Abstract
Serial coagulation investigations were performed in 4 children with the haemolytic uraemic syndrome treated with heparin by continuous infusion. 2 anuric patients showed consumption of factor V and fibrinogen early in the disease, with thrombocytopenia and raised fibrin degradation products. These changes regressed during heparin therapy and renal function fully recovered in both patients. A third patient with a mild form of the disease, normal urinary output, and only borderline thrombocytopenia did not develop demonstrable depletion of factor V or fibrinogen. In a further patient a secondary `wave' of consumption of platelets and perhaps fibrinogen was seen late in the course of the disease. These findings confirmed the occurrence of a consumptive coagulopathy in severe cases of haemolytic uraemic syndrome.
Full text
PDFSelected References
These references are in PubMed. This may not be the complete list of references from this article.
- ALKJAERSIG N., FLETCHER A. P., SHERRY S. xi-Aminocaproic acid: an inhibitor of plasminogen activation. J Biol Chem. 1959 Apr;234(4):832–837. [PubMed] [Google Scholar]
- Avalos J. S., Vitacco M., Molinas F., Peñalver J., Gianantonio C. Coagulation studies in the hemolytic-uremic syndrome. J Pediatr. 1970 Apr;76(4):538–548. doi: 10.1016/s0022-3476(70)80403-6. [DOI] [PubMed] [Google Scholar]
- BRAIN M. C., DACIE J. V., HOURIHANE D. O. Microangiopathic haemolytic anaemia: the possible role of vascular lesions in pathogenesis. Br J Haematol. 1962 Oct;8:358–374. doi: 10.1111/j.1365-2141.1962.tb06541.x. [DOI] [PubMed] [Google Scholar]
- BRECHER G., CRONKITE E. P. Morphology and enumeration of human blood platelets. J Appl Physiol. 1950 Dec;3(6):365–377. doi: 10.1152/jappl.1950.3.6.365. [DOI] [PubMed] [Google Scholar]
- Baker L. R., Rubenberg M. L., Dacie J. V., Brain M. C. Fibrinogen catabolism in microangiopathic haemolytic anaemia. Br J Haematol. 1968 Jun;14(6):617–625. doi: 10.1111/j.1365-2141.1968.tb00368.x. [DOI] [PubMed] [Google Scholar]
- Brain M. C., Baker L. R., McBride J. A., Rubenberg M. L., Dacie J. V. Treatment of patients with microangiopathic haemolytic anaemia with heparin. Br J Haematol. 1968 Dec;15(6):603–621. doi: 10.1111/j.1365-2141.1968.tb01582.x. [DOI] [PubMed] [Google Scholar]
- Brain M. C., Hourihane D. O. Microangiopathic haemolytic anaemia: the occurrence of haemolysis in experimentally produced vascular disease. Br J Haematol. 1967 Jan;13(1):135–142. doi: 10.1111/j.1365-2141.1967.tb08702.x. [DOI] [PubMed] [Google Scholar]
- Brain M. C. The haemolytic-uraemic syndrome. Semin Hematol. 1969 Apr;6(2):162–180. [PubMed] [Google Scholar]
- Bull B. S., Rubenberg M. L., Dacie J. V., Brain M. C. Microangiopathic haemolytic anaemia: mechanisms of red-cell fragmentation: in vitro studies. Br J Haematol. 1968 Jun;14(6):643–652. doi: 10.1111/j.1365-2141.1968.tb00370.x. [DOI] [PubMed] [Google Scholar]
- Corrigan J. J., Jr, Ray W. L., May N. Changes in the blood coagulation system associated with septicemia. N Engl J Med. 1968 Oct 17;279(16):851–856. doi: 10.1056/NEJM196810172791603. [DOI] [PubMed] [Google Scholar]
- Deykin D. The clinical challenge of disseminated intravascular coagulation. N Engl J Med. 1970 Sep 17;283(12):636–644. doi: 10.1056/NEJM197009172831207. [DOI] [PubMed] [Google Scholar]
