Thorax ultrasound in the management of pediatric pneumonias complicated with empyema

The aim of this work is to prospectively study the value of thoracic ultrasound (US) before pleural drainage in children with parapneumonic effusion (PPE). All children hospitalized for PPE, identified by thoracic radiography, underwent US to assess pleural loculation, echogenicity, and pleural fluid quantity. From August 2001 to July 2003, 52 children were examined. US was performed on 48 of these children, of whom 35 received chest tube drainage and 13 only received clinical treatment. US identified 38 patients with free flowing and 10 with loculated pleural fluid. About 25 of the free flowing (65.8%) and 10 (100%) of the loculated patients received chest tube drainage. Echogenicity was anechoic in 13, echoic without septations in 17 and echoic with septations in 18. Chest tube drainage was required in 6 anechoic (46.15%), 14 echoic without septations (82.35%), and 15 echoic with septations (83.33%). Quantity of fluid estimated by US varied from 20 to 860 ml. Effusion volume was higher in patients that were echoic with septations and loculated effusions. Pleural glucose and pH were lower, and LDH was higher in loculated PPE patients. In conclusion, US is an auxiliary exam for determining whether thoracic drainage is needed in parapneumonic effusion; loculated or echoic effusion should be drained, and free anechoic fluid needs further investigation.

Clinical and pathological assessment of 82 patients with cardiovascular diseases undergoing autopsy at the Hospital das Clínicas of the Faculdade de Medicina de Botucatu from 1988 to 1993

OBJECTIVE: To evaluated the clinical diagnostic, efficiency for basic death causes in patients dying of circulatory disease and de relative frequency of those diseases. METHODS: Analysis of medical record data of 82 patients, ages from 16 to 84 years old (68 over 40 years old), whose died of circulatory disease and had undergone necropsy in the period from 1988 to 1993 years in the University Hospital of Medicine Faculty of Botucatu-UNESP, Br. RESULTS: The functional class of patients were III or IV, in 78%, and 81.7% needed urgent hospitalization. By the clinical judgment the death were by ischemic heart disease in 32 (21 acute myocardial infarction), Chagas'disease in 12, valvopathy in 11, cardiomyopathy in 7, heart failure with no specification of cardiopathy in 11 and other causes in 9. At the necropsy the death cause was ischemic heart disease in 34 patients, valvopathy in 10, Chagas'disease in 10, cardiomyopathy in 5, and heart failure with no specification of cardiopathy in 2.The concordance taxes were in thhe same order: 94,6%, 90,0%, 83.3%, 71.4% and 28.5%. CONCLUSION: There was a great efficiency of clinical diagnosis for death cause in a general university hospital. The ischemic heart disease were the main causes of death.

Cytogenetic studies of Brazilian pediatric myelodysplastic syndrome cases: challenges and difficulties in a large and emerging country

Myelodysplastic syndromes (MDS) and juvenile myelomonocytic leukemia (JMML) are rare hematopoietic stem cell diseases affecting children. Cytogenetics plays an important role in the diagnosis of these diseases. We report here the experience of the Cytogenetic Subcommittee of the Brazilian Cooperative Group on Pediatric Myelodysplastic Syndromes (BCG-MDS-PED). We analyzed 168 cytogenetic studies performed in 23 different cytogenetic centers; 84 of these studies were performed in patients with confirmed MDS (primary MDS, secondary MDS, JMML, and acute myeloid leukemia/MDS+Down syndrome). Clonal abnormalities were found in 36.9% of the MDS cases and cytogenetic studies were important for the detection of constitutional diseases and for differential diagnosis with other myeloid neoplasms. These data show the importance of the Cooperative Group for continuing education in order to avoid a late or wrong diagnosis.

Bone marrow necrosis related to paracoccidioidomycosis: the first eight cases identified at autopsy

To report the first eight bone marrow necrosis (BMN) cases related to paracoccidioidomycosis (PCM) from patient autopsies with well-documented bone marrow (BM) histology and cytology.A retrospective evaluation was performed on BM specimens from eight autopsied patients from Botucatu University Hospital with PCM-related BMN. Relevant BMN literature was searched and analysed.All eight patients had acute PCM. Six had histological only (biopsies) and two cytological only (smears) specimens. Five biopsy specimens revealed severe and one mild coagulation patterned necrotic areas. Five had osteonecrosis. The cytological specimens also showed typical BMN patterns. Paracoccidioides brasiliensis yeast forms were visible within necrotic areas in all cases.

