tailieunhanh - Chapter 099. Disorders of Hemoglobin (Part 7)

Clinical Manifestations of Sickle Cell Trait Sickle cell trait is usually asymptomatic. Anemia and painful crises are exceedingly rare. An uncommon but highly distinctive symptom is painless hematuria often occurring in adolescent males, probably due to papillary necrosis. Isosthenuria is a more common manifestation of the same process. Sloughing of papillae with urethral obstruction has been reported, as have isolated cases of massive sickling or sudden death due to exposure to high altitudes or extremes of exercise and dehydration. Diagnosis Sickle cell syndromes are suspected on the basis of hemolytic anemia, RBC morphology (Fig. 99-4), and intermittent episodes of ischemic pain | Chapter 099. Disorders of Hemoglobin Part 7 Clinical Manifestations of Sickle Cell Trait Sickle cell trait is usually asymptomatic. Anemia and painful crises are exceedingly rare. An uncommon but highly distinctive symptom is painless hematuria often occurring in adolescent males probably due to papillary necrosis. Isosthenuria is a more common manifestation of the same process. Sloughing of papillae with urethral obstruction has been reported as have isolated cases of massive sickling or sudden death due to exposure to high altitudes or extremes of exercise and dehydration. Diagnosis Sickle cell syndromes are suspected on the basis of hemolytic anemia RBC morphology Fig. 99-4 and intermittent episodes of ischemic pain. Diagnosis is confirmed by hemoglobin electrophoresis and the sickling tests already discussed. Thorough characterization of the exact hemoglobin profile of the patient is important because sickle thalassemia and hemoglobin SC disease have distinct prognoses or clinical features. Diagnosis is usually established in childhood but occasional patients often with compound heterozygous states do not develop symptoms until the onset of puberty pregnancy or early adult life. Genotyping of family members and potential parental partners is critical for genetic counseling. Details of the childhood history establish prognosis and need for aggressive or experimental therapies. Factors associated with increased morbidity and reduced survival are more than three crises requiring hospitalization per year chronic neutrophilia a history of splenic sequestration or hand-foot syndrome and second episodes of acute chest syndrome. Patients with a history of cerebrovascular accidents are at higher risk for repeated episodes and require especially close monitoring using Doppler carotid flow measurements. Patients with severe or repeated episodes of acute chest syndrome may need lifelong transfusion support utilizing partial exchange transfusion if possible. Figure 99-4 .

• Y học thường thức
• Disorders of Hemoglobin
• bệnh học và điều trị
• bài giảng bệnh học
• tài liệu học ngành y
• Harrisons Internal Medicine
• Harrisons principles of internal medicine
• Internal medicine
• Quality of health care
• Developed countries
• Health care systems
• Chest discomfort
• Clinical medicine
• The cardiovascular system
• Critical care medicine
• The kidney and urinary tract
• The gastrointestinal system
• The joints and adjacent tissues
• Neoplasms of the Lung
• tài liệu y khoa
• bệnhbệnh học và điều trị
• Sodium and Water học và điều trị
• General considerations in clinical medicine
• Cardinal manifestations
• Presentation of diseases
• The environment and disease
• Regenerative medicine
• Introduction to clinical medicine
• The environment
• Disorders of the cardiovascular system
• Disorders of the respiratory system
• Disorders of the gastrointestinal system
• Infectious diseases
• The respiratory system
• Neurologic Disorders
• Moraxella Infections
• Haemophilus Infections
• Bệnh truyền nhiễm
• Tim mạch
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• Cơ xương khớp
• Acute rheumatic fever
• Antiphospholipid syndrome
• Inflammatory myopathies
• Relapsing polychondritis
• Pituitary tumor syndromes