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1.
Paediatr Drugs ; 22(6): 645-652, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32885390

RESUMO

Kawasaki disease (KD) is one of the most common vasculitides of childhood and the main cause of acquired heart disease in developed countries. Intravenous immunoglobulin (IVIG) in association with aspirin represents the main treatment for KD. However, 10-20% of patients fail to respond to standard treatment and have an increased risk of cardiac complications. There is currently no accepted protocol for treatment of resistant cases. Several authors highlighted the role of interleukin-1 (IL-1) as a mediator of inflammation in KD and suggested the possibility of using IL-1 or its receptor as a target of therapy. The use of IL-1 inhibitors in patients with KD has been reported in the scientific literature, but data are largely limited to individual case reports and small case series. We summarized the scientific literature related to the use of anakinra, analyzing preclinical and clinical data. Thirty-eight patients have been described so far, most of them with KD-related complications. Twenty-two were described in case reports and case series, while 16 were patients from the completed KAWAKINRA phase IIa study. Almost all patients received clinical benefit, and no relevant side effects were noted. Based on this evidence, in our opinion, anakinra may be considered as an option after the failure of the first IVIG infusion, especially in patients with coronary involvement.

2.
Clin Exp Rheumatol ; 38(6): 1255-1262, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32828142

RESUMO

OBJECTIVES: Chronic non-bacterial osteomyelitis (CNO) is a rare non-infectious bone inflammatory disorder; when multifocal, it is referred to as Chronic Recurrent Multifocal Osteomyelitis (CRMO). This study evaluates the demographic, clinical and radiological characteristics of a multi-centre cohort of patients with CNO/CRMO. METHODS: Demographic and clinical data of patients with an established diagnosis of CNO/CRMO followed at paediatric rheumatology centres across Europe (Italy, France, Slovenia) and India were retrospectively collected. RESULTS: There were no demographic differences across countries, but time to diagnosis was significantly longer in India (p=0.041). Pain was almost invariably present at disease onset; functional impairment was more frequent among Italian and Slovenian patients (p=0.001). The number of sites of bone involvement was similar between genders and countries, with long bone metaphises being the most common site. Raised acute phase reactants, detected in >50% of patients, were not associated with clinical manifestations or response to treatment. Comorbidities, evinced in 37% of patients, were equally distributed between genders and nationalities. Imaging approach was similar across countries, without any association between radiological findings and clinical manifestations. NSAIDs were almost invariably used as first-line treatment, but response rate was significantly lower in Italy (p=0.02). Methotrexate was used in 28% of case, with an overall rate of response of 82%. Health conditions and rate of permanent deformities were similar across different countries. CONCLUSIONS: The differences in clinical presentation, radiological features and response to treatment described in this multinational cohort of CNO/CRMO might provide novel insights into this still elusive disease.

3.
Pediatr Rheumatol Online J ; 18(1): 55, 2020 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-32650789

RESUMO

BACKGROUND: FBLIM1 gene has been recently demonstrated to be involved in the pathogenesis of bone sterile inflammation. The aim of the study is to evaluate the prevalence of FBLIM1 gene variants in a cohort of 80 Italian patients with Chronic Non-bacterial Osteomyelitis (CNO). METHODS: The coding regions of FBLIM1 gene were sequenced in a cohort of 80 patients with CNO using DNA extracted from blood lymphocytes, and PCR products were sequenced. Only rare (global MAF < 2%), coding variants detected were considered. Clinical evaluation of patients with rare variants and those without was performed. Fisher's exact test was used to compare categorical and ordinal data, and Student's t-test was used to analyze continuous data. RESULTS: Eighteen out of 80 patients (~ 22%) presented at least one rare coding variant in FBLIM1. Eight patients presented a variant never associated before with CNO. All patients presented classical features of CNO and no statistical difference between patients with presence of FBLMI1 variants and those without were found in terms of clinical manifestation, treatment, and outcome. CONCLUSION: Considering the high frequency of rare variants in our CNO cohort, our data seem to confirm a possible role of FBLIM1 in the pathogenesis of CNO suggesting that CNO is a disorder of chronic inflammation and imbalanced bone remodeling.

