Biomaterial Database

Clinical presentation of sickle cell-hemoglobin C disease. 1986

S Williams, and G H Maude, and G R Serjeant

Early symptoms were observed in a representative sample of 166 children with sickle cell-hemoglobin C disease diagnosed at birth. Symptoms were uncommon in the first year of life; in approximately 50% specific symptoms had developed by 5 years, but 22% remained without specific symptoms to 10 years. The age at presentation was significantly earlier in patients with low hemoglobin F levels, but was not influenced by heterozygous alpha-thalassemia-2. Painful crisis was the initial manifestation in 77% of the children; other symptoms included dactylitis (14%) and pneumococcal septicemia and acute splenic sequestration (4% each). The commonest nonspecific symptom was acute chest syndrome. The relatively mild early clinical course of sickle cell-hemoglobin C disease indicates that neonatal diagnosis does not have the same urgency as for homozygous sickle cell disease.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007238	Infarction	Formation of an infarct, which is NECROSIS in tissue due to local ISCHEMIA resulting from obstruction of BLOOD CIRCULATION, most commonly by a THROMBUS or EMBOLUS.	Infarct,Infarctions,Infarcts
D010020	Osteonecrosis	Death of a bone or part of a bone, either atraumatic or posttraumatic.	Aseptic Necrosis of Bone,Avascular Necrosis of Bone,Kienbock Disease,Necrosis, Aseptic, of Bone,Necrosis, Avascular, of Bone,Bone Necrosis,Kienbock's Disease,Kienboeck Disease,Kienboeck's Disease,Bone Aseptic Necrosis,Bone Avascular Necrosis,Bone Necroses,Kienboecks Disease,Necroses, Bone,Necrosis, Bone,Osteonecroses
D010146	Pain	An unpleasant sensation induced by noxious stimuli which are detected by NERVE ENDINGS of NOCICEPTIVE NEURONS.	Suffering, Physical,Ache,Pain, Burning,Pain, Crushing,Pain, Migratory,Pain, Radiating,Pain, Splitting,Aches,Burning Pain,Burning Pains,Crushing Pain,Crushing Pains,Migratory Pain,Migratory Pains,Pains, Burning,Pains, Crushing,Pains, Migratory,Pains, Radiating,Pains, Splitting,Physical Suffering,Physical Sufferings,Radiating Pain,Radiating Pains,Splitting Pain,Splitting Pains,Sufferings, Physical
D011018	Pneumonia, Pneumococcal	A febrile disease caused by STREPTOCOCCUS PNEUMONIAE.	Pneumococcal Pneumonia,Pneumococcal Pneumonias,Pneumonias, Pneumococcal
D001853	Bone Marrow	The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.	Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D006450	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.	SC Disease,Sickle Cell Hemoglobin C Disease,Disease, Hemoglobin SC,Disease, SC,Diseases, Hemoglobin SC,Diseases, SC,Hemoglobin SC Diseases,SC Disease, Hemoglobin,SC Diseases,SC Diseases, Hemoglobin