagnosis and treatment of the mother fetus, and neonate.[books.google.com]
  • Effectively monitor the pace of drug clearing in patients undergoing pharmacogenomic treatments with a new chapter on this groundbreaking new area.[books.google.com]

Prognosis

  • Prognosis The prognosis for patients with hemolytic anemia depends on the underlying cause. Overall, mortality rates are low in hemolytic anemias. However, the risk is greater in older patients and patients with cardiovascular impairment.[emedicine.medscape.com]
  • Course and prognosis Hemolytic episodes in the A– type of deficiency are usually self-limited, even if drug administration is continued. This is not the case in the more severe Mediterranean type of deficiency.[g6pd.org]
  • The prognosis of homozygous Hb C disease is excellent. An individual may inherit a Hb S gene from one parent and a Hb C gene from the other. The result of this double whammy is Hb SC disease.[web2.iadfw.net]

Etiology

  • Sickle Cell Disease Etiology Hemoglobin S (Hb S) is the predominant hemoglobin in the group of genetic disorders that encompass sickle cell disease (SCD).[obgynkey.com]
  • "An update on the etiology and diagnostic evaluation of a leukemoid reaction." European journal of internal medicine 17.6 (2006): 394-398. ul Haque, Anwar, and Noor ul Aan. "Leukemoid Reaction: Unusual Causes."[derangedphysiology.com]
  • Hemolytic anemia has multiple causes, and the clinical presentation can differ depending on the etiology.[emedicine.medscape.com]
  • The classification, symptomatology, and diagnostic procedures necessary for detection of such reactions and identification of the etiology are discussed in Chapter 9 and Chapter 11.[med-life.net]
  • It may be acquired through: immune etiologies(Rh, ABO or other blood group incompatibility, maternal autoimmune, drugs) infectious etiologies(bacterial sepsis, rubella, CMV, disseminated herpes simplex virus, adenovirus, toxoplasmosis, malaria, syphilis[oncologynurseadvisor.com]

Epidemiology

  • This text is divided into eight distinct sections, and includes coverage of the molecular and genetic basis of hemoglobinopathies and thalassemias, their epidemiology and genetic selection, and the diagnosis and special treatments of D*b and D*a thalassemias[books.google.com]
  • […] of Public Health Laboratories [APHL], 2015) Differential Diagnosis Sickle cell disease Congenital hemolytic anemias Glucose-6-phosphatase deficiency Pyruvate kinase deficiency Iron deficiency anemia Thalassemia Autoimmune hemolytic anemia Background Epidemiology[arupconsult.com]
  • […] intracranial pressure and brain herniation Elimination disorders Embryogenesis Emotional and behavioral disorders in children and adolescents Endarterectomy Endometrial cancer Endometriosis Endophthalmitis Environmental injuries Enzymes and biocatalysis Epidemiology[amboss.com]
  • Epidemiology Hemolytic anemia represents approximately 5% of all anemias. Acute AIHA is relatively rare, with an incidence of one to three cases per 100,000 population per year. [27] Hemolytic anemias are not specific to any race.[emedicine.medscape.com]
  • Epidemiology and genetics The Hb S gene is found primarily in populations of native tropical African origin (which include most African-Americans).[web2.iadfw.net]
Sex distribution
Age distribution

Pathophysiology

  • It stands as the definitive work on the genetics, pathophysiology, and clinical management of this wide range of disorders. Drs.[books.google.com]
  • Pathophysiological interactions cause unstable hemoglobins to exhibit altered solubility due to oxidation of amino acid residues in globin chains Prevents formation of intact hemoglobin tetramer through weakened binding of heme to globin Interferes with[arupconsult.com]
  • Useful For Suggests clinical disorders or settings where the test may be helpful Work-up of congenital hemolytic anemias Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Unstable[mayomedicallaboratories.com]
  • Pathophysiology The differential count on the peripheral blood smears gives us information for Anaemias or leukemias and any other abnormality of the blood cells. DLC and CBC are less expensive, easy to perform, and can be done in a short time.[labpedia.net]

Prevention

  • Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection.[books.google.com]
  • […] prevalent Inherited as autosomal dominant disorders although de novo variants do occur Pathophysiology Pathophysiological interactions cause unstable hemoglobins to exhibit altered solubility due to oxidation of amino acid residues in globin chains Prevents[arupconsult.com]
  • Porphyrias Portal hypertension Postmortem skin changes Postpartum hemorrhage Postpartum period Poststreptococcal glomerulonephritis Precancerous skin lesions Pregnancy Pregnancy loss Prenatal and postnatal physiology Prenatal care Preterm labor and birth Preventive[amboss.com]
  • Modern obstetric practice with administration of anti-D immunoprophylaxis to RhD negative women during pregnancy and within 72 hours postnatally has prevented 96% of cases of RhD sensitization in developed countries.[oncologynurseadvisor.com]
  • […] macrophage mediated clearance of antibody bound RBCs) cyclophosphamide or azathioprine (third line) role of IV Ig is uncertain (used in case reports) danazol (synthetic steroid, used previously) cold AIHA keep patient and fluids warm – avoidance of cold prevents[lifeinthefastlane.com]

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