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1.
BMC Pediatr ; 24(1): 229, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38561716

RESUMEN

BACKGROUND: Cardiovascular autonomic neuropathy (CAN) is a serious complication of diabetes, impacting the autonomic nerves that regulate the heart and blood vessels. Timely recognition and treatment of CAN are crucial in averting the onset of cardiovascular complications. Both clinically apparent autonomic neuropathy and subclinical autonomic neuropathy, particularly CAN pose a significant risk of morbidity and mortality in children with type 1 diabetes mellitus (T1DM). Notably, CAN can progress silently before manifesting clinically. In our study, we assessed patients with poor metabolic control, without symptoms, following the ISPAD 2022 guideline. The objective is is to determine which parameters we can use to diagnose CAN in the subclinical period. METHODS: Our study is a cross-sectional case-control study that includes 30 children diagnosed with T1DM exhibiting poor metabolic control (average HbA1c > 8.5% for at least 1 year) according to the ISPAD 2022 Consensus Guide. These patients, who are under the care of the pediatric diabetes clinic, underwent evaluation through four noninvasive autonomic tests: echocardiography, 24-h Holter ECG for heart rate variability (HRV), cardiopulmonary exercise test, and tilt table test. RESULTS: The average age of the patients was 13.73 ± 1.96 years, the average diabetes duration was 8 ± 3.66 years, and the 1-year average HbA1c value was 11.34 ± 21%. In our asymptomatic and poorly metabolically controlled patient group, we found a decrease in HRV values, the presence of postural hypotension with the tilt table test, and a decrease in ventricular diastolic functions that are consistent with the presence of CAN. Despite CAN, the systolic functions of the ventricles were preserved, and the dimensions of the cardiac chambers and cardiopulmonary exercise test were normal. CONCLUSIONS: CAN is a common complication of T1DM, often associated with the patient's age and poor glycemic control. HRV, active orthostatic tests, and the evaluation of diastolic dysfunctions play significant roles in the comprehensive assessment of CAN. These diagnostic measures are valuable tools in identifying autonomic dysfunction at an early stage, allowing for timely intervention and management to mitigate the impact of cardiovascular complications associated with T1DM.


Asunto(s)
Enfermedades del Sistema Nervioso Autónomo , Diabetes Mellitus Tipo 1 , Neuropatías Diabéticas , Humanos , Niño , Adolescente , Diabetes Mellitus Tipo 1/complicaciones , Estudios Transversales , Estudios de Casos y Controles , Hemoglobina Glucada , Neuropatías Diabéticas/diagnóstico , Neuropatías Diabéticas/etiología , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Enfermedades del Sistema Nervioso Autónomo/etiología , Frecuencia Cardíaca/fisiología
2.
BMC Pediatr ; 23(1): 92, 2023 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-36859277

RESUMEN

BACKGROUND: During Coronavirus disease of 2019 (COVID-19) pandemic, the WHO reported a noticeable increase in Kawasaki disease prevalence in countries where Kawasaki disease is rare. This newly seen disease, unlike typical Kawasaki disease, tends to appear at a later age, has prominent gastrointestinal findings, higher rates of myocarditis and coronary artery involvement and a greater need for admission to the intensive care unit (ICU). Induration of the Bacillus Calmette-Guerin (BCG) scar is a rare finding seen in multisystem inflammatory syndrome (MIS-C). This is the second reported case of erythema and induration of the BCG scar in a 1-year-old boy with MIS-C. CASE PRESENTATION: The Arabic boy presented with high resistant fever, nausea/vomiting, diarrhea, erythematous lips, and conjunctivitis. He later developed induration of his BCG scar, diffuse rash and desquamation on fingers and toes. He had a history of COVID-19 exposure as his IgG antibodies against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were positive. Based on his clinical findings and repeated lab results, he was diagnosed with MIS-C with Kawasaki features and treated with intravenous immune globulin (IVIG) followed by methylprenisolone and aspirin. CONCLUSIONS: Reaction at the BCG inoculation site is not a diagnostic criteria for Kawasaki, but it is seen clinically in 30-50% of the patients. We report the case of a 1-year-old boy diagnosed with MIS-C presenting with erythema and induration of BCG scar. Further studies are needed to explore this clinical presentation, especially in the countries that have BCG vaccination programs, and to determine the mechanisms of MIS-C.


