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1.
J Med Virol ; 92(2): 241-250, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31579937

RESUMO

BACKGROUND: Active infections of human herpesvirus 6B (HHV-6B) are frequent in immunocompromised recipients after transplantation. Nevertheless, they need to be distinguished from latent inherited chromosomally integrated genomes (iciHHV-6) present in about 1% of the population to avoid unnecessary administration of toxic antivirals. METHODS: A 5-year-old child presented with acute liver allograft rejection associated with HHV-6 DNA in plasma, which led to an unfavorable outcome. We investigated the possibility of HHV-6 infection derived from an iciHHV-6 present in the donor's liver using molecular and histopathology studies in various tissues, including quantification of HHV-6 DNA, genotyping, sequencing for antiviral resistance genes, relative quantification of viral transcripts, and detection of gB and gH viral proteins. RESULTS: The presence of iciHHV-6B was evidenced in the donor with signs of reactivation in the gallbladder and transplanted liver (detection of HHV-6B mRNA and late proteins). This localized expression could have played a role in liver rejection. Low viral loads in the recipient's plasma, with identical partial U39 sequences, were in favor of viral DNA released from the transplanted liver rather than a systemic infection. CONCLUSIONS: Determination of iciHHV-6 status before transplantation should be considered to guide clinical decisions, such as antiviral prophylaxis, viral load monitoring, and antiviral therapy.


Assuntos
Rejeição de Enxerto/virologia , Falência Hepática/virologia , Infecções por Roseolovirus/diagnóstico , Aloenxertos/virologia , Pré-Escolar , Cromossomos Humanos/genética , Cromossomos Humanos/virologia , DNA Viral/sangue , Evolução Fatal , Rejeição de Enxerto/diagnóstico , Herpesvirus Humano 6/genética , Herpesvirus Humano 6/isolamento & purificação , Humanos , Padrões de Herança , Falência Hepática/diagnóstico , Transplante de Fígado , Infecções por Roseolovirus/virologia , Integração Viral
2.
Pediatr Radiol ; 49(9): 1163-1170, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31004186

RESUMO

BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies. OBJECTIVE: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis. MATERIALS AND METHODS: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018. Two pediatric radiologists evaluated the chest imaging studies for the presence or absence of published radiologic findings of PPFE in adults, including platythorax, pneumothorax, upper lobe predominant pleural and septal thickening, and bronchiectasis. Platythorax indices were calculated from the normal chest CT exams of eight age- and gender-matched individuals obtained via the radiology search engine. RESULTS: The mean presentation age was 12.9 years (range: 7-16 years). Seven of the eight had a history of chemotherapy and radiation therapy for cancer. Three of the eight had undergone bone marrow transplantation and none had undergone lung transplantation. The mean time between chemotherapy, radiation therapy, and/or bone marrow transplantation and the presentation of PPFE was 8.4 years (range: 5.6-12.1 years). Most of the patients presented with dyspnea (63%), cough (50%) and/or pneumothorax (38%). The mean percentage of predicted FEV1 (forced expiratory volume in one second) was 14.1 (range: 7.7-27.5). All eight patients demonstrated platythorax, bronchiectasis, pleural and septal thickening (upper lobes in four, upper and lower lobes in four) and six had pneumothorax. Five underwent lung biopsies, four of whom developed pneumothoraces. CONCLUSION: Clinical and radiologic findings of pediatric PPFE are similar to those in adults, although a majority of the former have a history of treated cancer. Clinical presentation of restrictive lung disease, dyspnea, cough or spontaneous pneumothorax years after treatment for childhood cancer combined with platythorax, upper lobe pleural and septal thickening and traction bronchiectasis on chest CT establishes a presumptive diagnosis of PPFE.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/etiologia , Tomografia Computadorizada por Raios X , Adolescente , Transplante de Medula Óssea , Criança , Pré-Escolar , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Masculino , Neoplasias/tratamento farmacológico , Neoplasias/radioterapia , Testes de Função Respiratória
3.
Med Teach ; 41(7): 746-749, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30032720

RESUMO

Emotional intelligence (EI) is the ability to recognize, understand, and manage emotions in yourself and in others. EI has long been recognized as a critical component for individual and organizational success within the business realm, and there is emerging evidence that enhancing EI is equally important in the medical setting. EI can improve interpersonal communications, enable constructive conflict resolution, and promote a culture of professionalism. As healthcare becomes increasingly team-based, proficiency in EI will be required to build consensus among multidisciplinary stakeholders, and effect change in attitudes and behaviors that result in improved patient safety and clinical outcomes. Based on the existing literature and the authors' experiences, these 12 tips provide practical suggestions on how to introduce EI into a medical curriculum. These tips have broad applicability, and can be implemented in courses on topics such as professionalism, leadership development, empathy, patient safety, or wellness.


