Acute osteomyelitis in congenital hypofibrinogenemia
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《印度儿科学杂志》
Abstract
A 10-month-old female child presented with intermittent high grade fever, pain and diffuse swelling in the left knee joint with history of ecchymosis in different areas of the body. There was radiological features of acute osteomyelitis, low fibrinogen level and bacteremia due to the presence of coagulase positive staphylococcus aureus. The child responded nicely to the treatment for acute osteomyelitis and congenital hypofibrinogenemia. So, in congenital hypofibrinogenemia, a joint swelling might be a resultant of an acute osteomyelitis, not mere hemarthrosis.
Keywords: Congenital hypofibrinogenemia; Acute osteomyelitis; Hemarthrosis
How to cite this article:
Datta (Kanjilal) S, Bagchi S. Acute osteomyelitis in congenital hypofibrinogenemia. Indian J Pediatr 2006;73:529-530
How to cite this URL:
Datta (Kanjilal) S, Bagchi S. Acute osteomyelitis in congenital hypofibrinogenemia. Indian J Pediatr [serial online] 2006 [cited 2006 Jul 14];73:529-530. Available from: http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2006;volume=73;issue=6;spage=529;epage=530;aulast=Datta
In congenital hypofibrinogenemia blood does not clot normally due to a quantitative lack in the fibrinogen level since birth. It's a rare disease, which got first reported in 1935; and since then only 40 cases have been reported[1]. It usually presents as minor bleeding episodes, gastrointestinal, mucosal, post-surgical, post-truamatic bleeding in children. Osteomyelitis occurs mainly hematogenously in children and it's frequency is greater in infants and toddlers[2]. The present paper reports a case of congenital hypofibrinogenemia with acute osteomyelitis in an infant.
Case Report
A 10-month-old female child [Figure - 1] presented with complaints of intermittent high grade fever, pain and a diffuse swelling in the left knee joint for the last 10 days. There was history of ecchymosis, which first appeared spontaneously on the forehead when she was 2-month-old; and recurrences occurred in different areas of the body, like neck, back, hands etc. There was no history suggestive of purpuric spots, hemoptysis, hematemesis, melena, bleeding from umbilical stump or any other site. There was no history of trauma, massive blood transfusion, jaundice and contact with tuberculosis. There was no suggestive family history or history of intake of hypofibrinogenemia inducing drugs like coumarins, indandions, cholestyramine, sulphonamides or broad-spectrum antibiotics. General survey was normal excepting that the pulse rate was 140/min and temperature was 101o F. The maximum diameter of right knee joint was 18 cm, whereas that of the left knee joint was 24 cm. The left knee joint was red, hot, tender with all movements restricted, there was no bony abnormality elsewhere in the body. Systemic examination revealed no abnormality.
Considering 3 consecutive investigations, there was normal hemoglobin level(11.2gm%), a high white cell count (29,000/cu.mm, P-78%, L-20%, M-1%, E-1%, B-0)), raised ESR (32mm 1st hr), raised C-reactive protein level (9gm/l). In the peripheral smear there was no evidence of hemolysis or microangiopathy. The platelet count was 3,61,000/cu.mm. Bleeding time (Ivy) was 3 min 30 sec (normal- 0.5-6.0 min). The prothrombin time was 90.8 sec (control-14 sec) and APTT was 117.4 sec (control-27 sec). Thrombin time was 52 sec (control-10 sec). Serum fibrinogen level (44 mg/dl) was low (normal- 170-405mg/dl). Assays on von Willebrand Factor (120%) and other coagulation factors revealed nothing significant. Fibrin degradation product was negative. Liver function tests were normal. Blood culture and sensitivity revealed that there was bacteremia due to coagulase positive strain of Staphylococcus aureus sensitive to ampicillin. Skiagrams of left knee joint revealed features of acute osteomyelitis [Figure - 2]. There was an increase in the joint space probably due to effusion. The lower metaphysis of femur showed features of periosteal reaction and there was soft tissue swelling around the joint. USG of left knee joint showed collection of fluid with echogenic area. CT scan brain and USG of liver were normal.
