266
Brief clinical and laboratory observations
prothrombin levels from 2 to 10% o f normal have been reported to have epistaxis, bruising, mcnorrhagia, hematuria, and bleeding after surge D' and dental extractions. Despite the severe deficiency of prothrombin and marked prolongation of the prothrombin time in our patient, she has had only mild bleeding to date. lter activity has not been restricted. One episode of oral bleeding was managed with topical thrombin and epsilon-aminocaproic acid (Amicar), a treatment which has been used successfully by us for similar bleeding episodes in children with Factor VIII or Factor IX deficiency. Prothrombin has a biologic half-life of approximately 50 to 80 hours '~ and is present in both stored and freshfrozen plasma. The exact level necessary for hemoslasis is not known but is probably 10 to 15% for minor hemorrhages and 20 to 4070 for major trauma or surgery." Thus effective treatment is possible using plasma. The prothrombin complex concentrates, which also contain Factors Vll, IX, and X, have an increased risk of transmitting hepatitis compared to plasma and have been associated with thrombotic episodes, particularly in patients with liver disease. We feel that bleeding in congenital hypoprothrombinemia should be treated with plasma, unless this therapy is ineffective. Although prothrombin is present in stored plasma, the use of freshfrozen plasma will ensure the delivery of adequate amounts of active prothrombin. ADDENDUM A recent report describes a 31-year-old Chicano man with bleeding of moderate severity and biologic and immunologic levels of prothrombin similar to those in our patient (Montgomery RR, Otsaku A, and llathaway WE: Hypoprothrombinemia: Case report, Blood 51:299, 1978).
The Journal of Pediatrics August 1978
The authors thank Ms. Susannah McCord, Marge DeSipin, and Grace Terry for their expert technical assistance. REFERENCES !. Shapiro SS, Martinez J, and Ilolburn RR: Congenital dysprothrombinemia: An inherited structural disorder of human prothrombin, J Clin Invest 48:2251, 1969. 2. Kattlove HE, Shapiro SS, and Spivack M: Hereditary prothrombin deficiency, N Engl J Med 282:57, 1970. 3. Girolami A: The hereditary transmission of congenital "true" hypoprothrombinaemia, Br J Haematol 21:695, 1971. 4. Baudo F, de Cataldo F, Josso F, and Silvello L: Hereditary hypoprothrombinaemia, Acta Haematol 47:243, 1972. 5. Pina-Cabral JM, and Benvindo J: Congenital hypoprothrombinemia in a Portuguese family, Thromb Diath Haemorrh 30:451, 1973. 6. Josso F, Monasterio de Sanchez J, Lavergne JM, Menache D, and Soulier JP: Congenital abnormality of the prothrombin molecule (factor Ii) in four siblings: Prothrombin Barcelona, Blood 38:9, 1971. 7. Shapiro SS, Maldonado M, Fradera J and McCord S: Prothrombin San Juan: A complex new dysprothrombihernia, J Clin Invest 53:73a, 1974. 8. Girolami A, Bareggi G, Brunetti A, and Sticchi A: Prothrombin Padua: A "new" congenital dysprothrombinemia, J Lab Clin Med 84:654, 1974. 9. Kahn MJP, and Govaerts A: Prothrombin Brussels: A new congenital defective protein, Thromb Res 5:i41, 1974. 10. Shapiro SS and Martinez J: Ituman prothrombin metabolism in normal man and in hypocoagulable subjects, J Clin Invest 48:!292, 1969. I1. Johnson AJ, Aronson DL, and Williams WJ: P~'eparation and clinical use of plasma and plasma fractions, in Williams WJ, Beutler E, Erslev AJ, and Rundles RW, editors: ltematology, ed 2, New York, 1977, McGraw-llill Book Co., Inc., pp 1561-1582.
Urinar), catecholamines and metabolites in children Andr~ F. De Schaepdr~'ver, M.D.,* Carlos Hooft, M.D., Marie-Jeanne Delbeke, M.D., and Mare Van"den Noortgaete, M.D., Ghent, Belgium
DATA on the urinary excretion of catecholamines and metabolites by healthy children according to age are From the He)'mans lnstitttte of Phartnacology and the Pediatric Clinic, Ghent University Medical School. Aided b)' a grant from the National Fund for Medical Research Belgium (No. 3.0092. 74/20.499). *Reprint address: lle)'mans Institute of Pharmacolog)'. University of Ghent Medical School, De Pintelaan 135, B.9000 Ghent, Belgium
scarce and incomplete.'-' The present paper sets out the values of urinary catecholamines and metabolites found in essentially normal children from one month to 16 years of age. METHODS One-hundred and four children of both sexes, hospitalized for mild diseases, were studied while at bed rest. Twenty-four-hour urine specimens were collected and 0022-3476/78/0293-0266S00.30/0 9 1978 The C. V. Mosby Co.
