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1.
Pediatr Neonatol ; 60(4): 411-416, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30470618

RESUMO

OBJECTIVE: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia children. The aim of this retrospective study is to describe presenting features and clinical characteristics of ITP and evaluate clinical course, treatment modalities, and complications and determine the effects of preceding infection history, age, gender, treatment modality, and admission platelet count on chronicity. METHOD: Two hundred and eleven patients who were diagnosed ITP and followed-up in Department of Pediatric Hematology, Ankara Children Hematology Oncology Education and Research Hospital between January 2008 and September 2012 were included. Age of the patients, gender, date of admission, date of diagnosis, complaint in the application, previous infection and laboratory tests were recorded. RESULTS: Mean age of the patients on diagnosis was 5.4 ± 4.1 years. The female/male ratio was 1.03. The clinical courses were determined as acute or chronic in 72% and 28% of patients respectively. Mean age at diagnosis was significantly higher in chronic ITP (p < 0.01). Chronic course was significantly higher in female patients (p < 0.05). The most frequent complaint was bruises on the skin (68%). The most common physical examination findings were petechiae, purpura and ecchymosis (89%). Patients with a history of past infection (53.6%) and who had serologically positive infection (15.6%) frequently had acute course (p < 0.01). The most common serologically positive infection was Rubella. The mean platelet count was significantly higher in chronic ITP (p < 0.01). In the initial treatment of patients admitted in the acute phase, megadose methylprednisolone (MDMP) was used in 31% of patients, intravenous immune globulin (IVIG) in 55% of patients and anti-D in 2% of patients while 12% did not receive any treatment. There were no significant differences between the recurrence rate and treatment modality (p > 0.05). CONCLUSION: In our study, in females and in patients without any history of past infection, platelet count >20 × 109/L and initial diagnosis age > 10 years were found to increase the probability of chronic disease, which is compatible with the literature.


Assuntos
Glucocorticoides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Metilprednisolona/uso terapêutico , Púrpura Trombocitopênica Idiopática/terapia , Imunoglobulina rho(D)/uso terapêutico , Fatores Etários , Criança , Pré-Escolar , Doença Crônica , Infecções por Citomegalovirus/epidemiologia , Infecções por Citomegalovirus/imunologia , Progressão da Doença , Infecções por Vírus Epstein-Barr/epidemiologia , Infecções por Vírus Epstein-Barr/imunologia , Feminino , Hospitalização , Humanos , Lactente , Masculino , Contagem de Plaquetas , Prognóstico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/imunologia , Recidiva , Estudos Retrospectivos , Rubéola (Sarampo Alemão)/epidemiologia , Rubéola (Sarampo Alemão)/imunologia , Fatores Sexuais , Turquia/epidemiologia
2.
J Pediatr Endocrinol Metab ; 30(6): 683-691, 2017 May 24.
Artigo em Inglês | MEDLINE | ID: mdl-28525352

RESUMO

BACKGROUND: Endocrine organs are highly susceptible to effects of high-dose chemotherapy. The objective of the study was to evaluate endocrine and metabolic complications after hematopoietic stem cell transplantation (HSCT) in children. METHODS: The patients who underwent HSCT in our center from April 2010 to October 2014 with at least 1 year follow-up were analyzed retrospectively. RESULTS: One-hundred children (M/F:59/41; mean age 8.9±4.8 years, mean follow-up time 3.4±1.2 years) were included in the study. Female hypogonadism was the most common endocrine dysfunction (35.7%), followed by growth impairment (29.4%), malnutrition (27.4%), dyslipidemia (26%), low bone mineral density (BMD) (25%), hypothyroidism (13%) and insulin resistance (12%). Patients who underwent HSCT >10 years of age were significantly at risk for hypogonadism, metabolic syndrome, growth impairment and malnutrition (p<0.05). CONCLUSIONS: Endocrine or metabolic dysfunctions are more prevalent in children who are older than 10 years of age at HSCT. Children who underwent HSCT should be followed-up by a multidisciplinary team during puberty and adolescence.


