Table 1.

NOHARM AE definitions

Definition
Clinical AE terminology  
 Vaso-occlusive pain crisis/dactylitis Vaso-occlusive pain crisis: acute pain and tenderness in an area of the body, with or without swelling, with no other diagnostic explanation. 
Dactylitis: vaso-occlusive crisis (acute pain, tenderness, and swelling) localized to hands or feet. 
 Pneumonia/acute chest syndrome Pneumonia: history of fever or measured axillary temperature ≥37.5°C, with tachypnea and cough. 
Acute chest syndrome: signs of pneumonia plus chest pain and/or tenderness. 
 Clinical sepsis Measured fever and ill appearance, requiring IV antibiotics. 
 Acute splenic sequestration Increase in splenic size from last physical examination, accompanied by a decrease in hemoglobin of ≥2 g/dL. 
 Upper respiratory infection Child with general well appearance with rhinorrhea, nasal congestion, or cough. 
 Gastrointestinal related Diarrhea, vomiting, constipated, intestinal obstruction. 
 Malaria Measured fever (axillary temperature ≥37.5°C) or fever by history and Plasmodium species infection on blood smear. 
 Other infection Other infections, diagnosed clinically. 
 Other (eg, injury) Other diseases not included above diagnosed during visits for illness. 
Laboratory AEs*  
 Anemia Hemoglobin <6 g/dL 
 Reticulocytopenia ARC <80 × 109/L and hemoglobin <7 g/dL 
 Neutropenia Absolute neutrophil count (ANC) <1.0 × 109/L 
 Thrombocytopenia Platelet count <80 × 109/L 
 Elevated AST/ALT AST >150 IU/L, ALT >150 IU/L 
 Elevated bilirubin Total bilirubin >5 mg/dL 
Definition
Clinical AE terminology  
 Vaso-occlusive pain crisis/dactylitis Vaso-occlusive pain crisis: acute pain and tenderness in an area of the body, with or without swelling, with no other diagnostic explanation. 
Dactylitis: vaso-occlusive crisis (acute pain, tenderness, and swelling) localized to hands or feet. 
 Pneumonia/acute chest syndrome Pneumonia: history of fever or measured axillary temperature ≥37.5°C, with tachypnea and cough. 
Acute chest syndrome: signs of pneumonia plus chest pain and/or tenderness. 
 Clinical sepsis Measured fever and ill appearance, requiring IV antibiotics. 
 Acute splenic sequestration Increase in splenic size from last physical examination, accompanied by a decrease in hemoglobin of ≥2 g/dL. 
 Upper respiratory infection Child with general well appearance with rhinorrhea, nasal congestion, or cough. 
 Gastrointestinal related Diarrhea, vomiting, constipated, intestinal obstruction. 
 Malaria Measured fever (axillary temperature ≥37.5°C) or fever by history and Plasmodium species infection on blood smear. 
 Other infection Other infections, diagnosed clinically. 
 Other (eg, injury) Other diseases not included above diagnosed during visits for illness. 
Laboratory AEs*  
 Anemia Hemoglobin <6 g/dL 
 Reticulocytopenia ARC <80 × 109/L and hemoglobin <7 g/dL 
 Neutropenia Absolute neutrophil count (ANC) <1.0 × 109/L 
 Thrombocytopenia Platelet count <80 × 109/L 
 Elevated AST/ALT AST >150 IU/L, ALT >150 IU/L 
 Elevated bilirubin Total bilirubin >5 mg/dL 

The diagnosis of sickle-related clinical events followed published definitions,24  with modifications such as pneumonia and clinical sepsis.

ALT, alanine transferase; AST, aspartate transferase.

*

Laboratory AE definitions represent the values necessary for a grade 2 event.

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