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1.
J Pediatr Hematol Oncol ; 46(2): e137-e142, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38132565

RESUMO

OBJECTIVE: To determine the prevalence of iron overload in children with acute lymphoblastic leukemia (ALL) after treatment cessation and establish a cutoff value for serum ferritin level as an indicator of iron overload. BACKGROUND: Early detection and monitoring of iron overload in patients with leukemia is crucial. METHODS: In this prospective cohort study, 66 pediatric patients with ALL who were treated at a tertiary referral center affiliated with Shiraz University of Medical Sciences in Shiraz, Southern Iran, were investigated from July 2020 to December 2022. Serum ferritin levels were measured 6 months after treatment completion. T2* magnetic resonance imaging of the liver and heart was done for all patients. The receiver operating characteristic curve was used to illustrate the area under the receiver operating characteristic curve to assess the diagnostic value of serum ferritin level and total transfusion volume. RESULTS: A total of 24 patients (36.4%) had iron overload in the heart or liver based on T2 magnetic resonance imaging findings. Serum ferritin level was a highly accurate diagnostic marker for iron overload in pediatric patients with ALL, with a sensitivity of 95.8%, and specificity of 85.7% for a cutoff value of 238.5 ng/mL. Also, blood transfusion was a good predictor of iron overload a sensitivity of 75% and specificity of 81% for a cutoff value of 28.3 mL/kg. CONCLUSION: We identified specific cutoff values for serum ferritin and blood transfusion volume to predict iron overload with high sensitivity and specificity. These markers offer a cost-effective and accessible approach for periodic screening of iron deposition, particularly in resource-constrained settings.


Assuntos
Sobrecarga de Ferro , Leucemia , Humanos , Criança , Ferritinas , Estudos Prospectivos , Sobrecarga de Ferro/diagnóstico , Sobrecarga de Ferro/etiologia , Coração , Imageamento por Ressonância Magnética/métodos , Fígado/patologia , Leucemia/patologia
2.
Am J Blood Res ; 13(5): 143-151, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38023416

RESUMO

INTRODUCTION: Febrile neutropenia is a serious complication of cancer chemotherapy that can result in delays in treatment. This study evaluates the efficacy of A. ampeloprasum L. at neutrophil recovery time in children with chemotherapy-associated febrile neutropenia. METHODS: This single-center, parallel-group, double-blind, randomized clinical trial was conducted at an oncology hospital. Patients selected among childhood cancers with febrile neutropenia. Overall, 97 febrile neutropenic children were enrolled. The intervention group (n=49) was given A. ampeloprasum L. in capsules (500 mg twice daily) for seven days plus supportive care. The control group (n=48) was treated similarly with supportive care and placebo capsules. Total white blood cell (WBC) and absolute neutrophil counts (ANC) were checked daily and neutrophil recovery time in both groups was compared. RESULTS: Patients in the intervention group experienced shorter neutrophil recovery compared to the control group (4.02 ± 2.32 days vs. 6.38 ± 2.80 days, respectively, P less than 0.001). The intervention group was discharged from the hospital earlier than the control group with a mean of two days, but it did not reach statistical significance (P=0.133). Mean WBC and ANC were not significantly different in the two groups. Herbal medicine was well tolerated, and no adverse effect was reported. CONCLUSIONS: A fresh, lyophilized extract from deciduous leaves of A. ampeloprasum L. can effectively shorten the ANC recovery time leading to an earlier release from the hospital. The trial was registered in the Iranian Registry of Clinical Trials with registration No. IRCT2015051615666N2 (http://www.irct.ir/).