Apart from providing the child with hepatitis-B vaccine, intravenous antibiotic (for 4 weeks) and symptomatic treatment, we also started treating her with cryoprecipitate, at a quantity of 2 bags on the first day followed by 1 bag on alternate days. The child gradually improved after administration of 6 units of cryoprecipitate. Her fibrinogen level was raised to 100 mg/dl, with prothrombin time 44.2 sec and APTT 62.8 sec. Blood reports after 4 weeks showed decline in white cell count, C-reactive protein and ESR to normal level. The joint swelling also subsided after 4 weeks. The case was diagnosed as congenital hypofibrinogenemia with acute osteomyelitis of left knee joint.
Discussion
The history, clinical examinations and investigations made it evident that there was increase of PT, APTT and thrombin time which indicated that it might be either due to hypo/afibrinogenemia, dysfibrinogenemia, liver disease or disseminated intravascular coagulation. But low level of fibrinogen indicated that it was a case of congenital hypofibrinogenemia. It had not been a resultant of any acquired cause as there was no evidence of liver disorders or hepatoma, any drug-induced hypofibrinogenemia, disseminated intravascular coagulation, massive blood transfusion or any systemic diseases like leukemia, congenital heart disease, nephrotic syndrome or Gaucher's disease. But the age and the manifestations of hypofibrinogenemia in this case had strongly stamped it as congenital hypo/afibrinogenemia. Prolonged bleeding from umbilical stump may occur in the neonatal period[3] and a significant percentage may experience undue bleeding following birth trauma[4] in congenital hypo/afibrinogenemia which was absent in
this case. Recurrent spontaneously occurring ecchymotic spots are usually found[5] as in our case.
In a child of coagulation disorder, a swollen joint area always compels a pediatrician to ponder on hemarthrosis which occurs in 20% cases of congenital hypo/afibrinogenemia.[6] But in case of congenital hypofibrinogenemia, as this case suggests, a joint swelling might be a resultant of an acute osteomyelitis, not mere the hemarthrosis. Superficial skin infection by the bacteria like Staphylococcus may cause bacteremia. Due to the unique anatomy of the ends of long bones, which has a predilection for localization of blood borne bacteria and due to the presence of tiny vascular loops in the metaphysis of long bones, which has sluggish blood flow and low oxygen tension- the bacteria starts multiplying there.[2] In hemarthrosis, the accumulated blood might act as a good culture medium for the bacteria like Staphylococcus . The rupture of tiny loops causes proliferation of bacteria resulting acute osteomyelitis. However, in this case, needle aspiration from joint space was not attempted as it was a bleeding disorder, but the investigation results favored the diagnosis of acute osteomyelitis of left knee joint. Thus the case could be stamped as acute osteomyelitis in congenital hypofibrinogenemia and the disease showed excellent response to medical treatment. So a joint swelling in any patient with bleeding diathesis should not always be considered as hemarthrosis, but other causes of joint swelling should be considered and patients need appropriate investigation.
References
1. Rodgers GM, Greenberg CS. Inherited coagulation disorders. In: Wintrobe's Clinical Hematology. Lee GR, Foerster J, Lukens J, Paraskevas F, Green JP and Rodgers GM eds.10th ed. Baltimore, Williams and Wilkins; 1999. p. 1682-1732.
2. Nelson JD. Osteomyelitis and suppurative arthritis. In Nelson's Text book of Pediatrics. Behrman ER, Kliegman RM, Jenson HB eds.16th ed. Singapore. Harcourt Asia LTD. 2000. P.776-780.
3. Kuhle S, Mitchell L, Massicotte P and Andrew M. Hemostatic Disorders of the Newborn. In: Avery's Diseases of the Newborn . Taeusch HW, Ballard RA, Gleason CA eds. 8th ed. Philadelphia. Saunders. 2005. p.1145-1179.
