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1.
Cardiol Young ; 34(1): 218-220, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38037818

RESUMEN

Myocarditis is an inflammatory disease of the heart muscle that most commonly occurs after infectious diseases in childhood. The clinical picture of acute myocarditis ranges from asymptomatic infection to fulminant heart failure and sudden death (1). Most of the patients may present with nonspecific symptoms such as respiratory distress, chest pain, nausea, and vomiting (2). While rhythm abnormalities such as ventricular and supraventricular rhythm disorders can be observed in these patients, various degrees of atrioventricular blocks may rarely develop (3). In this article, we aimed to present a patient who developed second-degree, high-grade atrioventricular block after myocarditis and recovered completely after treatment.


Asunto(s)
Bloqueo Atrioventricular , Insuficiencia Cardíaca , Miocarditis , Humanos , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/terapia , Miocarditis/complicaciones , Miocarditis/diagnóstico , Miocardio , Ventrículos Cardíacos
2.
Cardiol Young ; 33(7): 1220-1222, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-36523262

RESUMEN

A 16-month-old girl was referred for tachycardia and upper respiratory tract infection. Echocardiographic examination revealed pericardial effusion, mild mitral regurgitation, and left ventricle systolic dysfunction. Patient was positive for Parainfluenza type 4 virus. Her laboratory tests revealed increased troponin I level. The patient was treated with intravenous immunoglobulin considering acute viral myopericarditis. Two weeks after treatment, midventricular hypertrophy was detected.


Asunto(s)
Neoplasias Cerebelosas , Meduloblastoma , Miocarditis , Pericarditis , Femenino , Humanos , Lactante , Miocarditis/diagnóstico , Ecocardiografía , Pericarditis/diagnóstico
3.
Echocardiography ; 39(6): 851-854, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35610733

RESUMEN

BACKGROUND: Accessory mitral valve tissue (AMVT) is an extremely rare causes left ventricular outflow tract (LVOT) obstruction and is usually incidentally detected in childhood. It is often associated with other cardiac and vascular congenital malformations. CASE PRESENTATION: In this case, we present a 15-year-old girl was diagnosed with AMVT by transesophageal echocardiography, resulting in LVOT obstruction during systole. Interestingly enough, the patient's accessory mitral valve remained undetected for years until he became symptomatic for wide ASD. Successful closure of the ASD with resection of the AMVT was performed with a transaortic approach. The patient was hemodynamically stable postoperatively. There were no abnormalities in the mitral valves and LVOT. CONCLUSION: It was also unusual to see AMVT with ASD instead of other frequently associated other congenital anomalies. Accessory mitral valve should be considered a rare but important cause of left ventricular outflow tract obstruction in childhood.


Asunto(s)
Ecocardiografía Tridimensional , Cardiopatías Congénitas , Defectos del Tabique Interatrial , Disfunción Ventricular Izquierda , Obstrucción del Flujo Ventricular Externo , Adolescente , Ecocardiografía Transesofágica , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/cirugía , Humanos , Masculino , Válvula Mitral/anomalías , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Disfunción Ventricular Izquierda/complicaciones , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/etiología , Obstrucción del Flujo Ventricular Externo/cirugía
4.
Pediatr Cardiol ; 43(2): 413-425, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34564734

RESUMEN

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p < 0.001), E/A ratio was significantly lower, and averaged E/e' ratio was significantly higher (median 1.5 vs. 1.8, p < 0.05; 8.9 vs. 6.3, p < 0.001 respectively) in patients compared to control. Patients were divided into 2 groups according to 3D LV ejection fraction (LVEF) on the echo-1: Group 1; LVEF < 55%, 26 patients, and group 2; LVEF ≥ 55%, 24 patients. E/e' ratio was significantly higher in group 1 than group 2 and control at discharge (median 7.4 vs. 6.9, p = 0.005; 7.4 vs. 6.3, p < 0.001 respectively). Coronary ectasia was detected in 2 patients (z score: 2.53, 2.6 in the right coronary artery), and resolved at discharge. Compared with group 2, group 1 had significantly higher troponin-I (median 658 vs. 65 ng/L; p < 0.001), NT-pro BNP (median 14,233 vs. 1824 ng/L; p = 0.001), procalcitonin (median 10.9 vs. 2.1 µg/L; p = 0.009), ferritin (median 1234 vs. 308 µg/L; p = 0.003). The most common findings were ventricular systolic dysfunction recovering during hospitalization, and persisting LV diastolic dysfunction in the reduced LVEF group at discharge. Coronary artery involvement was rare in the acute phase of the disease. Also, in MIS-C patients, the correlation between LV systolic dysfunction and markers of inflammation and cardiac biomarkers should be considered.


