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1.
J Pediatr Hematol Oncol ; 39(8): e504-e507, 2017 11.
Artículo en Inglés | MEDLINE | ID: mdl-28731923

RESUMEN

With improvements in molecular diagnostic methods, report of Human bocavirus (HBoV) as an etiologic agent in many studies on viral respiratory and gastrointestinal infections has been increasing. Two pediatric patients who presented with secondary hemophagocytic lymphohistiocytosis were examined for etiologic causes, including viruses. Whole bacterial and fungal cultures and viral serological studies were negative. Viral polymerase chain reaction of nasopharyngeal secretions showed HBoV. One was successfully treated with intravenous immunoglobulins, whereas the other died with multiorgan failure. Here we report 2 pediatric patients with secondary hemophagocytic lymphohistiocytosis and detection of HBoV as the sole agent, predicting an association.


Asunto(s)
Bocavirus Humano , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/etiología , Infecciones por Parvoviridae/complicaciones , Biomarcadores , Médula Ósea/patología , Preescolar , Exantema/patología , Resultado Fatal , Femenino , Bocavirus Humano/genética , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Lactante , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Masculino , Infecciones por Parvoviridae/diagnóstico , Infecciones por Parvoviridae/virología , Reacción en Cadena de la Polimerasa , Resultado del Tratamiento
2.
Pediatr Blood Cancer ; 63(4): 695-700, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26703084

RESUMEN

BACKGROUND: Fanconi anemia (FA) is a heterogeneous autosomal recessive (and rarely X linked) disorder, which is characterized by congenital malformations, progressive bone marrow failure, and predisposition to malignancies. Hematopoietic stem cell transplantation (HSCT) is the only definitive treatment for the hematological manifestations in FA. PROCEDURE: Twenty-seven patients with FA underwent HSCT using fludarabine (Flu) based regimen at our center between April 2004 and May 2014. One patient who developed acute leukemia before HSCT was excluded from the study. The remaining 26 patients were included. The median age of the patients at the time of transplantation was 9.6 years (range 5.6-17.0 years) and male/female ratio was 19/7. Donors were Human leukocyte antigen (HLA)-identical sibling in 18 patients, HLA-identical other relatives in six patients, and HLA 1-antigen mismatched sibling in two patients. Conditioning regimen consisted of Flu, cyclophosphamide, and antithymocyte globulin. RESULTS: All patients engrafted but one developed poor graft function and underwent second HSCT. Acute graft versus host disease (GVHD) (≥grade 2) occurred in two patients (7.6%) and chronic GVHD in one patient (3.9%). Three patients developed venoocclusive disease (11.5%). Survival rate was 96.2% (25/26) at a median follow-up of 54 months (10-131 months) and all patients who survived were in good clinical condition. None of the patients developed secondary malignancy during the follow-up period. CONCLUSIONS: The present study from Turkey, a middle-income country, shows successful transplant outcome with low toxicity using Flu-based conditioning in patients with FA who underwent HSCT from HLA-related donors.


Asunto(s)
Anemia de Fanconi/cirugía , Trasplante de Células Madre Hematopoyéticas/métodos , Agonistas Mieloablativos/administración & dosificación , Acondicionamiento Pretrasplante/métodos , Vidarabina/análogos & derivados , Adolescente , Suero Antilinfocítico/administración & dosificación , Suero Antilinfocítico/efectos adversos , Niño , Preescolar , Ciclofosfamida/administración & dosificación , Ciclofosfamida/efectos adversos , Femenino , Enfermedad Injerto contra Huésped/epidemiología , Humanos , Masculino , Agonistas Mieloablativos/efectos adversos , Donantes de Tejidos , Turquía , Vidarabina/administración & dosificación
3.
Pediatr Transplant ; 20(2): 276-83, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26614176