Autopsy: quality assurance in the ICU

Objective: To examine the correlation between the clinical diagnosis and autopsy findings in adult patients who died in an intensive care unit (ICU). To determine the rate of agreement of the basic and terminal causes of death and the types of errors in order to improve quality control of future care,Design, Retrospective study.Setting: Adult ICU in a university hospital.Patients: 30 adult patients who died in the ICU. with the exclusion of medicolegal cases.Methods and main results: Anatomo-clinical meetings were held to analyze the pre- and postmortem correlations in 30 consecutive autopsies at the ICU of the University Hospital, School of Medicine of Botucatu/ UNESP, from January 1994 to January 1997. The rate of correct clinical diagnoses of the basic cause was 66.7 %; in 23.3 % of cases, if the correct diagnosis was made, management would have been different, as would have been the evolution of the patient's course (Class I error): in 10 % of the cases the error would not have led to a change in management (Class II error). The rate of correct clinical diagnoses of terminal cause was 80 %.Conclusions: the rate of recognition of the basic cause was 66.7 %, which is consistent with the literature, but the Class I error rate was higher than that reported in the literature.

Mitochondrial Alterations in Nonalcoholic Fatty Liver Disease. Pediatric Case Description of Three Submitted Sequential Biopsies

Nonalcoholic fatty liver disease (NAFLD) is a clinical-pathological syndrome that encompasses a wide spectrum of morphologic alterations, ranging from simple hepatic steatosis to a more severe stage, known as nonalcoholic steatohepatitis (NASH). The purpose of this clinical report was to contribute to the understanding of mitochondrial alterations in NAFLD. The child (13-month-old) underwent initial biopsy in the year 2000 and was diagnosed with diffuse macro and microvesicular steatosis. Two additional biopsies were performed in 2001 and 2004. A high percentage of microvesicular steatosis was observed in the biopsies performed in 2000 and 2001. Mitochondrial size was slightly increased in the biopsy performed in the year 2000, significantly increased in 2001 and decreased in 2004. The presence of "mitochondrial hypertrophy" in the hepatocytes of an asymptomatic pediatric patient whose disease presentation was typical of NAFLD, excluding other pathological processes, allowed us to suspect that such a defect was considered the primary mitochondrial disorder.

Discrepancies between clinical diagnoses and autopsy findings

Autopsy examination is considered to be an essential element for medical auditing and teaching. Despite the significant progress in diagnostic procedures, autopsy has not always confirmed the clinical diagnosis. In the present study, we compared the diagnosis recorded on medical charts with reports of 96 autopsies performed at the University Teaching Hospital of the Faculdade de Medicina de Botucatu, Botucatu, SP, Brazil, between 1975 and 1982, and of 156 autopsies performed at the same institution between 1992 and 1996. The clinical diagnosis of the basic cause of death was confirmed at autopsy in 77% of cases. The percent confirmation fell to 60% when the immediate terminal cause of death was considered, and in 25% of cases, the terminal cause was only diagnosed at autopsy. The discrepancies between clinical and autopsy diagnosis were even larger for secondary diagnoses: 50% of them were not suspected upon clinical diagnosis. Among them, we emphasize the diagnosis of venous thromboses (83%), pulmonary embolisms (80%), bronchopneumonias (46%) and neoplasias (38%). Iatrogenic injuries were very frequent, and approximately 90% of them were not described in clinical reports. Our results suggest that highly sensitive and specific diagnostic tests are necessary but cannot substitute the clinical practice for the elaboration of correct diagnoses.

Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?

CONTEXTO E OBJETIVO: em crianças, a esteatose hepática pode se relacionar a erros inatos do metabolismo (EIMs) ou à doença hepática gordurosa não-alcoólica (DHGNA). O objetivo deste estudo foi avaliar e caracterizar esteatose de causa indeterminada por meio de análises morfológica e morfométrica em tecido hepático. TIPO DE ESTUDO E LOCAL: Estudo transversal nos Departamentos de Patologia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp) e Faculdade de Medicina de Botucatu da Universidade Estadual Paulista (FMB-Unesp). MÉTODOS: Foram utilizadas 18 biópsias hepáticas consecutivas obtidas de 16 pacientes com idade variando de 3 meses a 12 anos e 9 meses, inseridas num banco de dados no período do estudo, que foram analisadas por microscopia óptica e eletrônica. Na microscopia eletrônica, foi realizada determinação da densidade mitocondrial e da área superficial média das mitocôndrias nos hepatócitos. Dez pacientes com idade variando de 1 a 14 anos foram usados como grupo controle. RESULTADOS: Foi detectada esteatose pura, não acompanhada por fibrose ou outra alteração histológica. Foi verificado que, na predominância de esteatose microvesicular, houve aumento significativo da área mitocondrial média. CONCLUSÃO: A esteatose microvesicular pode estar relacionada à hepatopatia mitocondrial primária, principalmente devido à redução na β-oxidação ou parcial estagnação da fosforilação oxidativa. Por essas razões, esta forma de esteatose (que não pode ser chamada de pura) possivelmente represente uma fase inicial no amplo espectro da DHGNA. Chamamos a atenção para casos de esteatose no grupo pediátrico com predomínio da forma microvesicular, uma vez que pode haver associação com desordens mitocondriais.