4.
Clin Exp Rheumatol ; 38(2): 366-369, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31994478

RESUMO

OBJECTIVES: Chronic non-bacterial osteomyelitis (CNO) is a non-infectious inflammatory disease characterised by uni- or multi-focal bone lytic lesions. CNO mainly affects metaphysis of long bones, pelvis and shoulder girdle. Neurocranium lesions are extremely rare. The objective of the study is to describe the prevalence and clinical manifestations of CNO patients with neurocranium involvement in an Italian cohort of CNO patients. METHODS: This is a retrospective study. Medical records of patients with CNO admitted to eight paediatric rheumatology centres were reviewed. RESULTS: Among 86 patients with CNO enrolled in the study, three of them were female and presented neurocranium involvement - multifocal lesions. Two out of the 3 patients were completely asymptomatic for cranial involvement, while one of the 3 complained of cranial bossing. Cranial involvement was detected with bone scintigraphy and then confirmed by magnetic resonance imaging and/or computed tomography. Two patients presented fever and two with skin manifestations. Laboratory inflammatory markers were increased in two of them. All patients underwent bone biopsy confirming the diagnosis. They all received NSAIDs. Two patients received corticosteroids and then methotrexate and achieved clinical remission, while one patient received pamidronate. CONCLUSIONS: This is the first report of neurocranium involvement in a cohort of patients affected by CNO. In our cohort no patient showed significant signs attributable to cranial involvement.


Assuntos
Encéfalo/anormalidades , Osteomielite , Crânio/anormalidades , Cefalometria , Criança , Doença Crônica , Estudos de Coortes , Feminino , Humanos , Itália , Masculino , Osteomielite/complicações , Prevalência , Estudos Retrospectivos
5.
J Pediatr ; 218: 228-230.e1, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31748120

RESUMO

We describe 10 children with Alagille syndrome and inflammatory arthritis. In our centers, the prevalence of chronic arthritis in patients with Alagille syndrome is approximately 50 times higher compared with the general population. Arthritis was refractory to most treatment. Patients with Alagille syndrome should routinely be screened for musculoskeletal symptoms.


Assuntos
Síndrome de Alagille/complicações , Síndrome de Alagille/diagnóstico , Artrite Juvenil/complicações , Artrite Juvenil/diagnóstico , Adolescente , Síndrome de Alagille/epidemiologia , Artrite Juvenil/epidemiologia , Criança , Pré-Escolar , Doença Crônica , Meios de Contraste , Feminino , Humanos , Inflamação , Transplante de Fígado , Imagem por Ressonância Magnética , Masculino , Pediatria , Estudos Retrospectivos , Reumatologia , Inquéritos e Questionários , Punho/diagnóstico por imagem
6.
Isr Med Assoc J ; 21(7): 475-479, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31507124

RESUMO

BACKGROUND: Uveitis is an inflammatory disorder of the uveal tract of the eye that can affect both adults and children. Non-infectious uveitis can be an expression of a systemic autoimmune condition, or it can be idiopathic. It is a serious disease, associated with possible severe complications leading to visual impairment and blindness. For this reason, a prompt diagnosis and assessment of an appropriate treatment, with the collaboration of specialists such as ophthalmologists and rheumatologists, are extremely important. Many treatment options may be associated to side effects; therefore, clinicians should follow a stepladder approach starting with the least aggressive treatments to induce remission of inflammation. In this review, we reported the current evidence-based treatments for non-infectious uveitis in pediatric and adult patients with particular attention to the biologic response modifier treatment options. Important multicenter studies have demonstrated the efficacy of adalimumab, both in adults (VISUAL I, VISUAL II, VISUAL III) and in children (SYCAMORE, ADJUVITE), while for other agents data are still scarce.