4. AJ Mondhiry H,Ehman WC. Congenital afibrinogenemia. Am J Hematol 1994, 46: 343-347.
5. Awasthy N, Aggarwal KC, Gupta H and Saluja S. Congenital hypofibrinogenemia. In Indian Pediatrics 2004, 41 : 185-187.
6. Mamman EF. Fibrinogen abnormalities. Semin Thromb Hemot, 1988, 9: 1-6.(Datta (Kanjilal) Sumana, )
A 10-month-old female child presented with intermittent high grade fever, pain and diffuse swelling in the left knee joint with history of ecchymosis in different areas of the body. There was radiological features of acute osteomyelitis, low fibrinogen level and bacteremia due to the presence of coagulase positive staphylococcus aureus. The child responded nicely to the treatment for acute osteomyelitis and congenital hypofibrinogenemia. So, in congenital hypofibrinogenemia, a joint swelling might be a resultant of an acute osteomyelitis, not mere hemarthrosis.
Keywords: Congenital hypofibrinogenemia; Acute osteomyelitis; Hemarthrosis
How to cite this article:
Datta (Kanjilal) S, Bagchi S. Acute osteomyelitis in congenital hypofibrinogenemia. Indian J Pediatr 2006;73:529-530
How to cite this URL:
Datta (Kanjilal) S, Bagchi S. Acute osteomyelitis in congenital hypofibrinogenemia. Indian J Pediatr [serial online] 2006 [cited 2006 Jul 14];73:529-530. Available from: http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2006;volume=73;issue=6;spage=529;epage=530;aulast=Datta
In congenital hypofibrinogenemia blood does not clot normally due to a quantitative lack in the fibrinogen level since birth. It's a rare disease, which got first reported in 1935; and since then only 40 cases have been reported[1]. It usually presents as minor bleeding episodes, gastrointestinal, mucosal, post-surgical, post-truamatic bleeding in children. Osteomyelitis occurs mainly hematogenously in children and it's frequency is greater in infants and toddlers[2]. The present paper reports a case of congenital hypofibrinogenemia with acute osteomyelitis in an infant.
Case Report
A 10-month-old female child [Figure - 1] presented with complaints of intermittent high grade fever, pain and a diffuse swelling in the left knee joint for the last 10 days. There was history of ecchymosis, which first appeared spontaneously on the forehead when she was 2-month-old; and recurrences occurred in different areas of the body, like neck, back, hands etc. There was no history suggestive of purpuric spots, hemoptysis, hematemesis, melena, bleeding from umbilical stump or any other site. There was no history of trauma, massive blood transfusion, jaundice and contact with tuberculosis. There was no suggestive family history or history of intake of hypofibrinogenemia inducing drugs like coumarins, indandions, cholestyramine, sulphonamides or broad-spectrum antibiotics. General survey was normal excepting that the pulse rate was 140/min and temperature was 101o F. The maximum diameter of right knee joint was 18 cm, whereas that of the left knee joint was 24 cm. The left knee joint was red, hot, tender with all movements restricted, there was no bony abnormality elsewhere in the body. Systemic examination revealed no abnormality.
Considering 3 consecutive investigations, there was normal hemoglobin level(11.2gm%), a high white cell count (29,000/cu.mm, P-78%, L-20%, M-1%, E-1%, B-0)), raised ESR (32mm 1st hr), raised C-reactive protein level (9gm/l). In the peripheral smear there was no evidence of hemolysis or microangiopathy. The platelet count was 3,61,000/cu.mm. Bleeding time (Ivy) was 3 min 30 sec (normal- 0.5-6.0 min). The prothrombin time was 90.8 sec (control-14 sec) and APTT was 117.4 sec (control-27 sec). Thrombin time was 52 sec (control-10 sec). Serum fibrinogen level (44 mg/dl) was low (normal- 170-405mg/dl). Assays on von Willebrand Factor (120%) and other coagulation factors revealed nothing significant. Fibrin degradation product was negative. Liver function tests were normal. Blood culture and sensitivity revealed that there was bacteremia due to coagulase positive strain of Staphylococcus aureus sensitive to ampicillin. Skiagrams of left knee joint revealed features of acute osteomyelitis [Figure - 2]. There was an increase in the joint space probably due to effusion. The lower metaphysis of femur showed features of periosteal reaction and there was soft tissue swelling around the joint. USG of left knee joint showed collection of fluid with echogenic area. CT scan brain and USG of liver were normal.
