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BACKGROUND AND OBJECTIVE: Inhere we evaluated the diagnostic utility of Doppler sonography (DS) of the anterior cerebral artery (ACA), middle cerebral artery (MCA) and the basilar arteries (BA) based on resistive index (RI) for the diagnosis of asphyxia. METHODS: In this multi-centered cross-sectional study, neonates with clinical diagnosis of asphyxia, were considered for study. During the first 24 h, neonates underwent DS. MRI was done for each neonate during the first month, after discharge or during hospital admission, after obtaining clinical stability. Staging based on DS was compared with staging based on MRI. RESULTS: Overall, 34 patients entered the study. DS of the ACA, MCA, BA all had significant correlation with MRI findings (regarding severity of asphyxia) (r > 0.8 and p < 0.001). In the receiver-operating-characteristic analysis, ideal cut-off point for diagnoses of asphyxia based on ACA and BA was RI ≤ 0.62 [area under the curve (AUC) = 0.957 and 95% CI: 0.819-0.997; sensitivity = 95.65; specificity = 100; positive predictive value (PPV) = 100; negative predictive value (NPV) = 90.9 and negative likelihood ratio (NLR) = 0.043]. Regarding MCA, similarly, a RI ≤ 0.62 was ideal for differentiating between normal and asphyxiated neonates (AUC = 0.990 and 95% CI: 0.873-1; sensitivity = 91.30; specificity = 100; PPV = 91.2; NPV = 100 and NLR = 0.087). CONCLUSION: For evaluating neonates clinically suspected of asphyxia, especially in centers with limited facilities such as MRI, DS can be used as a first line diagnostic modality and RI of ≤ 0.62 is an appropriate cut-off for the diagnosis of perinatal asphyxia.
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Asfixia Neonatal , Hipóxia-Isquemia Encefálica , Asfixia , Asfixia Neonatal/diagnóstico por imagem , Estudos Transversais , Humanos , Recém-Nascido , Artéria Cerebral MédiaRESUMO
PURPOSE: Although mammography is a gold standard for breast cancer screening, the number of cancers that cannot be detected with mammography is substantial, especially in dense-breast (DB) women. Breast sonography can be a useful and powerful screening tool in these cases. The aim of this study is to assess the application of whole-breast sonography in the evaluation of breast lesions in women with DB tissue and estimate its accuracy in comparison with mammography. MATERIAL AND METHODS: A total of 207 asymptomatic DB women participated in this study. The breast tissue density was assessed using ACR BI-RADS. Patients underwent high-resolution ultrasonography of the breast in addition to physical examination and mammography. Different risk factors were also assessed. RESULTS: 152 of 207 (73.4%) cases who had mammography performed had DB, and 55 (26.6%) cases had very dense breasts (very DBs). None of the cases had a positive history of malignancy, while 19% of them had a positive history of breast cancer in first- or second-degree relatives. CONCLUSIONS: All findings were higher in cases with DB compared to very DBs except for fibroadenoma, which was detected more in cases with very DBs. Our study showed that the prevalence of different breast lesions had a significant relationship with the density of the breast. In our study, 48.3% of the cases were diagnosed with a lesion in their sonography result, although 81.0% of them were benign lesions, and the other 19.0% needed follow-up or biopsy evaluation. A substantial number of mammographically occult breast lesions, either benign or malignant, could be detected by ultrasound in DB tissue.
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Silent brain lesions might be associated with overt cerebrovascular accident over time in beta thalassemia major (BTM) and intermediate (BTI). Aspirin may be protective in these patients. We evaluated brain magnetic resonance imaging (MRI) in thalassemia patients to see whether aspirin is protective or not. A historical cohort study was conducted on 35 thalassemia patients, 22 BTI, and 13 BTM patients at Shiraz Hematology Research Center in 2018. Median age of the patients was 32 years and ranged from 8 to 42 years. Twenty-four patients (68.6%) were females. Overall frequency of white matter lesions (WMLs) in the first MRI was 10 patients (28.6%). After 3 years, 3 patients developed new lesions and the frequency of WMLs was 13 patients (37.1%) in the second MRI. Moreover, in 3 patients, number of WMLs increased. Patients with new lesions or more lesions compared to the baseline were significantly older than the other group (median age 36.5 years vs. 31 years, P = 0.046). Regarding aspirin consumption, only 1 patient (16.7%) of patients with new lesions was using aspirin compared to 10 (34.5%) of the other group (P = 0.640). The high-risk patients with thrombocytosis, splenectomy, severe iron overload, and older age (> 30 years) should be under close follow-up and evaluated on a regular periodic basis as well as brain MRI at least once every 3 years. Aspirin could be protective against new or progressive brain lesions so that low-dose aspirin is recommended in high-risk thalassemia patients.