Voh,ne 93 Number 2
Brief cfinical and laboratory observatimu
267
Table I. T w e n t y - f o u r - h o u r urinary excretion o f c a t e c h o l a m i n e s a n d m e t a b o l i t e s (~ -4- SD) in n o r m a l children according to age a n d surface a r e a
Age*
Brief clinical and laboratory observations
prothrombin levels from 2 to 10% o f normal have been reported to have epistaxis, bruising, mcnorrhagia, hematuria, and bleeding after surge D' and dental extractions. Despite the severe deficiency of prothrombin and marked prolongation of the prothrombin time in our patient, she has had only mild bleeding to date. lter activity has not been restricted. One episode of oral bleeding was managed with topical thrombin and epsilon-aminocaproic acid (Amicar), a treatment which has been used successfully by us for similar bleeding episodes in children with Factor VIII or Factor IX deficiency. Prothrombin has a biologic half-life of approximately 50 to 80 hours '~ and is present in both stored and freshfrozen plasma. The exact level necessary for hemoslasis is not known but is probably 10 to 15% for minor hemorrhages and 20 to 4070 for major trauma or surgery." Thus effective treatment is possible using plasma. The prothrombin complex concentrates, which also contain Factors Vll, IX, and X, have an increased risk of transmitting hepatitis compared to plasma and have been associated with thrombotic episodes, particularly in patients with liver disease. We feel that bleeding in congenital hypoprothrombinemia should be treated with plasma, unless this therapy is ineffective. Although prothrombin is present in stored plasma, the use of freshfrozen plasma will ensure the delivery of adequate amounts of active prothrombin. ADDENDUM A recent report describes a 31-year-old Chicano man with bleeding of moderate severity and biologic and immunologic levels of prothrombin similar to those in our patient (Montgomery RR, Otsaku A, and llathaway WE: Hypoprothrombinemia: Case report, Blood 51:299, 1978).
The Journal of Pediatrics August 1978
The authors thank Ms. Susannah McCord, Marge DeSipin, and Grace Terry for their expert technical assistance. REFERENCES !. Shapiro SS, Martinez J, and Ilolburn RR: Congenital dysprothrombinemia: An inherited structural disorder of human prothrombin, J Clin Invest 48:2251, 1969. 2. Kattlove HE, Shapiro SS, and Spivack M: Hereditary prothrombin deficiency, N Engl J Med 282:57, 1970. 3. Girolami A: The hereditary transmission of congenital "true" hypoprothrombinaemia, Br J Haematol 21:695, 1971. 4. Baudo F, de Cataldo F, Josso F, and Silvello L: Hereditary hypoprothrombinaemia, Acta Haematol 47:243, 1972. 5. Pina-Cabral JM, and Benvindo J: Congenital hypoprothrombinemia in a Portuguese family, Thromb Diath Haemorrh 30:451, 1973. 6. Josso F, Monasterio de Sanchez J, Lavergne JM, Menache D, and Soulier JP: Congenital abnormality of the prothrombin molecule (factor Ii) in four siblings: Prothrombin Barcelona, Blood 38:9, 1971. 7. Shapiro SS, Maldonado M, Fradera J and McCord S: Prothrombin San Juan: A complex new dysprothrombihernia, J Clin Invest 53:73a, 1974. 8. Girolami A, Bareggi G, Brunetti A, and Sticchi A: Prothrombin Padua: A "new" congenital dysprothrombinemia, J Lab Clin Med 84:654, 1974. 9. Kahn MJP, and Govaerts A: Prothrombin Brussels: A new congenital defective protein, Thromb Res 5:i41, 1974. 10. Shapiro SS and Martinez J: Ituman prothrombin metabolism in normal man and in hypocoagulable subjects, J Clin Invest 48:!292, 1969. I1. Johnson AJ, Aronson DL, and Williams WJ: P~'eparation and clinical use of plasma and plasma fractions, in Williams WJ, Beutler E, Erslev AJ, and Rundles RW, editors: ltematology, ed 2, New York, 1977, McGraw-llill Book Co., Inc., pp 1561-1582.
Urinar), catecholamines and metabolites in children Andr~ F. De Schaepdr~'ver, M.D.,* Carlos Hooft, M.D., Marie-Jeanne Delbeke, M.D., and Mare Van"den Noortgaete, M.D., Ghent, Belgium
DATA on the urinary excretion of catecholamines and metabolites by healthy children according to age are From the He)'mans lnstitttte of Phartnacology and the Pediatric Clinic, Ghent University Medical School. Aided b)' a grant from the National Fund for Medical Research Belgium (No. 3.0092. 74/20.499). *Reprint address: lle)'mans Institute of Pharmacolog)'. University of Ghent Medical School, De Pintelaan 135, B.9000 Ghent, Belgium
scarce and incomplete.'-' The present paper sets out the values of urinary catecholamines and metabolites found in essentially normal children from one month to 16 years of age. METHODS One-hundred and four children of both sexes, hospitalized for mild diseases, were studied while at bed rest. Twenty-four-hour urine specimens were collected and 0022-3476/78/0293-0266S00.30/0 9 1978 The C. V. Mosby Co.
Voh,ne 93 Number 2
Brief cfinical and laboratory observatimu
267
Table I. T w e n t y - f o u r - h o u r urinary excretion o f c a t e c h o l a m i n e s a n d m e t a b o l i t e s (~ -4- SD) in n o r m a l children according to age a n d surface a r e a
Age*
