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Outcome in children with purpura fulminans: report on 16 patients
Aytemiz Gürgey1, Selin Aytac, Guler Kanra
1Hacettepe University, Faculty of Medicine, Department of Pediatrics, Ankara, Turkey. agurgey@hacettepe.edu.tr
Insights
Purpura fulminans (PF) in young children is linked to severe infections, factor V G1691A mutation, and congenital heart disease, often requiring amputation. Early treatment improves survival rates, suggesting PF may not be a lethal disease.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Purpura fulminans (PF) is a severe, acute condition with high mortality, often linked to infections in children.
- Other underlying conditions may also contribute to PF development.
Purpose of the Study:
- To investigate underlying disorders and associated conditions in pediatric PF.
- To analyze the outcomes and risk factors for PF in children.
Main Methods:
- Retrospective analysis of 16 children (3.5 months to 12 years) diagnosed with PF.
- Evaluation of associated conditions, genetic mutations (Factor V G1691A), protein C/S deficiencies, and treatment outcomes.
- Assessment of amputation rates and survival.
Main Results:
- Children aged 4 years or younger (81%) were predominantly affected.
- Factor V G1691A mutation (46%) and protein deficiencies (Protein C: 37.5%, Protein S: 56%) were common.
- Amputation was required in 69% of younger children and 33% of older children, particularly those with Factor V mutation and severe infections.
Conclusions:
- Age under 4, Factor V G1691A mutation, and congenital heart disease are risk factors for PF in severe infections.
- The study suggests PF is treatable with improved survival rates, challenging its previously lethal perception.
- Changes in the etiological profile of PF may be influenced by immunization and healthcare advancements.
Abstract:
Purpura fulminans (PF) is a severe disorder of acute onset with high morbidity and mortality. In children, this rapidly progressive illness is usually associated with severe bacterial or viral infections. However, some other conditions may participate in the development of PF. Our objective was to investigate the underlying and associated disorders and the outcomes of the disease in 16 children, 7 males and 9 females ranging in age from 3.5 months to 12 years (median age, 2 years). Thirteen of the 16 children (81%) were 4 years of age or younger. The remaining 3 patients were 9, 10, and 12 years of age. Among these 13 infants and small children, 7 (43%) had infection, 2 infants had congenital cardiac disorders necessitating minor or major surgical intervention, and 1 infant and 3 children had different miscellaneous disorders. The factor V G1691A mutation was present in six of the 13 small children (46%). None of the 3 older children carried the mutation. Six (37.5%) of the 16 patients had protein C deficiencies, and 9 (56%) had protein S deficiencies. These deficiencies, except one for protein S, were acquired. Ten patients except two who were diagnosed at this center were treated with fresh frozen plasma. They were also given heparin. Nine (69%) of the 13 children 4 years of age or younger and one of the older children (33%) required amputation. Five of the six patients (83%) who had factor V G1691A mutation, and who also exhibited severe infection, required amputation. This study suggests that an age of 4 years or less is a risk factor for the development of PF during severe infections, especially in the presence of factor V G1691A mutation and congenital heart disease, necessitating major or minor surgical interventions. This study also shows that the amputation rate in 10 patients, after excluding the patients who had been referred to our center after development of sequelae, was 60%. The survival rate among these 10 patients may indicate that, with the treatment protocol, PF need not be regarded as a lethal disease any more. It is also suggested that effective immunization programs and better health care have probably resulted in some changes in the etiological profile of PF.