Assuntos
Educação Médica/organização & administração , Inteligência Emocional , Atitude do Pessoal de Saúde , Feedback Formativo , Processos Grupais , Humanos , Equipe de Assistência ao Paciente/organização & administração , Aprendizagem Baseada em Problemas/organização & administração , Autoavaliação (Psicologia) , Ensino/organização & administração
4.
Pediatr Nephrol ; 33(6): 1089-1092, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29546599

RESUMO

BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab. Both patients received two doses of rituximab and responded with remission. In addition, anti-PLA2R antibody levels normalized and/or significantly improved. CONCLUSIONS: Rituximab seems to be a safe and effective treatment option in children with idiopathic membranous nephropathy due to anti-PLA2R. Further studies are needed to evaluate this effectiveness.


Assuntos
Glomerulonefrite Membranosa/tratamento farmacológico , Imunossupressores/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Rituximab/uso terapêutico , Adolescente , Feminino , Humanos , Rim/patologia , Masculino , Receptores da Fosfolipase A2/imunologia
5.
J Pediatr Gastroenterol Nutr ; 65(4): 364-369, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28945205

RESUMO

Recurrent bile salt export pump (rBSEP) disease has been reported in progressive familial intrahepatic cholestasis type 2 (PFIC2) patients following liver transplantation (LT) and is often refractory to standard anti-cellular rejection immunosuppressants. The mechanism of rBSEP disease is proposed to be a form of type II hypersensitivity reaction with de novo anti-BSEP antibodies blocking the function of allograft BSEP. Utilization of C4d has not been evaluated in rBSEP. We describe a girl with 3 episodes of rBSEP with severe pruritus at 8.9, 10.3, and 11.0 years post-LT, respectively. Patient's serum reacted with normal liver canaliculi by indirect immunofluorescence (IF), whereas patient's liver showed canalicular immunoglobulin G deposition. The histologic features of all 3 liver biopsies recapitulate PFIC2 with cholestatic giant cell hepatitis. Canalicular BSEP expression was not detected in areas of feathery degeneration by immunohistochemistry, but was retained in morphologically normal liver. By direct IF, C4d showed diffuse sinusoidal staining in the third biopsy. Patient responded well to rituximab with or without intravenous immunoglobulin with subsiding symptoms and normalization of serum bile acid levels. In conclusion, rBSEP disease should be considered in the differential diagnosis when evaluating for rejection in a PFIC2 patient post-LT presenting with pruritus. A portion of liver core may be snap frozen in OCT medium for possible direct IF for C4d, that can serve as a surrogate marker for complement activation and antibody-mediated graft dysfunction.


Assuntos
Membro 11 da Subfamília B de Transportadores de Cassetes de Ligação de ATP/metabolismo , Colestase Intra-Hepática/diagnóstico , Rejeição de Enxerto/imunologia , Transplante de Fígado/efeitos adversos , Fígado/patologia , Colestase Intra-Hepática/tratamento farmacológico , Colestase Intra-Hepática/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Lactente , Rituximab/uso terapêutico
6.
Pediatr Nephrol ; 32(12): 2283-2291, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28717937

RESUMO

BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America. Renal outcomes were obtained using consensus definitions from the Childhood Arthritis and Rheumatology Research Alliance (CARRA). Logistic regression was used to detect predictors of complete renal response. RESULTS: A total of 56 children with MLN were identified (82% females, 44% black, 35% Hispanic) with a median follow-up time of 4.1 years. The mean age of MLN onset was 13.7 ± 3.4 years. On initial presentation 69% had nephrotic syndrome and 11% had acute kidney injury. Glucocorticoids were prescribed in 96% of patients and anti-malarials in 88%. Mycophenolate mofetil was the most common non-steroid immunosuppressive agent (69%), followed by rituximab (25%), cyclophosphamide (18%), and azathioprine (9%). Renin-angiotensin aldosterone system blocking agents were prescribed in 78% of patients. Of 37 patients with ≥2 years of follow-up, 74% achieved complete renal response at 24 months. No predictor variable of complete renal response was identified in this small cohort. Renal flares occurred in 48% of patients (86% proteinuric, 14% nephritic). On subsequent renal biopsy, 13% patients had developed proliferative nephritis. CONCLUSIONS: This single-center cohort of childhood-onset MLN showed favorable outcomes. Utilizing pediatric renal outcomes definitions, we found that response rates were high, as were rates of renal flare.