3.
J Orthop Surg Res ; 18(1): 907, 2023 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-38017431

RESUMO

BACKGROUND: Arthropathy is a common complication in patients with hemophilia. We examined the prevalence of this skeletal complication in patients with hemophilia who were registered at a Comprehensive Hemophilia Center in Shiraz, Southern Iran. MATERIALS AND METHODS: In this cross-sectional study, an orthopedic specialist visited 448 patients and conducted screenings for skeletal complications. The assessment included evaluating the type of hemophilia, disease severity, treatment modality, the presence of inhibitors, and the identification of skeletal complications. RESULTS: Ninety patients with hemophilia A, with a mean age (SD) of 31.6 (14.4) years, and 10 patients with hemophilia B, with a mean age of 30.5 (20.6) years, were assessed. The most frequently affected joints were the knee and ankle joints. In the univariate analysis, patients with severe disease were more likely to exhibit synovitis, a target joint, and bone disease compared to patients with non-severe disease. Additionally, a history of treated or active hepatitis and an annual bleeding rate showed significant associations with the target joint. In the multivariable logistic regression analysis, disease severity (OR 14.43, 95% CI 1.6-129.6) and a higher age at diagnosis (OR 1.06, 95% CI 1.00-1.13) increased the likelihood of developing osteoporosis. A history of hepatitis (OR 3.67, 95% CI 1.28-10.48) was identified as an independent risk factor for the target joint. CONCLUSION: Skeletal complications are a common occurrence in hemophilia. Regular consultations with orthopedic specialists, focusing on bleeding control and hepatitis prevention, are essential for reducing the impact of this debilitating complication.


Assuntos
Hemofilia A , Hemofilia B , Hepatite , Humanos , Adulto , Hemofilia A/complicações , Hemofilia A/epidemiologia , Hemartrose/diagnóstico , Hemartrose/etiologia , Hemartrose/prevenção & controle , Estudos Transversais , Hemofilia B/complicações , Hemofilia B/epidemiologia , Hemorragia , Hepatite/complicações
4.
Case Rep Oncol ; 16(1): 1041-1047, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37900809

RESUMO

Teratoma is a type of germ cell tumor layer that appears in the gonadal, sacrococcygeal, mediastinal, and retroperitoneal regions. Primary retroperitoneal teratoma is rare and asymptomatic but can present with symptoms due to a mass effect on neighboring organs. These tumors have to be considered in the differential diagnosis of a mass in the abdominal cavity of children to distinguish between Wilms' tumor, neuroblastoma, and other intra-abdominal lesions. We presented an infant boy with protrusion of the left upper quadrant of the abdomen and a palpable abdominal mass that had progressively enlarged. An abdominal computed tomography scan revealed a large retroperitoneal cystic, solid mass on the left side of the abdominal cavity, causing pressure on the left ureter. Also, hydronephrosis of the left kidney was seen with a decreased enhancement of the left kidney due to obstruction uropathy. The mass was suspicious on imaging for a retroperitoneal teratoma. The patient underwent laparotomy, and excision of the huge retroperitoneal mass was done. The final diagnosis was an immature teratoma grade 3, and the patient was discharged in good condition. Retroperitoneal teratomas are rare tumors in infants. These tumors would be an incident diagnosis or diagnosed by the mass effect of giant tumors on other organs. They must be considered in the differential diagnosis of intra-abdominal tumors in children. Hydronephrosis and obstructive uropathy can be rare consequences of the mass effects of these tumors.

5.
J Psychopharmacol ; 37(10): 1003-1010, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37466276

RESUMO

BACKGROUND: Due to the inflammatory factors in the pathophysiology of schizophrenia, Pentoxifylline, as anti-inflammatory medication, seems to improve the symptoms of schizophrenia. This study aims to evaluate the efficacy of Pentoxifylline as an adjunctive therapy on cognitive deficits and symptoms of schizophrenia. METHODS: This randomized double-blind placebo-controlled clinical trial was conducted on 52 patients diagnosed with chronic schizophrenia. All patients were divided into two, treatment and control groups. They received a 400 mg dose of Pentoxifylline and the placebo in the treatment and control groups, respectively, twice a day for 8 weeks. Then, they were tested with the Positive and Negative Syndrome Scale (PANSS), Wisconsin Card Sorting Test (WCST), digit span, Stroop test, and Rey Auditory and Verbal Learning Test at baseline and the end of the weeks 4 and 8. Data analysis was done by Kolmogorov-Smirnov test, t-test, Mann-Whitney test, chi-square test, and generalized estimating equation model. RESULTS: After analyzing the data, it was revealed that the positive symptoms of PANSS, the number of errors in the incongruent Stroop test, and reaction time in the congruent Stroop test were significantly lower in the treatment group (p < 0.05). Moreover, the number of categories of WCST was significantly higher in the treatment group (p < 0.05). There was no statistically significant difference in other parameters between the control and treatment groups (p > 0.05). CONCLUSIONS: As evidenced by the results of this study, Pentoxifylline can help alleviate schizophrenia cognitive deficits and can reduce psychotic symptoms. Therefore, it can potentially be useful for schizophrenia treatment.