RESUMEN

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.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Inmunoglobulina A/administración & dosificación , Inmunoglobulina M/administración & dosificación , Inmunoglobulinas Intravenosas/administración & dosificación , Adolescente , Anemia Aplásica/terapia , Niño , Femenino , Humanos , Inmunoglobulina G/química , Inmunoglobulinas Intravenosas/uso terapéutico , Leucemia/terapia , Masculino , Síndromes Mielodisplásicos/terapia , Estudios Prospectivos , Trasplante Homólogo , Resultado del Tratamiento , Talasemia beta/terapia
4.
J Pediatr Hematol Oncol ; 38(7): 539-43, 2016 10.
Artículo en Inglés | MEDLINE | ID: mdl-27548336

RESUMEN

The aim was to evaluate baseline demographic, clinical, and laboratory characteristics, treatment modalities, and outcome of children with idiopathic hypereosinophilic syndrome (HES) followed up in our center. Children who fulfilled the criteria of idiopathic HES followed up at Hacettepe University Faculty of Medicine, Pediatric Hematology Department between June 2004 and October 2013 were included in this study. Medical records of all children with idiopathic HES were reviewed to obtain regarding data. The mean age of 6 children with idiopathic HES was 52.8±44.3 months (13 to 132 mo) at diagnosis. Among 6 children with idiopathic HES; 2 had pulmonary involvement; 1 had cardiac and pulmonary involvement and splenomegaly; 1 had cardiac involvement and hepatosplenomegaly; 1 had cardiac and central nervous system involvement; and 1 had skin involvement. The mean follow-up duration was 36.5±31.4 months. Methyl prednisolone (MP) was used for the first-line therapy. Complete response was achieved with MP in 3 children. All steroid responsive children are alive; whereas 3 children who did not respond to MP had expired. In conclusion, cardiac and pulmonary involvement is the major causes of mortality in HES. Resistance to steroid therapy indicates poor prognosis.


Asunto(s)
Síndrome Hipereosinofílico/terapia , Niño , Preescolar , Femenino , Trasplante de Células Madre Hematopoyéticas , Humanos , Lactante , Masculino , Metilprednisolona/uso terapéutico
5.
J Pediatr Hematol Oncol ; 38(3): 232-4, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26907650

RESUMEN

Hypercalcemia is a rare complication of hematological malignancy in children. An 8-year-old girl with CALLA (+) Pre-B-cell ALL developed hypercalcemia during bone marrow relapse. She had nausea, vomiting, leg pain, polyuria, polydipsia, and muscle weakness. At the time of relapse, the ionized calcium level was 1.99 mmol/L. Rehydration with 0.9% saline and furosemide and methylprednisolone (MP) treatment were used for the treatment of hypercalcemia. The serum ionized calcium level increased to 2.2 mmol/L despite hydration, furosemide, and MP treatment. Then, a single-dose pamidronate (1 mg/kg/dose) was administered. Despite pamidronate treatment, the calcium level continued to rise. Next, calcitonin at a dose of 8 IU/kg/dose, 4 doses per day, was added to the treatment. After commencement of calcitonin treatment, her ionized calcium level decreased to normal reference ranges. In conclusion, because of the postponed effect of bisphosphonate treatment, pamidronate and calcitonin combination is an effective treatment option in the early resolution of malignancy-related hypercalcemia.


Asunto(s)
Conservadores de la Densidad Ósea/uso terapéutico , Hipercalcemia/tratamiento farmacológico , Hipercalcemia/etiología , Recurrencia Local de Neoplasia/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras B/complicaciones , Calcitonina/uso terapéutico , Niño , Difosfonatos/uso terapéutico , Femenino , Humanos
6.
J Pediatr Hematol Oncol ; 38(3): 240-2, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26907662

RESUMEN

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiologic condition characterized by headache, seizures, impaired vision, acute hypertension, and typical cranial MRI findings. OBSERVATION: A 10-year-old boy with FLT3-ITD-positive acute myelogenous leukemia who developed PRES during sorafenib treatment has been presented here. In English literature, there are 2 adult patients with metastatic cholangiocarcinoma or hepatocellular carcinoma who developed PRES under sorafenib treatment. Our patient is the first pediatric case with the diagnosis of acute myelogenous leukemia who developed PRES that might be attributed to sorafenib use. CONCLUSIONS: Thus, PRES might be a rare, potentially serious, but manageable, side effect of sorafenib that should be kept in mind by pediatric hematologists and oncologists.