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Aspirina/administração & dosagem , Imageamento por Ressonância Magnética , Acidente Vascular Cerebral , Talassemia beta , Adolescente , Adulto , Criança , Feminino , Seguimentos , Humanos , Masculino , Fatores de Risco , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/tratamento farmacológico , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Talassemia beta/complicações , Talassemia beta/diagnóstico por imagem , Talassemia beta/tratamento farmacológico , Talassemia beta/epidemiologiaRESUMO
PURPOSE: The aim of this study was to assess the incidence and risk factors of intraventricular hemorrhage (IVH) as well as the role of cord blood erythropoietin (EPO) level in predicting the possibility of IVH in premature neonates. MATERIALS AND METHODS: This prospective study included 140 preterm neonates born at hospitals affiliated to Shiraz University of Medical Sciences from May 2014 to April 2015. Complete blood count and cord blood EPO level was measured after birth. Brain ultrasonography was performed at 3 and 7-10 days after birth in these newborns. RESULTS: Brain ultrasonography showed IVH in 8.57% (12/140) until the third day and 20% (28/140) at 7-10 days of life in premature neonates. Early gestational age, low birth weight, low Apgar score, and failure to give prenatal steroid were significant risk factors for developing IVH. The mean level of cord blood EPO was 20.95 ± 21.09 mIU/mL in premature newborns without IVH and 15.82 ± 17.11 mIU/mL with IVH. There was no correlation between the cord blood EPO and IVH in premature newborns. CONCLUSION: Antenatal steroids therapy should be encouraged among women at risk of premature delivery. Our results showed that the cord blood EPO was not correlated with IVH in preterm neonates and further research is required to assess this relationship.
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Biomarcadores/sangue , Hemorragia Cerebral Intraventricular/sangue , Eritropoetina/sangue , Sangue Fetal/química , Doenças do Prematuro/sangue , Hemorragia Cerebral Intraventricular/etiologia , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro/sangue , Doenças do Prematuro/etiologia , Masculino , Estudos ProspectivosRESUMO
The aim of this study is to determine the frequency of silent cerebral ischemia (SCI) in a group of patients with ß-thalassemia major and correlate them with demographic parameters, blood and spleen status, volume and frequency of transfusions. In this cross-sectional study, 40 ß-thalassemic patients over 10 years old who had no neurologic deficit were studied. Brain MRI was performed to detect SCI. Silent cerebral ischemia was classified according to number and size. Silent cerebral ischemia was found in 15 patients (37.5 %). Mean number of SCI was 6.73 ± 10.33 (1-40), and mean size of the brain lesions was 3.07 ± 2.81 mm (1-11 mm). The patients with SCI were significantly older (31.1 ± 6.5 vs. 25 ± 6.8 years, P = 0.009), and most of them were splenectomized (80% vs. 36 %, P = 0.01). Interestingly, 10 out of 15 patients with SCI had platelet count less than 500,000/mm(3). Eight of these patients (80 %) were splenectomized. Other variables had no statistically significant association with the presence of SCI. Older age and splenectomized multitransfused ß-thalassemic patients even with normal platelet count have a higher incidence of SCI. The effect of splenectomy is more significant in statistical analysis. In splenectomized patients with a high platelet count and even with normal platelet count, aspirin therapy is indicated. Based on the results, it seems that regular blood transfusions are not going to have a significant effect on the number and size of SCI.