Assuntos
Antipsicóticos , Disfunção Cognitiva , Pentoxifilina , Esquizofrenia , Humanos , Esquizofrenia/complicações , Esquizofrenia/tratamento farmacológico , Pentoxifilina/uso terapêutico , Disfunção Cognitiva/tratamento farmacológico , Cognição , Método Duplo-Cego , Resultado do Tratamento , Quimioterapia Combinada , Escalas de Graduação Psiquiátrica
6.
Egypt Heart J ; 75(1): 21, 2023 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-36961611

RESUMO

BACKGROUND: Cardiotoxicity is a major concern following doxorubicin (DOX) use in the treatment of malignancies. We aimed to investigate whether deferoxamine (DFO) can prevent acute cardiotoxicity in children with cancer who were treated with DOX as part of their chemotherapy. RESULTS: Sixty-two newly-diagnosed pediatric cancer patients aged 2-18 years with DOX as part of their treatment regimens were assigned to three groups: group 1 (no intervention, n = 21), group II (Deferoxamine (DFO) 10 times DOX dose, n = 20), and group III (DFO 50 mg/kg, n = 21). Patients in the intervention groups were pretreated with DFO 8-h intravenous infusion in each chemotherapy course during and after completion of DOX infusion. Conventional and tissue Doppler echocardiography, serum concentrations of human brain natriuretic peptide (BNP), and cardiac troponin I (cTnI) were checked after the last course of chemotherapy. Sixty patients were analyzed. The level of cTnI was < 0.01 in all patients. Serum BNP was significantly lower in group 3 compared to control subjects (P = 0.036). No significant differences were observed in the parameters of Doppler echocardiography. Significant lower values of tissue Doppler late diastolic velocity at the lateral annulus of the tricuspid valve were noticed in group 3 in comparison with controls. By using Pearson analysis, tissue Doppler systolic velocity of the septum showed a marginally significant negative correlation with DOX dose (P = 0.05, r = - 0.308). No adverse effect was reported in the intervention groups. CONCLUSIONS: High-dose DFO (50 mg/kg) may serve as a promising cardioprotective agent at least at the molecular level in cancer patients treated with DOX. Further multicenter trials with longer follow-ups are needed to investigate its protective role in delayed DOX-induced cardiac damage. Trial registration IRCT, IRCT2016080615666N5. Registered 6 September 2016, http://www.irct.ir/IRCT2016080615666N5 .

7.
Clin Appl Thromb Hemost ; 29: 10760296221130335, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36630731

RESUMO

Data describing physicians' and patients' perspectives towards immune thrombocytopenia (ITP) management and impact of disease in Iran are limited. This ITP World Impact Survey was conducted between October 2019 and October 2020. Of the 114 patients included in the survey, 17 were aged ≤18 years. Forty-seven physicians, including 22 pediatric hematologists, participated in the survey. Fatigue and anxiety around stable platelet counts were frequent patient-reported symptoms at diagnosis and at survey completion. According to physicians, "watch-and-wait" was the preferred treatment option for mean (standard deviation) proportion of 50.1 (24.1) and 48.6 (21.8) of their adult and pediatric patients, respectively, following first diagnosis. Per adult and pediatric hematologists, the most prescribed treatments for newly diagnosed patients based on available answers were steroids (100%, n = 20/20; 89%, n = 16/18), respectively. Forty percent of adult (n = 10/25) and 38% of pediatric hematologists (n = 8/21) reported that ITP reduced patients' quality of life. Energy levels (46%, n = 52/112) and ability to concentrate on everyday activities (42%, n = 47/113) were the most affected aspects of patients' lives. This I-WISh study in Iran underlined the negative impact of ITP on patients.