Asunto(s)
COVID-19 , Disfunción Ventricular Izquierda , COVID-19/complicaciones , Niño , Ecocardiografía , Humanos , Laboratorios , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica , Disfunción Ventricular Izquierda/diagnóstico por imagen
5.
Cardiol Young ; : 1-2, 2022 Apr 27.
Artículo en Inglés | MEDLINE | ID: mdl-35475444

RESUMEN

Scimitar syndrome is a congenital anomaly in which some or all of right pulmonary veins drain into inferior caval vein. It is associated with anomalous systemic arteries arising from descending aorta supplying to right lung. Transcatheter embolisation of this artery prevents complications. We present a 2.5-year-old girl in which anomalous artery was embolised using Amplatzer PiccoloTM Occluder, for first time.

6.
Cardiol Young ; : 1-3, 2022 Nov 09.
Artículo en Inglés | MEDLINE | ID: mdl-36348619

RESUMEN

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

7.
Int J Clin Pract ; 75(9): e14471, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34107136

RESUMEN

OBJECTIVE: Although the initial reports of COVID-19 cases in children described that children were largely protected from severe manifestations, clusters of paediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease, toxic shock syndrome and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Our objective was to develop a protocol for the evaluation, treatment and follow-up of patients with MIS-C. METHODS: The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of paediatric critical care, cardiology, rheumatology, surgery, gastroenterology, haematology, immunology, infectious disease and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback. CONCLUSION: Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a paediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients' phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.


Asunto(s)
COVID-19 , Algoritmos , Niño , Humanos , SARS-CoV-2 , Síndrome , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/terapia
8.
Echocardiography ; 38(11): 1941-1947, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34755377

RESUMEN

OBJECTIVE: Arterial stiffness refers to arterial wall rigidity, particularly in central vessels, and is an independent predictor of cardiovascular disease in many chronic diseases. 25-Hydroxy (OH) vitamin D has beneficial effects on blood pressure, vascular endothelial function, and arterial stiffness; most importantly, its deficiency is common worldwide. Therefore, we aimed to elucidate the role of 25-OH vitamin D deficiency in arterial stiffness development and its relationship with arterial stiffness in healthy children. METHODS: This study included 80 patients with low levels of 25-OH vitamin D and 40 healthy control subjects. The study participants were then divided into three groups: group 1 consisted of patients with a deficient 25-OH D level of < 19.9 ng/ml, group 2 with an insufficient 25-OH D level between 20 and 29.9 ng/ml; group 3 were considered control group with a sufficient serum 25-OH vitamin D level of ≥30 ng/ml. Aortic strain, distensibility, stiffness index, and standard left ventricular measurements were calculated using M-mode echocardiographic data. RESULTS: Left ventricular mass index (LVMI) and inter-ventricular septal diastolic thickness (IVST) appeared to increase in group 1 compared to groups 2 and 3. Aortic strain and distensibility were significantly decreased in group 1, whereas aortic stiffness index and elastic modulus were significantly increased. The aortic stiffness index was negatively correlated with serum 25-OH vitamin D levels; however, aortic strain, aortic distensibility, and LVMI were positively correlated. CONCLUSIONS: Our study results revealed a significant relationship between 25-OH vitamin D levels indicative of a deficiency and aortic stiffness. Hence, we suggest that arterial stiffness may also occur in healthy children with a 25-OH vitamin D deficiency. Future in-depth studies are needed to understand the exact mechanisms underlying the aortic stiffness development associated with 25-OH vitamin D deficiency.


