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3.
JBJS Case Connect ; 10(1): e0525, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32224649

RESUMO

CASE: A 14-year-old boy presented with an 18-month history of progressive left wrist drop. Magnetic resonance imaging studies were concerning for mass infiltration of the posterior interosseous nerve (PIN). Surgical resection and pathology confirmed a variant of neuromuscular choristoma (NMC), infiltrated with and surrounded by proliferation of smooth muscle, rather than skeletal muscle. Given the wide-spanning nerve involvement, the patient underwent tendon transfers at the time of surgical resection. CONCLUSION: We report here the first case report of a NMC in the PIN and the first pathologically confirmed case with exclusive smooth muscle involvement without a skeletal muscle component.


Assuntos
Coristoma/patologia , Antebraço/patologia , Músculo Liso , Neuropatia Radial/diagnóstico , Adolescente , Coristoma/diagnóstico por imagem , Diagnóstico Diferencial , Antebraço/diagnóstico por imagem , Humanos , Masculino
4.
Hepatology ; 71(4): 1331-1349, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31469182

RESUMO

BACKGROUND AND AIMS: Alagille syndrome (ALGS) is a multisystem developmental disorder characterized by bile duct (BD) paucity, caused primarily by haploinsufficiency of the Notch ligand jagged1. The course of the liver disease is highly variable in ALGS. However, the genetic basis for ALGS phenotypic variability is unknown. Previous studies have reported decreased expression of the transcription factor SOX9 (sex determining region Y-box 9) in late embryonic and neonatal livers of Jag1-deficient mice. Here, we investigated the effects of altering the Sox9 gene dosage on the severity of liver disease in an ALGS mouse model. APPROACH AND RESULTS: Conditional removal of one copy of Sox9 in Jag1+/- livers impairs the biliary commitment of cholangiocytes and enhances the inflammatory reaction and liver fibrosis. Loss of both copies of Sox9 in Jag1+/- livers further worsens the phenotypes and results in partial lethality. Ink injection experiments reveal impaired biliary tree formation in the periphery of P30 Jag1+/- livers, which is improved by 5 months of age. Sox9 heterozygosity worsens the P30 biliary tree phenotype and impairs the partial recovery in 5-month-old animals. Notably, Sox9 overexpression improves BD paucity and liver phenotypes in Jag1+/- mice without ectopic hepatocyte-to-cholangiocyte transdifferentiation or long-term liver abnormalities. Notch2 expression in the liver is increased following Sox9 overexpression, and SOX9 binds the Notch2 regulatory region in the liver. Histological analysis shows a correlation between the level and pattern of SOX9 expression in the liver and outcome of the liver disease in patients with ALGS. CONCLUSIONS: Our results establish Sox9 as a dosage-sensitive modifier of Jag1+/- liver phenotypes with a permissive role in biliary development. Our data further suggest that liver-specific increase in SOX9 levels is a potential therapeutic approach for BD paucity in ALGS.


Assuntos
Síndrome de Alagille/genética , Síndrome de Alagille/patologia , Fígado/patologia , Fatores de Transcrição SOX9/genética , Animais , Ductos Biliares/anormalidades , Transdiferenciação Celular/genética , Criança , Pré-Escolar , Modelos Animais de Doenças , Hepatócitos/citologia , Heterozigoto , Humanos , Lactente , Proteína Jagged-1/genética , Fígado/anormalidades , Fígado/metabolismo , Camundongos , Camundongos Endogâmicos C57BL , Receptores Notch/genética , Receptores Notch/metabolismo , Índice de Gravidade de Doença , Transdução de Sinais
5.
Prenat Diagn ; 39(4): 287-292, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30707444

RESUMO

BACKGROUND: The ex utero intrapartum treatment (EXIT) is utilized to transition fetuses with prenatally diagnosed airway obstruction to postnatal life. We describe the unique clinical course, diagnosis, treatment, and outcomes of patients with cervical lymphatic malformation (CLM) managed with EXIT. METHODS: Review of fetuses with diagnosed CLM was delivered by EXIT (2001-2018) in a tertiary referral fetal center. Outcomes included survival, tracheostomy at discharge, neonatal course after delivery, and pulmonary hypoplasia. Data are reported as median [range] and rate (%). RESULTS: Out of 45 patients delivered by EXIT, 10 were delivered for CLM: seven had polyhydramnios, one had nonimmune hydrops, five delivered preterm, and three were emergency EXITs. The EXIT time and estimated blood loss were 125 minutes (95, 158) and 900 mL (500, 1500), respectively. Airway was secured in all. There was one neonatal death (day 8) with prematurity, sepsis, and pulmonary hypoplasia. Three out of nine were discharged with a tracheostomy. CONCLUSION: In CLM, close monitoring for structural neck involvement and development of polyhydramnios are important and may be an indication for EXIT as the optimal delivery mode. An experienced multidisciplinary team is a key factor for an effective approach to the obstructed airway in CLM.


