Sickle cell disease (SCD) refers to any one of the syndromes in which the sickle mutation is co-inherited with a mutation at the other beta globin allele that reduces or abolishes normal beta globin production. These include sickle cell anemia (homozygous sickle mutation), sickle-beta thalassemia, hemoglobin SC disease, and others.

hemolytic anemia and vaso-occlusion

individuals with sickle cell anemia (homozygous Hb S) and sickle-beta0-thalassemia have more severe manifestations

HbSS and HBS𝝱0 have a more severe phenotype

HPLC (i.e. High Performance Liquid Chromatography, hemoglobin fracture, or hemoglobin electrophoresis) can quantify the percentage of each hemoglobin variant.

Vaccinations

transcranial Doppler (TCD) ultrasonography identifies children with SCD who are at high risk for stroke.

extended analysis of the STOP trial further confirmed the reliability of TCD in identifying children with SCD at high risk for stroke and the efficacy of transfusion in reducing this risk. All 6 stroke events in the posttrial follow-up were predicted by abnormal TCD results, regardless of treatment status. In contrast, none of the patients with normal and conditional TCD results on follow-up developed stroke.

The only indicator of the risk of reversion to abnormal TCD results or stroke was the baseline TCD result, similar to our data from STOP

cardiovascular events or significant cardiovascular risk, and compared cardiac outcomes (specifically nonfatal MI, nonfatal CVA, or cardiovascular death) when treated to usual care (A1C of 7.0-7.9%) or intensive treatment (A1C of <6.0%). After 3.5 years, the study was prematurely terminated because of higher all-cause and higher cardiovascular death in the intensive treatment group.

with type 1 diabetes; lowering the A1C below 7% doubles the risk of death.

severe hypoglycemia had increased risk of major macrovascular and microvascular events, as well as a greater risk of death

medical nutrition therapy', or MNT) can reduce A1C levels by as much as 2% in adherent patients.

Counseling by a nutritionist is recommended for all patients with diabetes.

kept up to date on tetanus boosters, influenza vaccination, hepatitis B vaccination and pneumococcal vaccination.

LDL 125

Liver enzymes should be checked when T2DM is diagnosed, to screen for fatty liver disease.

The ADA recommends low-dose aspirin for the primary prevention of coronary artery disease in patients with type 2 diabetes who have increased cardiovascular risk (i.e., 10-year risk of MI of >10%

patients with T2DM who have glucose, lipids, blood pressure, tobacco, and albuminuria controlled do not have excess mortality

Options for evaluating a patient's glucose

FPG >126mg/dl and symptoms of diabetes can be diagnosed with diabetes.

cirrhosis severity scoring system

associated with increasing severity of hepatic dysfunction and increasing three-month mortality risk

Patients awaiting liver transplantation are ranked according to their MELD-Na score and stratified by blood type.

differ clinically according to the presence of ketoacidosis and, usually, the degree of hyperglycemia

DKA, metabolic acidosis is often the major finding, while the serum glucose concentration is generally below 800 mg/dL (44.4 mmol/L) and often in the 350 to 500 mg/dL

euglycemic DKA