Assuntos
Médicos , Púrpura Trombocitopênica Idiopática , Adulto , Humanos , Criança , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Irã (Geográfico) , Qualidade de Vida , Estudos Retrospectivos , Trombopoetina
8.
Cancer Rep (Hoboken) ; 6(4): e1784, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36700480

RESUMO

BACKGROUND: The survival of childhood leukemia has improved. We aimed to report the survival rate and the associated factors in children with acute leukemia during an 8-year follow-up. AIMS: This study investigates the 8-year survival rates of children with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) in Shiraz, the largest oncology center in Southern Iran. We also aimed to assess the independent factors associated with higher mortality in childhood leukemia. METHODS: Children 0-18 years with acute leukemia were followed from 2013 to 2021 in Shiraz, Iran. The 8-year overall survival (OS) and event-free survival (EFS) rates were estimated by the Kaplan-Meier method. Independent factors associated with survival were assessed by the Cox regression hazard modeling. RESULTS: We included 786 children, with 43.5% female, and a mean age of 6.32 ± 4.62 years. Patients with AML compared to ALL experienced more relapse (34.6% vs. 22.5%, p = .01) and death (31.7% vs. 11.3%, p < .001). The cumulative 8-year OS and EFS were 81% (95% confidence interval (CI), 74.3% to 86.1%) and 68.3% (95% CI, 63.5% to 72.7%) in ALL patients and 63.5% (95% CI, 52.1% to 72.9%) and 43% (95% CI, 33.1% to 52.6%) in AML patients. Multivariable analysis revealed that hepatomegaly (hazard ratio = 4, 95% CI, 1.0 to 22.3, p = .05) was the main independent risk factor of death in ALL patients. No definite risk factor was defined for AML patients. CONCLUSION: The survival of childhood leukemia has recently increased dramatically in low-middle income countries. Hepatomegaly was introduced as a potential risk factor for lower survival in ALL patients. Further multicenter studies are needed to confirm the validity of this association.


Assuntos
Leucemia Mieloide Aguda , Leucemia-Linfoma Linfoblástico de Células Precursoras , Criança , Humanos , Feminino , Lactente , Pré-Escolar , Masculino , Hepatomegalia , Protocolos de Quimioterapia Combinada Antineoplásica , Estudos Retrospectivos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia Mieloide Aguda/tratamento farmacológico
9.
J Pediatr Hematol Oncol ; 45(1): e97-e102, 2023 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-36162044

RESUMO

BACKGROUND: The SARS-CoV-2 infection has been associated with potentially endothelial damage and coagulation cascade activation that cause thrombosis. There is limited information on thrombosis and anticoagulant therapy in children with coronavirus disease 2019 (COVID-19). AIMS: This study evaluates the outcome of thromboprophylaxis in children younger than 18-year old with COVID-19 infection. METHODS: A retrospective study was conducted on 184 hospitalized pediatric patients with confirmed COVID-19 infection. A designed questionnaire was made to collect all demographic, clinical, and laboratory data. According to World Health Organization, the patients were classified as asymptomatic/mild, moderate, severe, and critically ill. RESULTS: The mean age of the patients was 7.04±5.9 (1 wk to younger than 18 y). Overall, 33 patients received anticoagulant therapy. All patients who passed away (n=19) belonged to the critical group. One patient (1.28%) was complicated with deep vein thrombosis despite taking thromboprophylaxis, and 1 (1.28%) with pulmonary thromboembolism while the patient did not take an anticoagulant. CONCLUSIONS: Our data showed a lower rate of thrombosis (1.4%) than adult patients with COVID-19. It may underline the role of anticoagulants in moderate to severe/critically ill children with COVID-19 infection. Expert opinion and personal experience are necessary, while we have a significant knowledge gap in understanding COVID-19-associated coagulopathy and thrombotic risk in children.


