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Intervenciones para el tratamiento de la colestasis intrahepática en pacientes con anemia de células falciformes

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Appendices

Appendix 1. Glossary of medical terms

Term

Definition

Source

Acute hepatic injury

Acute hepatic injury can be recognized by the presence of jaundice or non‐specific symptoms of acute illness accompanied
by elevation of AST or ALT (or both) activities.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf).

Albumin

Albumin is the most abundant plasma protein produced by hepatocytes. Rate of production is dependent on several factors, including supply of amino acids, plasma oncotic pressure, levels of inhibitory cytokines (particularly IL‐6), and number of functioning hepatocytes.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Alanine aminotransferase

(ALT)

Aspartate aminotransferase (AST, also sometimes termed serum glutamic oxaloacetic transaminase (SGOT)) and alanine aminotransferase (ALT, also sometimes
termed serum glutamic pyruvate transaminase (SGPT)) are widely distributed in cells throughout the body. AST is found primarily in heart, liver, skeletal muscle,
and kidney, while ALT is found primarily in liver and kidney, with lesser amounts in heart and skeletal muscle

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Alkaline phosphatase

Alkaline phosphatase (ALP), involved in metabolite transport across cell membranes, is found, in decreasing order of abundance, in placenta, ileal mucosa, kidney, bone, and liver. Bone, liver, and kidney alkaline phosphatase share a common protein structure, coded for by the same gene.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf).

Anemia, sickle cell

A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.

MeSH (Medical Subject Headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).

Aspartate aminotransferase (AST)

Aspartate aminotransferase (AST, also sometimes termed SGOT) and alanine aminotransferase (ALT, also sometimes termed SGPT) are widely distributed in cells throughout the body. AST is found primarily in heart, liver, skeletal muscle, and kidney, while ALT is found primarily in liver and kidney, with lesser amounts in heart and skeletal muscle.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Bilirubin

Daily production of unconjugated bilirubin is 250 to 350 mg, mainly from senescent erythrocytes (65). Clearance at normal values is 5 mg/kg/day, or about 400 mg/day in adults; the rate does not increase significantly with hemolysis (66). The half‐life of unconjugated bilirubin is < 5 minutes (67). UDP‐glucuronyltransferase catalyzes rapid conjugation of bilirubin in the liver; conjugated bilirubin is excreted into bile and is essentially absent from blood in normal individuals. Delta bilirubin (‐bilirubin, also sometimes termed biliprotein) is produced by reaction of conjugated bilirubin with albumin (68); it has a half‐life of about 17 ‐ 20 days (the same as albumin), accounting for prolonged jaundice in patients recovering from hepatitis or obstruction.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Chronic hepatic injury

Chronic hepatic injury is a relatively common disorder with minimal symptoms, yet with long term risk of significant morbidity and mortality. It is defined pathologically by ongoing hepatic necrosis and inflammation in the liver, often accompanied by fibrosis.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Exchange transfusion, whole blood

Repetitive withdrawal of small amounts of blood and replacement with donor blood until a large proportion of the blood volume has been exchanged.

MeSH (Medical Subject Headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).

Gamma‐glutamyl transferase

Gamma‐glutamyltransferase (GGT), a membrane bound, enzyme, is present in decreasing order of abundance in proximal renal tubule, liver, pancreas (ductules and acinar cells), and intestine. GGT activity in serum comes primarily from liver. The half‐life of GGT in humans is about seven to 10 days; in alcohol‐associated liver injury, the half‐life increases to as much as 28 days, suggesting impaired clearance.

NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf)

Hemoglobin SC disease

One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia.

 MeSH (Medical Subject Headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).

Hemoglobin, sickle

It is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain.

 Ballas 2012

Hyperbilirubinemia

A condition characterized by an abnormal increase of bilirubin in the blood, which may result in jaundice. Bilirubin, a breakdown product of heme, is normally excreted in the bile or further catabolized before excretion in the urine.

MeSH (Medical Subject Headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).

Jaundice

A clinical manifestation of hyperbilirubinemia, characterized by the yellowish staining of the skin; mucous membrane; and sclera. Clinical jaundice usually is a sign of liver dysfunction.

MeSH (Medical Subject Headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).

Prothrombin time

Prothrombin time (PT) measures the time required for plasma to clot after addition of tissue factor and phospholipid; it is affected by changes in the activity of factors X, VII, V, II (prothrombin) and I (fibrinogen). All of these factors are synthesized in the liver, and three (II, VII, and X) are activated by a vitamin K‐dependent enzyme through addition of a second, ‐carboxyl group on glutamic acid residues.

 NACB 2000 (http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/Hepatic.pdf).

Sickle cell trait

The condition of being heterozygous for hemoglobin S.

 MeSH (medical subject headings). National Library of Medicine (http://www.ncbi.nlm.nih.gov/mesh).