Assuntos
Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/terapia , Vasos Linfáticos/anormalidades , Pescoço/anormalidades , Assistência Perinatal/métodos , Diagnóstico Pré-Natal , Adolescente , Adulto , Feminino , Doenças Fetais/diagnóstico , Doenças Fetais/mortalidade , Doenças Fetais/terapia , Humanos , Lactente , Cuidado do Lactente/métodos , Mortalidade Infantil , Recém-Nascido , Anormalidades Linfáticas/mortalidade , Vasos Linfáticos/cirurgia , Masculino , Pescoço/patologia , Parto/fisiologia , Gravidez , Diagnóstico Pré-Natal/métodos , Estudos Retrospectivos , Adulto Jovem
6.
Prenat Diagn ; 39(4): 269-279, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30609053

RESUMO

OBJECTIVES: To describe and compare placental and amniotic histology in women who underwent a fetoscopic myelomeningocele repair to those who underwent an open hysterotomy myelomeningocele repair. Also, we intended to compare findings from both prenatal repair groups to age-matched control pregnant patients. METHODS: Placental and membrane histopathology from 43 prenatally repaired spina bifida cases (17 fetoscopic and 26 open) and 18 healthy controls were retrospectively assessed. Quantitative assessment of histopathology included apoptosis count and maternal and fetal underperfusion scores. Qualitative assessment included the detection of pigmented macrophages and/or signs of placental/amniotic inflammation. Associations between the duration of surgery or the duration of CO2 insufflation and quantitative histological parameters were tested. RESULTS: Fetoscopic surgery cases did not show significant differences in any of the studied parameters when compared against controls. No differences were detected either when compared with open repaired cases, except for lower proportion of pigmented laden macrophages in the fetoscopic group (11.8% vs 61.5%, P < 0.01). No associations between the duration of surgery or the duration of CO2 exposure and any of the quantitative histological parameters were detected. CONCLUSIONS: These preliminary results support the lack of detrimental effects of the use of heated and humidified CO2 gas for uterine insufflation to fetal membranes and placenta.


Assuntos
Âmnio/patologia , Fetoscopia/estatística & dados numéricos , Defeitos do Tubo Neural/cirurgia , Técnicas de Abdome Aberto/estatística & dados numéricos , Doenças Placentárias/epidemiologia , Placenta/patologia , Adulto , Âmnio/cirurgia , Estudos de Casos e Controles , Feminino , Doenças Fetais/epidemiologia , Doenças Fetais/patologia , Doenças Fetais/cirurgia , Terapias Fetais/métodos , Terapias Fetais/estatística & dados numéricos , Fetoscopia/métodos , Humanos , Meningomielocele/epidemiologia , Meningomielocele/patologia , Meningomielocele/cirurgia , Defeitos do Tubo Neural/epidemiologia , Técnicas de Abdome Aberto/métodos , Placenta/cirurgia , Doenças Placentárias/diagnóstico , Doenças Placentárias/patologia , Gravidez , Estudos Retrospectivos , Útero/patologia , Útero/cirurgia , Adulto Jovem
7.
J Ultrasound Med ; 38(9): 2485-2491, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30620078

RESUMO

Superb Microvascular Imaging (SMI; Canon Medical Systems, Tustin, CA) uses clutter suppression to extract flow signals at rapid frame rates, which provides high-resolution vessel-branching details without the need for contrast agents. The potential diagnostic benefits of SMI, as described in other areas of medicine, requires further exploration during pregnancy. In this pictorial essay, we demonstrate the complementary use of SMI compared to conventional Doppler ultrasound and how it may improve our ability to characterize placental microvascular patterns without the need for ultrasound contrast agents.


