Kawasaki disease: clinical experience in a university hospital

OBJETIVO: A doença de Kawasaki é uma vasculite sistêmica aguda de etiologia desconhecida. Seu diagnóstico baseia-se em critérios clínicos. O objetivo deste estudo foi descrever os casos de pacientes com doença de Kawasaki internados no Hospital Universitário da Universidade de São Paulo entre janeiro/2000 e junho/2008. MÉTODOS: Dentre todos os pacientes internados na Enfermaria de Pediatria no período acima, foram selecionados aqueles cujo CID de alta foi doença de Kawasaki. Realizou-se estudo descritivo por meio da análise dos prontuários dessas crianças. RESULTADOS: Foram encontrados 18 casos. A média de internações foi de 2,1 casos/ano. A idade variou de três meses a nove anos. A proporção meninos:meninas foi 1:1,25. Receberam outros diagnósticos prévios 17 pacientes, sendo escarlatina em 2/3 dos casos. O tempo de febre antes do diagnóstico variou de cinco a 11 dias. Nove crianças apresentaram quatro sinais sugestivos de doença de Kawasaki; oito apresentaram cinco sinais e uma apresentou dois sinais, o que foi considerado doença de Kawasaki incompleta. Receberam gamaglobulina 15 crianças (entre o sexto e o décimo dias de evolução) e 11 (73%) ficaram afebris após infusão da medicação. Os demais tiveram febre até 24 horas após a administração. Todos os pacientes realizaram ecocardiograma e três apresentaram aneurisma leve da coronária. CONCLUSÕES: A doença de Kawasaki é habitualmente confundida com outras doenças, o que causa retardo no tratamento e aumento no risco de complicações cardíacas.OBJECTIVE: Kawasaki disease is an acute systemic vasculitis of unknown etiology. Its diagnosis is based on clinical criteria. This study aimed to describe Kawasaki disease cases treated at the University Hospital of Universidade de São Paulo, from January/2000 to June/2008. METHODS: Among all patients admitted to the pediatric ward during this period, patients whose discharge ICD was Kawasaki disease were selected. A descriptive study was carried out by analyzing the records of these children. RESULTS: 18 cases were found, with an average of 2.1 cases/year. Patients varied from three to nine years old. The boys/girls ratio was 1/1.25. Seventeen patients had previously been misdiagnosed with other diseases, being 2/3 of them scarlet fever. Prior to diagnosis, fever had persisted for five to 11 days. Nine patients showed four suggestive signs of Kawasaki disease, eight patients showed five signs, and one patient had two suggestive signs, which was considered as incomplete Kawasaki disease. Gammaglobulin was administered to 15 children (between the 6th-10th day of the disease), with 11 (73%) of them having no fever following the administration. The other ones had fever for up to 24 hours following gammaglobolin use. All patients were evaluated by echocardiograms, and three of them showed mild coronary aneurysm. CONCLUSIONS: Kawasaki disease is usually misdiagnosed, thereby delaying treatment and increasing the risk of heart

Cat-scratch disease presenting as multiple hepatic lesions: case report and literature review

Although infectious diseases are the most prevalent cause of fevers of unknown origin (FUO), this diagnosis remains challenging in some pediatric patients. Imaging exams, such as computed tomography (CT) are frequently required during the diagnostic processes. The presence of multiple hypoattenuating scattered images throughout the liver associated with the history of cohabitation with cats should raise the suspicion of the diagnosis of cat-scratch disease (CSD), although the main etiologic agent of liver abscesses in childhood is Staphylococcus aureus. Differential diagnosis by clinical and epidemiological data with Bartonella henselae is often advisable. The authors report the case of a boy aged 2 years and 9 months with 16-day history of daily fever accompanied by intermittent abdominal pain. Physical examination was unremarkable. Abdominal ultrasound performed in the initial work up was unrevealing, but an abdominal CT that was performed afterwards disclosed multiple hypoattenuating hepatic images compatible with the diagnosis of micro abscesses. Initial antibiotic regimen included cefotaxime, metronidazole, and oxacillin. Due to the epidemiology of close contact with kittens, diagnosis of CSD was considered and confirmed by serologic tests. Therefore, the initial antibiotics were replaced by clarithromycin orally for 14 days followed by fever defervescence and clinical improvement. The authors call attention to this uncommon diagnosis in a child presenting with FUO and multiple hepatic images suggestive of micro abscesses

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 arthropodborne 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 typicalcutaneous 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

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 arthropodborne 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

Meningoencefalite concomitante a herpes zoster oftálmico em escolar previamente hígido