Asunto(s)
Rigidez Vascular , Deficiencia de Vitamina D , Niño , Ecocardiografía , Humanos , Turquía , Vitamina D , Deficiencia de Vitamina D/complicaciones
9.
Pediatr Int ; 63(12): 1433-1440, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34157184

RESUMEN

BACKGROUND: Premature ventricular contractions and supraventricular contractions are common rhythm disorders requiring comprehensive investigation in children. The aim of the study was to evaluate the heart rate variability (HRV) in premature ventricular contractions (PVCs) and supraventricular contractions (PSVCs) in children. METHODS: The study compared the characteristics of HRV in 175 children with PVCs and 160 children with PSVCs who underwent 24-h Holter monitoring, with 101 healthy children. RESULTS: Significant differences were found between standard deviation of all normal RR intervals (SDNN), standard deviation of average RR intervals in all 5 min segments of registration (SDANN), root mean square of the successive differences (rMSSD), and the proportion of NN50 divided by total number of NNs (pNN50) values of the patient and control groups. The PVCs group had a significantly lower high frequency (HF) and higher low frequency (LF)/HF ratio and the PSVCs group had a significantly higher LF and higher LF/HF ratio compared to the control group. No significant correlation existed between frequency and the SDNN index, rMSSD, and pNN50 values of the PVCs group. The receiver operating characteristics analysis showed a significantly changed LF/HF ratio when premature contractions exceeded 60 beats per hour in children with PVCs. CONCLUSIONS: This study indicated an increased sympathetic tone and a significantly decreased vagal tone in children with PVCs and PSVCs. HRV can be used increasingly as a non-invasive method in the follow-up of children with premature cardiac contractions.


Asunto(s)
Electrocardiografía Ambulatoria , Corazón , Niño , Frecuencia Cardíaca , Humanos
10.
Pediatr Cardiol ; 42(2): 264-268, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33006644

RESUMEN

Coronavirus disease of 2019 (COVID-19) is a cause of significant morbidity and mortality worldwide. Although COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported. The mechanism of cardiac injury and arrhythmias is unclear. Also, drugs currently used to treat the COVID-19 may prolong the QT interval and may have a proarrhythmic propensity. The study aims to investigate the effects of COVID-19 infection with asymptomatic and mild symptoms on trans-myocardial repolarization parameters in children without treatment. A total of 105 COVID-19 patients were compared with 40 healthy children. The patient and control group data were compared by calculating the QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e, Tp-e dispersion, Tp-e/QT ratio, and Tp-e/QTc ratio on the 12-lead surface electrocardiogram. The mean age was determined as 11.2 ± 0.3 years in the patient group, and 10.8 ± 2.1 years in the control group. In the COVID-19 group, QTd, QTcd, Tp-e, Tp-e dispersion, Tp-e/QT ratio and Tp-e/QTc ratio were statistically higher than the control group. The ventricular repolarization was impaired even in asymptomatic children with COVID-19 infection. These results suggest the need to further assess the long terms risks of prolonged QT dispersion in the setting of COVID-19 infection.


Asunto(s)
Arritmias Cardíacas/etiología , COVID-19/complicaciones , Adolescente , Arritmias Cardíacas/fisiopatología , Niño , Preescolar , Electrocardiografía/métodos , Femenino , Humanos , Lactante , Síndrome de QT Prolongado/complicaciones , Masculino , Factores de Riesgo , SARS-CoV-2
11.
Echocardiography ; 36(9): 1666-1674, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31452268