Assuntos
Microvasos/diagnóstico por imagem , Doenças Placentárias/diagnóstico por imagem , Placenta/irrigação sanguínea , Ultrassonografia Doppler/métodos , Ultrassonografia Pré-Natal/métodos , Feminino , Humanos , Gravidez
8.
J Pediatr Gastroenterol Nutr ; 68(1): 96-103, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30234762

RESUMO

OBJECTIVES: Systematic study of allograft liver histology in children undergoing orthotopic liver transplantation (LT) for cystic fibrosis-related liver disease (CFLD). METHODS: Retrospective clinicopathologic review of explants and allograft liver biopsies from 13 children and adolescents with CFLD. RESULTS: In this study, the median age at LT for CFLD was 15.7 years. Notably, 10 of 13 (77%) CF explants had >5% steatosis and 8 of 13 (61.5%) demonstrated variable fibrosis. The median age, sex, type of transplant (liver vs liver-lung), pancreatic insufficiency status, body mass index (BMI) percentile, genotype, and prevalence of diabetes were comparable in those with and without explant steatosis. More than half of allograft biopsies showed significant steatosis (17/31, 54.8%) and lobular inflammation (16/31, 51.6%). Hepatocyte ballooning was less frequent (5/31, 16.1%). Overall, 6 patients (46.2%) had allograft steatosis that worsened over time in 2 patients (33%). None had advanced fibrosis (≥stage 3). Patients with allograft steatosis had significantly more biopsies, were more likely to be "liver only" recipients, had a shorter interval since transplant and higher body mass index percentile (although <85). Patients without explant steatosis never demonstrated allograft steatosis, whereas 60% of patients with explant steatosis (n = 6) developed varying degrees of allograft steatosis. The degree of explant steatosis did not predict its severity in allografts (P = 0.3). CONCLUSION: This is the first study highlighting the development of allograft steatosis in CF patients. Our findings suggest that allograft steatosis in patients with CF may be related to pre-existing steatosis in native livers, regardless of other risk factors and may have implications on patient management and long-term graft/patient survival.


Assuntos
Fibrose Cística/complicações , Fígado Gorduroso/epidemiologia , Hepatopatias/cirurgia , Transplante de Fígado/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Adolescente , Aloenxertos/patologia , Biópsia , Criança , Pré-Escolar , Fibrose Cística/patologia , Fígado Gorduroso/etiologia , Feminino , Humanos , Fígado/patologia , Hepatopatias/etiologia , Hepatopatias/patologia , Transplante de Fígado/métodos , Masculino , Complicações Pós-Operatórias/etiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Transplante Homólogo
10.
Pediatr Infect Dis J ; 37(12): 1294-1298, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29634623

RESUMO

BACKGROUND: Ureaplasma spp. is a known risk factor for bronchopulmonary dysplasia in premature infants. Emerging research suggests treatment with azithromycin or clarithromycin in the first days of life (DOLs) reduces bronchopulmonary dysplasia in Ureaplasma spp. positive infants. Side effects of these antibiotics make it imperative to optimize reliable noninvasive screening procedures to identify infants who would benefit from treatment. METHODS: The aim of this study was to determine the best site and time to screen for Ureaplasma spp. in 24- to 34-week premature infants. Oral, nasal, gastric and tracheal cultures were collected and placed immediately in 10B broth media. Polymerase chain reaction verified culture results and identified the Ureaplasma spp. RESULTS: Cultures yielded a Ureaplasma spp. incidence of 80/168 = 47.6% [95% confidence interval (CI): 40-56]. Nasal cultures had greater sensitivity to detect Ureaplasma spp. than oral cultures (P = 0.008): however, a significant proportion of infants with Ureaplasma spp. would have been missed (12/79 = 15.2%, 95% CI: 8%-25%, P < 0.001) if oral cultures were not obtained. For all sites, the collection at DOL 7-10 were more likely to be positive than the collection at DOL 1-2: however, a significant proportion (5/77 = 6.5%, 95% CI: 2-15, P < 0.001) of infants with Ureaplasma spp. would have been missed if the DOL 1-2 cultures were not obtained. CONCLUSIONS: For optimal Ureaplasma spp. detection in 24- to 34-week premature infants, cultures need to be taken both early and late in the first 10 DOLs both from nasal and oral secretions.