Assuntos
Doenças do Sistema Endócrino/etiologia , Doenças Hematológicas/complicações , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Síndrome Metabólica/etiologia , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Turquia
3.
Pediatr Transplant ; 21(3)2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28078791

RESUMO

HSCT is a curative treatment in TM, but conditioning and immunosuppressive treatment may affect bone metabolism. In this retrospective study, we aimed to compare BMD, vitamin D status, and growth in children with TM who underwent HSCT to those in children with TD TM. Twenty-three children with TM who underwent HSCT (mean age 7.1 years [1.03-14.7]) and 24 children with TD thalassemia (mean age 9.8 years [1.6-14]) were recruited. Lumbar spine BMD of TD thalassemia patients was higher than those in patients who had HSCT at both baseline and second-year assessments (P=.009, P<.001, respectively). However, BMD Z scores or serum 25-OH vitamin D levels were not different in two groups. Being >10 years of age was a significant risk factor for low BMD, height, and weight Z score for both groups. Patients who underwent HSCT with Pesaro risk class II or III had higher risk for low BMD compared to those risk class I patients (P=.044). In conclusion, children with TM who were >10 years at HSCT are at risk for low BMD and growth retardation. HSCT had no effect on BMD deficit in children with TM.


Assuntos
Densidade Óssea , Transplante de Células-Tronco , Vitamina D/sangue , Talassemia beta/sangue , Absorciometria de Fóton , Adolescente , Peso Corporal , Criança , Pré-Escolar , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Vértebras Lombares/efeitos dos fármacos , Masculino , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Talassemia beta/terapia
4.
J Int Adv Otol ; 13(1): 136-139, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27879229

RESUMO

OBJECTIVE: In this study, we aimed to detect the incidences of ototoxicity in patients with hemoglobinopathies taking deferoxamine (DFO), deferiprone, and deferasirox using the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events (CTCAE) scale to obtain more objective data. MATERIALS AND METHODS: Fifty-five transfusion-dependent patients were evaluated in this study. The NCI CTCAE scale was used to assess ototoxicity levels. The average ferritin and hemoglobin levels, the type of iron chelator, and the duration of therapy of all the patients were recorded. RESULTS: Ototoxicity was observed in 15 patients (31.9 %), all of whom were taking DFO. The median age was 19.5 (6-43) in patients without ototoxicity and 29 (16-50) in those with ototoxicity; this difference was statistically significant (p<0.05). The median ferritin and pre-tx Hb levels were 1391 ng/mL and 9.06 mg/dL, respectively, in patients with ototoxicity and 986.7 ng/mL and 9.24 mg/dL, respectively, in those without ototoxicity; these differences were not significant (p>0.05). Ototoxicity was not observed in the eight patients who used only deferasirox and deferiprone. CONCLUSION: The ototoxicity incidence with DFO at doses below 50 mg/kg/day was 27.3%. Deferiprone and deferasirox were not associated with ototoxic effects in patients taking these drugs.


Assuntos
Otopatias/induzido quimicamente , Quelantes de Ferro/efeitos adversos , Talassemia/tratamento farmacológico , Adolescente , Adulto , Terapia por Quelação/métodos , Criança , Estudos Transversais , Otopatias/diagnóstico , Otopatias/epidemiologia , Feminino , Humanos , Incidência , Quelantes de Ferro/administração & dosagem , Masculino , Pessoa de Meia-Idade , Turquia/epidemiologia
5.
Pediatr Transplant ; 21(1)2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27813281

RESUMO

The aim of this study was to investigate the effects of donor characteristics on CD34+ cell yield in BM harvest. Between April 2010 and November 2013, consecutive donors who underwent BM harvesting in our BM transplantation unit were retrospectively investigated. Donors were classified into two groups: those who donated BM without mobilization (steady-state BM donors) and those who received G-CSF for stem cell mobilization (G-CSF-primed BM donors). Donor characteristics (age, gender, race, body weight, BMI, and laboratory factors including donor's leukocyte, platelet, and monocyte) and their relationship with total nuclear cell and CD34+ cell numbers has been evaluated. A total of 64 healthy related donors (29 males/35 females, median age 11.2 years; 49 [76.6%] younger than 18 and 36 [56.3%] younger than 12 years) were included in the study. The median CD34+ cell yield in the harvest was 0.12×106 /L (0.02-0.21) in SS-BM donors and 0.18×106 /L (0.09-0.67) in GP-BM donors (P=.03). Median of CD34+ cell count given to recipients was 2.6×106 /recipient body weight (1.3-19.3) in SS-BM yields and 3.8×106 /recipient body weight (1.1-10.2) in GP-BM yields, respectively. Multiple regression analysis showed that donor height and pre-G-CSF platelet were the most important parameters to obtain a sufficient BM harvest. Our data suggest that the shorter donors and the donors with higher thrombocyte counts may offer more hematopoietic stem cell. The height and thrombocyte count of the donors should be taken into consideration before planning the targeted CD34+ cell count especially for pediatric donors.