INTRODUÇÃO O vírus Varicella-Zoster é o agente da varicela, doença auto-limitada comum \ud da faixa etária pediátrica. Cerca de 20% dos casos evoluem com herpes zoster em algum \ud momento da vida, devido a reativação do vírus dos gânglios nervosos ou reexposição. O \ud envolvimento do ramo oftálmico do nervo trigêmio, defido como zoster oftálmico, tem como \ud complicação mais descrita a nevralgia pós-herpética, podendo evoluir com outras alterações \ud locais agudas e tardias. Meningoencefalites concomitante a herpes zoster são pouco descritas na \ud literatura. DESCRIÇÃO DO CASO Paciente de 9 anos, com antecedente de varicela com 1 ano \ud de idade, com queixa de vômitos e cefaléia há dois dias, associada a queda do estado gerale \ud hiporexia. Referiu aparecimento de lesões vesico-bolhosas dolorosas em região periorbitária \ud esquerda há 1 dia e evoluiu com agitação psicomotora e confusão mental. No exame físico de \ud entrada apresentava-se sonolento, com lesões vesico-bolhosas em dermátomo do ramo oftálmico \ud do nervo trigêmio, sem sinais de irritação meníngea ou déficits motores, sem alterações visuais \ud ou oculares. Realizada tomografia de crânio e eletroencefalograma sem alterações. Coletado \ud líquor que revelou líquido límpido e incolor, com aumento de celularidade às custas de \ud linfócitos, glicorraquia normal, bacterioscopia negativa e culturas negativas. Feita hipótese \ud diagnóstica inicial de herpes zoster oftálmico complicado com meningoencefalite e introduzido \ud aciclovir. Paciente evoluiu bem, com melhora do estado geral, remissão dos sintomas \ud neurológicos e melhora das lesões de pele. Evidenciado PCR positivo para o vírus varicela-zoster \ud (VVZ) no líquor. DISCUSSÃO Encontramos poucas descrições na literatura de herpes zoster \ud oftálmico associado à alterações neurológicas. A presença da PCR positiva no liquor foi \ud fundamental para o diagnóstico. CONCLUSÃO O VVZ pode reativar na forma de herper \ud zoster oftálmico e acometer o sistema nervoso central. Apesar de evento raro em crianças, \ud especialmente nas imunocompetentes, a presença da PCR positiva liquor confirmou a \ud meningoencefalite

The ASTRO-H X-ray Observatory

The joint JAXA/NASA ASTRO-H mission is the sixth in a series of highly successful X-ray missions initiated by the Institute of Space and Astronautical Science (ISAS). ASTRO-H will investigate the physics of the high-energy universe via a suite of four instruments, covering a very wide energy range, from 0.3 keV to 600 keV. These instruments include a high-resolution, high-throughput spectrometer sensitive over 0.3-2 keV with high spectral resolution of Delta E < 7 eV, enabled by a micro-calorimeter array located in the focal plane of thin-foil X-ray optics; hard X-ray imaging spectrometers covering 5-80 keV, located in the focal plane of multilayer-coated, focusing hard X-ray mirrors; a wide-field imaging spectrometer sensitive over 0.4-12 keV, with an X-ray CCD camera in the focal plane of a soft X-ray telescope; and a non-focusing Compton-camera type soft gamma-ray detector, sensitive in the 40-600 keV band. The simultaneous broad bandpass, coupled with high spectral resolution, will enable the pursuit of a wide variety of important science themes.Comment: 22 pages, 17 figures, Proceedings of the SPIE Astronomical Instrumentation "Space Telescopes and Instrumentation 2012: Ultraviolet to Gamma Ray

Effects of high EPA and high DHA fish oils on changes in signaling associated with protein metabolism induced by hindlimb suspension in rats

© 2016 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.The effects of either eicosapentaenoic (EPA)- or docosahexaenoic (DHA)-rich fish oils on hindlimb suspension (HS)-induced muscle disuse atrophy were compared. Daily oral supplementations (0.3 mL/100 g b.w.) with mineral oil (MO) or high EPA or high DHA fish oils were performed in adult rats. After 2 weeks, the animals were subjected to HS for further 2 weeks. The treatments were maintained alongside HS. At the end of 4 weeks, we evaluated: body weight gain, muscle mass and fat depots, composition of fatty acids, cross-sectional areas (CSA) of the soleus muscle and soleus muscle fibers, activities of cathepsin L and 26S proteasome, and content of carbonylated proteins in the soleus muscle. Signaling pathway activities associated with protein synthesis (Akt, p70S6K, S6, 4EBP1, and GSK3-beta) and protein degradation (atrogin-1/MAFbx, and MuRF1) were evaluated. HS decreased muscle mass, CSA of soleus muscle and soleus muscle fibers, and altered signaling associated with protein synthesis (decreased) and protein degradation (increased). The treatment with either fish oil decreased the ratio of omega-6/omega-3 fatty acids and changed protein synthesis-associated signaling. EPA-rich fish oil attenuated the changes induced by HS on 26S proteasome activity, CSA of soleus muscle fibers, and levels of p-Akt, total p70S6K, p-p70S6K/total p70S6K, p-4EBP1, p-GSK3-beta, p-ERK2, and total ERK 1/2 proteins. DHA-rich fish oil attenuated the changes induced by HS on p-4EBP1 and total ERK1 levels. The effects of EPA-rich fish oil on protein synthesis signaling were more pronounced. Both EPA- and DHA-rich fish oils did not impact skeletal muscle mass loss induced by non-inflammatory HS

Involvement of Girdin in the Determination of Cell Polarity during Cell Migration

Cell migration is a critical cellular process that determines embryonic development and the progression of human diseases. Therefore, cell- or context-specific mechanisms by which multiple promigratory proteins differentially regulate cell migration must be analyzed in detail. Girdin (girders of actin filaments) (also termed GIV, Gα-interacting vesicle associated protein) is an actin-binding protein that regulates migration of various cells such as endothelial cells, smooth muscle cells, neuroblasts, and cancer cells. Here we show that Girdin regulates the establishment of cell polarity, the deregulation of which may result in the disruption of directional cell migration. We found that Girdin interacts with Par-3, a scaffolding protein that is a component of the Par protein complex that has an established role in determining cell polarity. RNA interference-mediated depletion of Girdin leads to impaired polarization of fibroblasts and mammary epithelial cells in a way similar to that observed in Par-3-depleted cells. Accordingly, the expression of Par-3 mutants unable to interact with Girdin abrogates cell polarization in fibroblasts. Further biochemical analysis suggests that Girdin is present in the Par protein complex that includes Par-3, Par-6, and atypical protein kinase C. Considering previous reports showing the role of Girdin in the directional migration of neuroblasts, network formation of endothelial cells, and cancer invasion, these data may provide a specific mechanism by which Girdin regulates cell movement in biological contexts that require directional cell movement