[Heterozygous protein C deficiency: apropos of 2 cases with cerebral venous thrombosis in the neonatal period]. / Déficit hétérozygote en protéine C: à propos de deux cas avec thromboses veineuses cérébrales en période néonatale.
Arch Pediatr
; 7(2): 158-62, 2000 Feb.
Article
de Fr
| MEDLINE
| ID: mdl-10701061
ABSTRACT
UNLABELLED Thrombotic accidents in the newborn, particularly cerebrovascular accidents, are reported in case of abnormalities in the coagulation system and rarely in heterozygous protein C deficiency; a low protein C level could be either physiological or acquired. CASE REPORT Two cases of heterozygous protein C deficiency are reported in neonates. Severe neurologic distress was associated with bloody cerebrospinal fluid, and hemorrhagic lesions due to cerebral sinovenous occlusion were visualised by cerebral imaging. The course was severe. One case was associated with renal thrombosis. Mutation in the 168 proline/leucine was detected by molecular biology in the neonates and their mothers. In one case a treatment with protein C had no beneficial effect. CONCLUSION:
Cerebral sinus venous thrombosis has to be sought by magnetic resonance imaging in the case of neurologic distress with profound cerebral hemorrhage in the newborn. A low level of protein C has to be interpreted with caution. The diagnosis of a heterozygous deficiency status can only be made through molecular biology. The effect of treatment with protein C concentrate is questionable.
Recherche sur Google
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Veines de l'encéphale
/
Déficit en protéine C
/
Thrombose intracrânienne
Type d'étude:
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limites:
Humans
/
Male
/
Newborn
Langue:
Fr
Journal:
Arch Pediatr
Année:
2000
Type de document:
Article
Pays d'affiliation:
France