Assuntos
Antígenos CD34/metabolismo , Medula Óssea/patologia , Transplante de Células-Tronco , Adolescente , Adulto , Plaquetas/imunologia , Peso Corporal , Criança , Pré-Escolar , Feminino , Fator Estimulador de Colônias de Granulócitos/metabolismo , Humanos , Lactente , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Análise de Regressão , Estudos Retrospectivos , Transplante Homólogo , Adulto Jovem
6.
Pediatr Transplant ; 20(2): 276-83, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26614176

RESUMO

There are few studies evaluating the use of IgM-enriched IVIG (Pentaglobin(®) ) in HSCT recipients. This study aimed to compare the efficacy of prophylactic use of IVIG versus prophylactic use of Pentaglobin(®) within the first 100 days after allogeneic HSCT. We performed a prospective, randomized study of the use of prophylactic IVIG versus prophylactic use of Pentaglobin(®) in patients after allogeneic HSCT. The first dose of IVIG or Pentaglobin(®) was given before conditioning regimen and after transplant was given on day +1, +8, +15, and +22. And then, it was given if IgG level was below 400 mg/dL. Twenty-seven patients in IVIG group and 32 patients in Pentaglobin(®) group were included in the study. There were no significant differences in the duration of neutropenia, hospitalization, fever, and in the number of pyrexial episode, septicemia, bacteremia, local infection, CMV infection, acute GVHD, VOD, and adverse events between the IVIG group and Pentaglobin(®) group. Randomized placebo-controlled trials are needed to conclude that utilization of IVIG or Pentaglobin(®) has no beneficial effect in HSCT.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Imunoglobulina A/administração & dosagem , Imunoglobulina M/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Adolescente , Anemia Aplástica/terapia , Criança , Feminino , Humanos , Imunoglobulina G/química , Imunoglobulinas Intravenosas/uso terapêutico , Leucemia/terapia , Masculino , Síndromes Mielodisplásicas/terapia , Estudos Prospectivos , Transplante Homólogo , Resultado do Tratamento , Talassemia beta/terapia
9.
Biol Blood Marrow Transplant ; 21(11): 1888-94, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26265462

RESUMO

Granulocyte colony stimulating factor (G-CSF) is sometimes administered to donors before bone marrow (BM) harvest. G-CSF-primed (G-BM) and unprimed BM (U-BM)-derived mesenchymal stem cells (MSC) were obtained from 16 healthy donors and were expanded in vitro. Their proliferative characteristics, morphology, and differentiation capacity were examined. Supernatants of the second passage of MSCs were evaluated for transforming growth factor ß1, hepatocyte growth factor, and prostaglandin E2 (PGE2) levels and compared with controls. The analyses of cytokines in the G-BM- and U-BM-derived MSCs supernatants revealed that PGE2 levels were significantly lower in the G-CSF-primed samples. These cytokines were also measured in BM plasma. The level of hepatocyte growth factor in G-BM plasma was significantly increased. The current study is the first to show the effects of G-CSF on the BM microenvironment of healthy human donors. The preliminary data suggest that G-BM- and U-BM-derived MSCs have similar morphologic/phenotypic properties and differentiation capacity but differ in their secretory capacity. Significant changes in cytokine levels of BM plasma in G-CSF-primed donors were also demonstrated. These findings suggest that BM MSCs and changes in the BM microenvironment may contribute to the effects of G-CSF on inflammation and immunomodulation.


