Afibrinogenemia With Intracerebral Hematoma

Montgomery, Robert A.; Natelson, Stephen E.

doi:10.1001/archpedi.1977.02120180069012

Cited by 9 publications

(2 citation statements)

References 9 publications

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“…The pattern of clinical presentation of afibrinogenemia found in this large Iranian series is similar to that stemming from the analysis of single cases or small series of patients (14-17). Central nervous system bleeding (16,17) or recurrent abortions (14, 15) could be observed in only three cases in the Iranian series.…”

Section: Clinical Manifestationsmentioning

confidence: 84%

Rare Coagulation Disorders

Peyvandi¹,

Pm²

1999

Thromb Haemost

184

176

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SummaryThe type of hemorrhagic manifestations that occur in patients with recessively transmitted coagulation disorders and their optimal treatment are not well established as for hemophilia A and B and von Willebrand disease, due to the rarity of these disorders. In a Muslim country like Iran where consanguineous marriages are frequent these disorders are less rare. We chose to evaluate the pattern of bleeding symptoms in 237 Iranian patients with the inherited deficiencies of fibrinogen, factor II, combined factor V and factor VIII, factor V, factor VII and factor X. Considering “severe” life-endangering hemorrhages such as those in the CNS, gastrointestinal tract and from the umbilical cord and those potentially handicapping such as hematomas and hemarthroses; and “mild” epistaxis, menorrhagia, hematuria, oral and postsurgical bleeding, it would appear the most severe diseases are factor X and factor II deficiencies. For the remaining defects only a minority of patients, even those with unmeasurable plasma levels, had life-endangering hemorrhages or muscoloskeletal disabilities as a consequence of hemarthroses and hematomas. The relatively mild severity of clinical manifestations in recessive coagulation disorders commands safety as the primary criterion in the choice of replacement material for treatment. Hence, virally inactivated plasma and factor concentrates should be the products of choice.

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Section: Clinical Manifestationsmentioning

confidence: 84%

Rare Coagulation Disorders

Peyvandi¹,

Pm²

1999

Thromb Haemost

184

176

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“…During spinal surgical procedures, especially complex procedures, blood coagulation disorders should be considered preoperatively during the preparation of the patient for the operation. Once a blood coagulation disorder has been diagnosed in the preoperative stages and after necessary measures have been taken, lifethreatening complications can be avoided and successful surgical treat-ments can be performed (2). Herein, we describe the diagnosis of postoperative fibrinogen deficiency in a patient who was taken into surgery for lumbar degenerative disc disease.…”

Section: Introductionmentioning

confidence: 99%

The Perioperative Importance of Congenital Fibrinogen Deficiency in Spinal Surgery Procedure

Kaner¹,

Özer

Sasani

et al. 2010

ELECTRON J GEN MED

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Demonstration of a double hereditary pattern for congenital afibrinogenemia

Girolami

Cappellato

Falezza

et al. 1981

Blut

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Six patients with congenital afibrinogenemia belonging to four kindreds were studied with regard to hereditary pattern. In two families the hereditary pattern appeared to be autosomal recessive; in the two other pedigrees, on the contrary, the pattern seems autosomal intermediate. In the first type, all family members, excluding the patients, showed normal fibrinogen levels; in the second type, family members could be divided into two groups: normal and heterozygotes. The heterozygotes had fibrinogen levels of 192 +/- 30 mg/dl, definitely lower than that of a normal control population. The average level of the normal relatives was 361 +/- 81.9 mg/dl, practically identical to that of a normal control group unrelated to the homozygotes. In the past these differences were thought to be secondary to variances in fibrinogen assays from one laboratory to the other. It now appears that they are real ones since they can be observed in the same laboratory using the same fibrinogen technique. It must be concluded that congenital afibrinogenemia shows two patterns of hereditary transmission, one autosomal recessive and the other autosomal intermediate.

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Afibrinogenemia With Intracerebral Hematoma

Cited by 9 publications

References 9 publications

Rare Coagulation Disorders

Rare Coagulation Disorders

The Perioperative Importance of Congenital Fibrinogen Deficiency in Spinal Surgery Procedure

Demonstration of a double hereditary pattern for congenital afibrinogenemia

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