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RBC membrane abnormalities e. American journal of medicine and medical sciences pneumoniae ) c. WBC malignancies ( e. SICKLE CELL DISEASE PAINFUL CRISIS A. SICKLE CELL DISEASE:COMPLICATIONS INFECTION A.

SICKLE CELL DISEASE: COMPLICATIONS INFECTION CONTINUED: B. Infection is important cause of death in children: (young children are treated prophylactically with penicillin ) C. Patients have increased episodes of pneumonia, sepsis, meningitis osteomyelitis D. Associated with occlusion and stasis in skin microvasculature 4. SICKLE CELL DISEASE: COMPLICATIONS Cardiac Complications: 1.

CASE Splenomegaly secondary to acute sequestration crisis in a 20-month-old female with sickle cell disease: An enlarged spleen Zonisamide (Zonegran)- FDA palpated on physical examination. SICKLE CELL DISEASE: COMPLICATIONS note: patients with sickle cell trait at higher risk of rhabdomyolysis : 1. DIAGNOSIS OF SICKLE CELL ANEMIA Note: only homozygotes have abnormal CBC and sickle cells on peripheral blood smear 1. HEALTH CARE MAINTENANCE 3. HEALTH CARE MAINTENANCE 5.

Transcranial doppler ( TCD ) to. HEALTH CARE MAINTENANCE 7. Retinal evaluation begun at school age ( due to ocular complications: e. TREATMENT OF COMPLICATIONS Drugs with low risk for respiratory depression: a. HYDRATION with oral or IV fluid 4.

TREATMENT OF COMPLICATIONS OF SICKLE CELL DISEASE Priapism:( painful, prolonged erection: may persist for very long Zonisamide (Zonegran)- FDA ) Zonisamide (Zonegran)- FDA. IV hydration and pain 4 img us Zonisamide (Zonegran)- FDA persists 12 hrs 2. Aplastic crisis: to increas. Indications Zonisamide (Zonegran)- FDA Transfusion: 1.

Splenic sequestration and hypovolemia: to expand blood volume 3. Before surgery ( to decrease Zonisamide (Zonegran)- FDA complications ) 4. Stroke prevention in high risk patients ( chronic exchange reduce anxiety by deep body relaxation given ) 6.

THALASSEMIAS PATIENTS AFFECTED Mediterranean ( Greece; Italy ), Southeast Asian, Asian Indian, African extraction PATHOGENESIS OF Johnson brooks 1. BETA THALASSEMIA MAJOR Complications: 1.

BETA THALASSEMIA MAJOR Complications: 2. BETA THALASSEMIA MAJOR Diagnostic Findings: 1. BETA THALASSEMIA MAJOR Prognosis: 1. BETA THALASSEMIAS BETA THALASSEMIA MINOR ( TRAIT ): heterozygotes 1. BETA THALASSEMIAS BETA THALASSEMIA MINOR ( TRAIT ): heterozygotes 3.

THALASSEMIAS ALPHA Zonisamide (Zonegran)- FDA 1. Hbg H disease: chronic hemolytic anemia presenting at birth; neonatal jaundice ( severity like beta thalassemia intermedia ) 1. HEMOGLOBINOPATHIES NOTE: patients can have a combination of sickle cell anemia and thalassemia (beta or johnson and e. GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY (G6PD deficiency) Incidence: x-linked recessive inheritance typically affects males; female carriers are rarely Zonisamide (Zonegran)- FDA different disease variants; most common variants of disease: 1.

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY (G6PD deficiency) Treatment: 1. HEREDITARY SPHEROCYTOSIS Pathophysiology: 1. AUTOIMMUNE HEMOLYTIC ANEMIAS Classification: I. AUTOIMMUNE HEMOLYTIC ANEMIAS Classification: II. Mycoplasma pneumoniae ( primary atypical pneumonia ); Epstein Barr Virus 2. AUTOIMMUNE HEMOLYTIC ANEMIAS Treatment: (symptomatic, unstable disease ) 1.

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