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Transfusão de Sangue/tendências , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/epidemiologia , Talassemia beta/diagnóstico , Talassemia beta/epidemiologia , Adolescente , Adulto , Isquemia Encefálica/terapia , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Talassemia beta/terapiaRESUMO
PURPOSE: In this study, we aimed to assess the new trends in characteristics, molecular subtypes, and imaging findings of breast cancer in very young women. METHODS: We retrospectively reviewed the database of a primary breast cancer referral center in southern Iran in 342 cases of 30-year-old or younger women from 2001 to 2020. Pathologic data, including nuclear subtype and grade, tumor stage, presence of in situ cancer, imaging data including lesion type in mammogram and ultrasound, and treatment data were recorded. Descriptive statistics were applied. Differences between categorical values between groups were compared using Pearson's Chi-square test. RESULTS: The mean age was 27.89 years. The tumor type was invasive ductal carcinoma in 82 % of cases. Fourteen patients (4.4 %) had only in situ cancer, and 170 patients had in situ components (49.7 %). Molecular subtypes were available in 278 patients, including 117 (42.1 %) Luminal A, 64 (23.0 %) Luminal B, 58 (20.9 %) triple negative, and 39 (14 %) HER2 Enriched. In those with mammograms available, 63 (30.1 %) had no findings, 53 (25.3 %) had mass, 27 (12.9 %) had asymmetry, whether focal or global, 21 (10 %) had microcalcifications solely, and 45 (21.5 %) had more than one finding. Microcalcifications were significantly more common in Luminal cancers than HER2 and triple-negative cancers (p = 0.041). CONCLUSION: Our study shows the most common subtype to be Luminal A cancer, with 74 % of the tumors being larger than 2 cm at the time of diagnosis. Irregular masses with non-circumscribed margins were the most common imaging findings.
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Neoplasias da Mama , Mamografia , Ultrassonografia Mamária , Humanos , Feminino , Estudos Retrospectivos , Adulto , Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/patologia , Mamografia/métodos , Ultrassonografia Mamária/métodos , Irã (Geográfico)/epidemiologia , Adulto Jovem , Mama/diagnóstico por imagem , Mama/patologia , Estadiamento de NeoplasiasRESUMO
We aimed to determine the frequency of asymptomatic brain lesions in a group of patients with ß-thalassemia intermedia (ß-TI) and to evaluate correlation of asymptomatic brain lesions with splenectomy, thrombocytosis, blood transfusions, and clinical parameters. Ninety five neurologically intact patients with ß-TI were randomly enrolled in this cross-sectional study. Diffusion-weighted imaging brain MRI was performed in every patient to detect cerebral white matter lesions (WML). We found an overall frequency of 15 (15.8 %) for WMLs, 14 (23.7 %) in splenectomized, and 1 (2.8 %) in nonsplenectomized patients. The presence of WML was significantly associated with splenectomy (P = 0.008) and thrombocytosis (P = 0.009). However, after adjustment for splenectomy, thrombocytosis was not significantly associated with the presence of WML (P > 0.05). The number of patients with regular blood transfusions and normal MRI was not significantly higher compared to those with abnormal findings (52.5 % vs. 26.7 %; P = 0.092). In untransfused patients, hydroxyurea (HU) administration was associated with a lower incidence of WML (P < 0.001). Although in univariate analysis either splenectomy or thrombocytosis showed significant correlation with the presence of single or multiple WMLs, thrombocytosis by itself did not significantly contribute in developing asymptomatic brain lesions. The lack of significant correlation between lesions and regular blood transfusions could be related to the treatment with HU in untransfused patients, which increased fetal hemoglobin levels and improved the morphology and the pathological indices of the red blood cells. Larger prospective studies are suggested for the accurate evaluation of the correlation of these factors with developing asymptomatic brain lesions.
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Encefalopatias/etiologia , Cérebro/patologia , Talassemia beta/fisiopatologia , Adolescente , Adulto , Transfusão de Sangue , Encefalopatias/epidemiologia , Criança , Estudos de Coortes , Terapia Combinada , Estudos Transversais , Imagem de Difusão por Ressonância Magnética , Feminino , Fármacos Hematológicos/uso terapêutico , Humanos , Hidroxiureia/uso terapêutico , Incidência , Irã (Geográfico)/epidemiologia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Esplenectomia/efeitos adversos , Trombocitose/etiologia , Adulto Jovem , Talassemia beta/tratamento farmacológico , Talassemia beta/patologia , Talassemia beta/terapiaRESUMO
Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of the study: Our aim was to investigate the efficacy and side effects of the Sinopharm vaccine in patients with hemoglobinopathies in Iran and the frequency of breakthrough infection after a full course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 ß-thalassemia major, 118 ß-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of the China Sinopharm vaccine and received the second dose of the vaccine 28 days apar. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzyme-linked immunosorbent assay (Pishtazteb ELISA commercial kit), including a surrogate virus neutralization test (sVNT), for detection of SARS-CoV-2 immunoglobulins (IgA, IgM, IgG), total neutralizing antibody (NAb). Results: The mean age of patients was 35.0 ± 8.5 (from 18 to 70) years, and 55.6% were positive for the antibody. Overall, 48.2% of the studied population had at least one side effect after vaccination. The most frequent side effects were fever and chills, dizziness, and body pain. A total of 90 (20.7%) vaccinated patients developed breakthrough infections after two doses of Sinopharm vaccination. Disease severity was recorded, and it was classified as mild in 77.8%, moderate in 13.6%, and severe in 7.4% of patients. One 28-year-old woman with ß-thalassemia major died eight days after diagnosing a breakthrough SARS-CoV-2 infection. Conclusion: No safety concerns were identified in patients who received two doses of the Sinopharm vaccine. Its efficacy was not optimal due to the lack of effect on new variations of the virus. However, our data show that it seems to be protective against the severity of COVID-19 infection in patients with hemoglobinopathies. The frequency of breakthrough infections after two doses of Sinopharm vaccination supports the evolving dynamic of SARS-CoV-2 variants requiring special challenge since such infection may represent a risk for vulnerable patients.