Assuntos
Infecções por Ureaplasma/diagnóstico , Ureaplasma/isolamento & purificação , Feminino , Idade Gestacional , Humanos , Incidência , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Reação em Cadeia da Polimerase/métodos , Infecções por Ureaplasma/epidemiologia
11.
Pediatr Dev Pathol ; 20(6): 490-497, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28468559

RESUMO

Cystic epithelium in mesenchymal hamartoma (MH) is typically biliary type. Mucinous differentiation of the epithelium and increased hepatocellular component may pose a diagnostic challenge. We studied MH in 7 children (6 M, 1 F; age 4 months to 8 years, median 1 year). Resected tumors varied from 3.0 to 17.0 cm. All tumors showed biliary epithelium in the cystic component with strong and diffuse reactivity for CK7 and CK19. Expression of CK20 and CDX2 was additionally seen in 2 tumors, rare/focal in 1, and diffuse with mucinous differentiation in the other. Strong and continuous nuclear reactivity for Sox9 was seen in the cyst epithelium in all 7 tumors including focal staining in mucinous areas. It was also positive in the hepatocellular component. CD56 and vimentin were variably positive in the cystic epithelium of all cases. Alpha-fetoprotein showed patchy weak staining in the hepatocellular component and was negative in the cystic epithelium. Hepar-1 showed focal staining in the cystic epithelium in 4/7 cases and diffuse in the hepatocellular component. Both patients showing CK20 and CDX2 expression had abnormal chromosomal analysis: one with balanced translocation between chromosomes 2 and 19 and other with loss of chromosome Y. Among others, one showed 46,XY,inv(9)(p11q12), one did not grow cells, and the remaining 3 had normal karyotype. Six patients underwent resection and one had liver transplantation. All were alive and well with a median follow-up of 1.5 years. In conclusion, mucinous epithelium can be seen in MH. Expression of Sox9 in the cyst epithelium and hepatocytes suggests a common origin for these components. Expression of vimentin indicates an overlapping epithelial-mesenchymal phenotype. Hepatic MH can show divergent epithelial differentiation including both foregut and hindgut phenotype, which may provide insights into the pathogenesis of this rare neoplasm but does not seem to affect the outcome in this limited series.


Assuntos
Epitélio/patologia , Hamartoma/patologia , Neoplasias Hepáticas/patologia , Biomarcadores Tumorais/metabolismo , Criança , Pré-Escolar , Epitélio/metabolismo , Feminino , Seguimentos , Hamartoma/diagnóstico , Hamartoma/metabolismo , Humanos , Imuno-Histoquímica , Lactente , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/metabolismo , Masculino
12.
Arch Pathol Lab Med ; 141(5): 678-683, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28301225

RESUMO

CONTEXT: - Image-guided, fine-needle aspiration-assisted core needle biopsy with an on-site evaluation by a pathologist (FNACBP) of osseous lesions is not a common practice in pediatric institutions. OBJECTIVES: - To evaluate the diagnostic adequacy and accuracy of FNACBP for pediatric osseous lesions and to compare the adequacy with procedures that do not use fine-needle aspiration. DESIGN: - Six-year, retrospective review of 144 consecutive children biopsied for osseous lesions with and without fine-needle aspiration assistance. RESULTS: - Pathologic diagnosis was achieved in 79% (57 of 72) of the core biopsies without an on-site evaluation, 78% (32 of 41) of the open biopsies (9 with intraoperative consultation), and 97% (30 of 31) of the FNACBPs as the initial diagnostic procedure. Three FNACBP cases were preceded by nondiagnostic open biopsies. Among 34 lesions sampled by FNACBP, 33 (97%) succeeded with diagnostic tissue, with most (30 of 33; 91%) being neoplasms, including 16 malignant (48%), 13 benign (39%), and 1 indeterminate (3%) lesions. The most-common diagnoses were osteosarcoma (9 of 33; 27%) and Langerhans cell histiocytosis (7 of 33; 21%). In cases with follow-up information available, 93% (28 of 30) of the FNACBP-rendered diagnoses were clinically useful, allowing initiation of appropriate therapy. The FNACBP procedure had 100% specificity, sensitivity, and positive predictive value for all 14 malignant lesions, with the sensitivity being 88% in benign lesions. Most FNACBP procedures (32 of 34; 94%) yielded adequate material for ancillary testing. A gradual upward trend was observed for the choice of FNACBP as an initial diagnostic procedure for osseous lesions. CONCLUSIONS: - The FNACBP procedure yields sufficient material for diagnosis and ancillary studies in pediatric, osseous lesions and may be considered an initial-diagnostic procedure of choice.