Assuntos
COVID-19 , Trombose , Tromboembolia Venosa , Humanos , Criança , Adolescente , COVID-19/complicações , Anticoagulantes/uso terapêutico , Estudos Retrospectivos , Estado Terminal , SARS-CoV-2 , Tromboembolia Venosa/etiologia , Trombose/etiologia , Trombose/prevenção & controle , Trombose/tratamento farmacológico
10.
J Med Case Rep ; 16(1): 422, 2022 Nov 03.
Artigo em Inglês | MEDLINE | ID: mdl-36329514

RESUMO

BACKGROUND: Recently, an unknown hepatitis outbreak among children has concerned many individuals worldwide. These cases are frequently reported, mainly from Europe and other countries. In this study, we present two similar patients, who, to the best of our knowledge, are the first cases reported in the Middle East (Shiraz, Fars Province, Iran). Unlike in similar cases reported up until 30 April 2022, our patients' hepatitis eventually resulted in aplastic anemia. CASE PRESENTATION: In this study, we present cases of two Iranian boys aged 13 and 8 years with hepatitis of unknown origin who developed aplastic anemia in the course of hospitalization. CONCLUSIONS: Hepatitis-associated aplastic anemia is a well-known immune-mediated form of aplastic anemia that we detected in our patients and treated with immunosuppressive therapy. One patient established a satisfactory response to the treatment, but unfortunately, the other was declared brain dead.


Assuntos
Anemia Aplástica , Hepatite , Criança , Masculino , Humanos , Anemia Aplástica/complicações , Irã (Geográfico)/epidemiologia , Hepatite/complicações , Surtos de Doenças , Terapia de Imunossupressão
11.
Clin Lab ; 68(7)2022 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-35975541

RESUMO

BACKGROUND: Understanding the determinants of long-term overall survival (OS) of thalassemia patients (TPs) is the mainstay of care. METHODS: As a retrospective survey, we assessed the data of 769 TPs who had regular follow-up and blood transfusion for at least 30 years from 1990 - 2019. We utilized semi-parametric proportional hazards mixture cure-rate regression to discover the factors with a significant effect on short- and long-term OS separately. RESULTS: The 25- and 30-year OS for the TPs were calculated to be 98.7% and 90.4%, respectively. Each five-year age escalation was associated with a 30% decrease in the probability of being short-term survivors (HR = 1.06, p = 0.047). Parental family relationship influenced both cured (OR = 3.00, p = 0.017) and uncured (HR = 0.50, p = 0.046) TPs. Moreover, the type of iron chelation drug, liver iron concentration, and normal EF results had a significant effect on long-term OS. Aging, parental consanguinity, liver and cardiac siderosis, higher ferritin levels, and low hemoglobin level were associated with poorer prognosis in TPs. CONCLUSIONS: However, deferoxamine followed by multiple drugs as iron chelation, severe liver siderosis, and abnormal EF declined the probability of long-term OS among TPs. This can be considered by health policy decision-makers to enforce the screening program more strictly.


Assuntos
Sobrecarga de Ferro , Siderose , Talassemia , Talassemia beta , Humanos , Ferro , Quelantes de Ferro , Prognóstico , Estudos Retrospectivos , Siderose/complicações , Talassemia/terapia , Talassemia beta/complicações , Talassemia beta/terapia
12.
Blood Coagul Fibrinolysis ; 33(3): 159-161, 2022 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-35165218

RESUMO

Diagnosis of inherited platelet glycoprotein disorders is based on specific laboratory techniques such as aggregometry and flow cytometry. Flowcytometry is a powerful method, but equivocal results are produced in some cases. New cluster of differentiation markers could resolve the diagnostic dilemmas. Abnormal expression of CD9 in Bernard-Soulier syndrome (BSS) is recently reported. We aimed to determine the diagnostic significance of CD9 expression in a cohort of Iranian patients with inherited platelet glycoprotein defects. Twelve BSS, 21 Glanzmann thrombasthenia and 16 healthy controls were included in the present study. Flowcytometric diagnosis of BSS and Glanzmann thrombasthenia was made by analysis of CD41/61 and CD42a/42b CD markers. Moreover, phycoerythrin-labelled anti CD9 was examined in patients and healthy controls. The mean fluorescence intensity (MFI) of CD9 among the three groups was compared using suitable statistical methods and a P value of less than 0.05 considered statistically significant. Mean MFI of CD9 was 990.0 in BSS patients versus 421.2 and 317.3 in individuals with Glanzmann thrombasthenia and healthy controls, respectively (P < 0.05). Between the two-group comparison of means by the Mann-Whitney test revealed a P value of less than 0.001 for BSS group versus GT (2.4-fold) and BSS versus healthy controls (2.9-fold). CD9 molecule also expressed differently in patients with Glanzmann thrombasthenia in comparison with healthy controls (P < 0.001), although with a less magnitude (1.3-fold). According to our findings, CD9 is a potential biomarker for laboratory diagnosis of inherited glycoprotein defects, especially to elucidate the ambiguous results in BSS cases.