Asunto(s)
Antineoplásicos/efectos adversos , Leucemia Mieloide Aguda/tratamiento farmacológico , Niacinamida/análogos & derivados , Compuestos de Fenilurea/efectos adversos , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Tirosina Quinasa 3 Similar a fms/genética , Niño , Humanos , Leucemia Mieloide Aguda/genética , Masculino , Recurrencia Local de Neoplasia/tratamiento farmacológico , Niacinamida/efectos adversos , Terapia Recuperativa/métodos , Sorafenib
7.
Biol Blood Marrow Transplant ; 21(11): 1888-94, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26265462

RESUMEN

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.


Asunto(s)
Células de la Médula Ósea/efectos de los fármacos , Factor Estimulante de Colonias de Granulocitos/farmacología , Leucocitos Mononucleares/efectos de los fármacos , Células Madre Mesenquimatosas/efectos de los fármacos , Adolescente , Células de la Médula Ósea/citología , Células de la Médula Ósea/inmunología , Diferenciación Celular/efectos de los fármacos , Proliferación Celular/efectos de los fármacos , Niño , Preescolar , Técnicas de Cocultivo , Medios de Cultivo Condicionados/química , Dinoprostona/genética , Dinoprostona/metabolismo , Femenino , Expresión Génica , Factor de Crecimiento de Hepatocito/genética , Factor de Crecimiento de Hepatocito/metabolismo , Humanos , Leucocitos Mononucleares/citología , Leucocitos Mononucleares/inmunología , Masculino , Células Madre Mesenquimatosas/citología , Células Madre Mesenquimatosas/inmunología , Cultivo Primario de Células , Donantes de Tejidos , Factor de Crecimiento Transformador beta1/genética , Factor de Crecimiento Transformador beta1/metabolismo
8.
J Clin Immunol ; 35(2): 189-98, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25627830

RESUMEN

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.


Asunto(s)
Estudios de Asociación Genética , Factores de Intercambio de Guanina Nucleótido/deficiencia , Factores de Intercambio de Guanina Nucleótido/genética , Adolescente , Adulto , Niño , Preescolar , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Humanos , Inmunoglobulina E/sangre , Inmunoglobulina E/inmunología , Incidencia , Lactante , Infecciones/diagnóstico , Infecciones/epidemiología , Infecciones/etiología , Síndrome de Job/complicaciones , Síndrome de Job/diagnóstico , Síndrome de Job/genética , Síndrome de Job/inmunología , Síndrome de Job/mortalidad , Síndrome de Job/terapia , Recuento de Linfocitos , Subgrupos Linfocitarios/inmunología , Subgrupos Linfocitarios/metabolismo , Masculino , Persona de Mediana Edad , Mutación , Neoplasias/epidemiología , Neoplasias/etiología , Fenotipo , Adulto Joven
9.
Pediatr Transplant ; 19(5): E126-9, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25996881

RESUMEN

Vascular complications are important causes of allograft loss in renal transplantation. A two and a half-month-old boy was diagnosed with posterior urethral valve and progressed to end-stage renal disease at eight yr of age. During the HD period, a central venous catheter was replaced three times for repeated thrombosis. The boy was found to be homozygous for FVL and heterozygous for both MTHFR (C677T) and PAI. At the age of 12, renal transplantation was performed from a deceased donor. Postoperative anticoagulation therapy was initiated with continuous intravenous administration of heparin at the dose of 10 IU/kg/h. HD was performed for the first three days. By the fourth day of transplantation, his urine output had increased gradually. Heparin infusion was continued for 18 days during hospitalization at the same dosage. Thereafter, he was discharged with LMWH. On the third month after transplantation, his serum creatinine level was 1.1 mg/dL and eGFR was 75.7 mL/min/1.73 m(2). He has still been using LMWH, and his eGFR was 78.7 mL/min/1.73 m(2) eight months after transplantation. Postoperative low-dose heparin treatment is a safe strategy for managing a patient with multiple thrombotic risk factors.