Asunto(s)
COVID-19 , Síndrome Mucocutáneo Linfonodular , Mycobacterium bovis , Masculino , Niño , Humanos , Lactante , Cicatriz , Vacuna BCG , SARS-CoV-2
3.
BMC Pediatr ; 23(1): 360, 2023 07 13.
Artículo en Inglés | MEDLINE | ID: mdl-37442972

RESUMEN

BACKGROUND: Pulmonary complications, such as airway leak syndrome, are common in preterm neonates; however, bronchial rupture is a rarely seen phenomenon. CASE PRESENTATION: In this case, we present a preterm newborn who developed pneumomediastinum and pneumothorax. The pneumothorax persisted, despite placement of a thorax tube, requiring a thoracotomy to detect and treat the bronchial rupture. CONCLUSION: Physicians should have a high suspicion of bronchial rupture in patients with persistent air leak syndrome, even after thorax tube placement and continuous negative pressure implementation.


Asunto(s)
Neumotórax , Recién Nacido , Humanos , Neumotórax/diagnóstico por imagen , Neumotórax/etiología , Neumotórax/terapia , Bronquios , Síndrome , Tórax , Toracotomía/efectos adversos
4.
Echocardiography ; 40(11): 1292-1299, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37805960

RESUMEN

Maternal hyperoxygenation (MH) has been studied as a diagnostic tool to evaluate pulmonary vasculature and as a treatment option to improve the growth of fetal left heart in fetuses with left-sided cardiac defects. Chronic maternal hyperoxygenation (CMH) therapy leads to an improvement in fetal pulmonary blood flow resulting in an enhanced venous return to the left heart with increased gestational age. With this manipulation it is anticipated to augment blood flow directed remodeling of the left heart structures and to improve left heart growth spanning from the mitral valve to the aortic isthmus. However, there are concerns about CMH therapy with regard to fetal complications with growth restriction and fetal brain development. Now, with two successful cases we try to discuss this fetal treatment option and related concerns.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico , Embarazo , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Corazón Fetal/diagnóstico por imagen , Edad Gestacional , Hemodinámica , Terapia por Inhalación de Oxígeno/métodos , Ultrasonografía Prenatal/métodos
5.
Cardiol Young ; 33(5): 810-812, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-36217801

RESUMEN

Left ventricular non-compaction is a very rare, still unclassified congenital cardiomyopathy. Nine distinct subtypes of functional and anatomical left ventricular non-compaction have been identified. Studies on the prognosis and mortality of subtypes are ongoing. Our study presented the first restrictive subtype left ventricular non-compaction case with family history and MYH7 gene mutation.


Asunto(s)
Fibroelastosis Endocárdica , Cardiopatías , Humanos , Mutación , Ventrículos Cardíacos , Cadenas Pesadas de Miosina/genética , Miosinas Cardíacas/genética
6.
Mycoses ; 58(10): 578-81, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26214750

RESUMEN

With advances in medical sciences, an increase in survival rates of low birth weight; increased incidence in use of catheter and antibiotics, and total parenteral nutrition are reported, therefore, the rate of fungal infections in late and very late onset neonatal sepsis have increased. Although fungal endocarditis rarely occur in newborns, it has a high morbidity and mortality. Antifungal therapy is often insufficient in cases who develop fungal endocarditis and surgical treatment is not preferred due to its difficulty and high mortality. Herein, fungal endocarditis in a preterm newborn treated with single-dose recombinant tissue plasminogen activator in addition to antifungal therapy is presented and relevant literature has been reviewed. The vegetation completely disappeared following treatment and no complication was observed.


Asunto(s)
Antifúngicos/uso terapéutico , Candida albicans/aislamiento & purificación , Candidiasis Invasiva/tratamiento farmacológico , Endocarditis/tratamiento farmacológico , Enfermedades del Prematuro/tratamiento farmacológico , Enfermedades del Prematuro/microbiología , Activador de Tejido Plasminógeno/uso terapéutico , Encéfalo/microbiología , Candida albicans/crecimiento & desarrollo , Candidiasis Invasiva/microbiología , Ecocardiografía , Endocarditis/microbiología , Escherichia coli/aislamiento & purificación , Femenino , Fibrinolíticos/uso terapéutico , Corazón/microbiología , Humanos , Recién Nacido , Recien Nacido Prematuro , Recién Nacido de muy Bajo Peso , Sepsis/tratamiento farmacológico , Sepsis/microbiología
7.
Pediatr Hematol Oncol ; 31(7): 597-606, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24684386

RESUMEN

OBJECTIVES: Cardiology follow up is important in thalassemia major patients. The object of this study is to define parameters which can be used in the early detection of cardiac impairment. MATERIAL AND METHODS: Forty seven beta thalassemia major patients (mean age 16.3 ± 4.47 years; 22 boys, 25 girls) whose left ventricular systolic functions were normal and a healthy control group of fifty age and gender matched children were included in the study. M-mode echocardiographic measurements, systolic and diastolic functions with PW and tissue Doppler and heart rate variabilities (HRVs) were compared between the two groups. The patients were also grouped according to MRT2*, ferritin and left ventricular diastolic diameters (LVDds) to compare the echocardiographic and Holter parameters among them. RESULTS: None of the children in the study group had symptomatic congestive heart failure. PW Doppler late diastolic forward flow in pulmonary artery was higher in the thalassemia group when compared with the control group (P = 0.01) indicating decreased compliance of the right ventricle. While the systolic and diastolic functions were normal, all the HRV parameters in the thalassemia group were significantly lower than the control group (P = 0.005). CONCLUSIONS: Significant decrease in HRV and increase in PW late diastolic forward flow in pulmonary artery in the absence of systolic or diastolic dysfunction, points out that these parameters can be useful in detection of early cardiac impairment.


