HISTIOCITOSE DE CÉLULAS DE LANGERHANS AUTOLIMITADA E DE INÍCIO TARDIO: RELATO DE UMA ENTIDADE RARÍSSIMA

RESUMO Objetivo: Relatar um caso de histiocitose de células de Langerhans autolimitada e de início tardio. Descrição do caso: Paciente com 4 meses e meio de idade do sexo feminino, apresentando uma erupção cutânea eritematosa purpúrea, foi submetida a uma biópsia de pele, sendo diagnosticada com histiocitose de células de Langerhans cutânea isolada. As lesões regrediram em poucos meses e ela foi diagnosticada, retrospectivamente, com histiocitose de células de Langerhans autolimitada e de início tardio, após não apresentar nenhum envolvimento cutâneo ou sistêmico durante um seguimento de quatro anos. Comentários: A histiocitose de células de Langerhans autolimitada caracteriza-se pela proliferação clonal das células de Langerhans e apresenta-se com lesões cutâneas, sendo uma variante autolimitada rara de histiocitose. A doença só pode ser diagnosticada de forma retrospectiva, após o paciente não apresentar nenhum envolvimento sistêmico durante vários anos. Embora existam casos de manifestações ao nascimento ou durante o período neonatal, apenas alguns casos de histiocitose de células de Langerhans de idade tardia foram relatados. Lesões purpúreas que aparecem após o período neonatal podem sugerir histiocitose de células de Langerhans autolimitada e de início tardio. Uma vez confirmado o diagnóstico por biópsia cutânea, tais pacientes devem ser acompanhados regularmente, pois pode haver comprometimento sistêmico

Long-term Quality of Life in Patients with Thalassemia Major following Hematopoetic Stem Cell Transplantation in Childhood

WOS:000600556201329[No Abstract Available

Does mold sensitivity cause adenoid hypertrophy in children?

Objective: Chronic adenoid infections, and allergic inflammation lead to development of adenoid hypertrophy. One of the major causes of allergic rhinitis due to allergic inflammation is indoor allergens, mainly house dust mites and molds. In this study, it was aimed to investigate the frequency of mold fungi sensitivity in the etiology of allergic rhinitis caused by adenoid hypertrophy in children

Reference values for airway resistance measured by the interrupter technique in preschool children

Objective: In preschool children, diagnosis of asthma is difficult because of inability to use spirometry objectively. Instead of spirometry, other techiques that do not require sedation and cooperation to evaluate pulmonary functions have been used.. Interrupter technique that measures the resistance of the airway, is one of these methods. Insufficient reference values hamper the use of this technique commonly. In this study, to determine the reference values of Turkish preschool children for interrupter technique is intended

Prevalence of psychiatric disorders and suicidality among children and adolescents with thalassemia major-A Turkish sample

The current study examined psychiatric diagnoses and suicidality in children with transfusion-dependent thalassemia major via a semi-structured interview (K-SADS). Although 4 subjects were diagnosed with major depressive disorder, 24 subjects (40.7%) had depressed mood and irritability, whereas anhedonia and boredom were detected in 23 (39.0%) subjects. Suicidal thoughts were expectedly more prevalent (28.8%) than suicidal intention (5.1%) and suicide attempts (1.7%). Children with thalassemia major may be an under-risk population for subclinical forms of depression and suicidality. Offering routine screening for mental health problems can lead to early identification and treatment

Persistent pulmonary mass-like lung consolidations and chest wall extension in a newborn diagnosed with chronic granulomatous disease

[No Abstract Available

HISTIOCITOSE DE CÉLULAS DE LANGERHANS AUTOLIMITADA E DE INÍCIO TARDIO: RELATO DE UMA ENTIDADE RARÍSSIMA

RESUMO Objetivo: Relatar um caso de histiocitose de células de Langerhans autolimitada e de início tardio. Descrição do caso: Paciente com 4 meses e meio de idade do sexo feminino, apresentando uma erupção cutânea eritematosa purpúrea, foi submetida a uma biópsia de pele, sendo diagnosticada com histiocitose de células de Langerhans cutânea isolada. As lesões regrediram em poucos meses e ela foi diagnosticada, retrospectivamente, com histiocitose de células de Langerhans autolimitada e de início tardio, após não apresentar nenhum envolvimento cutâneo ou sistêmico durante um seguimento de quatro anos. Comentários: A histiocitose de células de Langerhans autolimitada caracteriza-se pela proliferação clonal das células de Langerhans e apresenta-se com lesões cutâneas, sendo uma variante autolimitada rara de histiocitose. A doença só pode ser diagnosticada de forma retrospectiva, após o paciente não apresentar nenhum envolvimento sistêmico durante vários anos. Embora existam casos de manifestações ao nascimento ou durante o período neonatal, apenas alguns casos de histiocitose de células de Langerhans de idade tardia foram relatados. Lesões purpúreas que aparecem após o período neonatal podem sugerir histiocitose de células de Langerhans autolimitada e de início tardio. Uma vez confirmado o diagnóstico por biópsia cutânea, tais pacientes devem ser acompanhados regularmente, pois pode haver comprometimento sistêmico

Metabolic syndrome and risk factors after hematopoietic stem cell transplantation in children and adolescents

Objectives: The early and late complications after hematopoietic stem cell transplantation (HSCT) determine the patients' prognosis and life quality. We aim to determine the metabolic syndrome development frequency after HSCT in children to find out the risk factors and compare them with healthy adolescents. Methods: Thirty-six children who underwent HSCT at least two years ago were analyzed prospectively and cross-sectionally. Our study included 18 healthy children between the ages of 11 and 17 as a control group. All of the cases were assessed in terms of metabolic syndrome (MS) through the use of Modified WHO Criteria. Results: The patients' median age was 10.6 (5.1-17) years, the median time of follow-up after HCST was 4.1 (2-13.5) years and 70% were male. Two cases were diagnosed with MS (5.6%). When considered in terms of the subcomponents of MS, 2 cases (5.6%) were found to have obesity, 17 cases (47%) abnormal glucose tolerance, 11 cases (30.7%) dyslipidemia, and 3 cases (8.6%) hypertension. The MS rate was not different when compared with the 11-17 year-old healthy control group (0 vs. 11%, p=0.48). Myeloablative conditioning regimen (65 vs. 20%) and the increased age at which HSCT was performed were considered to be risk factors in terms of insulin resistance (p=0.025 and 0.002). Conclusions: Age and conditioning regimens were found to be the risk factors for insulin resistance development. The long-term follow-up of the cases who had undergone HSCT in childhood in terms of MS and its sub-components is important in order to increase life quality