Assuntos
Glomerulonefrite Membranosa/tratamento farmacológico , Rim/patologia , Nefrite Lúpica/tratamento farmacológico , Adolescente , Criança , Estudos de Coortes , Feminino , Seguimentos , Glomerulonefrite Membranosa/diagnóstico , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Modelos Logísticos , Nefrite Lúpica/diagnóstico , Masculino , Estudos Retrospectivos , Texas , Resultado do Tratamento
7.
Am J Hum Genet ; 92(6): 996-1000, 2013 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-23731537

RESUMO

Infantile myofibromatosis (IM) is the most common benign fibrous tumor of soft tissues affecting young children. By using whole-exome sequencing, RNA sequencing, and targeted sequencing, we investigated germline and tumor DNA in individuals from four distinct families with the familial form of IM and in five simplex IM cases with no previous family history of this disease. We identified a germline mutation c.1681C>T (p.Arg561Cys) in platelet-derived growth factor receptor ß (PDGFRB) in all 11 affected individuals with familial IM, although none of the five individuals with nonfamilial IM had mutations in this gene. We further identified a second heterozygous mutation in PDGFRB in two myofibromas from one of the affected familial cases, indicative of a potential second hit in this gene in the tumor. PDGFR-ß promotes growth of mesenchymal cells, including blood vessels and smooth muscles, which are affected in IM. Our findings indicate p.Arg561Cys substitution in PDGFR-ß as a cause of the dominant form of this disease. They provide a rationale for further investigations of this specific mutation and gene to assess the benefits of targeted therapies against PDGFR-ß in aggressive life-threatening familial forms of the disease.


Assuntos
Mutação de Sentido Incorreto , Miofibromatose/congênito , Receptor beta de Fator de Crescimento Derivado de Plaquetas/genética , Sequência de Aminoácidos , Feminino , Genes Dominantes , Estudos de Associação Genética , Mutação em Linhagem Germinativa , Heterozigoto , Humanos , Masculino , Modelos Moleculares , Miofibromatose/genética , Linhagem , Estrutura Terciária de Proteína , Receptor Notch3 , Receptor beta de Fator de Crescimento Derivado de Plaquetas/química , Receptores Notch/genética , Análise de Sequência de DNA
8.
J Clin Immunol ; 36(4): 377-387, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-27048656

RESUMO

Inherently defective immunity typically results in either ineffective host defense, immune regulation, or both. As a category of primary immunodeficiency diseases, those that impair immune regulation can lead to autoimmunity and/or autoinflammation. In this review we focus on one of the most recently discovered primary immunodeficiencies that leads to immune dysregulation: "Copa syndrome". Copa syndrome is named for the gene mutated in the disease, which encodes the alpha subunit of the coatomer complex-I that, in aggregate, is devoted to transiting molecular cargo from the Golgi complex to the endoplasmic reticulum (ER). Copa syndrome is autosomal dominant with variable expressivity and results from mutations affecting a narrow amino acid stretch in the COPA gene-encoding COPα protein. Patients with these mutations typically develop arthritis and interstitial lung disease with pulmonary hemorrhage representing a striking feature. Immunologically Copa syndrome is associated with autoantibody development, increased Th17 cells and pro-inflammatory cytokine expression including IL-1ß and IL-6. Insights have also been gained into the underlying mechanism of Copa syndrome, which include excessive ER stress owing to the impaired return of proteins from the Golgi, and presumably resulting aberrant cellular autophagy. As such it represents a novel cellular disorder of intracellular trafficking associated with a specific clinical presentation and phenotype.


