Systemic juvenile idiopathic arthritis in French Afro-Caribbean children, a retrospective cohort study.

INTRODUCTION: The epidemiology and clinical presentation of systemic juvenile idiopathic arthritis (sJIA) in the Afro-Caribbean population is not well described. METHODS: Retrospective study conducted between January 2000 and January 2022 in the French Overseas Departments of America. Clinical data were obtained from multiple sources: computerized hospital archives, registries of referring pediatricians, and the French National Registry for rare diseases. The disease studied was sJIA defined according to international criteria. RESULTS: Twenty-five patients were identified. Mean age at diagnosis was 7.5 years (range: 1.2-14.9 years) and mean duration of follow-up was 5.2 years (range: 0.5-16 years). All patients had joint involvement at diagnosis with 68% presenting inflammatory arthritis and 32% inflammatory joint pain. Sixteen percent had coronary involvement at onset. More than half (52%) suffered from macrophage activation syndrome (MAS) during childhood (32% at onset). The mean number of flares in childhood was 2 (Range: 1-5). Sixty-eight percent of patients had disease control during childhood without biotherapy. The most frequent second line treatment was anakinra (7/8). There was no difference in clinical or biological severity according to gender. The median duration of treatment during childhood was 5 months (range: 2-144) and 72% had a cumulative treatment duration of less than one year. CONCLUSION: These patients of Afro-Caribbean origin suffering from sJIA showed some specificities, such as a higher rate of MAS and coronary involvement at onset. The incidence per year was stable over a 20-year period. Overall outcomes during childhood were similar to western countries.

Age-specific characteristics of neutrophilic dermatoses and neutrophilic diseases in children.

BACKGROUND: Our suggested 'modern' concepts of 'neutrophilic dermatoses' (ND) and 'neutrophilic disease' were based on observations in adult patients and have not been studied in paediatric patients. Only a minority of ND occurs in children, and little is known about age-specific characteristics. OBJECTIVES: To describe age-specific characteristics of ND in children and to study whether our suggested 'modern' classification of ND may be applied to children. METHODS: We conducted a retrospective multicentre study in a French cohort of 27 paediatric patients diagnosed with pyoderma gangrenosum (PG) or Sweet's syndrome (SS). RESULTS: Demographics and distribution of typical/atypical forms were similar in patients diagnosed with PG and SS. Atypical ND were more frequent in infants (90%), when compared to young children (60%) and adolescents (33%). Neutrophilic disease was observed in 17/27 patients and was most frequent in infants. Neutrophilic disease of the upper respiratory tract, as well as cardiac neutrophilic disease, was only observed in infants, whereas other locations were similarly found in infants, young children and adolescents. In infants and young children, ND were associated with a large spectrum of general diseases, whereas in adolescents associations were limited to inflammatory bowel disease and Behçet's disease. CONCLUSIONS: Our study describes the concept of ND in paediatric patients and shows that they have some characteristics different from ND occurring in adults. ND occurring in infants can be associated with a large spectrum of general diseases. Occurrence of neutrophilic disease is frequent in children. Thus, ND occurring in young paediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes.

[Streptococcus pneumoniae-induced hemolytic uremic syndrome: a serotype-3-associated case]. / Syndrome hémolytique et urémique secondaire àStreptococcus pneumoniae de sérotype 3.

Hemolytic uremic syndrome (HUS) is the primary cause of acute renal failure in children younger than 3 years of age. It usually occurs after a diarrheal illness due to Shiga-toxin-producing Escherichia coli. Streptococcus pneumoniae (SP)-induced HUS remains rare, involving 5% of all cases of HUS in children, but its frequency has increased over the last decade. The incidence of HUS following invasive pneumococcal infections is estimated at 0.4 to 0.6%. We report here the case of a 3.5-year-old child who presented SP serotype-3-associated HUS. The diagnosis was suspected by the patient's multiple organ failure. The pathogenesis involves the activation of the Thomsen-Friedenreich antigen. To prevent transfusion-associated hemolysis, it is recommended that fresh-frozen plasma or unwashed blood products should be avoided when possible. Our patient was transfused with 4 units of unwashed red blood cell and 2 units of fresh-frozen plasma. No special complication was noted. The risk of immediate complications requires close clinical and biological monitoring, and the possibility of starting dialysis immediately. Twenty-five to 35% of SP-HUS patients exhibit long-term renal aftereffects. The acute mortality rate depends on the site of infection. The increased frequency of SP-HUS may be related to the new ecology of serotypes created by widespread Prevenar7(®) vaccination.

Prospective and descriptive study of adult dengue cases in an emergency department, in Martinique.

OBJECTIVE: Knowing about the clinical aspects of dengue in endemic zones is essential to implementation of appropriate case management protocols and public health interventions. PATIENTS AND METHODS: The authors made a 4-year prospective, observational study of dengue-infected patients admitted to the emergency department of the Fort-de-France University Hospital. RESULTS: Two hundred and sixty-three male and 297 female patients were included. The median age was 37 years (range: 14-91). The diagnosis was based on a positive RT-PCR (463 patients) or on the presence of specific IgM (97 patients). Two hundred and seventy-seven patients (49.5%) presented with dengue fever without complications. According to WHO criteria, 95 patients (17%) developed plasma leakage, including 39 patients (7%) diagnosed with DHF, and 10 (1.8%) diagnosed with DSS. Among the other patients without plasma leakage, 84 (15%) had isolated thrombocytopenia, 14 (2.5%) had internal bleeding, and 90 (16%) had unusual manifestations. Seven patients died (1.3%): fulminant hepatitis (two), myocarditis (one), encephalitis (one), acute respiratory failure (one), gangrenous cholecystitis (one), and post-traumatic intracranial hemorrhage (one). The other patients recovered. Seven patients were pregnant (1.3%) from 6 to 27 weeks of amenorrhea and carried their pregnancy to term without complications. CONCLUSION: With this experience, we were able to develop appropriate case management protocols for patients during dengue epidemics.

Congenital leukemia cutis preceding monoblastic leukemia by 3 months.

We report an infant born with a cutaneous nodular eruption and neutropenia. Skin biopsy specimens revealed an immature dermal infiltrate suggestive of leukemia cutis, but repeated peripheral blood and bone marrow examinations failed to demonstrate malignant cells. The eruption resolved spontaneously. At the age of 3 months, a second occurrence of maculopapular skin lesions led to discovery of an acute monoblastic leukemia with (9;11)(p21-22;q23) translocation. Congenital acute leukemia is a rare disease associated with skin infiltration in 25% to 30% of patients. Usually the diagnosis is easily made by peripheral blood examination and/or bone marrow aspirate. However, skin involvement may precede acute leukemia by several weeks. Although very rare, this event must be kept in mind.