Fibrinogen Lima. A new dysfibrinogenaemia with a high-molecular-weight alpha-chain and effective polymerization.
A patient with transient microhaematuria was studied. Coagulation tests revealed prolonged thrombin and reptilase times concomitant with abnormal fibrin polymerization rate (also abnormal in both parents). In the patient and her patients, the abnormal fibrin polymerization rate was only slightly corrected by addition of calcium ions. The alpha-chain had a molecular weight higher than normal and there was deficient formation of alpha-polymers. The molecule showed a more anodal migration than the control. The abnormality described has been classified as Fibrinogen Lima.
Arocha-Piñango
C L
CL
Instituto Venezolano de Investigaciones Científicas, Caracas, Venezuela.
Rodriguez
S
S
Nagy
H
H
Perez Requejo
J L
JL
D000347:Afibrinogenemia / Q000097:blood / Q000150:complications / Q000235:genetics*; D001797:Blood Protein Electrophoresis; D002118:Calcium / Q000494:pharmacology; D002648:Child; D003241:Consanguinity; D005260:Female; D005340:Fibrinogen / Q000235:genetics; D015241:Fibrinogens, Abnormal / Q000235:genetics / Q000302:isolation & purification* / Q000378:metabolism; D006417:Hematuria / Q000209:etiology*; D006801:Humans; D010446:Peptide Fragments / Q000235:genetics; D011108:Polymers; D013918:Thrombin Time
D015241:Fibrinogens, Abnormal; D010446:Peptide Fragments; D011108:Polymers; C072592:fibrinogen Lima; D005340:Fibrinogen; D002118:Calcium