Assuntos
Células da Medula Óssea/efeitos dos fármacos , Fator Estimulador de Colônias de Granulócitos/farmacologia , Leucócitos Mononucleares/efeitos dos fármacos , Células-Tronco Mesenquimais/efeitos dos fármacos , Adolescente , Células da Medula Óssea/citologia , Células da Medula Óssea/imunologia , Diferenciação Celular/efeitos dos fármacos , Proliferação de Células/efeitos dos fármacos , Criança , Pré-Escolar , Técnicas de Cocultura , Meios de Cultivo Condicionados/química , Dinoprostona/genética , Dinoprostona/metabolismo , Feminino , Expressão Gênica , Fator de Crescimento de Hepatócito/genética , Fator de Crescimento de Hepatócito/metabolismo , Humanos , Leucócitos Mononucleares/citologia , Leucócitos Mononucleares/imunologia , Masculino , Células-Tronco Mesenquimais/citologia , Células-Tronco Mesenquimais/imunologia , Cultura Primária de Células , Doadores de Tecidos , Fator de Crescimento Transformador beta1/genética , Fator de Crescimento Transformador beta1/metabolismo
10.
Hemoglobin ; 39(4): 247-50, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26120034

RESUMO

Thalassemia is an autosomal recessive inherited blood disorder. It is prevalent in Mediterranean countries such as Sardinia, Greece, Cyprus, Turkey, Lebanon and also Southeast Asia. Our aim was to investigate the carrier prevalence of thalassemia and other hemoglobinopathies in adolescents who live in Mugla Province, Turkey. We analyzed retrospectively the surveys conducted at primary schools between 1997 and 2013. Complete blood count (CBC) and high performance liquid chromatography (HPLC) were used to screen for thalassemia and hemoglobinopathies. Patients were diagnosed as having thalassemia trait if the mean corpuscular volume (MCV) was ≤ 80.0 fL, mean corpuscular hemoglobin (Hb) was ≤ 27.0 pg and Hb A2 levels were ≥ 3.5%. A total of 164,814 students were analyzed. The median age of the students was 13.5 years (minimum 13.0, maximum 14.0). The total number of students with abnormal HPLC results was 5861 (3.8%). There was a significant decrease in the newborn of new thalassemia patients found with screening programs for hemoglobinopathies in Mugla Province from 1997 to 2013. The number of students with abnormal HPLC results for thalassemia, sickle cell disease and other Hb traits were 3.2, 0.15 and 0.4%, respectively. It is important to recognize that including Hb, MCV, red blood cell (RBC) count and HPLC tests for carrier screening are necessary to find hemoglobinopathies. Our study supported that the number of new patients significantly decreased using these screening programs from 1997 to 2013.


Assuntos
Hemoglobinopatias/epidemiologia , Adolescente , Feminino , Seguimentos , Hemoglobinopatias/genética , Hemoglobinas Anormais/genética , Heterozigoto , Humanos , Masculino , Programas de Rastreamento , Prevalência , Turquia/epidemiologia , Talassemia beta/epidemiologia , Talassemia beta/genética
11.
Turk J Haematol ; 32(3): 228-33, 2015 09.
Artigo em Inglês | MEDLINE | ID: mdl-25912774

