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A bleeding syndrome in infants due to acquired prothrombin complex deficiency: a survey of 93 affected infants
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Severe prothrombin complex deficiency caused a bleeding syndrome in 93 infants, with high rates of intracranial hemorrhage. Vitamin K therapy led to rapid improvement, highlighting its crucial role in treating this condition.
Area of Science:
- Pediatric Hematology
- Nutritional Deficiencies
- Neonatal Medicine
Background:
- A bleeding syndrome was observed in 93 infants, primarily breast-fed and aged 2 weeks to 1 year.
- Major manifestations included hemorrhagic diathesis, pallor, and mild hepatomegaly, with no significant preceding illnesses.
Purpose of the Study:
- To report on a bleeding syndrome in infants caused by severe prothrombin complex deficiency.
- To analyze the clinical manifestations, pathogenesis, and outcomes of this condition.
Main Methods:
- Retrospective case series analysis of 93 infants with severe prothrombin complex deficiency.
- Clinical data collection on manifestations, laboratory findings, treatment, and outcomes.
- Evaluation of vitamin K deficiency as the primary cause.
Main Results:
- High incidence of intracranial bleeding (63%), including subdural and subarachnoid hemorrhage.
- Rapid clinical and laboratory improvement was noted following vitamin K therapy.
- Mortality rate decreased from 35% to 17% after 1969, indicating improved management.
Conclusions:
- Severe prothrombin complex deficiency, likely due to vitamin K deficiency, is the cause of this bleeding syndrome in infants.
- Prompt diagnosis and early vitamin K treatment are critical for improving prognosis.
- The exact causes of vitamin K deficiency in this cohort remain undetermined.
Abstract:
A bleeding syndrome due to severe prothrombin complex deficiency is reported in 93 infants. Most were breast fed (98 per cent), aged 2 weeks to 1 year and there were no serious preceding or associated diseases. Hemorrhagic diathesis, pallor and mild hepatomegaly were the major manifestations. The incidence of intracr anial bleeding was strikingly high (63 per cent) particularly with subdural and subarachnoid hemorrhage. Acute onset, short course and rapid clinical and laboratory improvement after vitamin K therapy were observed. Mortality rate was 35 per cent but has been reduced to 17 per cent since 1969. The location of bleeding, prompt diagnosis and early treatment are the major factors affecting prognosis. Severe prothrombin complex deficiency due to vitamin K deficiency accounted for the pathogenesis of bleeding. Possible causes of vitamin K deficiency were discussed but definite conclusions could not be drawn.