Assuntos
Adalimumab/administração & dosagem , Fatores Imunológicos/administração & dosagem , Uveíte/tratamento farmacológico , Adulto , Criança , Comportamento Cooperativo , Medicina Baseada em Evidências , Humanos , Inflamação/tratamento farmacológico , Uveíte/diagnóstico , Uveíte/patologia
7.
Int J Mol Sci ; 20(2)2019 Jan 21.
Artigo em Inglês | MEDLINE | ID: mdl-30669566

RESUMO

Corticosteroids are the mainstay of therapy for many pediatric disorders and sometimes are life-saving. Both endogenous and synthetic derivatives diffuse across the cell membrane and, by binding to their cognate glucocorticoid receptor, modulate a variety of physiological functions, such as glucose metabolism, immune homeostasis, organ development, and the endocrine system. However, despite their proved and known efficacy, corticosteroids show a lot of side effects, among which growth retardation is of particular concern and specific for pediatric age. The aim of this review is to discuss the mechanism of action of corticosteroids, and how their genomic effects have both beneficial and adverse consequences. We will focus on the use of corticosteroids in different pediatric subspecialties and most common diseases, analyzing the most recent evidence.


Assuntos
Glucocorticoides/farmacologia , Glucocorticoides/uso terapêutico , Adolescente , Fatores Etários , Anti-Inflamatórios/farmacologia , Anti-Inflamatórios/uso terapêutico , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Terapia de Reposição Hormonal , Humanos , Fatores Imunológicos/farmacologia , Fatores Imunológicos/uso terapêutico , Lactente , Recém-Nascido , Receptores de Glucocorticoides/metabolismo , Resultado do Tratamento
8.
Pediatr Rheumatol Online J ; 16(1): 46, 2018 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-29996864

RESUMO

BACKGROUND: Conventional pharmacological therapies for the treatment of juvenile idiopathic arthritis (JIA) consist of non-biological, disease-modifying antirheumatic drugs, among which methotrexate (MTX) is the most commonly prescribed. However, there is a lack of consensus-based clinical and therapeutic recommendations for the use of MTX in the management of patients with JIA. Therefore, the Methotrexate Advice and RecommendAtions on Juvenile Idiopathic Arthritis (MARAJIA) Expert Meeting was convened to develop evidence-based recommendations for the use of MTX in the treatment of JIA. METHODS: The preliminary executive committee identified a total of 9 key clinical issues according to the population, intervention, comparator, outcome (PICO) approach, and performed an evidence-based, systematic, literature review. During the subsequent Expert Meeting, the relevant evidence was assessed and graded, and 10 recommendations were made. RESULTS: Recommendations relating to the efficacy, optimal dosing and route of administration and duration of treatment with MTX in JIA, and to the issue of folic acid supplementation to prevent MTX side effects, use of MTX in the treatment of chronic JIA-associated uveitis, combination treatment with biologic agents, and the use of vaccinations in patients with JIA were developed. The selected topics were considered to represent clinically important issues facing clinicians caring for patients with JIA. Evidence was insufficient to formulate recommendations for the use of biomarkers predictive of treatment response. CONCLUSIONS: These consensus recommendations provide balanced and evidence-based recommendations designed to have broad value for physicians and healthcare clinicians involved in the clinical management of patients with JIA.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Metotrexato/uso terapêutico , Consenso , Humanos , Guias de Prática Clínica como Assunto
9.
Ital J Pediatr ; 44(1): 74, 2018 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-29954416

RESUMO

BACKGROUND: Sarcoidosis in pediatric age is uncommon and challenging diagnosis, because manifestations can be significantly variable and non-specific since it is a multisystem disease, and virtually any organ system may be involved. CASE PRESENTATION: In this report, we describe the case of a 12-year-old girl presenting with fatigue and weight loss, with a painless hepato-splenomegaly without additional clinical signs on physical examination. In our patient, once we had ruled out infections, malignancies and granulomatous diseases of childhood, we made diagnosis of sarcoidosis, finding suggestive histological features in two different tissues (liver and lymph nodes) with lung involvement. CONCLUSIONS: Our case points out that pediatricians should consider sarcoidosis in the differential diagnosis in case of systemic symptoms, even in absence of other specific clinical clues, because they represent the most common clinical manifestations on presentation in children, in order to refer promptly the young patient to specialist evaluation.