Asunto(s)
Factor V/genética , Trasplante de Riñón , Metilenotetrahidrofolato Reductasa (NADPH2)/genética , Mutación , Inhibidor 1 de Activador Plasminogénico/genética , Cateterismo Venoso Central , Niño , Creatinina/sangre , Tasa de Filtración Glomerular , Heparina/uso terapéutico , Heterocigoto , Homocigoto , Humanos , Donadores Vivos , Masculino , Persona de Mediana Edad , Insuficiencia Renal/genética , Insuficiencia Renal/cirugía , Factores de Riesgo , Trombofilia/genética , Trombosis/terapia , Resultado del Tratamiento
10.
J Pediatr Hematol Oncol ; 37(1): e19-22, 2015 01.
Artículo en Inglés | MEDLINE | ID: mdl-25522351

RESUMEN

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.


Asunto(s)
Antifúngicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Micosis/prevención & control , Adolescente , Anfotericina B/uso terapéutico , Caspofungina , Niño , Equinocandinas/uso terapéutico , Femenino , Humanos , Lipopéptidos , Masculino , Estudios Retrospectivos
11.
Transfus Apher Sci ; 52(3): 332-8, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25779226

RESUMEN

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.


Asunto(s)
Células de la Médula Ósea/citología , Factor Estimulante de Colonias de Granulocitos/química , Antígenos HLA/metabolismo , Movilización de Célula Madre Hematopoyética/métodos , Trasplante de Células Madre Hematopoyéticas , Adolescente , Adulto , Antígenos CD34/metabolismo , Niño , Preescolar , Supervivencia sin Enfermedad , Anemia de Fanconi/terapia , Femenino , Enfermedad Injerto contra Huésped , Células Progenitoras de Granulocitos y Macrófagos/citología , Humanos , Lactante , Tiempo de Internación , Leucemia Mieloide Aguda/terapia , Masculino , Síndromes Mielodisplásicos/terapia , Neutrófilos/citología , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Estudios Retrospectivos , Trasplante Homólogo , Resultado del Tratamiento , Adulto Joven , Talasemia beta/terapia
12.
Clin Exp Nephrol ; 19(3): 506-13, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24993948

RESUMEN

BACKGROUND: The aim of this study was to describe the incidence and contributory risk factors for thromboembolic complications in children with nephrotic syndrome (NS) and thrombosis. METHODS: Among 188 children with the diagnosis of NS (80 girls; mean age: 12.6 ± 5.4 years) followed up in our hospital for the last 5 years, 17 (9.0 %) children (16 boys) identified as having thromboembolic complications. All 17 children with NS and thrombosis were screened for laboratory risk factors for thrombosis. The diagnosis was confirmed by cranial magnetic resonance imaging, doppler ultrasonography, and echocardiography. RESULTS: Among 17 children with thrombosis, 14 (82.3 %) were found to have focal segmental glomerulosclerosis (FSGS) as underlying pathology by renal biopsy. The mean age of the thrombotic children was 4.5 ± 3.2 years at the diagnosis of NS and that was 7.1 ± 4.9 years at the time of thrombosis. The mean time from NS diagnosis to the first thrombosis development was 2.6 ± 2.3 years. Thrombosis occurred during the first year of NS in 9/17 (52.9 %) children. Most of the children (88.2 %) had venous thrombosis. Among the screened risk factors, high factor VIII level (64.7 %) was the leading factor followed by decreased antithrombin III level (29.4 %). Furthermore, 4 children had central venous catheters and 2 had infection as clinical risk factors for thrombosis. CONCLUSION: In this case series, subtype of FSGS, active disease state of NS, central venous catheters, and some inherited and acquired thrombotic risk factors have been identified as contributory factors for the development of thrombosis in children with NS.