Assuntos
Proteína Coatomer/genética , Síndromes de Imunodeficiência , Artrite/genética , Artrite/imunologia , Artrite/patologia , Humanos , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/patologia , Nefropatias/genética , Nefropatias/imunologia , Nefropatias/patologia , Pneumopatias/genética , Pneumopatias/imunologia , Pneumopatias/patologia
10.
J Pediatr Hematol Oncol ; 34(7): e304-7, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22935665

RESUMO

Sinonasal teratocarcinosarcoma is an extremely rare and aggressive neoplasm with <100 cases described in the literature and only 3 reported pediatric cases. We describe a 15-year-old male with a case of sinonasal teratocarcinosarcoma with extension to the cribriform plate. He presented with epistaxis, nasal obstruction, and frontal headaches and was treated with resection and postoperative chemoradiation. He is currently doing well without evidence of recurrence 45 months after therapy.


Assuntos
Carcinossarcoma/patologia , Neoplasias Nasais/patologia , Neoplasias dos Seios Paranasais/patologia , Teratoma/patologia , Adolescente , Carcinossarcoma/terapia , Humanos , Masculino , Neoplasias Nasais/terapia , Neoplasias dos Seios Paranasais/terapia , Teratoma/terapia
11.
Ultrastruct Pathol ; 36(1): 1-18, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22292732

RESUMO

The detection of microscopic hematuria in a child's urine prompts evaluation for renal and urinary bladder causes. Microscopic hematuria identified during a routine physical examination by the pediatrician is much more common than macroscopic hematuria. Persistent microscopic hematuria is particularly worrisome and may require a percutaneous needle core kidney biopsy to determine whether the etiology is secondary to glomerular disease, tubulointerstitial disease, urinary tract infection, urinary tract structural abnormalities, medications, or toxins. This paper reviews the epidemiology, pathologic features, pathogenesis, treatment, and outcome of familial hematuria (Alport syndrome [hereditary nephritis]), thin basement membrane nephropathy), IgA nephropathy, Henoch-Schönlein purpura, and acute postinfectious glomerulonephritis.


Assuntos
Hematúria/diagnóstico , Hematúria/etiologia , Hematúria/terapia , Nefropatias/complicações , Nefropatias/diagnóstico , Nefropatias/terapia , Adolescente , Criança , Humanos
12.
PLoS One ; 17(2): e0264130, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35213596

RESUMO

The global COVID-19 pandemic has highlighted the need for rapid, accurate and accessible nucleic acid tests to enable timely identification of infected individuals. We optimized a sample-to-answer nucleic acid test for SARS-CoV-2 that provides results in <1 hour using inexpensive and readily available reagents. The test workflow includes a simple lysis and viral inactivation protocol followed by direct isothermal amplification of viral RNA using RT-LAMP. The assay was validated using two different instruments, a portable isothermal fluorimeter and a standard thermocycler. Results of the RT-LAMP assay were compared to traditional RT-qPCR for nasopharyngeal swabs, nasal swabs, and saliva collected from a cohort of patients hospitalized due to COVID-19. For all three sample types, positive agreement with RT-LAMP performed using the isothermal fluorimeter was 100% for samples with Ct <30 and 69-91% for samples with Ct <40. Following validation, the test was successfully scaled to test the saliva of up to 400 asymptomatic individuals per day as part of the campus surveillance program at Rice University. Successful development, validation, and scaling of this sample-to-answer, extraction-free real-time RT-LAMP test for SARS-CoV-2 adds a highly adaptable tool to efforts to control the COVID-19 pandemic, and can inform test development strategies for future infectious disease threats.


Assuntos
Teste para COVID-19 , COVID-19/diagnóstico , Nasofaringe/virologia , Nariz/virologia , Vigilância da População/métodos , SARS-CoV-2/isolamento & purificação , Saliva/virologia , COVID-19/virologia , Humanos , Técnicas de Diagnóstico Molecular/métodos , Técnicas de Amplificação de Ácido Nucleico/métodos , RNA Viral/genética , Sensibilidade e Especificidade
13.
J Pediatric Infect Dis Soc ; 10(5): 599-606, 2021 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-33491073