- Gilchrist G. S., Lieberman E., Ekert H., Fine R. N., Grushkin C. Heparin therapy in the haemolytic-uraemic syndrome. Lancet. 1969 Jun 7;1(7606):1123–1126. doi: 10.1016/s0140-6736(69)91644-4. [DOI] [PubMed] [Google Scholar]
- HARDISTY R. M., MACPHERSON J. C. A one-stage factor VIII (antihaemophilic globulin) assay and its use on venous and capillary plasma. Thromb Diath Haemorrh. 1962 May 15;7:215–228. [PubMed] [Google Scholar]
- Hammond D., Lieberman E. The hemolytic uremic syndrome. Renal cortical thrombotic microangiopathy. Arch Intern Med. 1970 Nov;126(5):816–822. [PubMed] [Google Scholar]
- Hathaway W. E. Care of the critically ill child: the problem of disseminated intravascular coagulation. Pediatrics. 1970 Nov;46(5):767–773. [PubMed] [Google Scholar]
- Hitzig W. H., Straub P. W., Lo S. S., Frick P. G. Disseminated intravascular coagulation. Clinical experience with anticoagulant therapy in the management of disseminated intravascular coagulation in children. Proc R Soc Med. 1968 Nov;61(11 Pt 1):1138–1138. [PMC free article] [PubMed] [Google Scholar]
- Katz J., Lurie A., Kaplan B. S., Krawitz S., Metz J. Coagulation findings in the hemolytic-uremic syndrome of infancy: similarity to hyperacute renal allograft rejection. J Pediatr. 1971 Mar;78(3):426–434. doi: 10.1016/s0022-3476(71)80222-6. [DOI] [PubMed] [Google Scholar]
- Lanzkowsky P., McCrory W. Disseminated intravascular coagulation as a possible factor in the pathogenesis of thrombotic microangiopathy (hemolytic-uremic syndrome). J Pediatr. 1967 Mar;70(3):460–462. doi: 10.1016/s0022-3476(67)80157-4. [DOI] [PubMed] [Google Scholar]
- Merskey C., Kleiner G. J., Johnson A. J. Quantitative estimation of split products of fibrinogen in human serum, relation to diagnosis and treatment. Blood. 1966 Jul;28(1):1–18. [PubMed] [Google Scholar]
- Moncrieff M. W., Glasgow E. F. Haemolytic-uraemic syndrome treated with heparin. Br Med J. 1970 Jul 25;3(5716):188–191. doi: 10.1136/bmj.3.5716.188. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Monnens L., Schretlen E. Intravascular coagulation in an infant with the hemolytic-uremic syndrome. Acta Paediatr Scand. 1967 Jul;56(4):436–441. doi: 10.1111/j.1651-2227.1967.tb15403.x. [DOI] [PubMed] [Google Scholar]
- Rubenberg M. L., Regoeczi E., Bull B. S., Dacie J. V., Brain M. C. Microangiopathic haemolytic anaemia: the experimental production of haemolysis and red-cell fragmentation by defibrination in vivo. Br J Haematol. 1968 Jun;14(6):627–642. doi: 10.1111/j.1365-2141.1968.tb00369.x. [DOI] [PubMed] [Google Scholar]
- STEFANINI M. New one-stage procedures for the quantitative determination of prothrombin and labile factor. Am J Clin Pathol. 1950 Mar;20(3):233–240. doi: 10.1093/ajcp/20.3.233. [DOI] [PubMed] [Google Scholar]
- Sharpstone P., Evans R. G., O'Shea M., Alexander L., Lee H. A. Haemolytic-uraemic syndrome: survival after prolonged oliguria. Arch Dis Child. 1968 Dec;43(232):711–716. doi: 10.1136/adc.43.232.711. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Uttley W. S. Serum levels of fibrin-fibrinogen degradation products in the haemolytic-uraemic syndrome. Arch Dis Child. 1970 Aug;45(242):587–589. doi: 10.1136/adc.45.242.587. [DOI] [PMC free article] [PubMed] [Google Scholar]