Asunto(s)
Síndrome Nefrótico/complicaciones , Trombosis de la Vena/epidemiología , Trombosis de la Vena/etiología , Adolescente , Edad de Inicio , Antitrombina III/metabolismo , Aspirina/uso terapéutico , Infarto Encefálico/tratamiento farmacológico , Infarto Encefálico/etiología , Catéteres Venosos Centrales/efectos adversos , Niño , Preescolar , Factor VIII/metabolismo , Femenino , Fibrinolíticos/uso terapéutico , Estudios de Seguimiento , Glomeruloesclerosis Focal y Segmentaria/complicaciones , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Incidencia , Lactante , Masculino , Metilenotetrahidrofolato Reductasa (NADPH2)/genética , Mutación , Síndrome Nefrótico/sangre , Síndrome Nefrótico/diagnóstico , Factores de Riesgo , Trombectomía , Factores de Tiempo , Turquía/epidemiología , Trombosis de la Vena/sangre , Trombosis de la Vena/terapia
13.
J Clin Lab Anal ; 29(4): 259-62, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24840114

RESUMEN

BACKGROUND: Early life-threatening cardiotoxicity and cardiac death have been reported after hematopoietic stem cell transplantation (HSCT). The purpose of the current study was to evaluate cardiac toxicity of conventional chemotherapy followed by HSCT with cardiac markers: heart-type fatty acid binding protein (H-FABP), glycogen phosphorylase BB (GPBB), high sensitive C reactive protein (hsCRP) cardiac troponin I, (cTnI), creatine kinase MB (CK-MB mass) and myoglobin. METHODS: A total of 20 children who underwent HSCT for malignant and non-malignant diseases were included in this study. Blood samples were collected from all patients in 0th, 7th and 21st day for evaluating these cardiac biomarkers. The patients' echocardiography was assessment before and after one-month of HSCT. RESULTS: Serum 21st H-FABP level was significantly higher when compared with the 0th day H-FABP level (P < 0.05) . 7th day hsCRP level was significantly higher than 0th and 21st day levels (P < 0.05). Interestingly, 7th day GPBB level was significantly lower than 0th and 21st day levels (P < 0.05). Myoglobin, CK-MB mass and cTnI biomarkers remained within the reference range in all patients. CONCLUSIONS: This study showed that H-FABP and hsCRP both seem to be promising markers for evaluation of cardiotoxicity in HSCT process and probably superior to GPBB, cTnI, CK-MB mass and myoglobin.


Asunto(s)
Biomarcadores/metabolismo , Trasplante de Células Madre Hematopoyéticas , Miocardio/metabolismo , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Adulto Joven
14.
Pediatr Cardiol ; 36(4): 862-6, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25577226

RESUMEN

Improvement in long-term survival in patients with acute childhood leukemia has led to the need for monitorization of chemotherapy-related morbidity and mortality. This study included 60 patients with acute lymphoblastic leukemia that were in remission for at least 2 years and 30 healthy controls. Systolic and diastolic function of myocardium was evaluated using conventional echocardiography and tissue Doppler imaging of the left ventricle, interventricular septum and right ventricle. Median age of patients was 11.7 years (range 10-14.9 years), and the median duration of remission was 4 years (range 2.5-5 years). All patients were treated with a low cumulative dose of adriamycin (100 mg/m(2)) according to the St. Jude Total-XIIIA protocol. The ejection fraction (EF) and fractional shortening were normal in the patient and control groups, even though EF values were significantly lower in the patients (69.5 ± 2.3 vs. 72.7 ± 3 %, P < 0.01). Myocardial systole (S m), early diastole (E m) and late diastole (A m) velocities in all segments of the myocardium were significantly lower in the patient group (P < 0.01 for all segments). Cardiotoxicity was noted in all segments of the myocardium in the patient group, despite the fact that they were all treated with a low cumulative dose of adriamycin. Based on these findings, we think that there is no safe dose for anthracyclines and periodic echocardiographic evaluation of both the left and right ventricles must be performed in all patients treated with anthracyclines, even at low doses.