RESUMO

BACKGROUND: Distinctions between HHV-6 primary infection in seronegative patients and HHV-6 reactivation in seropositive patients remains largely undescribed in pediatric liver transplant (LT) recipients. METHODS: We implemented pretransplant serology testing of HHV-6 in a large pediatric hospital and retrospectively assessed the incidence, manifestations and outcomes of HHV-6 infections over a 3-year period. RESULTS: Among 101 pediatric LT recipients, 96 had pretransplant HHV-6 serologies; 34 (35.4%) were seronegative and 62 (64.6%) seropositive. Posttransplantation, 8/25 (32%) seronegative patients had HHV-6 DNAemia (primary infection) compared to 2/48 (4%) seropositive patients (p=0.002). Compared to seropositive patients, seronegative patients with HHV-6 DNAemia were younger, and had symptoms of fever and/or elevated aminotransferases in association with higher viral loads, in the first month post-transplant. More than 90% of seronegative patients and 77.8% of seropositive patients had HHV-6 detected by PCR in liver biopsy obtained for concerns of allograft rejection, but most had no detectable concomitant DNAemia. Active replication of virus in the liver was confirmed by in situ hybridization in select cases. While HHV-6 infection occurred among patients on prophylaxis doses of antivirals for CMV, HHV-6 DNAemia and presenting symptoms resolved on treatment doses. CONCLUSIONS: HHV-6 DNA-emia occurred more frequently in seronegative pediatric LT recipients, usually in the early posttransplant period, and was subsequently detected in allograft biopsies. HHV-6 cannot be ruled out as a cause of hepatitis in the absence of allograft tissue testing and specialized virological assays, as HHV-6 may disrupt local allograft immune homeostasis while evading traditional screening methods using blood or plasma. The assessment of pre-transplant HHV-6 serological status may be important for risk stratification and post-transplant management of pediatric LT recipients.


Assuntos
Herpesvirus Humano 6 , Transplante de Fígado , Criança , DNA Viral , Feminino , Humanos , Incidência , Estudos Retrospectivos
14.
Pediatr Blood Cancer ; 54(7): 1041-4, 2010 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-20162687

RESUMO

We describe a male with a large abdominal mass, most likely originating from the liver, with capsule rupture and tumor dissemination into the abdominal cavity. Adherence of the tumor to the diaphragm and lower right colon also were noted. A comprehensive evaluation of the mass revealed no tumor-defining histopathologic, immunocytochemical, ultrastructural, cytogenetic, or translocation features. The malignant tumor was found to have a novel translocation (X;19)(q13;13.3), which has not been reported in small round cell tumors of childhood or adults. The final diagnosis rendered was an undifferentiated small round cell tumor of uncertain cell of origin.


Assuntos
Neoplasias Abdominais/genética , Neoplasias Abdominais/patologia , Carcinoma de Células Pequenas/genética , Carcinoma de Células Pequenas/patologia , Cromossomos Humanos Par 19/genética , Cromossomos Humanos X/genética , Neoplasias Abdominais/metabolismo , Carcinoma de Células Pequenas/metabolismo , Criança , Humanos , Imuno-Histoquímica , Masculino , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Translocação Genética
15.
Pediatr Nephrol ; 25(3): 535-8, 2010 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-19956975

RESUMO

Pulmonary renal syndromes are unusual, but frequently life-threatening manifestations of a distinct group of disorders in the pediatric age group. Although IgA nephropathy is a common cause of hematuria, it is an extremely rare cause of pulmonary renal syndrome, causing high mortality, and has mostly been reported in adult patients. We describe the youngest patient with this presentation reported to date, a 14-year-old male, who presented with end stage renal disease and pulmonary hemorrhage and was found to have IgA nephropathy by renal biopsy and pulmonary capillaritis by open lung biopsy. His lung disease was successfully treated with immunosuppressive medications. Despite this being a rare manifestation of IgA nephropathy, clinicians need to be aware of this presentation as it is potentially fatal, but amenable to aggressive immunosuppression.


Assuntos
Injúria Renal Aguda/complicações , Glomerulonefrite por IGA/complicações , Hemorragia/complicações , Pneumopatias/complicações , Injúria Renal Aguda/diagnóstico por imagem , Injúria Renal Aguda/patologia , Adolescente , Anti-Inflamatórios/uso terapêutico , Biópsia , Glomerulonefrite por IGA/diagnóstico por imagem , Glomerulonefrite por IGA/patologia , Hemorragia/diagnóstico por imagem , Hemorragia/patologia , Humanos , Rim/patologia , Pulmão/patologia , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Masculino , Metilprednisolona/uso terapêutico , Alvéolos Pulmonares/patologia , Diálise Renal , Tomografia Computadorizada por Raios X
17.
Pediatr Nephrol ; 24(6): 1187-92, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19219463

RESUMO

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children's Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.


