Investigation of primary immune deficiency after severe bacterial infection in children: A population-based study in western France.

BACKGROUND: Infectious diseases are still an important cause of morbidity and mortality in high-income countries and may preferentially affect predisposed children, especially immunocompromised children. We aimed to evaluate the frequency of recommended immunological tests in children with community-onset severe bacterial infection (COSBI) admitted to a pediatric intensive care unit. We also assessed the frequency and described the typology of diagnosed primary immune deficiency (PID). METHODS: We conducted a retrospective observational epidemiological study in six university hospitals in western France. All children from 1 month to 16 years of age admitted to hospital for bacterial meningitis, purpura fulminans, or meningococcal disease between August 2009 and January 2014 were included. We analyzed the frequency, type, and results of the immunological tests performed on children with meningitis, purpura fulminans, or a meningococcemia episode. RESULTS: Among the 143 children included (144 episodes), 84 (59%) and 60 (41%) had bacterial meningitis and purpura fulminans or meningococcemia, respectively: 72 (50%) had immunological tests and 8% had a complete immunological investigation as recommended. Among the 72 children examined for PID, 11 (15%) had at least one anomaly in the immunological test results. Two children had a diagnosis of PID (one with C2 deficit and the other with C8 deficit) and seven other children had possible PID. Thus, the prevalence of a definite or possible diagnosis of PID was 12% among the children examined. CONCLUSION: PID is rarely investigated after COSBI. We raise awareness of the need for immunological investigations after a severe infection requiring PICU admission.

[DRESS complicated by hemophagocytic lymphohistiocytosis in an infant treated for congenital toxoplasmosis]. / DRESS compliqué d'un syndrome d'activation macrophagique chez un nourrisson traité pour une toxoplasmose congénitale.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe disease that may be complicated by hemophagocytic lymphohistiocytosis but this is rarely described in children. PATIENTS AND METHODS: We report the case of a 5-week old infant hospitalized in a pediatric intensive care unit for hemophagocytic lymphohistiocytosis with prolonged fever, splenomegaly, cytopenia, fibrinogen≤1.5g/L, ferritin≥500µg/L, and soluble IL-2 receptor≥2400U/mL. As a result of the presence of a diffuse skin rash, eosinophilia and multiple organ failure that started three weeks after the initiation of a congenital toxoplasmosis treatment, association with DRESS was suggested. DISCUSSION: Exposure to sulfadiazine remains the main factor leading to DRESS in this case. This is probably the trigger event, secondarily complicated by hemophagocytic lymphohistiocytosis, although in our case the diagnosis was made subsequently. The unfortunately poor outcome of this association is probably exacerbated in fragile patients such as young infants. CONCLUSION: Clinicians should be aware of the possibility of DRESS of every early onset associated with hemophagocytic lymphohistiocytosis linked to a treatment started during the neonatal period to avoid any delay in care that might adversely affect the prognosis.

[Acute leukemia revealed by bone magnetic resonance imaging: Two pediatric case reports]. / Leucémie aiguë découverte par imagerie par résonance magnétique osseuse : deux observations pédiatriques.

Bone and joint pain are common causes of pediatric consultation for a variety of etiologies. The causes are mostly traumatic or infectious or have an inflammatory origin. Acute leukemia (AL) can sometimes begin with osteoarticular signs and radiological investigations such as magnetic resonance imaging (MRI) can help guide the diagnosis. We report on two cases of pediatric AL revealed by osteoarticular signs, with MRI helpful in the diagnostic procedure. In these children, the first signs were recurrent joint pain, with no blood count anomalies. There was a delay between the onset of arthralgia and diagnosis due to atypical presentation of the disease. MRI guided the investigations to the diagnosis of AL. The persistence of pain or inflammation must lead to considering a bone MRI and a complete blood count must be repeated in case of persistent inflammatory bone pain to avoid a missed diagnosis of blood disease.