Assuntos
Hepatopatias/diagnóstico , Fígado/diagnóstico por imagem , Sarcoidose/diagnóstico , Biópsia , Criança , Diagnóstico Diferencial , Feminino , Humanos , Imagem por Ressonância Magnética , Doenças Raras
10.
J Rheumatol ; 45(10): 1418-1421, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29907671

RESUMO

OBJECTIVE: This study aimed to test fecal calprotectin (FC) as a screening tool to identify inflammatory bowel disease (IBD) among patients with juvenile idiopathic arthritis (JIA). METHODS: FC level < 100 g/kg was considered normal. Patients with 2 consecutive FC dosage ≥ 100 g/kg underwent endoscopic evaluation. RESULTS: There were 113 patients with JIA enrolled. FC was raised in 7 patients out of 113. All patients had IBD. In 3/7 patients, high FC levels were the only sign consistent with IBD. CONCLUSION: FC is a useful, economical, and noninvasive diagnostic tool to identify JIA patients with underlying IBD.


Assuntos
Artrite Juvenil/complicações , Fezes/química , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/diagnóstico , Complexo Antígeno L1 Leucocitário/análise , Adolescente , Biomarcadores/análise , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Programas de Rastreamento , Estudos Retrospectivos , Adulto Jovem
11.
Arthritis Care Res (Hoboken) ; 70(7): 1046-1051, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-28973842

RESUMO

OBJECTIVE: To assess the time in remission after discontinuing biologic therapy in patients with juvenile idiopathic arthritis (JIA). METHODS: We enrolled 135 patients followed in 3 tertiary-care centers. The primary outcome was to assess, once remission was achieved, the time in remission up to the first flare after discontinuing treatment. Mann-Whitney U test, Wilcoxon's signed rank test for paired samples, chi-square tests, and Fisher's exact test were used to compare data. Pearson's and Spearman's correlation tests were used to determine correlation coefficients for different variables. To identify predictors of outcome, Cox regression model and Kaplan-Meier curves were constructed, each one at the mean of entered covariates. RESULTS: The majority of enrolled patients flared after stopping treatment with biologics (102 of 135, 75.6%) after a median followup time in remission off therapy of 6 months (range 3-109 months). A higher probability of maintaining remission after discontinuing treatment was present in systemic-onset disease compared to the rest of the JIA patients (Mantel-Cox χ2 = 8.31, P < 0.004). In analysis limited to children with JIA with polyarticular and oligoarticular disease, patients who received biologics >2 years after achieving remission had a higher probability of maintaining such remission off therapy (mean ± SD 18.64 ± 3.3 months versus 11.51 ± 2.7 months [P < 0.009]; Mantel-Cox χ2 = 9.06, P < 0.002). No other clinical variable was significantly associated with a long-lasting remission. CONCLUSION: Children with oligoarticular and polyarticular JIA who stop treatment before 2 years from remission have a higher chance of relapsing after biologic withdrawal.


Assuntos
Antirreumáticos/administração & dosagem , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Produtos Biológicos/administração & dosagem , Suspensão de Tratamento/tendências , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Recidiva , Indução de Remissão/métodos , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
12.
Pediatr Rheumatol Online J ; 15(1): 87, 2017 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-29287595