Assuntos
Biópsia por Agulha Fina/métodos , Biópsia com Agulha de Grande Calibre/métodos , Biópsia Guiada por Imagem/métodos , Neoplasias/diagnóstico por imagem , Adolescente , Osso e Ossos/diagnóstico por imagem , Osso e Ossos/patologia , Criança , Pré-Escolar , Condrossarcoma , Feminino , Humanos , Lactente , Masculino , Neoplasias/patologia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Pediatria , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tumor Rabdoide , Sarcoma de Ewing , Adulto Jovem
13.
Rev. Esc. Enferm. USP ; 51: e03294, 2017. tab, graf
Artigo em Inglês, Português | LILACS, BDENF - Enfermagem | ID: biblio-956655

RESUMO

RESUMO Objetivo Verificar a associação entre as características macroscópicas do cordão umbilical, a gestação de alto risco e as repercussões neonatais. Método Estudo transversal, realizado no período de janeiro de 2012 a janeiro de 2015 em uma maternidade pública de Goiânia/GO. A população foi constituída de 126 puérperas com diagnóstico de gestação de alto risco e 139 clinicamente normais (grupo controle). Foram avaliadas características macroscópicas do cordão umbilical, doenças de base maternas, fetais e neonatais, idade gestacional, índice de Apgar, peso ao nascer, perímetro cefálico e paridade. Os dados foram analisados descritivamente. Resultados Participaram do estudo 265 puérperas e seus respectivos recém-nascidos. As características mais frequentes do cordão umbilical de puérperas com gestação de alto risco e grupo controle foram a ausência de nós verdadeiros (97,6% e 2,4%, respectivamente), comprimento entre 35 e 70 centímetros e inserção paracentral (81,7% e 18,3%). Houve diferença estatística entre o grupo gestação de alto risco e extremos de idade materna (p=0,004). Conclusão A análise e a descrição das características do cordão umbilical, realizadas pelo/a enfermeiro/a, trazem informações importantes sobre o prognóstico neonatal. Essa atribuição subsidia a prática clínica e visa à segurança ao binômio durante todo o período perinatal.


RESUMEN Objetivo Verificar la asociación entre las características macroscópicas del cordón umbilical, gestación de alto riesgo y repercusiones neonatales. Método Estudio transversal, realizado en el período de enero de 2012 a enero de 2015 en una maternidad pública de Goiânia/GO. La población estuvo constituida de 126 puérperas con diagnóstico de gestación de alto riesgo y 139 clínicamente normales (grupo control). Fueron evaluadas características macroscópicas del cordón umbilical, enfermedades de base maternas, fetales y neonatales, edad gestacional, índice de Apgar, peso al nacer, perímetro cefálico y paridad. Los datos fueron analizados descriptivamente. Resultados Participaron en el estudio 265 puérperas y sus respectivos recién nacidos. Las características más frecuentes del cordón umbilical de puérperas con gestación de alto riesgo y grupo control fueron la ausencia de nudos verdaderos (el 97,6% y el 2,4%, respectivamente), longitud entre 35 y 70 centímetros e inserción paracentral (el 81,7% y el 18,3%). Hubo diferencia estadística entre el grupo gestación de alto riesgo y extremos de edad materna (p=0,004). Conclusión El análisis y la descripción de las características del cordón umbilical, realizadas por el/la enfermero/a, brindan informaciones importantes acerca del pronóstico neonatal. Dicha atribución subsidia la práctica clínica y tiene como fin la seguridad del binomio durante todo el período perinatal.


ABSTRACT Objective To verify the association between the macroscopic characteristics of the umbilical cord, high-risk pregnancy and neonatal repercussions. Method A cross-sectional study carried out from January 2012 to January 2015 in a public maternity hospital in Goiânia/GO. The study population consisted of 126 recent puerperal women with diagnosis of high-risk pregnancy, and 139 clinically normal women (control group). Macroscopic features of the umbilical cord, maternal, fetal and neonatal diseases, gestational age, Apgar score, birth weight, head circumference and parity were evaluated. Data were descriptively analyzed. Results 265 puerperal women and their respective newborns participated in the study. The most frequent characteristics of the umbilical cord of those with high-risk pregnancy and those from the control group were the absence of true knots (97.6% and 2.4%, respectively), length between 35 and 70 centimeters and paracentral insertion (81.7% and 18.3%). A statistical difference was observed between the high-risk pregnancy group and extremes of maternal age (p=0.004). Conclusion The analysis and description of the characteristics of the umbilical cord carried out by the nurse lend important information about the neonatal prognosis. This evaluation subsidizes clinical practice and seeks to ensure the safety of the (mother-baby) binomial throughout the perinatal period.