RESUMEN

OBJECTIVES: The aim was to evaluate the role of tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), to identify myocardial dysfunction, and to evaluate myocardial segmental deformation in acute viral myocarditis. METHODS: Twenty-one patients and twelve healthy children were studied prospectively. The TDI and STE were performed before and after treatment. The myocardial velocities (Sm , Em , and Am ) and time intervals (isovolumic contraction, isovolumic relaxation, and ejection times [ET]) at interventricular septum (IVS), left, and right ventricular basal segments were examined by TDI. The left ventricular global longitudinal strain (LVGLS) and strain rate (LVGLSR), left ventricular global circumferential strain (LVGCS) and strain rate (LVGCSR), and right ventricular global longitudinal strain (RVGLS) and strain rate (RVGLSR) were examined by STE. RESULTS: Sm and Em at IVS and at LV, ET at IVS, ET at RV, ET at LV were significantly lower in patients before treatment than controls. LVGLS, LVGLSR, LVGCS, LVGCSR, RVGLS, RVGLSR were significantly decreased in patients before treatment than controls. There was significant improvement for LVGLS, LVGLSR, LVGCS, LVGCSR, and RVGLS in patients after treatment. Sm , Em , and Am at LV were significantly lower in patients before treatment than in patients after treatment. In spite of improvements, Sm , Em , and ET at IVS, LVGLS, LVGLSR, LVGCS, LVGCSR were significantly lower in patients after treatment than controls. CONCLUSIONS: The TDI and STE were useful methods for detection of early myocardial dysfunction and evaluation of treatment outcomes in acute viral myocarditis.


Asunto(s)
Miocarditis/diagnóstico por imagen , Miocarditis/fisiopatología , Ultrasonografía Doppler/métodos , Enfermedad Aguda , Adolescente , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Biomarcadores/sangre , Niño , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Miocarditis/tratamiento farmacológico , Miocarditis/virología , Estudios Prospectivos , Resultado del Tratamiento
12.
Echocardiography ; 35(3): 380-387, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29239028

RESUMEN

AIM: To investigate myocardial deformation and function during treatment for Kawasaki disease (KD) in children. METHODS: We performed speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI) in 15 children with KD and 15 healthy children during treatment for KD. STE was performed for longitudinal and circumferential strain (S) and strain rate (SR) at the left ventricle (LV) and for longitudinal S and SR at the right ventricle (RV). TDI was performed at the base of interventricular septum (IVS), LV, and RV. RESULTS: Among TDI parameters, Em and ejection time (ET) at IVS, ET at LV and ET at RV obtained obtained before treatment were significantly lower in patients with KD compared to controls. After treatment, in spite of improvements, ET at IVS and ET at RV remained significantly lower in patients with KD compared to controls. Left ventricular global longitudinal and circumferential S and SR values obtained before treatment were significantly lower in patients with KD compared to controls. Left ventricular S and SR values were found to be increased after treatment. However, left ventricular global circumferential S value remained significantly lower in patients with KD compared to controls. There were no significant differences in right ventricular global longitudinal S and SR values between patients and controls before treatment. CONCLUSION: During acute phase, patients with KD have reduced global left ventricular S and SR which may be more sensitive indicators of myocardial inflammation. This study showed gradual improvements in left ventricular myocardial function during treatment for KD.


Asunto(s)
Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/fisiopatología , Preescolar , Ecocardiografía Doppler/métodos , Femenino , Humanos , Masculino , Síndrome Mucocutáneo Linfonodular/terapia , Resultado del Tratamiento
13.
Echocardiography ; 34(3): 383-389, 2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-28139073

RESUMEN

BACKGROUND: The purpose of this study is to determine early myocardial dysfunction in ß-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS: In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition. The myocardial functions were evaluated by conventional echocardiography, TDI and STE. RESULTS: When basal lateral left ventricular and basal septal wall TDI values were compared between the patient group and control group, only isovolumic contraction time values were significantly longer in the patients. The global circumferential strain was significantly lower in the patients. When evaluated as segmental, longitudinal strain values of basal inferoseptum and circumferential strain values of anteroseptum, anterior, and inferolateral segments were significantly lower in the patients. In the patients, global longitudinal and circumferential strains in the group who had pathological T2* values were significantly lower than the group who did not. In addition, circumferential strain values in anteroseptum, anterolateral, inferior, and inferoseptum segments were significantly lower in the patients with T2* values<20 ms than those with T2* values≥20 ms. CONCLUSION: Although T2* MRI is the most sensitive test detecting myocardial iron load, TDI and STE can be used for screening myocardial dysfunction. The abnormal strain values, especially circumferential, may be detected as the first finding of abnormal iron load and related to T2* values.