Asunto(s)
Antraciclinas/efectos adversos , Antibióticos Antineoplásicos/efectos adversos , Cardiotoxicidad/diagnóstico por imagen , Cardiotoxicidad/etiología , Ecocardiografía Doppler , Sobrevivientes/estadística & datos numéricos , Adolescente , Antraciclinas/administración & dosificación , Antraciclinas/uso terapéutico , Niño , Diagnóstico por Imagen , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Leucemia/tratamiento farmacológico , Masculino
15.
Pediatr Blood Cancer ; 61(4): 763-4, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24115609

RESUMEN

Homozygous protein C deficiency is among rare causes of thrombophilia. Herein, we present a neonate with purpura fulminans, disseminated intravascular coagulation and severe intracranial hemorrhage who was found to have plasma protein C level of 4%. The molecular work-up revealed a novel homozygous mutation of T903C (amino acid position Leu 270 Pro) located in a catalytic domain region of PROC gene. Asymptomatic course in patients with low but measurable levels of protein C levels has been reported, which is different than observed in our patient who had a very severe course despite plasma protein C level of 4%.


Asunto(s)
Coagulación Intravascular Diseminada/etiología , Hemorragias Intracraneales/etiología , Mutación/genética , Deficiencia de Proteína C/complicaciones , Proteína C/genética , Púrpura Fulminante/etiología , Coagulación Intravascular Diseminada/patología , Homocigoto , Humanos , Recién Nacido , Hemorragias Intracraneales/patología , Masculino , Fenotipo , Pronóstico , Deficiencia de Proteína C/genética , Púrpura Fulminante/patología
16.
Pediatr Transplant ; 18(4): 405-11, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24802348

RESUMEN

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%).


Asunto(s)
Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedades del Sistema Nervioso/etiología , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Inmunosupresores/efectos adversos , Incidencia , Masculino , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/epidemiología , Evaluación del Resultado de la Atención al Paciente , Estudios Retrospectivos , Factores de Riesgo , Trasplante Homólogo/efectos adversos
17.
Pediatr Transplant ; 18(4): E130-3, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24724984

RESUMEN

CDA are a group of inherited, rare diseases that are characterized by dyserythropoiesis and ineffective erythropoiesis associated with transfusion dependency in approximately 10% of cases. For these latter patients, the only curative treatment is HSCT. There are very limited data on HSCT experience in this rare disease. Herein, we report a five-yr six-month-old girl with compound heterozygous mutations in SEC23B gene, who was diagnosed to have CDA type II and underwent successful HSCT from her matched sibling donor.


Asunto(s)
Anemia Diseritropoyética Congénita/terapia , Trasplante de Células Madre Hematopoyéticas , Anemia Diseritropoyética Congénita/genética , Preescolar , Femenino , Marcadores Genéticos , Heterocigoto , Humanos , Mutación , Hermanos , Proteínas de Transporte Vesicular/genética
18.
J Pediatr Hematol Oncol ; 36(1): e39-41, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23389505

RESUMEN

BACKGROUND: Although splenic abnormalities are common in patients with lupus, spontaneous rupture of spleen is extremely rare. OBSERVATIONS: A 15-year-old boy with new-onset Evans syndrome subsequently diagnosed as systemic lupus erythematosus developed spontaneous rupture of spleen during the course of his illness. Despite the severe thrombocytopenia, he was managed conservatively with gradual regression of hematoma without further complication. CONCLUSIONS: Splenic rupture may occur spontaneously in the course of systemic lupus erythematosus. We conclude that conservative treatment of splenic rupture may be preferred especially in immunocompromised patients to avoid surgical complications.


