Epidemiological and prognostic aspects of purpura fulminans in children. Regional survey

A regional survey (Rh?ne-Alpes region) about fulminans purpura has been performed in the pediatric services and the pediatric intensive care units. Between January 1982 and July 1986, 101 cases have been observed. 93.5% (72/77) of the bacteriologically documented cases were due to Neisseria meningitidis. Fatality rate is 34.6%, 58.6% in infants younger than 1 year to 14.3% in children older than 2 years. Between 1982 and 1985 fatality rate was reduced from 54.5% to 27.6%. This study confirms the interest of the severity score established by the French Pediatric Intensive Care Club.     

Topical nitroglycerin and pain in purpura fulminans

Topical nitroglycerin has been previously described as an adjunctive therapy to increase perfusion to areas of purpura affected in purpura fulminans. We report a case of purpura fulminans in which topical nitroglycerin was found to provide analgesia after its application to purpuric lesions. The broader role for the use of topical nitroglycerin in pain management deserves further study and evaluation.     

Severe and fatal forms of COVID-19 in children

ObjectivesThe aim of this study was to describe severe forms of novel coronavirus disease 2019 in children, including patient characteristics, clinical, laboratory, and imaging findings, as well as the disease management and outcomes.MethodsThis was a retrospective, single-center, observational study conducted in a pediatric intensive and high-dependency care unit (PICU, HDU) in an urban hospital in Paris. All patients, aged from 1 month to 18 years, admitted for confirmed or highly suspected SARS-CoV-2 were included.ResultsWe analyzed the data of 27 children. Comorbidities (n = 19, 70%) were mainly neurological (n = 7), respiratory, (n = 4), or sickle cell disease (n = 4). SARS-CoV-2 PCR results were positive in 24 children (nasopharyngeal swabs). The three remaining children had a chest CT scan consistent with COVID-19. Respiratory involvement was observed in 24 patients (89%). Supportive treatments were invasive mechanical ventilation (n = 9), catecholamine (n = 4), erythropheresis (n = 4), renal replacement therapy (n = 1), and extracorporeal membrane oxygenation (n = 1). Five children died, of whom three were without past medical history.ConclusionThis study highlighted the large spectrum of clinical presentation and time course of disease progression as well as the non-negligible occurrence of pediatric life-threatening and fatal cases of COVID-19 mostly in patients with comorbidities. Additional laboratory investigations are needed to further analyze the mechanism underlying the variability of SARS-Cov-2 pathogenicity in children.     

Diagnosis and management of neonatal purpura fulminans

Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated. The clinical presentation is that of acute disseminated intravascular coagulation and hemorrhagic skin necrosis. The management includes an acute phase of replacement therapy with fresh frozen plasma or protein C concentrate and a maintenance therapy that includes anticoagulation with warfarin or low molecular weight heparin. This review focuses on the management of severe protein C deficiency.     

Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans

Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. Administration of recombinant human activated protein C (rhAPC) has been successfully applied in adults with MPF and pediatric studies are under way. We report three pediatric patients with MPF treated with rhAPC as compassionate therapy. In two of these patients, positive clinical and laboratory effects were observed and both children achieved full recovery. The remaining patient died after 36 hours from refractory multiorgan failure. No rhAPC-related adverse effects were detected. The reported cases highlight the usefulness of rhAPC in children with MPF at least as a rescue compassionate treatment. Further clinical trials are needed to better delineate its efficacy and administration schedule in children.     

Grading of hemorrhage in children with idiopathic thrombocytopenic purpura

OBJECTIVE: To develop an instrument to allow semiquantitative assessment of hemorrhage in children with idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: Bleeding severity was graded on a scale of 0 to 4 in 4 different sites (overall, oral, epistaxis, and skin) on the basis of history during the previous 24 hours and physical examination. RESULTS: Children with ITP (n = 54) were assessed on 109 different occasions by multiple observers, including 81 measurements by one of the authors. Grade of bleeding correlated inversely with platelet count. Grade 3 or 4 hemorrhage was infrequently encountered except involving the skin, where assessment was difficult. Grade 4 mucosal or internal hemorrhage was noted in 7 patients; none had life-threatening or fatal bleeding. Interrater agreement in grading of overall and mouth bleeding and epistaxis was acceptable. CONCLUSIONS: We conclude that scoring of hemorrhage is possible in children with ITP and that the grade of hemorrhage may represent a clinically meaningful end point in future studies.     

A rare association between group A Streptococcus purpura fulminans and diffuse alveolar hemorrhage in a young girl: A case report

We report the case of a 7-year-old girl with septic shock and coagulopathy associated with purpura fulminans (PF) and diffuse alveolar hemorrhage (DAH) due to group A Streptococcus (GAS) infection identified with 16S ribosomal RNA analysis performed on the skin biopsy. GAS infection with PF associated with DAH is rare in healthy young children but pediatricians should be aware of this condition because of the poor prognosis. The initial treatment for circulatory failure and severe disseminated intravascular coagulation as well as the prompt initiation of antibiotic treatment may be crucial for the outcomes of S. pyogenes PF.