Apart from providing the child with hepatitis-B vaccine, intravenous antibiotic (for 4 weeks) and symptomatic treatment, we also started treating her with cryoprecipitate, at a quantity of 2 bags on the first day followed by 1 bag on alternate days. The child gradually improved after administration of 6 units of cryoprecipitate. Her fibrinogen level was raised to 100 mg/dl, with prothrombin time 44.2 sec and APTT 62.8 sec. Blood reports after 4 weeks showed decline in white cell count, C-reactive protein and ESR to normal level. The joint swelling also subsided after 4 weeks. The case was diagnosed as congenital hypofibrinogenemia with acute osteomyelitis of left knee joint.
Discussion
The history, clinical examinations and investigations made it evident that there was increase of PT, APTT and thrombin time which indicated that it might be either due to hypo/afibrinogenemia, dysfibrinogenemia, liver disease or disseminated intravascular coagulation. But low level of fibrinogen indicated that it was a case of congenital hypofibrinogenemia. It had not been a resultant of any acquired cause as there was no evidence of liver disorders or hepatoma, any drug-induced hypofibrinogenemia, disseminated intravascular coagulation, massive blood transfusion or any systemic diseases like leukemia, congenital heart disease, nephrotic syndrome or Gaucher's disease. But the age and the manifestations of hypofibrinogenemia in this case had strongly stamped it as congenital hypo/afibrinogenemia. Prolonged bleeding from umbilical stump may occur in the neonatal period[3] and a significant percentage may experience undue bleeding following birth trauma[4] in congenital hypo/afibrinogenemia which was absent in
this case. Recurrent spontaneously occurring ecchymotic spots are usually found[5] as in our case.
In a child of coagulation disorder, a swollen joint area always compels a pediatrician to ponder on hemarthrosis which occurs in 20% cases of congenital hypo/afibrinogenemia.[6] But in case of congenital hypofibrinogenemia, as this case suggests, a joint swelling might be a resultant of an acute osteomyelitis, not mere the hemarthrosis. Superficial skin infection by the bacteria like Staphylococcus may cause bacteremia. Due to the unique anatomy of the ends of long bones, which has a predilection for localization of blood borne bacteria and due to the presence of tiny vascular loops in the metaphysis of long bones, which has sluggish blood flow and low oxygen tension- the bacteria starts multiplying there.[2] In hemarthrosis, the accumulated blood might act as a good culture medium for the bacteria like Staphylococcus . The rupture of tiny loops causes proliferation of bacteria resulting acute osteomyelitis. However, in this case, needle aspiration from joint space was not attempted as it was a bleeding disorder, but the investigation results favored the diagnosis of acute osteomyelitis of left knee joint. Thus the case could be stamped as acute osteomyelitis in congenital hypofibrinogenemia and the disease showed excellent response to medical treatment. So a joint swelling in any patient with bleeding diathesis should not always be considered as hemarthrosis, but other causes of joint swelling should be considered and patients need appropriate investigation.
References
1. Rodgers GM, Greenberg CS. Inherited coagulation disorders. In: Wintrobe's Clinical Hematology. Lee GR, Foerster J, Lukens J, Paraskevas F, Green JP and Rodgers GM eds.10th ed. Baltimore, Williams and Wilkins; 1999. p. 1682-1732.
2. Nelson JD. Osteomyelitis and suppurative arthritis. In Nelson's Text book of Pediatrics. Behrman ER, Kliegman RM, Jenson HB eds.16th ed. Singapore. Harcourt Asia LTD. 2000. P.776-780.
3. Kuhle S, Mitchell L, Massicotte P and Andrew M. Hemostatic Disorders of the Newborn. In: Avery's Diseases of the Newborn . Taeusch HW, Ballard RA, Gleason CA eds. 8th ed. Philadelphia. Saunders. 2005. p.1145-1179.
4. AJ Mondhiry H,Ehman WC. Congenital afibrinogenemia. Am J Hematol 1994, 46: 343-347.
5. Awasthy N, Aggarwal KC, Gupta H and Saluja S. Congenital hypofibrinogenemia. In Indian Pediatrics 2004, 41 : 185-187.
6. Mamman EF. Fibrinogen abnormalities. Semin Thromb Hemot, 1988, 9: 1-6.(Datta (Kanjilal) Sumana, )