Pediatric Antiphospholipid Syndrome: Clinical and Immunologic Features of 121 Patients in an International Registry

OBJECTIVES. The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome.PATIENTS and METHODS. A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday.RESULTS. As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vessel thrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologic manifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-beta(2)-glycoprotein I antibodies in 67%, and lupus anticoagulant in 72%. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations.CONCLUSIONS. Clinical and laboratory characterization of patients with pediatric antiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups. Pediatrics 2008; 122: e1100-e1107

Staphylococcus Aureus Contamination in a Pediatric Dental Clinic

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Moebius Syndrome: Clinical Manifestations in a Pediatric Patient

Moebius syndrome is a congenital, nonprogressive disorder clinically characterized by loss of facial expression, impaired stomatognathic system functions, incapacity to close the eyelids, and several oral impairments. The purpose of this paper was to present the clinical manifestations and the dental treatment in a 5-year, 2-month-old male Moebius syndrome patient. The child presented with facial asymmetry, difficulty performing facial mimic movements and pronouncing some letters, and compromised suction, mastication, breathing, and deglutition. An intraoral examination revealed hypofunction of the perioral muscles, cheeks and tongue, ankyloglossia, anterior open bite, and absence of carious lesions and dental anomalies. The dental treatment consisted of frenectomy and further placement of a removable orthodontic appliance with a palatal crib for correction of the anterior open bite. After 12 months of follow-up, anterior open bite decreased and speech, deglutition, and mastication improved. (Pediatr Dent 2009;31:289-93) Received March 8, 2008 vertical bar Lost Revision July 22, 2008 vertical bar Revision Accepted July 28, 2008

Longitudinal clinical and radiographic evaluation of severely intruded permanent incisors in a pediatric population

Intrusion is defined as the axial dislodgment of the tooth into its socket and is considered one of the most severe types of dental trauma. This longitudinal outcome study was undertaken to evaluate clinically and radiographically severely intruded permanent incisors in a population of children and adolescents. All cases were treated between September 2003 and February 2008 in a dental trauma service. Clinical and radiographic data were collected from 12 patients (eight males and four females) that represented 15 permanent maxillary incisors. Mean age at the time of injury was 8 years and 9 months (range 7-14 years and 8 months). Mean time elapsed to follow-up was 26.6 months (range 10-51 months). The analysis of data showed that tooth intrusion was twice as frequent in males. The maxillary central incisors were the most commonly intruded teeth (93.3%), and falling at home was the main etiologic factor (60%). More than half of the cases (53.3%) were multiple intrusions, 73.3% of the intruded teeth had incomplete root formation and 66.6% of the teeth suffered other injuries concomitant to intrusion. Immediate surgical repositioning was the treatment of choice in 66.7% of the cases, while watchful waiting for the tooth to return to its pre-injury position was adopted in 33.3% of the cases. The teeth that suffered additional injuries to the intrusive luxation presented a fivefold increased relative risk of developing pulp necrosis. The immature teeth had six times more chances of presenting pulp canal obliteration that the mature teeth and a lower risk of developing root resorption. The most frequent post-injury complications were pulp necrosis (73.3%), marginal bone loss (60%), inflammatory root resorption (40%), pulp canal obliteration (26.7%) and replacement root resorption (20%). From the results of this study, it was not possible to determine whether the type immediate treatment had any influence on the appearance of sequelae like pulp necrosis and root resorption after intrusive luxation, but the existence of additional injuries and the stage of root development influenced the clinical case outcome in a negative and positive manner, respectively.