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Isquemia Encefálica/diagnóstico por imagem , Voluntários Saudáveis , Imageamento por Ressonância Magnética/métodos , Talassemia beta/complicações , Adolescente , Adulto , Transfusão de Sangue , Isquemia Encefálica/complicações , Infarto Cerebral/complicações , Infarto Cerebral/diagnóstico por imagem , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Talassemia beta/terapiaRESUMO
BACKGROUND: Acarbose and repaglinide are two safe and effective antidiabetic agents that are especially in wide use in Asian and Middle Eastern countries. These two prandial agents share some outstanding qualities that their newer counterparts do not. While globally available in generic versions, both are oral and cheap. There is a paucity of data regarding their comparative efficacy. Herein, a head-to-head comparison of the efficacy of the two in treatment of postprandial hyperglycemia of newly-diagnosed type 2 diabetes was investigated. MATERIALS AND METHODS: One hundred and sixty-four newly-diagnosed type 2 diabetes patients with fasting plasma glucose levels of <7.2 mmol/L (130 mg/dL) but 2-hour postprandial glucose (2hPPG) levels of >10 mmol/L (180 mg/dL) were consecutively alternated between acarbose- and repaglinide-treatment for 6 months. RESULTS: Per protocol analysis, 67% of acarbose-treated patients versus 85% of repaglinide-treated patients achieved 2hPPG levels of <10 mmol/L (180 mg/dL) (Pâ¯=â¯0.05). Treatment adherence rates were 52.4% and 72%, respectively (P < 0.02). Thirteen of the repaglinide-treated and 2 of acarbose-treated patients reported at least one episode of hypoglycemia (P < 0.03). Fasting plasma glucose, 2hPPG, glycated hemoglobin and basal insulin requirement decreased more significantly with repaglinde than acarbose (P, <0.05, <0.04, <0.04 and <0.03, respectively). Weight increased with repaglinide and decreased with acarbose (Pâ¯=â¯0.03). There were no significant changes in LDL levels with either treatment (Pâ¯=â¯0.58), but triglycerides decreased more significantly with acarbose treatment (Pâ¯=â¯0.03) CONCLUSIONS: Significantly higher rates of treatment-adherence and at-target glycemic levels were seen with repaglinide treatment. Weight decreased with acarbose and increased with repaglinide treatment. Hypoglycemic episodes were much less frequent with acarbose treatment.
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Acarbose/farmacologia , Carbamatos/farmacologia , Diabetes Mellitus Tipo 2/tratamento farmacológico , Hiperglicemia/metabolismo , Hipoglicemiantes/farmacologia , Piperidinas/farmacologia , Adulto , Idoso , Glicemia/análise , Feminino , Humanos , Irã (Geográfico) , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: Compared with newer prandial anti-diabetes agents, repaglinide and acarbose are unique in being globally available in generic versions, being oral, and being the cheapest of all. The aim of this study was to compare their efficacy when used alone or in combination. METHODS: In a randomized, double-blind, prospective study, 358 recently diagnosed type 2 diabetes (T2D) patients, who on a combined therapy with metformin and insulin glargine had a fasting plasma glucose (FGP) of <7.2 mmol/L but a 2-h postprandial plasma glucose (2hPPG) >10 mmol/L, were assigned to three groups of additional treatment with either repaglinide, acarbose, or repaglinide-plus-acarbose for 4 months. RESULTS: With intention-to-treat analysis, 63% of repaglinide group, 45.4 percent of acarbose group, and 75.7% of repaglinide-plus-acarbose group reached the primary endpoint of 2hPPG < 10 mmol/L while maintaining FPG < 7.2 mmol/L. Treatment adherence rate was 75.6% with repaglinide, 61.4% with acarbose, and 81.3% with repaglinide-plus-acarbose (p = 0.001). Among the groups, weight was significantly lower in acarbose group (p < 0.05). Twenty-one percent of repaglinide patients, 4.9% of acarbose subjects, and 10.3% of repaglinide-plus-acarbose cases reported at least one episode of hypoglycemia (p < 0.005). HbA1C and basal insulin requirement were significantly lower in repaglinide group (p = 0.004, p = 0.0002). Triglycerides were lowest in acarbose group (p = 0.005). CONCLUSIONS: Both acarbose and repaglinide were vastly effective in lowering postprandial hyperglycemia of recently diagnosed T2D. When combined, they were even more efficacious and the disease had a better outcome. Compared with newer peers, these two are particularly useful where and when cost consideration in diabetes treatment is a prime concern.