Asunto(s)
Ecocardiografía/métodos , Sobrecarga de Hierro/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Disfunción Ventricular Izquierda/complicaciones , Disfunción Ventricular Izquierda/diagnóstico por imagen , Talasemia beta/complicaciones , Adolescente , Ecocardiografía Doppler , Femenino , Corazón/diagnóstico por imagen , Corazón/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Humanos , Sobrecarga de Hierro/sangre , Masculino , Reproducibilidad de los Resultados , Talasemia beta/sangre
14.
Pediatr Cardiol ; 37(1): 201-7, 2016 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-26396115

RESUMEN

Echocardiography is the mainstay of screening and disease surveillance in isolated left ventricular non-compaction (iLVNC). The aim of our study is to determine the early regional and global myocardial functional changes and whether the myocardial changes that cannot be detected by conventional echocardiography could be detected by tissue Doppler imaging (TDI) or two-dimensional speckle-tracking echocardiography (STE) in iLVNC cases without symptoms. Longitudinal and circumferential strain (S) and strain rates (SR) as determined by STE in 20 children aged 12.1 ± 3.3 years was compared with those in 20 controls. All children underwent echocardiographic assessment using two-dimensional, tissue Doppler and speckle-tracking echocardiography. iLVNC patients who had normal systolic function by ejection and shortening fractions were included in this study. According to the TDI in all three segments [the non-compacted (NC), neighboring NC (NNC) and compacted (C) segments], isovolumic contraction time, isovolumic relaxation time and myocardial performance index values were significantly higher, while ejection time were significantly lower in the iLVNC group. According to STE in two segments (NC and NNC-segments) longitudinal S and SR values and also circumferential S and SR values were significantly lower in the iLVNC group compared with the control group; whereas, in the global measurements both longitudinal and circumferential S and SR values in all three segments were significantly lower in the iLVNC group compared with the control group. We believe that TDI and STE that evaluates myocardial deformation can be used for the detection of early myocardial dysfunction in the iLVNC patients who are subclinical and whose left ventricular functions were detected as normal by conventional methods with normal ejection and shortening fractions.


Asunto(s)
Ecocardiografía/métodos , Ventrículos Cardíacos/fisiopatología , No Compactación Aislada del Miocardio Ventricular/fisiopatología , Adolescente , Niño , Femenino , Humanos , Masculino , Miocardio , Reproducibilidad de los Resultados , Volumen Sistólico
15.
Pediatr Transplant ; 19(7): E185-8, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26282574

RESUMEN

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non-specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage-IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas/efectos adversos , Hipertensión Pulmonar/etiología , Neuroblastoma/terapia , Preescolar , Humanos , Hipertensión Pulmonar/diagnóstico , Masculino , Estadificación de Neoplasias , Neuroblastoma/patología , Trasplante Autólogo
16.
Pediatr Hematol Oncol ; 31(5): 481-7, 2014 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-24933192

RESUMEN

The medical records of 16 patients diagnosed as intracardiac thrombus were searched. The size, location and outcome of thrombus together with demographic data of patients were assessed. The median age of the patients was 2.2 years. Six patients were newborn and two patients were infant. The median size of thrombus was 9 mm. The localization was right atrium in seven, right ventricle in five, left ventricle in one, pulmonary artery in one, and superior vena cava in two patients. There was prematurity in five, ciyanotic congenital heart disease in one, blood culture positivity in three, malignancy in four, nephrotic syndrome in one, indwelling catheters in 10, and acquired or genetic thrombophilia in six patients as risk factors. In the treatment, the first choice was tissue plasminogen activator in two patients, heparin infusion in one patient and low molecular weight heparin in remaining 12 patients. In nine patients, therapy included parenteral antimicrobials together with anticoagulants. The result was complete resolution in 15 patients and in one patient thrombus was surgically removed. The median time was 16 (2-70) days for 50% resolution and 26 (3-93) days for complete resolution. There was a statistically significant (P = .027 and r = 0.5) correlation between the size and the complete resolution time. There was no anticoagulant therapy related major complication. In patients with intracardiac thrombus, selection of anticoagulant therapy may decrease the risk of complications. Surgery is rarely required and thrombolytics are not usually necessary for resolution of thrombus.