Assuntos
Imunoglobulina M/análise , Nefrose Lipoide/patologia , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/imunologia , Síndrome Nefrótica/patologia , Biópsia , Quimioterapia Adjuvante , Criança , Pré-Escolar , Estudos de Coortes , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Feminino , Imunofluorescência , Mesângio Glomerular/imunologia , Mesângio Glomerular/patologia , Humanos , Imunossupressores/uso terapêutico , Rim/cirurgia , Masculino , Nefrose Lipoide/tratamento farmacológico , Síndrome Nefrótica/tratamento farmacológico , Estudos Retrospectivos , Prevenção Secundária , Resultado do Tratamento
18.
Pediatr Dermatol ; 26(3): 323-7, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19706097

RESUMO

In the past pyogenic granuloma (PG) in infancy has been easily confused clinically and histopathologically with infantile hemangioma (IH). In 2000 North and colleagues discovered that IH is immunopositive for GLUT-1, a glucose transporter which is also expressed in placental capillaries (1). GLUT-1 staining is negative in pyogenic granuloma. We report two newborns with congenital disseminated PG who were otherwise healthy. In both of these cases, negative GLUT-1 staining supported the proper diagnosis.


Assuntos
Granuloma Piogênico/congênito , Feminino , Granuloma Piogênico/patologia , Humanos , Lactente , Recém-Nascido , Masculino
19.
Pediatr Clin North Am ; 66(1): 87-99, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30454753

RESUMO

Childhood-onset systemic lupus erythematosus (SLE) is a subset of SLE with an onset before 18 years of age. Patients with early onset SLE tend to have a greater genetic component to their disease cause, more multisystemic involvement, and a more severe disease course, which includes greater risks for developing nephritis and end-stage kidney disease. Five- and 10-year mortality is lower than in adult-onset SLE. Although patient and renal survival have improved with advances in induction and maintenance immunosuppression, accumulation of irreversible damage is common. Cardiovascular and infectious complications are frequent, as are relapses during adolescence and the transition to adulthood.


Assuntos
Corticosteroides/uso terapêutico , Imunossupressores/uso terapêutico , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/tratamento farmacológico , Biópsia , Criança , Diagnóstico Diferencial , Humanos , Nefrite Lúpica/patologia
20.
J Clin Virol ; 43(1): 66-72, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18539521

RESUMO

BACKGROUND: BK virus (BKV), JC virus (JCV) and simian virus 40 (SV40) are nonenveloped DNA viruses, members of the family Polyomaviridae. BK and JC viruses establish persistent infections in humans, and evidence suggests that SV40 can infect humans, as well. Whether persistence occurs in the lymphoid system is unknown. METHODS: Paraffin-embedded tonsils from 220 immunocompetent children (mean age 9.3 years) were examined by polymerase chain reaction (PCR) to detect viral DNA of BKV, JCV, SV40, and Epstein-Barr virus (EBV). RESULTS: Polyomavirus-specific DNA sequences were detected in 8.3% (29/351) of specimens collected from 220 children. Twenty-one (9.5%) children had polyomavirus DNA present in at least one tonsil, with sequences identified as SV40 (n=20) and BKV (n=1). Polyomavirus JCV was not detected. Among patients positive for SV40, 8 of 14 (57%) contained viral DNA in both available tonsils. EBV DNA was detected in 99 (28.2%) samples from 67 (30.5%) patients. Eleven samples (3.1%) from 8 (3.6%) children were positive for both polyomavirus and EBV. SV40-positive children were significantly older than the SV40-negative subjects (P<0.001). T-antigen expression was detected in an SV40 DNA-positive tonsil by immunohistochemistry. CONCLUSIONS: These results suggest that SV40 can infect tonsils, that lymphoid tissue may represent a site for polyomavirus persistence, and that immunohistochemistry is not a useful detection assay when there are very few virus-infected cells in a tissue.


Assuntos
Tonsila Palatina/virologia , Doenças Faríngeas/diagnóstico , Infecções por Polyomavirus/diagnóstico , Vírus 40 dos Símios/isolamento & purificação , Infecções Tumorais por Vírus/diagnóstico , Adolescente , Antígenos Transformantes de Poliomavirus/genética , Antígenos Transformantes de Poliomavirus/isolamento & purificação , Criança , Pré-Escolar , Feminino , Herpesvirus Humano 4/isolamento & purificação , Humanos , Imunocompetência , Masculino , Tonsila Palatina/patologia , Doenças Faríngeas/virologia , Reação em Cadeia da Polimerase , Infecções por Polyomavirus/virologia , Vírus 40 dos Símios/genética
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