RESUMO

INTRODUCTION: Venous thromboembolism (VTE) in children who undergo hematopoietic stem cell transplantation (HSCT) has high morbidity. The aim of this study is to assess the incidence of VTE in allogeneic pediatric HSCT recipients and the contribution of pretransplant prothrombotic risk factors to thrombosis. METHODS: We retrospectively evaluated 92 patients between April 2010 and November 2012 undergoing allogeneic HSCT who had completed 100 days post-HSCT. Before HSCT, coagulation profiles; acquired and inherited prothrombotic risk factors including factor V Leiden, prothrombin G20210A, methylenetetrahydrofolate reductase (MTHFR) C677T, and MTHFR A1298C mutations; and serum homocysteine and lipoprotein(a), plasma antithrombin III, protein C, and protein S levels were obtained from all patients. RESULTS: In the screening of thrombophilia, 8 patients (9%) were heterozygous for factor V Leiden, 5 (6%) were homozygous for MTHFR 677TT, 12 (14%) were homozygous for MTHFR 1298CC, and 2 (2%) were heterozygous for prothrombin G20210A mutation. We observed VTE in 5 patients (5.4%); a prothrombotic risk factor was found in 3 out of these 5 patients, while 4 out of 5 patients had central venous catheters. It was determined there was no significant relationship between VTE and inherited prothrombotic risk factors. DISCUSSION AND CONCLUSION: VTE after HSCT seems to be a low-frequency event that may be due to low-dose, low-molecular-weight heparin prophylaxis, and the role of inherited prothrombotic risk factors cannot be entirely excluded without a prospective study.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Tromboembolia Venosa/etiologia , Resistência à Proteína C Ativada/complicações , Resistência à Proteína C Ativada/epidemiologia , Resistência à Proteína C Ativada/genética , Adolescente , Aloenxertos , Cateterismo Venoso Central/efeitos adversos , Criança , Pré-Escolar , Enoxaparina/administração & dosagem , Enoxaparina/uso terapêutico , Fator V/genética , Feminino , Doenças Hematológicas/terapia , Hepatopatia Veno-Oclusiva/prevenção & controle , Humanos , Síndromes de Imunodeficiência/terapia , Incidência , Lactente , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Neoplasias/terapia , Mutação Puntual , Protrombina/genética , Estudos Retrospectivos , Fatores de Risco , Trombofilia/epidemiologia , Trombofilia/genética , Turquia/epidemiologia , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/prevenção & controle
12.
Transfus Apher Sci ; 52(3): 332-8, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25779226

RESUMO

In this study, we aimed to determine the effect(s) of G-CSF priming on graft and transplantation parameters and compare these findings with those obtained without priming. A total of 64 pediatric patients transplanted from HLA-matched family donors were enrolled in the study. Twenty-nine patients received G-CSF primed marrow (G-BM group) and 35 patients received steady state bone marrow (S-BM group). Number of total nucleated cells (TNC) and CD34(+) cells, CFU-GM colony number, neutrophil and platelet engraftment times, total length of stay in hospital, overall and disease free survival, and occasions of acute and chronic GvHD has been compared between these two groups. Granulocyte colony stimulating factor primed bone marrow (G-BM) yielded higher numbers of CD34(+) cells, TNCs, and CFU-GM colony numbers compared to those obtained in S-BM. The neutrophil engraftment time, platelet engraftment time, length of stay in hospital, overall survival and disease free survival were not different between G-BM and S-BM groups. Also the cumulative incidence of grades II-IV acute and chronic GvHD were similar. It was observed that the use of G-CSF did not increase the risk of acute or chronic GvHD. We concluded that use of G-CSF for stem cell mobilization is an effective and safe method in children.


Assuntos
Células da Medula Óssea/citologia , Fator Estimulador de Colônias de Granulócitos/química , Antígenos HLA/metabolismo , Mobilização de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas , Adolescente , Adulto , Antígenos CD34/metabolismo , Criança , Pré-Escolar , Intervalo Livre de Doença , Anemia de Fanconi/terapia , Feminino , Doença Enxerto-Hospedeiro , Células Progenitoras de Granulócitos e Macrófagos/citologia , Humanos , Lactente , Tempo de Internação , Leucemia Mieloide Aguda/terapia , Masculino , Síndromes Mielodisplásicas/terapia , Neutrófilos/citologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Estudos Retrospectivos , Transplante Homólogo , Resultado do Tratamento , Adulto Jovem , Talassemia beta/terapia
13.
J Clin Immunol ; 35(2): 189-98, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25627830