DNA methylation patterns of the CDHI, RARB, and SFN genes in choroid plexus tumors

Genetic and epigenetic alterations in choroid plexus tumors, a rare neuroepithelial neoplasm most frequently detected in children, are poorly characterized. Epigenetic silencing associated with aberrant CpG island methylation is one mechanism leading to the loss of tumor suppressor functions in cancer cells. Using methylation-specific polymerase chain reaction, the methylation patterns of the genes CDH1 (E-cadherin), RARB (retinoic acid receptor, beta), and SFN (stratifin; 14-3-3 sigma) were retrospectively investigated in eight choroid plexus tumors (five papillomas, two atypical papillomas, and one carcinoma), as well as in two normal cortexes obtained after autopsy from male individuals aged 6 months and 64 years. Among the six pediatric tumors, the mean age at diagnosis was 1.8 years old (range, 0.2-6) and the two adult tumors were detected in a 66-year-old man and a 45-year-old woman. A high frequency of hypermethylation was detected in CDH1 and SFN genes in tumoral and normal cortex tissues. Tumor-specific RARB hypermethylation was observed in four papillomas. Further studies are required to evaluate the role of aberrant methylation in choroid plexus tumor progression. (c) 2007 Elsevier B.V. All rights reserved.

The Pediatric Rheumatology International Trials Organization American-College-of-Rheumatology provisional criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: Prospective validation of the definition of improvement

Objective. To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE).Methods. Thirty-seven experienced pediatric rheumatologists from 27 countries, each of whom had specific experience in the assessment of juvenile SLE patients, achieved consensus on 128 patient profiles as being clinically improved or not improved. Using the physicians' consensus ratings as the gold standard measure, the chi-square, sensitivity, specificity, false-positive and false-negative rates, area under the receiver operating characteristic curve, and kappa level of agreement for 597 candidate definitions of improvement were calculated. Only definitions with a kappa value greater than 0.7 were retained. The top definitions were selected based on the product of the content validity score multiplied by its kappa statistic.Results. The definition of improvement with the highest final score was at least 50% improvement from baseline in any 2 of the 5 core set measures, with no more than 1 of the remaining worsening by more than 30%.Conclusion. PRINTO proposes a valid and reproducible definition of improvement that reflects well the consensus rating of experienced clinicians and that incorporates clinically meaningful change in core set measures in a composite end point for the evaluation of global response to therapy in patients with juvenile SLE. The definition is now proposed for use in juvenile SLE clinical trials and may help physicians to decide whether a child with SLE responded adequately to therapy.

The Brazilian pediatric myelodysplastic cooperative group strategies: are they relevant to improve educational approach and correct diagnosis?

Brazil is a wide country with huge contrasts. Its peculiarities can highlight environmental factors that could influence the frequencies of different cancers. The standard treatment and results achieved from several different areas of the country may not be found in others. The establishment of a national cooperative group has the potential to improve outcomes. The The Brazilian Cooperative Group on Pediatric Patients with Myelodysplastic Syndrome (BCG-MDS-PED) was first organized in January 1997 as a working group of hematologists, pediatric oncologists, pediatric-hematologists, molecular biologists and other professionals in order to study pediatric (age < 18 years) MDS. Six distinct subcommittees constituted with members from several universities: cytology, histopathology, clinical, cytogenetics, molecular biology and epidemiology. The goals of the BCG-MDS-PED were: (i) to offer support for diagnosis and orientation for treatment; (ii) educational Support for the colleagues all over the country and (iii) research on pathogenesis and new approaches for pediatric MDS patients. There are socio-economical differences among the five regions of the country. The BCG-MDS-PED believes that it is absolutely necessary to Study the clinical, cellular, molecular and epidemiological aspects of MDS, taking in account these peculiar differences among populations and regions. Since 1997, 114 pediatric cases were referred to the BCG-MDS-PED from 21 centres. Seven Brazilian states have sent cases to the group, 31 patients were referred from universities, 73 patients from pediatric oncology units (foundations) and 10 patients came from private clinics. Some of these patients have been followed up and/or treated by the physician who referred them to the BCG-MDS-PED for confirmation of the initial diagnosis. The majority of these physicians have required orientation on diagnostic and treatment issues, as well as to complete cytogenetic and molecular studies. From these 114 patients, 64 patients were confirmed as MDS. We believe that, the more numerous the MDS-studied cases, the more experienced will be the referee group on clinical and laboratory features on childhood MDS in Brazil. (C) 2002 Published by Elsevier B.V. Ltd.