RESUMO

BACKGROUND: Chronic Non-Bacterial Osteomyelitis (CNO) is an inflammatory disorder that primarily affects children. Although underestimated, its incidence is rare. For these reasons, no diagnostic and no therapeutic guidelines exist. The manuscript wants to give some suggestions on how to deal with these patients in the every-day clinical practice. MAIN BODY: CNO is characterized by insidious onset of bone pain with local swelling. Systemic symptoms such as fever, skin involvement and arthritis may be sometimes present. Radiological findings are suggestive for osteomyelitis, in particular if multiple sites are involved. CNO predominantly affects metaphyses of long bones, but clavicle and mandible, even if rare localizations of the disease, are very consistent with CNO diagnosis. CNO pathogenesis is still unknown, but recent findings highlighted the crucial role of cytokines such as IL-1ß and IL-10 in disease pathogenesis. Moreover, the presence of non-bacterial osteomyelitis among autoinflammatory syndromes suggests that CNO could be considered an autoinflammatory disease itself. Differential diagnosis includes infections, malignancies, benign bone tumors, metabolic disorders and other autoinflammatory disorders. Radiologic findings, either with Magnetic Resonance or with Computer Scan, may be very suggestive. For this reason in patients in good clinical conditions, with multifocal localization and very consistent radiological findings bone biopsy could be avoided. Non-Steroidal Anti-Inflammatory Drugs are the first-choice treatment. Corticosteroids, methotrexate, bisphosphonates, TNFα-inhibitors and IL-1 blockers have also been used with some benefit; but the choice of the second line treatment depends on bone lesions localizations, presence of systemic features and patients' clinical conditions. CONCLUSION: CNO may be difficult to identify and no consensus exist on diagnosis and treatment. Multifocal bone lesions with characteristic radiological findings are very suggestive of CNO. No data exist on best treatment option after Non-Steroidal Anti-Inflammatory Drugs failure.


Assuntos
Osteomielite/tratamento farmacológico , Anti-Inflamatórios não Esteroides/uso terapêutico , Criança , Diagnóstico Diferencial , Difosfonatos/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Osteomielite/diagnóstico
14.
Eur J Phys Rehabil Med ; 53(4): 508-515, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28084060

RESUMO

BACKGROUND: The Spinal Cord Independence Measure (SCIM III) is a scale of independence in the activities of daily life, specifically designed for spinal cord injury subjects. AIM: The aim of this study was to calculate the minimal clinically important difference (MCID) of the SCIM III according to distribution and anchor based approach. DESIGN: Prospective study. SETTING: Four Spinal Cord Units in Italy. POPULATION: Patients with acute/subacute spinal cord injury/lesion. METHODS: The scores of the total SCIM and of the four subscale was recorded at admission and discharge. Clinical significance was calculated according to anchor based methodology using a global rating of change questionnaire. The accuracy of MCID values in predicting a judgment of small improvement by the patients has been assessed by means of the area under the receiving operating curves (aROC). RESULTS: Total SCIM MCID values varied from 12 for patients with complete tetraplegia to 45.3 for those with incomplete thoracic lesions. The MCID of self-care varied from 3.3 to 8.5 and from 10 to 18 for respiration and sphincter management, depending on the level and severity of the lesion. With regard to mobility (room and toilet), the MCID varied from 1 to 3 and from 2.5 to 7.26 for mobility (indoors and outdoors). The aROC was between good and excellent for all these values. CONCLUSIONS: The results provide benchmarks for clinicians and researchers to interpret whether patients' change score on the SCIM III can be interpreted as true or clinically meaningful and to make clinical judgments about the patients' progress. CLINICAL REHABILITATION IMPACT: Our data could be useful for both clinicians and researchers. At the beginning of rehabilitation clinicians may have an idea of the minimal improvement of the patient (based on his neurological status) that could have an impact on patient's life. At the end of rehabilitation process, it is possible to control if the patient achieved an improvement that is true and significant. Researchers could also use these criteria to evaluate the clinical significance of an intervention by calculating the number of subjects in the treatment and control groups (or in two different treatment groups) who achieved a change calculated as the natural recovery plus the MCID.