Assuntos
Humanos , Feminino , Recém-Nascido , Adulto , Cordão Umbilical/anatomia & histologia , Cordão Umbilical/fisiopatologia , Recém-Nascido , Gravidez de Alto Risco , Estudos Transversais , Enfermagem Materno-Infantil , Maternidades
14.
Hum Pathol ; 53: 137-41, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-26980027

RESUMO

We hereby report an unusual gastric tumor arising from the pyloric wall of the stomach in a 9-year-old child harboring the exceptionally rare translocation t(7;12) resulting in ACTB-GLI1 gene fusion. This tumor has been previously classified as pericytoma with t(7;12) and described in 6 patients, 2 of them children. We discuss the challenges in recognizing this rare entity and the importance of the molecular studies in establishing the correct diagnosis. Our case is the first report of this type arising in the stomach of a child.


Assuntos
Actinas/genética , Biomarcadores Tumorais/genética , Cromossomos Humanos Par 12 , Cromossomos Humanos Par 7 , Fusão Gênica , Neoplasias Gástricas/genética , Translocação Genética , Proteína GLI1 em Dedos de Zinco/genética , Biomarcadores Tumorais/análise , Criança , Feminino , Predisposição Genética para Doença , Humanos , Imuno-Histoquímica , Cariotipagem , Fenótipo , Neoplasias Gástricas/química , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia
15.
J Pediatr Surg ; 50(3): 382-7, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25746693

RESUMO

BACKGROUND: Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature. METHODS: A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013. RESULTS: Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size. CONCLUSIONS: Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.


Assuntos
Diagnóstico por Imagem , Hiperplasia Nodular Focal do Fígado/diagnóstico , Imageamento por Ressonância Magnética/métodos , Biópsia/métodos , Criança , Diagnóstico Diferencial , Humanos
17.
Rev. bras. ginecol. obstet ; 36(1): 23-28, 01/2014. tab
Artigo em Português | LILACS | ID: lil-702025

RESUMO

OBJETIVO: Avaliar as variações do peso corporal e dos órgãos internos de crianças autopsiadas no período perinatal e sua relação com a causa de morte. MÉTODOS: Foram incluídos 153 casos de autópsias perinatais realizadas em um hospital universitário do Sudeste do Brasil. Informações sobre causa de morte perinatal, data da autópsia, idade gestacional, peso perinatal e dos órgãos foram recuperadas dos protocolos de autópsia e do prontuário da mãe e/ou do recém-nascido. Foram definidos quatro grupos de causa de morte: malformações congênitas, hipóxia/anóxia perinatal, infecção ascendente e membrana hialina. Encéfalo, fígado, pulmões, coração, baço, timo e suprarrenais foram analisados. RESULTADOS: O peso das crianças com hipóxia/anóxia perinatal (1.834,6±1.090,1 g versus 1.488 g), membrana hialina (1.607,2±820,1 g versus 1.125 g) e infecção ascendente (1.567,4±1.018,9 g versus 1.230 g) foi maior do que o esperado para a idade gestacional. O peso dos pulmões foi maior nos casos com infecção ascendente (36,6±22,6 g versus 11 g) e menor nos casos com malformação congênita (22,0±9,5 g versus 40 g). O peso do baço foi maior nos casos que apresentaram infecção ascendente (8,6±8,9 g versus 3,75 g ). O peso das suprarrenais foi menor nos casos com malformação congênita (3,9±2,1 g versus 5,5 g), o do timo foi menor nos casos com miscelânea (3,7±1,2 g versus 7,5 g) e o do baço foi menor nos casos com imaturidade pulmonar (0,4±0,1 g versus 1,7 g). Todos esses resultados apresentaram diferenças significativas. CONCLUSÕES: Este estudo demonstra que as variações do peso das crianças e de seus órgãos ...