Asunto(s)
Fibrinolíticos/administración & dosificación , Cardiopatías , Heparina de Bajo-Peso-Molecular/administración & dosificación , Trombolisis Mecánica , Trombosis , Adolescente , Niño , Preescolar , Femenino , Cardiopatías/diagnóstico , Cardiopatías/terapia , Humanos , Lactante , Recién Nacido , Masculino , Factores de Riesgo , Trombosis/diagnóstico , Trombosis/terapia
17.
Pediatr Emerg Care ; 30(5): 331-4, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24759488

RESUMEN

Syncope is a transient loss of consciousness as a result of global cerebral hypoperfusion. It is generally benign but may be a sign of pathology. The purpose of this study was to analyze the frequency of syncope due to cardiac, neurocardiogenic, neurologic, situational, psychiatric, and other causes and make a differential diagnosis of syncope types according to detailed medical history and further investigations. We examined prospectively 268 children presented to pediatric polyclinics as well as cardiology and neurology departments (age range, 1-18 years) with a primary complaint of syncope for the study. Cardiac syncope was diagnosed in 12 patients, neurocardiogenic syncope in 232, neurologic syncope in 9, psychiatric syncope in 9, situational in 4, and benign paroxysmal positional vertigo in 2. The neurologic syncope group consists of patients diagnosed with epilepsy after evaluation. Eight patients in the cardiac syncope group were found to have diseases such as long QT syndrome, and the remaining patients had hypertrophic cardiomyopathy, atrioventricular nodal reentry tachycardia, ventricular tachycardia, and a second-degree heart block that can cause sudden death. In conclusion, syncope is a common problem in childhood that requires hospitalization. Because it may be the first finding of an underlying malignant cardiac or neurologic disease, clinicians must be very careful during medical evaluation. An electrocardiogram and a medical history including the details of the event, chronic diseases, and familial diseases are among the most important steps for the right diagnosis and prognosis. Instead of a routine procedure, further diagnostic workup should be directed according to medical history for high yield. Convulsive movements may be defined in all types of syncope related with cerebral hypoxia, and this may lead to a misdiagnosis of seizure by the clinician.


Asunto(s)
Síncope/diagnóstico , Síncope/etiología , Adolescente , Niño , Preescolar , Diagnóstico Diferencial , Ecocardiografía , Electrocardiografía , Electroencefalografía , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Factores de Riesgo
18.
Mol Syndromol ; 15(2): 156-160, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38585546

RESUMEN

Introduction: Primary carnitine deficiency (PCD) is a rare autosomal recessive disorder caused by loss of function mutations in the solute carrier family 22 member 5 (SLC22A5) gene that encodes a high-affinity sodium-ion-dependent organic cation transporter protein (OCTN2). Carnitine deficiency can result in acute metabolic decompensation or, in a more insidious presentation, cardiomyopathy. Cardiomyopathy associated with PCD often presents with life-threatening heart failure. This presentation also usually includes skeletal muscle myopathy. Early recognition of this disorder and treatment with carnitine can avoid life-threatening complications related to cardiomyopathy. Case Presentation: Herein, we present a 10-month-old male patient with PCD, which was diagnosed while investigating the etiology of dilated cardiomyopathy and confirmed by molecular genetic analysis. Conclusion: Homozygous c.254_265 insGGCTCGCCACC (p.I89Gfs) pathogenic variant of the SLC22A5 gene was detected. With oral L-carnitine supplementation, the free carnitine level increased up to 14 µmol/L and the symptoms disappeared. LVEF increased by 45-70%. We would like to emphasize that this problem is a combination of the metabolic decompensation and the cardiac phenotypes, which are usually separated to either phenotype.