Assuntos
Síndrome de Bernard-Soulier , Transtornos Plaquetários , Trombastenia , Síndrome de Bernard-Soulier/diagnóstico , Biomarcadores/metabolismo , Plaquetas/metabolismo , Humanos , Irã (Geográfico) , Complexo Glicoproteico GPIb-IX de Plaquetas/metabolismo , Tetraspanina 29/metabolismo , Trombastenia/diagnóstico
14.
BMC Pulm Med ; 21(1): 338, 2021 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-34715834

RESUMO

Severe coronavirus disease 2019 (COVID-19) accompanies hypercytokinemia, similar to secondary hemophagocytic lymphohistiocytosis (sHLH). We aimed to find if HScore could predict disease severity in COVID-19. HScore was calculated in hospitalized children and adult patients with a proven diagnosis of COVID-19. The need for intensive care unit (ICU), hospital length of stay (LOS), and in-hospital mortality were recorded. The median HScore was 43.0 (IQR 0.0-63.0), which was higher in those who needed ICU care (59.7, 95% CI 46.4-72.7) compared to those admitted to non-ICU medical wards (38.8, 95% CI 32.2-45.4; P = 0.003). It was also significantly higher in patients who died of COVID-19 (105.1, 95% CI 53.7-156.5) than individuals who survived (41.5, 95% CI 35.8-47.1; P = 0.005). Multivariable logistic regression analysis revealed that higher HScore was associated with a higher risk of ICU admission (adjusted OR = 4.93, 95% CI 1.5-16.17, P = 0.008). The risk of death increased by 20% for every ten units increase in HScore (adjusted OR 1.02, 95% CI 1.00-1.04, P = 0.009). Time to discharge was statistically longer in high HScore levels than low levels (HR = 0.41, 95% CI 0.24-0.69). HScore is much lower in patients with severe COVID-19 than sHLH. Higher HScore is associated with more ICU admission, more extended hospitalization, and a higher mortality rate. A modified HScore with a new cut-off seems more practical in predicting disease severity in patients with severe COVID-19.


Assuntos
COVID-19/diagnóstico , Índice de Gravidade de Doença , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/mortalidade , COVID-19/patologia , COVID-19/terapia , Teste para COVID-19 , Criança , Pré-Escolar , Cuidados Críticos/estatística & dados numéricos , Síndrome da Liberação de Citocina/diagnóstico , Síndrome da Liberação de Citocina/virologia , Feminino , Mortalidade Hospitalar , Hospitalização , Humanos , Lactente , Irã (Geográfico)/epidemiologia , Tempo de Internação/estatística & dados numéricos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Análise de Sobrevida , Adulto Jovem
15.
Glob Pediatr Health ; 8: 2333794X211042238, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34471652

RESUMO

Malignant bone tumors (MBT) account for 3% to 5% of cancers in children younger than 15 years. We aimed to report the outcome of children with MBT in 10 years in Southern Iran. During the study period, 100 patients (57 Osteosarcoma, 43 Ewing sarcoma) with an M/F ratio of 1.56 and a median age of 13.8 years (3.8-17.9) were diagnosed. Metastasis occurred in 27% of patients, mostly in the first 3 months of diagnosis. The mean survival time of MBT altogether was 94.1 months (95% CI: 86.5-101.7). The 5-year overall survival and event-free survivals were 85.2% (95% CI: 74%-91.8%) and 69.2% (95% CI: 56%-79%), respectively. Metastasis was the only independent risk factor of death in our study cohort (Hazard ratio 36.7, 95% CI: 4.8-282.6, P = .001) MBT in children mostly occur in adolescent boys. About one-third of them become metastatic, which is significantly associated with poor outcomes.