PURPOSE: To evaluate changes in body and internal organ weight of autopsied children in the perinatal period and their relationship with the cause of death. METHODS: One hundred and fifty three cases of perinatal autopsies performed at a university hospital in Southeastern Brazil ere included. Information about cause of perinatal death, date of autopsy, gestational age, perinatal weight and organ weight was obtained from the autopsy protocols and medical records of the mother and/or the newborn. Four groups of causes of death were defined: congenital malformations, perinatal hypoxia/anoxia, ascending infection and hyaline membrane. Brain, liver, lungs, heart, spleen, thymus and adrenals were analyzed. RESULTS: The weight of children with perinatal hypoxia/anoxi (1,834.6±1,090.1 g versus 1,488 g), hyaline membranes (1,607.2±820.1 g versus 1,125 g) and ascending infection (1,567.4±1,018.9 g versus 1,230 g) was higher than expected for the population. Lung weight was higher in cases with ascending infection (36.6±22.6 g versus 11 g) and lower in cases with congenital malformations (22.0±9.5 g versus 40 g). Spleen weight was higher in children with ascending infection (8.6±8.9 g versus 3.75 g ) and adrenal weight was lower in cases with congenital malformations (3.9±2.1 g versus 5.5 g). Thymus weight was lower in cases with miscellaneous causes (3.7±1.2 g versus 7.5 g) and spleen weight was lower in patients with lung immaturity (0.4±0.1 g versus 1.7 g). All results showed significant differences. CONCLUSIONS: This study demonstrates that variations in the weight of children and the weight of their organs are related to the types of cause of perinatal death. These data may contribute to a better interpretation of autopsy findings and their anatomical and clinical relationship. .


Assuntos
Feminino , Humanos , Recém-Nascido , Masculino , Peso Corporal , Causas de Morte , Morte Perinatal , Autopsia , Tamanho do Órgão
18.
Pathol Res Pract ; 208(12): 699-704, 2012 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-23102809

RESUMO

The aim this work was to compare the distribution of cellular phenotypes of the LF in the FVC to the ones in the subglottic region in pediatric autopsy, relating this distribution to age and different causes of death. We analyzed 60 larynges of newborns and children autopsied in the period from 1993 to 2003. The fragments were prepared in order to perform histochemical and immunohistochemical techniques. The morphological analysis showed cases that presented LF only in FVC (35%), LF only in the subglottic region (20%), lack of LF in FVC (30%) and lymphoid aggregates, which did not characterize an LF (15%). The cases of LF in the subglottic region were significantly younger compared to the ones that presented LF in the FVC (p=0.017). The LF in the subglottic region was bigger than the LF in the FVC (p=0.020). There was no significant difference between the cause of death and cellular phenotype for both FVC and the subglottic region. In conclusion, the cells that make up the LF in the FVC in newborns and children younger than one year have functional characteristics similar to LF cells in the subglottic region, suggesting that there are similarities with LALT.


Assuntos
Tecido Linfoide/anatomia & histologia , Prega Vocal/anatomia & histologia , Fatores Etários , Antígenos CD/metabolismo , Autopsia , Biomarcadores/metabolismo , Causas de Morte , Células Dendríticas Foliculares/citologia , Células Dendríticas Foliculares/metabolismo , Feminino , Humanos , Lactente , Recém-Nascido , Tecido Linfoide/metabolismo , Macrófagos/citologia , Macrófagos/metabolismo , Masculino , Infecções Respiratórias/imunologia , Infecções Respiratórias/mortalidade , Infecções Respiratórias/patologia , Linfócitos T/citologia , Linfócitos T/metabolismo , Prega Vocal/metabolismo
19.
Rev. bras. ginecol. obstet ; 34(4): 153-157, abr. 2012. ilus, graf, tab
Artigo em Português | LILACS | ID: lil-624743