Assuntos
Atividades Cotidianas , Avaliação da Deficiência , Diferença Mínima Clinicamente Importante , Traumatismos da Medula Espinal/diagnóstico , Traumatismos da Medula Espinal/reabilitação , Adulto , Idoso , Estudos de Coortes , Feminino , Humanos , Escala de Gravidade do Ferimento , Itália , Masculino , Pessoa de Meia-Idade , Paraplegia/reabilitação , Satisfação do Paciente , Modalidades de Fisioterapia , Estudos Prospectivos , Quadriplegia/reabilitação , Curva ROC , Recuperação de Função Fisiológica , Centros de Reabilitação , Resultado do Tratamento
15.
Acta Paediatr ; 105(7): e328-33, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27059298

RESUMO

AIM: The aim of this Italian study was to describe the clinical features, treatment options and outcomes of a cohort of patients with chronic nonbacterial osteomyelitis (CNO). METHODS: This was a retrospective cohort study. Laboratory data, diagnostic imaging, histological features and clinical course are reported. RESULTS: We enrolled 47 patients diagnosed with CNO. Bone pain was the leading symptom, and multifocal disease was present in 87% of the patients. The majority of the bone lesions were located in the appendicular skeleton (58%). Extraosseous manifestations were present in 34% of the patients, and renal involvement was detected in four patients. Inflammatory indices were increased in 80%, and bone x-rays were negative in 15% of the patients. Nonsteroidal anti-inflammatory drugs (NSAIDs) were the first therapy for all patients, achieving clinical remission in 27%. A good response to NSAIDs was significantly associated with a better prognosis. Bisphosphonates were used in 26 patients, with remission in 73%. Only six patients (13%), all with spine involvement, developed sequelae. CONCLUSION: We found a possible association between CNO and renal disease. Bisphosphonates were more likely to lead to clinical remission when NSAIDs and corticosteroids had failed. Vertebral localisation was the only risk factor for potential sequelae.


Assuntos
Difosfonatos/uso terapêutico , Nefropatias/complicações , Osteomielite/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Doença Crônica , Humanos , Osteomielite/complicações , Estudos Retrospectivos
17.
J Basic Clin Physiol Pharmacol ; 26(2): 161-3, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25153234

RESUMO

BACKGROUND: The use of vascular endothelial growth factor (VEGF) inhibitors may cause fetal harm and systemic side effects in the mother, so these drugs are contraindicated in pregnancy. We report a case of inadvertent administration of two intravitreal bevacizumab injections in a woman with diabetes mellitus type 1, 5 days before ovulation (±3 days) and during the 5th gestational week, respectively. The patient had a past history of both miscarriage and requirement for cesarean section for preeclampsia. METHODS: Observational case report. RESULTS: The patient did not have any drug-related adverse event and delivered a healthy full-term infant, reaching all developmental milestones appropriately during infancy. CONCLUSIONS: Intravitreal drug injections did not result in any detectable adverse event in the mother and infant although she had a significant past obstetric history. However, there have been no studies evaluating the effects of bevacizumab in pregnant women and suggesting that intravitreal drug injection in this patient is safe. Until more is known about this, it seems reasonable to avoid treatment with this drug a few weeks before and during pregnancy.


Assuntos
Inibidores da Angiogênese/administração & dosagem , Bevacizumab/administração & dosagem , Diabetes Mellitus Tipo 1/tratamento farmacológico , Adulto , Inibidores da Angiogênese/efeitos adversos , Bevacizumab/efeitos adversos , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/fisiopatologia , Feminino , Humanos , Recém-Nascido , Injeções Intravítreas , Gravidez , Complicações na Gravidez/tratamento farmacológico , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores
18.
J Pediatr Hematol Oncol ; 37(3): e194-7, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24942030

RESUMO

Teno Torque virus, member of the family of Anelloviridae, has been associated with many autoimmune diseases such as idiopathic hepatitis, systemic lupus erythematosus, and multiple sclerosis. Its viral load tends to be higher in the bone marrow and in tissues with high turnover rate. We report here a case of an 11-month-old infant affected by acute myeloid leukemia who underwent hematopoietic stem cell transplantation, and after 6 months had autoimmune hepatitis and atopic dermatitis. Extremely high-cytokine IP-10 and eotaxin levels were found in her sera, and serological tests and RT-PCR for viruses showed positive results exclusively for Teno Torque virus.