RESUMO

Mutations in DOCK8 result in autosomal recessive Hyper-IgE syndrome with combined immunodeficiency (CID). However, the natural course of disease, long-term prognosis, and optimal therapeutic management have not yet been clearly defined. In an international retrospective survey of patients with DOCK8 mutations, focused on clinical presentation and therapeutic measures, a total of 136 patients with a median follow-up of 11.3 years (1.3-47.7) spanning 1693 patient years, were enrolled. Eczema, recurrent respiratory tract infections, allergies, abscesses, viral infections and mucocutaneous candidiasis were the most frequent clinical manifestations. Overall survival probability in this cohort [censored for hematopoietic stem cell transplantation (HSCT)] was 87 % at 10, 47 % at 20, and 33 % at 30 years of age, respectively. Event free survival was 44, 18 and 4 % at the same time points if events were defined as death, life-threatening infections, malignancy or cerebral complications such as CNS vasculitis or stroke. Malignancy was diagnosed in 23/136 (17 %) patients (11 hematological and 9 epithelial cancers, 5 other malignancies) at a median age of 12 years. Eight of these patients died from cancer. Severe, life-threatening infections were observed in 79/136 (58 %); severe non-infectious cerebral events occurred in 14/136 (10 %). Therapeutic measures included antiviral and antibacterial prophylaxis, immunoglobulin replacement and HSCT. This study provides a comprehensive evaluation of the clinical phenotype of DOCK8 deficiency in the largest cohort reported so far and demonstrates the severity of the disease with relatively poor prognosis. Early HSCT should be strongly considered as a potential curative measure.


Assuntos
Estudos de Associação Genética , Fatores de Troca do Nucleotídeo Guanina/deficiência , Fatores de Troca do Nucleotídeo Guanina/genética , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Humanos , Imunoglobulina E/sangue , Imunoglobulina E/imunologia , Incidência , Lactente , /epidemiologia , Síndrome de Job/complicações , Síndrome de Job/diagnóstico , Síndrome de Job/genética , Síndrome de Job/imunologia , Síndrome de Job/mortalidade , Síndrome de Job/terapia , Contagem de Linfócitos , Subpopulações de Linfócitos/imunologia , Subpopulações de Linfócitos/metabolismo , Masculino , Pessoa de Meia-Idade , Mutação , Neoplasias/epidemiologia , Neoplasias/etiologia , Fenótipo , Adulto Jovem
14.
J Pediatr Hematol Oncol ; 37(1): e19-22, 2015 01.
Artigo em Inglês | MEDLINE | ID: mdl-25522351

RESUMO

Invasive fungal infections (IFIs) constitute a leading cause of morbidity and infection-related mortality among hematopoietic stem cell transplant (HSCT) recipients. With the use of secondary prophylaxis, a history of IFI is not an absolute contraindication to allo-HSCT. However, still, IFI recurrence remains a risk factor for transplant-related mortality. In this study, of the 105 children undergoing HSCT between April 2010 and February 2013, 10 patients who had IFI history before transplantation and had undergone allo-HSCT were evaluated retrospectively to investigate results of secondary prophylaxis. In conclusion, our study shows that amphotericin B and caspofungin was successful as secondary antifungal prophylaxis agents with no relapse of IFI. In addition, after engraftment, secondary prophylaxis was continued with voriconazole orally in 4 patients that yielded good results.


Assuntos
Antifúngicos/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Micoses/prevenção & controle , Adolescente , Anfotericina B/uso terapêutico , Caspofungina , Criança , Equinocandinas/uso terapêutico , Feminino , Humanos , Lipopeptídeos , Masculino , Estudos Retrospectivos
16.
Exp Clin Transplant ; 12(5): 462-8, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25299374

RESUMO

OBJECTIVES: The purpose of this study was to evaluate the effectiveness of acyclovir prophylaxis and preemptive ganciclovir treatment in preventing cytomegalovirus disease in children who underwent hematopoietic stem cell transplant. MATERIALS AND METHODS: We reviewed the clinical records of 66 children (36 boys, 30 girls; mean age, 9 ± 5 y; age range, 2-20 y) who underwent hematopoietic stem cell transplant at Ankara Children's Hematology and Oncology Hospital, Bone Marrow Transplantation Unit, between April 2010 and March 2012. RESULTS: In these 66 children, 61 children (92.4%) received allogeneic transplant; 50 children (76.9%) received a myeloablative regimen; and 14 children (21.2%) received anti-thymocyte globulin as part of the conditioning regimen. All children received acyclovir prophylaxis from the beginning of conditioning regimen until 100 days after transplant, and children received preemptive treatment with ganciclovir when cytomegalovirus DNAemia ≥ 400 copies/mL on 2 tests or ≥ 1000 copies/mL on 1 test. There were 19 children (28.8%) who had cytomegalovirus reactivation during median follow-up 381 days (range, 100-720 d). Cytomegalovirus disease was observed in only 2 patients (10.5%); 1 patient had cytomegalovirus hepatitis and 1 patient had cytomegalovirus gastrointestinal disease. Both patients were cured of cytomegalovirus with treatment for 1 month. There was no death attributable to cytomegalovirus reactivation and/or disease. Febrile neutropenia, acute graft-versus-host disease, and steroid use were more frequent in patients who had cytomegalovirus than did not have cytomegalovirus reactivation. The risk of cytomegalovirus reactivation was increased 5-fold in patients who used steroids. CONCLUSIONS: Acyclovir prophylaxis and preemptive treatment with ganciclovir may be effective in preventing cytomegalovirus disease in most children who have hematopoietic stem cell transplant.