Asunto(s)
Anemia Hemolítica Autoinmune/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Rotura del Bazo/diagnóstico , Rotura del Bazo/etiología , Trombocitopenia/diagnóstico , Adolescente , Diagnóstico Diferencial , Humanos , Masculino , Rotura Espontánea
19.
Transfus Apher Sci ; 50(3): 467-72, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24656491

RESUMEN

The study was designed to compare colony forming capacity of granulocyte-colony stimulating factor (G-CSF) stimulated bone marrow (G-BM) with standard unstimulated bone marrow (U-BM) of healthy donors of pediatric patients. CFU-Assay results of 26 healthy donors of pediatric patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) were analyzed retrospectively. 13 of donors received 10 µg/kg per day of G-CSF as a single injection for 3 consecutive days and other 13 of donors had unstimulated BM. Colony forming capacity of hematopoietic stem cells evaluated with Colony Forming Unit-Assay (CFU-Assay) with in semi-solid agar culture medium after 14-18 days of culture period. CFU-Assay results of G-BM and U-BM (expressed as means) were; Burst Forming Unit-Erythroid (BFU-E): 15.20 × 10(4)/kg and 8.38 × 10(4)/kg, Colony Forming Unit-Granulocyte Macrophage (CFU-GM): 10.35 × 10(4)/kg and 5.67 × 10(4)/kg, Colony Forming Unit-Erythroid (CFU-E): 0.59 × 10(4)/kg and 0.33 × 10(4)/kg, CFU-Granulocyte Erythroid Macrophage Megakaryocyte (CFU-GEMM): 0.52 × 10(4)/kg and 0.53 × 10(4)/kg respectively. BFU-E and CFU-GM capacity of G-BM was increased and statistically significantly different than standard U-BM (p ⩽ 0.01). In conclusion, increased colony forming capacity of hematopoietic stem cells of G-BM when compared with standard unstimulated BM could be a major advantage for transplantation.


Asunto(s)
Médula Ósea/metabolismo , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Movilización de Célula Madre Hematopoyética , Trasplante de Células Madre Hematopoyéticas , Células Madre Hematopoyéticas/metabolismo , Adolescente , Adulto , Aloinjertos , Células Cultivadas , Niño , Preescolar , Femenino , Células Madre Hematopoyéticas/citología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo
20.
Pediatr Hematol Oncol ; 31(7): 607-15, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24854890

RESUMEN

The endocrinological complications in ß-thalassemia major patients do affect the life quality to a large extend. In this study, the endocrinological complications of 47 ß-thalassemia patients, who have been followed-up at our hospital's pediatric hematology department, were evaluated. Out of ß-thalassemia major cases included to this study, the 55.3% was male and 44.7% was female. The patients' mean levels of ferritin, whose mean age was 10.0 ± 4.5 years (2-20 years), were 2497 ± 1469 ng/mL (472-8558 ng/mL). At least one endocrinological pathology in 27 out of 47 (57.4%) and more than one endocrinological pathology in 14 out of 47 (29.7%) thalassemia patients were observed. The most frequently observed complication in followed-up cases was vitamin D insufficiency and deficiency (78.2%). The other complications in decreasing order were pubertal failure (41.6%), growth retardation (25.5%), decreased bone-mineral density (22.2%), secondary hyperparathyroidism (11.5%), overt hypothyroidism (4.25%), subclinical hypothyroidism (2.12%), and impaired glucose tolerance (2.12%). There was no statistically significant difference between serum mean ferritin level and endocrin complications (P > .05). Four patients (8.5%) had decreased signal intensity in pituitary magnetic resonance imaging (MRI) but this finding was not associated with ferritin levels (P = .87). MRI parameters were similar between patients with and without gonadal dysfunction. Mean height of the pituitary gland was 4.98 ± 1.1 mm (3-9 mm) and this was similar to those normal values in the literature. Ferritin levels were not correlated with pituitary height (P > .05). Beta thalassemia major, having the potential of leading to multisystemic complications, is a chronic disease that should be treated and followed-up by a multidisciplinary approach. Due to frequently encountered endocrinological complications, beta thalassemic patients should be followed-up regularly by hematology and endocrinology departments in coordination.


Asunto(s)
Enfermedades del Sistema Endocrino/etiología , Talasemia beta/complicaciones , Adolescente , Densidad Ósea , Niño , Preescolar , Femenino , Ferritinas/sangre , Humanos , Masculino , Deficiencia de Vitamina D/etiología , Adulto Joven
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