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Acarbose , Diabetes Mellitus Tipo 2 , Acarbose/uso terapêutico , Glicemia , Carbamatos , Diabetes Mellitus Tipo 2/tratamento farmacológico , Hemoglobinas Glicadas , Humanos , Hipoglicemiantes/uso terapêutico , Insulina , Piperidinas , Estudos ProspectivosRESUMO
There are several slightly invasive methods to detect colorectal carcinoma (CRC) including colonoscopy and sigmoidoscopy; but there is no noninvasive, accurate screening test. It is recommended to initiate screening at the age of 50 for non-familial CRC. Laboratory tests are routinely suggested if internal observation and imaging are recommended for further evaluation. Spectroscopic-based imaging, such as magnetic resonance spectroscopy (MRS) is an interesting and promising tool with the potential to be an alternative to some minimally invasive procedures, such as biopsy. Accordingly, MRS might be a suitable substitution for invasive methods, such as colonoscopy. This article aimed to review the studies that have evaluated the MRS technique as a screening tool in CRC.
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Neoplasias Colorretais/diagnóstico por imagem , Espectroscopia de Ressonância Magnética/métodos , Colo/diagnóstico por imagem , Humanos , Reto/diagnóstico por imagemAssuntos
Doença de Graves/complicações , Neoplasias Induzidas por Radiação/complicações , Neoplasias da Glândula Tireoide/complicações , Adolescente , Biópsia por Agulha Fina , Carcinoma , Carcinoma Papilar , Feminino , Doença de Graves/radioterapia , Humanos , Radioisótopos do Iodo/uso terapêutico , Neoplasias Induzidas por Radiação/patologia , Câncer Papilífero da Tireoide , Neoplasias da Glândula Tireoide/patologiaRESUMO
BACKGROUND: Breast pain is one of the most common complaints of women attending a gynecology clinic and general practitioners. This pain can cause anxiety and, consequently, reduce the woman's quality of life. The objective of this study was to assess the severity of pain and anxiety in patients with breast pain and normal examination, before and after breast sonography. MATERIALS AND METHODS: Among the patients with breast pain and normal examination who came for breast sonography in three centers in Shiraz during 2011-2012, 51 patients agreed to cooperate in this project. These patients filled out the questionnaire for evaluation of pain and anxiety before and after breast sonography. RESULTS: Sonography findings indicated that 88% of patients have normal sonography without any finding. The average amounts of pain severity before and after sonography were 3.3 and 2.4, respectively (p < 0.005). The average amounts of anxiety severity in patients before and after sonography were 51.9 and 37.9, respectively (p < 0.005). CONCLUSION: This study shows the severity of pain and anxiety after ultrasonography decreased significantly. It can be concluded that ultrasound findings, which reassure the patients that they do not have specific pathology, can reduce the pain and anxiety by its impact on psychological status of the patient.
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Increased demand for interventional radiology techniques has interventionists performing a large number of these procedures. Measurements and calculations have shown that the radiation doses received by these specialists can exceed the threshold of radiation-induced deterministic effects unless radiation protection procedures and devices are used. Proper usage of radiation protection devices can protect them from radiation-induced effects, even with a high workload. Occupational radiation protection entails proper training of interventionists to increase their awareness about available appropriate protection tools and equipment, and devices that can be used to minimize exposure, such as needle holders, tubing extensions, and injectors. This study introduces a device that can be used to fix the catheter to prevent the physician from holding the catheter by hand. The authors, also, discuss the importance of radiation protection training along with the training on new medical equipment, which can be applied to reduce the radiation dose.