16.
BMC Infect Dis ; 21(1): 732, 2021 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-34340686

RESUMO

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation. We aimed to describe the clinical and laboratory findings of HLH patients secondary to Visceral leishmaniasis (VL) and their treatment outcome during a 4-year follow-up period compared to primary HLH. METHOD: Forty children with primary HLH confirmed by genetic study and 20 children with HLH secondary to VL confirmed by a blood or bone marrow polymerase chain reaction from 2014 to 2018 in Shiraz, Fars province, Southern Iran, were enrolled. RESULTS: The median age at diagnosis was 11.5 months (range 1-170), and 56.7% were male. Fever and splenomegaly were the most frequent clinical presentations. 93.3% of the subjects had an HScore > 169, which had a good correlation with HLH-2004 criteria (r = 0.371, P = 0.004). Patients with primary HLH experienced more thrombocytopenia (P = 0.012) and higher alanine transaminase (P = 0.016), while patients with VL-associated HLH had higher ferritin (P = 0.034) and erythrocyte sedimentation rate (P = 0.011). Central nervous system (CNS) involvement occurred in 38.3% of patients. The mortality rate was higher in patients with CNS disease (61% vs. 35%, P = 0.051). The 3-yr overall survival rate was 35.9%. (24% in primary HLH and 100% in VL-associated HLH, P < 0.001). In Cox regression analysis, platelet count < 100,000/ µ l (hazard ratio 4.472, 95% confidence interval 1.324-15.107, P = 0.016) correlated with increased mortality in patients with primary HLH. CONCLUSION: VL is a potential source of secondary HLH in regions with high endemicity. Treatment of the underlying disease in VL-associated HLH is sufficient in most cases, with no need to start etoposide-based chemotherapy.


Assuntos
Leishmaniose Visceral/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/parasitologia , Adolescente , Alanina Transaminase/sangue , Sedimentação Sanguínea , Doenças do Sistema Nervoso Central/complicações , Criança , Pré-Escolar , Feminino , Ferritinas/sangue , Febre , Seguimentos , Humanos , Lactente , Recém-Nascido , Irã (Geográfico) , Linfo-Histiocitose Hemofagocítica/mortalidade , Linfo-Histiocitose Hemofagocítica/terapia , Masculino , Reação em Cadeia da Polimerase , Esplenomegalia/diagnóstico , Taxa de Sobrevida , Trombocitopenia/complicações , Resultado do Tratamento
17.
Thromb J ; 19(1): 45, 2021 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-34176487

RESUMO

BACKGROUND: Statins are a potential treatment for venous thromboembolism (VTE) prophylaxis complementary to conventional anticoagulants without associated bleeding complications. This study aimed to compare pro-thrombotic activities of different classes of lipid-lowering drugs in an active comparator design and determine whether there is a relation between statin versus fibrate/niacin use and pro-coagulant factor outcomes. METHODS: This is a cross-sectional analysis of participants from the Netherlands Epidemiology of Obesity study using any class of lipid-lowering drugs, including any types of statins, niacin, and fibrates. We performed linear regression analyses to determine fibrinogen, factor (F) VIII, FIX, and FXI activity in statins versus fibrate/niacin users and adjusted for age, sex, tobacco smoking, body mass index (BMI), hypertension, diabetes, and prevalent cardiovascular disease. RESULTS: Among 1043 participants, the mean age was 58.4 ± 5.2 years, 61% were men, and the mean BMI was 31.3 ± 4.5 kg/m2. Clinical characteristics were balanced between statin and fibrate/niacin users. Statin users had lower mean FXI (18.3 IU/dL, 95% confidence interval (CI) 9.4 to 27.3) levels compared to fibrate/niacin users. The level of FVIII (15.8 IU/dL, 95% CI - 0.003 to 31.6), and FIX (11.3 IU/dL, 95% CI - 0.4 to 23.2) were lower in statin users than fibrate/niacin users with marginal statistical significance. CONCLUSION: Current statin use was associated with lower plasma levels of FXI than fibrate/niacin use. The effects on coagulation factors may, in part, explain the benefit of statin therapy rendered in primary and secondary prevention of VTE.