19.
Pediatr Hematol Oncol ; 30(3): 239-45, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23387876

RESUMEN

The aim was to assess the myocardial functions in infants with iron deficiency anemia (IDA). The findings of 22 infants with IDA and 16 healthy infants were compared. Complete blood count in all infants, serum iron level, total iron binding capacity (TIBC), and serum ferritin level in patients were evaluated. Echocardiography with tissue Doppler imaging (TDI) was performed in all infants. The mean hemoglobin was 8.5 versus 11 g/dL, hematocrit was 26.7 versus 33.1% and the mean corpuscular volume (MCV) was 59.8 versus 72.2 fL in patients and controls, respectively. The median serum iron level was 14.5 µg/dL, TIBC was 444 µg/dL, and serum ferritin level was 4.9 ng/mL in patients. There were significant differences between patients and controls for the myocardial performance indexes of the left ventricle (LV) (0.57 vs. 0.45, P < .05), interventricular septum (IVS) (0.56 vs. 0.48, P < .05), and the right ventricle (RV) (0.56 vs. 0.41, P < .05). The similar significances were also present for the ejection times (ET) of the LV (173.1 vs. 212.1 ms, P < .05), IVS (173.7 vs. 195.6 ms, P < .05), and RV (169.9 vs. 213.3 ms, P < .05). In spite of negatively significant correlations between hemoglobin, hematocrit, MCV and MPI, the correlations between these parameters and ET were positively significant. However, there were no correlations between serum iron level, TIBC, serum ferritin level, and the TDI parameters of patients. According to these findings, we could say that, the IDA affects the myocardial functions in a negative manner.


Asunto(s)
Anemia Ferropénica/metabolismo , Ferritinas/metabolismo , Hemoglobinas/metabolismo , Hierro/metabolismo , Miocardio/metabolismo , Anemia Ferropénica/patología , Estudios de Casos y Controles , Ecocardiografía Doppler en Color , Índices de Eritrocitos , Estudios de Seguimiento , Hematócrito , Humanos , Lactante , Miocardio/patología , Pronóstico , Estudios Prospectivos
20.
Pediatr Hematol Oncol ; 30(2): 71-9, 2013 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-23151153

RESUMEN

Cardiac rhabdomyomas (CRs) are the most common heart tumors in children and closely associated with tuberous sclerosis complex (TSC). This study was performed to assess the presentation type, clinical course, treatment modalities, and outcome of the patients with rhabdomyoma, associated with TSC. We reviewed our patients with cardiac rhabdomyomas (CRs), who had received a diagnosis of TSC previously or during the follow-up period between June 1996 and January 2012, retrospectively. Thirty-two patients with TSC were evaluated and among them 11 patients (34%) were associated with CRs. Five patients (45%) had multiple tumors and consequently a total of 29 CRs were analyzed in our study. The median follow-up period was 2 years (range: 1 week-15 years). Clinical presentation was cardiac murmur in three patients, cyanosis in two patients and arrhythmia in one patient. Five patients were asymptomatic at the diagnosis and CRs were detected during routine cardiac evaluation for TSC. Cardiac tumors were diagnosed prenatally in two patients. Spontaneous regression rate was 31% and we experienced a complete regression of a tumor with an echogenic bordered tissue defect and septal thinning in a patient. Three patients had hemodynamically significant tumor obstruction; two of them underwent surgery. The other patient, who had multiple CRs, was treated medically with everolimus because of high-risk potential of surgery. Although surgical resection is the preferred treatment in most of the patients with hemodynamic instability, we need novel alternative medical therapies in some critically ill patients who cannot be operated due to various reasons.


Asunto(s)
Neoplasias Cardíacas , Rabdomioma , Esclerosis Tuberosa , Antineoplásicos/administración & dosificación , Niño , Preescolar , Everolimus , Femenino , Estudios de Seguimiento , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/mortalidad , Neoplasias Cardíacas/fisiopatología , Neoplasias Cardíacas/terapia , Humanos , Lactante , Recién Nacido , Masculino , Diagnóstico Prenatal , Estudios Retrospectivos , Rabdomioma/diagnóstico , Rabdomioma/mortalidad , Rabdomioma/fisiopatología , Rabdomioma/terapia , Sirolimus/administración & dosificación , Sirolimus/análogos & derivados , Tasa de Supervivencia , Esclerosis Tuberosa/diagnóstico , Esclerosis Tuberosa/mortalidad , Esclerosis Tuberosa/fisiopatología , Esclerosis Tuberosa/terapia
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