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Catéteres , Exposição Ocupacional/prevenção & controle , Exposição à Radiação/prevenção & controle , Proteção Radiológica/instrumentação , Radiografia Intervencionista/instrumentação , Fita Cirúrgica , Desenho de Equipamento , Análise de Falha de Equipamento , Exposição Ocupacional/análise , Exposição à Radiação/análiseRESUMO
OBJECTIVE: To compare endotracheal tube tip-to-carina distance obtained by ultrasonography vs. that obtained by chest X-ray in neonates. METHODS: After endotracheal intubation of 40 neonates, chest X-ray and, within one hour, ultrasonography was obtained for each patient for measurement of endotracheal tube tip-to-carina distance. RESULTS: Means of endotracheal tube tip-to-carina distances were not significantly different by both modalities (mean difference 0.157 cm, P= 0.06). In addition, an intraclass correlation was observed between them (r2= 0.61, 95% CI= 0.26, 0.79). CONCLUSION: Ultrasonography and chest X-ray are equally accurate for determination of endotracheal tube tip-to-carina in infants. As ultrasonography is more easily available and is safer than X-ray, it may be a better modality for confirming proper placement of endotracheal tube in neonates.
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Intubação Intratraqueal , Traqueia/diagnóstico por imagem , Ultrassonografia , Estudos Transversais , Feminino , Humanos , Lactente , Recém-Nascido , Irã (Geográfico) , Masculino , Radiografia TorácicaRESUMO
Evaluation of joints in children with haemophilia is important in detecting abnormalities, staging their severity and following the effects of treatment. The aim of this study is to evaluate the correlation of FISH score (Functional Independence Score in Haemophilia) with the scores obtained by MRI and sonography for the diagnosis, evaluation and classification of arthropathy in severe haemophilia. In this cross-sectional study on 25 severe haemophilia patients, FISH, MRI and sonography procedures were performed in the elbow or knee joint. All patients' information, including age, type of haemophilia, affected joint, scores of MRI, sonography and FISH, dose of factor consumed, weight and prophylaxis protocol were collected and analysed. Among the 25 patients (age range of 11-70 years), 22 patients were haemophilia A and three patients were haemophilia B. Affected joints were right knee in 12 patients, left knee in nine and right elbow in four. There was only a statistically significant negative correlation between FISH and MRI Additive (A) scale (rsâ=â-0.537, Pâ=â0.006). Considering cartilage loss domain (related MRI A scale: 13-20), 20 patients (80%) were classified in this group with FISH scores ranged from 17 to 22. On the basis of our results, FISH scores in severe haemophilia patients were negatively correlated with MRI A scale. Also, it seems that a FISH score less than 22 could be considered as loss of cartilage; however, due to the small number of our patients, it needs further assessment in different populations.
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Articulação do Cotovelo/patologia , Hemartrose/diagnóstico , Hemofilia A/diagnóstico , Hemofilia B/diagnóstico , Articulação do Joelho/patologia , Adolescente , Adulto , Idoso , Cartilagem/diagnóstico por imagem , Cartilagem/patologia , Criança , Estudos Transversais , Articulação do Cotovelo/diagnóstico por imagem , Feminino , Hemartrose/classificação , Hemartrose/diagnóstico por imagem , Hemartrose/etiologia , Hemofilia A/classificação , Hemofilia A/complicações , Hemofilia A/diagnóstico por imagem , Hemofilia B/classificação , Hemofilia B/complicações , Hemofilia B/diagnóstico por imagem , Humanos , Articulação do Joelho/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Projetos de Pesquisa , Índice de Gravidade de Doença , Ultrassonografia de IntervençãoRESUMO
OBJECTIVE AND IMPORTANCE: Extramedullary hematopoiesis (EMH) is evidenced by erythropoietic masses, which occurs as a compensatory mechanism to overcome hypoxia during chronic anemia. EMH masses in spinal cord could lead to cord compression and neurological symptoms. Besides transfusion, radiotherapy, and surgery, hydroxyurea (HU) is also a treatment strategy in EMH. CLINICAL PRESENTATION: We described four cases of beta thalassemia with EMH who were treated with HU as a monotherapy. INTERVENTION (AND TECHNIQUE): HU therapy was done in all patients without any transfusion during therapy. CONCLUSION: HU is a good treatment option for patients with EMH and it could be a substitute for radiotherapy and invasive surgery or regular blood transfusion.