18.
Iran J Nurs Midwifery Res ; 26(1): 18-24, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33954094

RESUMO

BACKGROUND: Social stigma is the most common and challenging burden of care on the family of people with Borderline Personality Disorder (BPD) In Iran, despite the cultural and social influences, this issue has been less studied. Therefore, present study was conducted to determine the lived experiences of caregivers of patients with BPD of social stigma. MATERIALS AND METHODS: This qualitative study was performed at Ibn Sina hospital in Mashhad, Iran from 2017 to 2019. Participants were selected by purposive and snowball sampling method. Data were collected through semi-structured interviews. Data saturation was achieved after 16 interviews. Finally, the data were analyzed by the method proposed by Diekelmann (1989). RESULTS: In data analysis, one main theme and two sub-themes emerged. The main themes include Black shadow. Two sub-themes consisted of society dagger and secrecy. The sub-theme of society dagger included the two common meanings (inner turmoil in response to the stigma of others and weakening of family status among relatives and acquaintances). The sub-theme of secrecy comprised of the three common meanings (concealment of disease, hide hospitalization, and seclusion). CONCLUSIONS: An understanding of the experience of family stigma can lead to the development of supportive strategies to manage this problem among caregivers of patients with BPD. Nurses can support caregivers by offering them opportunities to discuss how stigma is disrupting their caregiving roles. They can also support the caregivers in negotiating the experienced social and emotional distress and when necessary, refer them to the other members of healthcare teams.

19.
Asian Pac J Cancer Prev ; 22(S1): 43-47, 2021 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-33576211

RESUMO

OBJECTIVE: Although combined chemotherapy regimen leads to 80% remission in children with acute lymphocytic leukemia (ALL), malnutrition and altered serum trace elements as a consequence of chemotherapy agents, have become the new issue to deal with. With the aim to evaluate each trace element in childhood ALL, we investiguâtes six main trace elements before and after induction chemotherapy while considering age, gender and chemotherapy protocol as confounding factors. METHODS: Thirty-six newly diagnosed ALL children were recruited, and trace elements were assessed by atomic absorption spectrometry technique. Trace elements (Zinc, Copper, Manganese, Magnesium, Chromium and Iron) decreased significantly after induction chemotherapy. RESULTS: Considering the confounding factors, mean difference of elements decreased significantly, except for Chromium. Its mean difference was only significant in children younger than 10 and those who had received standard risk chemotherapy. CONCLUSION: In conclusion, all the studied trace elements decreased significantly after induction chemotherapy session in ALL children. This highlights the importance of complementary and supplementary management. A larger cohort study with longer follow up is warranted to elucidate the long-term effect of chemotherapy on these trace elements on the general health status, quality of life or risk of relapse in ALL children.


Assuntos
Antineoplásicos/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Oligoelementos/análise , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Prognóstico , Oligoelementos/metabolismo
20.
Spat Spatiotemporal Epidemiol ; 36: 100389, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33509421

RESUMO

BACKGROUND: Using maps and spatial analysis are technologies to evaluate the magnitude and spatial distribution of disease in epidemiology investigations. We aimed to conduct a Bayesian spatial analysis on epidemiologic data of transfusion-dependent ß-thalassemia (TDT) patients. METHODS: In this cross-sectional study, data of all TDT patients diagnosed during 1955-2018 in all counties of Fars Province were obtained from data registry of the Organization of Special Diseases of Shiraz University of Medical Sciences in Shiraz, Fars Province, Iran. Besag, York, and Mollie's (BYM) model was used for mapping. RESULTS: The estimated relative risk ranged from 0.23 to 1.66 for TDT patients. The highest and lowest relative risks of TDT were observed in Larestan located in Southern and Abadeh in Northern Fars Province respectively. CONCLUSIONS: Determining the accurate geographical distribution of a chronic disease such as ß-thalassemia can be an essential prerequisite in allocation of regional health system resources.


Assuntos
Talassemia beta , Teorema de Bayes , Estudos Transversais , Humanos , Incidência , Irã (Geográfico)/epidemiologia , Talassemia beta/epidemiologia , Talassemia beta/terapia
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