Asunto(s)
Ecocardiografía Doppler , Cardiopatías/diagnóstico , Frecuencia Cardíaca/fisiología , Imagen por Resonancia Magnética , Talasemia beta/complicaciones , Adolescente , Adulto , Niño , Diástole , Femenino , Ferritinas/sangre , Humanos , Masculino , Talasemia beta/fisiopatología
8.
J Cardiothorac Surg ; 18(1): 38, 2023 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-36653817

RESUMEN

BACKGROUND: The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. METHODS: 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. RESULTS: Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months-27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. CONCLUSION: Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Niño , Humanos , Centros de Atención Terciaria , Resultado del Tratamiento , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Morbilidad , Estudios Retrospectivos
9.
Diagnostics (Basel) ; 13(14)2023 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-37510124

RESUMEN

The most common cause of death in patients with Duchenne muscular dystrophy (DMD) is cardiomyopathy. Our aim was to investigate the relationship between the Tpeak-Tend (Tp-e) interval and the premature ventricular contraction (PVC) burden and therefore early arrhythmic risk and cardiac involvement in DMD patients. Twenty-five patients with DMD followed by pediatric cardiology were included in the study. Those with a frequency of <1% PVC in the 24 h Holter were assigned to Group 1 (n = 15), and those with >1% were assigned to Group 2 (n = 10). Comparisons were made with healthy controls (n = 27). Left ventricular ejection fraction (LVEF) was lowest in Group 2 and highest in the control group (p < 0.001). LV end-diastolic diameter was greater in Group 2 than in Group 1 and the control group (p = 0.005). Pro-BNP and troponin levels were higher in Group 1 and Group 2 than in the control group (p = 0.001 and p < 0.001, respectively). Tp-e interval was longer in Group 2 compared to Group 1 and the control group (p < 0.001). The LVEF (OR 0.879, 95% CI 0.812-0.953; p = 0.002) and Tp-e interval (OR 1.181, 95% CI 1.047-1.332; p = 0.007) were independent predictors of PVC/24 h frequency of >1%. A Tp-e interval > 71.65 ms predicts PVC > 1%, with a sensitivity of 80% and a specificity of 90% (AUC = 0.842, 95% CI (0.663-1.000), p = 0.001). Determination of Tp-e prolongation from ECG data may help in the determination of cardiac involvement and early diagnosis of arrhythmic risk in DMD.

10.
Pediatr Hematol Oncol ; 29(4): 386-8, 2012 May.
Artículo en Inglés | MEDLINE | ID: mdl-22568804

RESUMEN

Approximately 10% of neonates with Down syndrome may develop a form of megakaryoblastic leukemia that usually disappear spontaneously during the first months of the life. Although it seems to have a benign course, it may also be lethal and severe in some cases, especially in the form of hydrops and/or cardiopulmonary failure. Herein, we report a male infant with Down syndrome who was admitted with respiratory distress due to severe pericardial effusion leading to pericardial tamponade in the first 2 weeks of life. Pericardiosentesis and pericardial tube replacement in combination with steroid therapy was performed. He responded well to these therapies and his leukemia resolved on the fourth month of life. This case suggests that severe pericardial effusion and pericardial tamponade may be life-threatening complications of transient leukemia of Down syndrome and also it may be managed successfully with appropriate treatments.


Asunto(s)
Taponamiento Cardíaco/terapia , Síndrome de Down/terapia , Leucemia Megacarioblástica Aguda/terapia , Taponamiento Cardíaco/complicaciones , Síndrome de Down/complicaciones , Humanos , Recién Nacido , Leucemia Megacarioblástica Aguda/complicaciones , Masculino , Derrame Pericárdico/complicaciones , Derrame Pericárdico/terapia , Inducción de Remisión , Síndrome de Dificultad Respiratoria del Recién Nacido/complicaciones , Síndrome de Dificultad Respiratoria del Recién Nacido/terapia
11.
Pediatr Hematol Oncol ; 29(3): 220-34, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22475298