RESUMO

OBJETIVO: Avaliar a influência das complicações maternas, da prematuridade, dos parâmetros antropométricos do feto e de condições do recém-nascido nos diferentes graus de corioamnionite. MÉTODOS: Foram analisadas 90 placentas provenientes de partos realizados no Hospital de Clinicas da Universidade Federal do Triângulo Mineiro com diagnóstico de inflamação no exame anatomopatológico. A revisão dos prontuários foi realizada para adquirir informações materno-fetais relevantes. A infecção foi classificada em: grau I - deciduíte; grau II - corioamnionite; grau III - corioamnionite e vasculite; grau IV - sepse neonatal; e grau V - morte fetal e pneumonite. RESULTADOS: Dentre as gestantes analisadas, 50,0% não apresentaram intercorrências, 15,0% apresentaram amniorrexe prematura, 15,0% infecção do trato urinário, 7,5% síndromes hipertensivas, 7,5% infecção transvaginal, 5,0% infecção hematogênica e 11,1% outras complicações. Mais da metade dos neonatos eram do sexo masculino e 72,2% a termo. Analisando o grau de corioamnionite, 56,7% apresentaram o grau I, 22,2 % grau II, 4,4% grau III, 10,0% grau IV e 6,7% grau V. Na análise estatística foi utilizado o Teste Χ2 para a análise de variáveis qualitativas e o Teste de Spearman para a análise das correlações. Os graus mais elevados de corioamnionite foram observados nos casos que apresentaram intercorrências maternas. Foram observadas correlações negativas entre todos os parâmetros fetais e o grau de corioamnionite, sendo significativa em relação ao peso, ao comprimento, à circunferência torácica e ao Apgar no primeiro e quinto minuto. CONCLUSÕES: Os diferentes padrões de corioamnionite estão relacionados a diferentes manifestações clínicas materno-fetais, influenciando nas condições de vida do recém-nascido e na gravidade de lesões morfológicas encontradas em natimortos.


PURPOSE: To evaluate the influence of maternal complications, prematurity, fetal anthropometric parameters and conditions of the newborn on different degrees of chorioamnionitis. METHODS: We analyzed 90 placentas from deliveries performed at the General Hospital of Triângulo Mineiro Federal University with a diagnosis of inflammation in the anatomopathological exams. We reviewed the medical records to obtain relevant maternal and fetal information. The infections were classified as grade I - deciduitis; grade II - chorioamnionitis; grade III - chorioamnionitis and vasculitis; grade IV - neonatal sepsis and grade V - fetal death and pneumonitis. RESULTS: Among the pregnant women analyzed, 50.0% had no complications, 15.0% had ruptured membranes, 15.0% urinary tract infection, 7.5% hypertensive disorders, 7.5% transvaginal infection, 5.0% hematogenous infection, and 11.1% other complications. More than a half the neonates were males and 72.2% were born at term. Analysis of the degree of chorioamnionitis showed that 56.7% had grade I, 22.2% grade II, 4.4% grade III, 10.0% grade IV, and 6.7% grade V. Data were analyzed statistically by the Χ2 test for qualitative variables and by the Spearman test for correlation analysis. The higher grades of chorioamnionitis were observed in cases of maternal complications. We observed negative correlations between all parameters and the degree of fetal chorioamnionitis, which were significant regarding weight, length, thoracic circumference and Apgar score in the first and fifth minutes. CONCLUSIONS: The different patterns of chorioamnionitis were related to different maternal and fetal clinical features, affecting the life conditions of the newborn and the severity of morphological lesions found in stillbirths.


Assuntos
Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Adulto Jovem , Corioamnionite/diagnóstico , Índice de Gravidade de Doença
20.
Fetal Pediatr Pathol ; 31(5): 265-72, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22432721

RESUMO

Problems with the foreskin are common reasons for pediatric surgery consultations. We collected the foreskin of 40 patients for 2 years and these samples were divided into groups with and without previous topical corticosteroid. We carried out histochemical hematoxylin & eosin and Picrosirius analyses of the foreskin. Collagen fibers and inflammatory infiltrate was higher in samples from patients who had complications related to phimosis. Fibrosis was higher in patients who used topical corticosteroid. A histopathologic study of the foreskin may provide an additional analysis of patients undergoing circumcision and it can also improve the accuracy of surgical indication.


Assuntos
Betametasona/administração & dosagem , Circuncisão Masculina , Prepúcio do Pênis/patologia , Glucocorticoides/administração & dosagem , Fimose/tratamento farmacológico , Administração Tópica , Adolescente , Criança , Pré-Escolar , Colágeno/metabolismo , Fibrose/induzido quimicamente , Fibrose/patologia , Prepúcio do Pênis/metabolismo , Humanos , Masculino , Fimose/etiologia , Fimose/cirurgia , Estudos Retrospectivos
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