Assuntos
Aciclovir/administração & dosagem , Antivirais/administração & dosagem , Infecções por Citomegalovirus/prevenção & controle , Ganciclovir/administração & dosagem , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Adolescente , Fatores Etários , Biomarcadores/sangue , Criança , Pré-Escolar , Citomegalovirus/genética , Citomegalovirus/isolamento & purificação , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/virologia , DNA Viral/sangue , Esquema de Medicação , Feminino , Humanos , Masculino , Fatores de Risco , Fatores de Tempo , Condicionamento Pré-Transplante , Resultado do Tratamento , Turquia , Carga Viral , Adulto Jovem
18.
Pediatr Transplant ; 18(4): 405-11, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24802348

RESUMO

In this study, we retrospectively examined the data of children who underwent allo-HSCT from HLA-matched family donors. We analyzed the incidence, etiological factors, clinical characteristics, possible reasons, risk factors, and follow-up of neurologic complications. BU-based conditioning regimens were used in most of the cases (n = 62). The median duration of follow-up for the 89 patients was 20 months (range 1-41 months). Eleven percent of transplanted children developed one or more neurological symptoms after HSCT with a median observation time of two months (range -6 days to 18 months). The median age of the four girls and six boys with neurological complication was 13 yr (range 5.3-17.6 yr). Cylosporine A neurotoxicity was diagnosed in five children, four of them were PRES. The rest of complications were BU and lorazepam toxicity, an intracranial hemorrhage, a sinovenous thrombosis, and a transient ischemic attack during extracorpereal photopheresis. No difference was found between groups of neurological complication according to age, gender, diagnosis, hospitalization time, neutrophil and platelet engraftment time, stem cell source, and conditioning regimen, acute and chronic GVHD or VOD. Neurological complication was the cause of death in one patient (1.1%).


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Doenças do Sistema Nervoso/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Imunossupressores/efeitos adversos , Incidência , Masculino , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/epidemiologia , Avaliação de Resultados da Assistência ao Paciente , Estudos Retrospectivos , Fatores de Risco , Transplante Homólogo/efeitos adversos
20.
J Pediatr Hematol Oncol ; 36(1): e42-5, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23774160

RESUMO

BACKGROUND: Visceral leishmaniasis (VL) triggered genetic hemophagocytic lymphohistiocytosis (HLH) is clinically challenging. OBSERVATIONS: One-year-old VL-HLH patient improved after liposomal-amphotericin-B therapy, but subsequently deteriorated, although bone marrow amastigotes disappeared. Symptoms resolved after 8 weeks of HLH-2004 therapy but recurred upon cessation. Homozygous UNC13D gene 627delT mutation was identified however stem cell donor was unavailable. The patient died at age 4 years after central nervous system attacks and HLH recurrences. CONCLUSIONS: VL in HLH patients does not exclude a genetic etiology and requires structured clinical management. VL should be excluded in all HLH patients in endemic regions before immunochemotherapy, which is recommended for VL-HLH patients unresponsive to VL treatment and/or reactivated.


Assuntos
Leishmaniose Visceral/complicações , Leishmaniose Visceral/diagnóstico , Linfo-Histiocitose Hemofagocítica/genética , Linfo-Histiocitose Hemofagocítica/parasitologia , Proteínas de Membrana/genética , Diagnóstico Diferencial , Feminino , Humanos , Lactente
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