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1.
Ital J Pediatr ; 50(1): 59, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38561850

RESUMO

BACKGROUND: The aim of this study is to compare two groups of celiac patients: the first one, in which diagnosis was based on a "biopsy sparing" approach according to the 2012 ESPGHAN criteria, and the second one, based on the biopsy approach like the one of the 1991 Revised Criteria, in order to find relevant difference for sex, M/F ratio, age at diagnosis, clinical features at the onset, presence and prevalence of concomitant autoimmune disorders. METHODS: Our study involves 61 patients having the Celiac Disease (CD) onset from February 2013 to February 2020. The 32 patients who received diagnosis according "biopsy sparing" criteria were enrolled in group (1) The 29 patients who received diagnosis by duodenal biopsy were enrolled in group (2) Prevalence of comorbidities was analysed through chi-square test. RESULTS: In group 1 the prevalence of comorbidities such as Insulin-Dependent Diabetes Mellitus (IDDM) and thyroiditis was of 53%, while in group 2 it was only of 24%. Analysing the IDDM prevalence between the two groups we found a relevant difference. At the same time, the prevalence of thyroiditis was also significantly different. In group 1, male patients, in particular, would seem to have a higher incidence of CD related autoimmune disorders. CONCLUSIONS: An increased prevalence of IDDM, thyroiditis and juvenile idiopathic arthritis (JIA) in the first group would show that the "biopsy sparing" approach could expose patients to a greater length of disease activity that might be responsible for the onset of such comorbidities. Further studies should be carried out on more numerous samples of patients in order to confirm or not these data.


Assuntos
Artrite Juvenil , Doença Celíaca , Diabetes Mellitus Tipo 1 , Tireoidite , Humanos , Masculino , Artrite Juvenil/epidemiologia , Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , Comorbidade , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/epidemiologia , Diabetes Mellitus Tipo 1/complicações , Prevalência , Tireoidite/complicações , Tireoidite/epidemiologia , Feminino
2.
J Pediatr Hematol Oncol ; 43(6): e886-e890, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-33122582

RESUMO

Rasburicase is a recombinant urate oxidase enzyme indicated for tumor lysis syndrome, a potential life-threatening oncologic emergency that occurs most commonly during initial chemotherapy for hematological malignancies. As a result of the defects in the physiological antioxidant pathway, erythrocytes of patients with glucose-6-phosphate dehydrogenase deficiency are not protected against the oxidizing stress exerted by hydrogen peroxide generated with the administration of rasburicase. The authors report a 14-year-old patient, diagnosed with T-cell acute lymphoblastic leukemia, who developed methemoglobinemia and hemolytic anemia with low oxygen saturation after starting steroids, hyperhydratation, and rasburicase administration. The complications resolved with supportive therapy only.


Assuntos
Metemoglobinemia/induzido quimicamente , Leucemia-Linfoma Linfoblástico de Células T Precursoras/tratamento farmacológico , Urato Oxidase/efeitos adversos , Adolescente , Anemia Hemolítica/induzido quimicamente , Anemia Hemolítica/diagnóstico , Humanos , Masculino , Metemoglobinemia/diagnóstico , Leucemia-Linfoma Linfoblástico de Células T Precursoras/diagnóstico , Proteínas Recombinantes/efeitos adversos , Proteínas Recombinantes/uso terapêutico , Urato Oxidase/uso terapêutico
3.
Brain Pathol ; 26(4): 542-6, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-26462994

RESUMO

Tauopathies are sporadic or familial neurodegenerative diseases characterized by the accumulation of phosphorylated tau in neurons and glial cells and include encephalitis related to measles virus such as subacute sclerosing panencephalitis. We describe a 45-year-old woman, with a history of lymphoma treated with immunosuppressant therapy who underwent an open biopsy of the right frontal cortex for a suspect of encephalitis, and died 4 days later. The neuropathological assessment on the bioptic sample revealed edema, severe gliosis and microglial activation, with lymphomonocytic perivascular cuffing and neurons containing both nuclear and cytoplasmic eosinofilic inclusions that ultrastructurally appeared as tubular and curvilinear non-membrane-bound 12-18 nm structures, leading to the diagnosis of measles inclusion-bodies encephalitis. The biopsy specimen showed several cortical neurons with intense perikaryal immunoreactivity for anti-tau antibodies recognizing phosphorylated epitopes while on autoptic specimens no phosphorylated tau immunoreactivity was detected. Our findings suggest that in specific conditions biopsy-derived human tau may be phosphorylated at sites that may result not phosphorylated in autopsy-derived specimens, most likely caused by post-mortem dephosphorylation.


Assuntos
Neurônios/patologia , Mudanças Depois da Morte , Panencefalite Esclerosante Subaguda/patologia , Proteínas tau/metabolismo , Autopsia , Biópsia , Feminino , Humanos , Pessoa de Meia-Idade , Neurônios/metabolismo , Fosforilação
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