Assuntos
Doenças Autoimunes/etiologia , Autoimunidade/imunologia , Infecções por Vírus de DNA/virologia , Dermatite Atópica/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatite/etiologia , Leucemia Mieloide Aguda/imunologia , Torque teno virus/patogenicidade , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/patologia , Infecções por Vírus de DNA/imunologia , Infecções por Vírus de DNA/patologia , DNA Viral/genética , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/patologia , Feminino , Hepatite/tratamento farmacológico , Hepatite/patologia , Humanos , Lactente , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/virologia , Prognóstico , Torque teno virus/genética , Torque teno virus/isolamento & purificação , Carga Viral
19.
Acta Paediatr ; 103(11): e495-500, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25040148

RESUMO

AIM: Pain is a neglected problem in children with cognitive impairments, and few studies compare the clinical use of specific pain scales. We compared the Non-Communicating Children's Pain Checklist Postoperative Version (NCCPC-PV), the Echelle Douleur Enfant San Salvador (DESS) and the Children's Hospital of Eastern Ontario Pain Scale (CHEOPS). The first two were developed for children with cognitive impairment, and the third is a more general pain scale. METHODS: Two external observers and the child's caregiver assessed 40 children with cognitive impairment for pain levels. We assessed inter-rater agreement, correlation, dependence on knowledge of the child's behaviour, simplicity and adequacy in pain rating according to the caregiver for all three scales. RESULTS: The correlation between the NCCPC-PV and the DESS was strong (Spearman correlation coefficient = 0.76) and better than between each scale and the CHEOPS. Although the DESS showed better inter-rater agreement, it was more dependent on familiarity with the child and was judged more difficult to use by all observers. The NCCPC-PV was the easiest use and the most appropriate for rating the child's pain. CONCLUSION: The NCCPC-PV was the easiest to use for pain assessment in cognitively impaired children and should be adopted in clinical settings.


Assuntos
Transtornos Cognitivos , Medição da Dor/métodos , Adolescente , Lista de Checagem , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Dor/diagnóstico , Dor Pós-Operatória/diagnóstico
20.
Pediatr Emerg Care ; 30(4): 257-61, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24694881

RESUMO

Acquired long QT syndrome (LQTS) is a disorder of cardiac repolarization most often due to specific drugs, hypokalemia, or hypomagnesemia that may precipitate torsade de pointes and cause sudden cardiac death. Common presentations of the LQTS are palpitations, presyncope, syncope, cardiac arrest, and seizures. An abnormal 12-lead electrocardiogram obtained while the patient is at rest is the key to diagnosis. The occurrence of drug-induced LQTS is unpredictable in any given individual, but a common observation is that most patients have at least 1 identifiable risk factor in addition to drug exposure. The cornerstone of the management of acquired LQTS includes the identification and discontinuation of any precipitating drug and the correction of metabolic abnormalities, such as hypokalemia or hypomagnesemia. Most of the episodes of torsade de pointes are short-lived and terminate spontaneously. We propose a management protocol that could be useful for the daily practice in the emergency pediatric department to reduce the risk of acquired QT prolongation.


Assuntos
Síndrome do QT Longo/diagnóstico , Adolescente , Morte Súbita Cardíaca/etiologia , Eletrocardiografia , Feminino , Clínicos Gerais , Humanos , Síndrome do QT Longo/tratamento farmacológico , Síndrome do QT Longo/etiologia , Ondansetron/efeitos adversos , Ondansetron/uso terapêutico , Fatores de Risco , Antagonistas da Serotonina/efeitos adversos , Antagonistas da Serotonina/uso terapêutico
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