Maki Hirose

Dengue fever as a cause of hemophagocytic lymphohistiocytosis

Dengue is endemic in more than 100 countries in Southeast Asia, the Americas, the western Pacific, Africa and the eastern Mediterranean regions. The virus is transmitted by Aedes mosquitoes. Dengue disease is the most prevalent arthropod-borne viral disease in humans and is a global and national public health concern in several countries. A seasonal pattern of dengue disease is consistently observed. The highest incidences usually correspond to the period of highest rainfall and humidity, providing suitable conditions for Aedes aegypti breeding and survival. In Brazil for instance it is from January to June. Dengue may cause marked changes in bone marrow that result in hypocellularity and, consequently, thrombocytopenia and leucopenia, along with an increase in hematocrit, which is secondary to capillary leakage. However, those abnormalities are usually self-limited, and do not warrant further investigations, such as a marrow biopsy or a myelogram. The occurrence of persistent reactive hemophagocytosis is uncommon and usually leads to serious adverse outcomes. The authors report the case of an 8-year old girl complaining of high-grade fever, malaise, headache, abdominal pain and a cutaneous rash. Laboratory examination revealed atypical lymphocytosis on peripheral blood count, hyperbilirrunemia, abnormal liver enzymes and clotting tests. Serology was positive for dengue. Because of the persistence of fever and laboratory examinations were consistent with hemophagocytic lymphohistiocytosis (HLH) a bone marrow aspiration was performed, which confirmed the presence of hemophagocytosis. Hence we report a rare presentation of dengue accompanied by self-limited HLH that hopefully evolve to favorable outcome.

Acute Hemorrhagic Edema of Infancy: an unusual diagnosis for the general pediatrician

Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis, clinically characterized by the classical triad: palpable purpuric skin lesions, edema and fever, and is commonly misdiagnosed as Henoch-Schönlein purpura. In addition to its sudden onset, AHEI is also characterized by its self-limited course with complete and spontaneous recovery occurring between 1 and 3 weeks. Because of the scarcity of studies on therapy with corticosteroids, the conservative approach is usually recommended. The authors report an unusual case of an one-year-old boy who presented with typical cutaneous rash of AHEI and orchitis, the latter showing complete resolution after less than 24 hours of prednisolone therapy. The authors call attention to this entity mainly as a differential diagnosis of Henoch-Schönlein purpura and to the importance of new studies to establish the benefits of corticosteroid therapy for AHEI.

Otomastoiditis caused by Sphingomonas paucimobilis: case report and literature review

Sphingomonas paucimobilis is an aerobic Gram-negative bacillus that, although rare in humans, most commonly infects immunocompromised and hospitalized patients. Among the 59 pediatric cases of S. paucimobilis infection reported in the literature, the most common diagnosis involves isolated bacteremia. These cases are related to sporadic or epidemic infections. Death related to this infection occurred in only one case. The authors report a case of an 11-year-old boy with the diagnosis of Sphingomonas paucimobilis otomastoiditis and a thorough review of the literature on this infection in pediatrics. The patient presented a 20-day history of fever, otalgia, otorrhea, and progressive retroauricular swelling with protrusion of the left ear; despite 15 days of amoxicillin regimen. His past medical history included chronic bilateral otitis media, but no cause of immunosuppression was found. A brain computed tomography scan showed left otomastoiditis associated with a large circumscribed fluid collection with deep involvement of the soft tissues of the temporal region, including the subperiosteal space. Blood tests showed neutrophilia and elevated C-reactive protein. Surgical manipulation of the cited collection drained a large amount of a fetid purulent secretion. Ceftazidime and clindamycin were empirically initiated. The outcome was favorable, with fever defervescence and resolution of the scalp deformation. Culture of the drained secretion was positive for S. paucimobilis. Ciprofloxacin was scheduled for a further 10 days after discharge. The follow-up showed complete recovery. As far as we know, this is the first case of S. paucimobilis otomastoiditis, complicated with subperiosteal abscess in an immunocompetent child. The authors call attention to the increasing number of reports on S. paucimobilis infection over the years, and therefore to the importance of this pathogen, which was previously underestimated.

PO-0200 Sphingomonas Paucimobilis: A Cause Of Otomastoiditis Complicated With Subperiosteal Abscess In An Immunocompetent Child

We present the first case of Sphingomonas paucimobilis otomastoiditis, complicated with subperiosteal abscess in an immunocompetent child. Case Report A 11-year-old boy with previous diagnosis of chronic bilateral otitis media presented with 20 days of fever, otalgia, otorrhea, progressive retroauricular swelling with protrusion of the left ear and worsening of the symptoms despite 15 days of Amoxicilin. A CT scan was performed (Figure 1). Blood tests showed WBC 22,4 × 109 (neutrophils 81,2%) and CRP of 142. Surgical drainage of the abscess found a large amount of purulent foetid secretion. We empirically initiated Ceftazidime and Clindamycin. He remained well, with defervescence and resolution of the scalp deformation. Control test showed WBC 7,4 × 109 (neutrophils 58,7%) and CRP of 6. Culture of the abscess grew Sphingomonas paucimobilis. The patient was discharged with Cyprofloxacin and returned after 1 month in our clinic recovered. Discussion S. paucimobilis is an aerobic gram-negative bacillus that rarely infects humans, most commonly immunocompromised and hospitalised patients. We searched the literature for S. Paucimobilis infections in children and found 47 reported cases (Table 1). The most common diagnosis is isolated bacteremia and there are no previous report of otomastoiditis. It is related to sporadic or epidemic infections, leading to just one published case of death in children. S. paucimobilis infection has been increasingly reported over the years and it’s a more important pathogen than previously thought. Abstract PO-0200 Figure 1 CT scan showing left otomastoiditis (yellow arrow) associated with a large collection exterior to the temporal bone (red arrow) Abstract PO-0200 Table 1 Clinical characteristics of paediatric cases of Sphingomonas paucimobilis infection in our literature review

Doença de Kawasaki: experiência clínica em hospital universitário

OBJETIVO: A doenca de Kawasaki e uma vasculite sistemica aguda de etiologia desconhecida. Seu diagnostico baseia-se em criterios clinicos. O objetivo deste estudo foi descrever os casos de pacientes com doenca de Kawasaki internados no Hospital Universitario da Universidade de Sao Paulo entre janeiro/2000 e junho/2008. METODOS: Dentre todos os pacientes internados na Enfermaria de Pediatria no periodo acima, foram selecionados aqueles cujo CID de alta foi doenca de Kawasaki. Realizou-se estudo descritivo por meio da analise dos prontuarios dessas criancas. RESULTADOS: Foram encontrados 18 casos. A media de internacoes foi de 2,1 casos/ano. A idade variou de tres meses a nove anos. A proporcao meninos:meninas foi 1:1,25. Receberam outros diagnosticos previos 17 pacientes, sendo escarlatina em 2/3 dos casos. O tempo de febre antes do diagnostico variou de cinco a 11 dias. Nove criancas apresentaram quatro sinais sugestivos de doenca de Kawasaki; oito apresentaram cinco sinais e uma apresentou dois sinais, o que foi considerado doenca de Kawasaki incompleta. Receberam gamaglobulina 15 criancas (entre o sexto e o decimo dias de evolucao) e 11 (73%) ficaram afebris apos infusao da medicacao. Os demais tiveram febre ate 24 horas apos a administracao. Todos os pacientes realizaram ecocardiograma e tres apresentaram aneurisma leve da coronaria. CONCLUSOES: A doenca de Kawasaki e habitualmente confundida com outras doencas, o que causa retardo no tratamento e aumento no risco de complicacoes cardiacas.