RESUMEN

The aim of the study was to evaluate cardiac function and early cardiac dysfunction of patients followed as thalassemia major. In this study, the authors compared 100 patients, diagnosed as thalassemia major with mean age 11.84 ± 4.35, with 60 healthy control subjects at the same age between 2008 and 2011. Early diagnosis of iron overload that may occur after repeated transfusions is important in this patient group. To detect early iron accumulation, the authors compared ferritin with the echo findings, the 24-hour Holter, and cardiac magnetic resonance imaging (MRI) T2* values in the patients of same age and sex, treated with chelators, without heart failure, nonsplenectomized, and do not differ in the presence of hepatitis C. Ferritin levels, left ventricular systolic functions (ejection fraction [EF], shortening fraction [SF]), left ventricular measurements, left ventricular diastolic functions, T2* image on cardiac magnetic resonance, heart rate variables in 24 hours, and Holter rhythm were evaluated to show the early failure of cardiac functions. In this study the authors confirmed that iron-related cardiac toxicity damages electrical activity earlier than myocardial contractility. Left ventricular diastolic diameter (LVDd), left ventricular mass (LVM), and LV systolic diameter (LVDs) levels were significantly higher in the patient group with ectopia. Patients with ectopia are the ones in whom LVM and LVDd are increased. In thalassemia major patients with ectopia, LF/HF ratio was markedly increased, QTc dispersion was clearly found higher in patients with ectopia rather than nonectopic patients. The standard deviation all normal RR interval series (SDNN) was found clearly lower in thalassemia major group with ectopia than control group because it is assumed that increase in cardiac sympathetic neuronal activity is related to exposure to chronic diastolic and systolic failure.


Asunto(s)
Insuficiencia Cardíaca/etiología , Talasemia beta/fisiopatología , Adolescente , Estudios de Casos y Controles , Niño , Insuficiencia Cardíaca/diagnóstico , Pruebas de Función Cardíaca , Humanos , Sobrecarga de Hierro , Imagen por Resonancia Magnética , Turquía/epidemiología , Talasemia beta/complicaciones
12.
Pediatr Hematol Oncol ; 28(5): 380-94, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21699467

RESUMEN

The present study was designed to evaluate the significance of echocardiography versus cardiac troponin I levels in early detection of anthracycline dependent cardiotoxicity in acute lymphoblastic leukemia (ALL) patients. A total of 276 pediatric ALL patients were included in the study prospectively along 3 phases of data collection lasted from 2002 to 2009; including phase I (March 2002 to February 2003; n = 25; 53.3% females), phase II (September 2003 to April 2004; n = 35; 57.1% females), and phase III (January 2005 to June 2009; n = 216; 52.7% females) with respect to cumulative anthracycline doses applied. Anthracycline was administered in accordance with berlin-Franfurt-Munich (BFM)-2000 protocol in doses of 30 to 350 mg/m(2) (in the first phase) and 30 to 240 mg/m(2) (in the following phases). Evaluation of cardiotoxicity was performed via echocardiography and measurement of cardiac troponin I levels. Patients in each phase were homogenous in terms of gender and age. Diastolic dysfunction determined via reduction E/A ratio below the cutoff value was demonstrated to deteriorate earlier than systolic functions and alteration in cardiac enzymes. Being similar between dose groups, cTnI levels were shown to rise in the presence of congestive heart failure. In conclusion, anthracycline cardiotoxicity appears to be detected in an earlier stage by using diastolic parameters compared to systolic parameters and cardiac enzymes.


Asunto(s)
Antraciclinas/efectos adversos , Antibióticos Antineoplásicos/efectos adversos , Ecocardiografía/métodos , Corazón/efectos de los fármacos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Troponina I/sangre , Niño , Preescolar , Diástole/efectos de los fármacos , Diagnóstico Precoz , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/inducido químicamente , Humanos , Lactante , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatología , Pronóstico , Estudios Prospectivos , Sístole/efectos de los fármacos
13.
J Clin Ultrasound ; 39(7): 427-30, 2011 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-21688274

RESUMEN

We report the case of a male fetus with tuberous sclerosis complex (TSC), in whom multiple cardiac rhabdomyomas and renal angiomyolipomas were detected at 33 weeks by ultrasound with additional brain lesions detected on MRI, all confirmed after birth. DNA analysis of the TSC2 gene detected a de novo mutation in the TSC2 gene. Postnatal follow-up and neurological examination were normal, as were the results of Holter monitoring.


Asunto(s)
Resultado del Embarazo , Esclerosis Tuberosa/diagnóstico por imagen , Ultrasonografía Prenatal/métodos , Angiolipoma/diagnóstico , Angiolipoma/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Edad Gestacional , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/diagnóstico por imagen , Humanos , Recién Nacido , Neoplasias Renales/diagnóstico , Neoplasias Renales/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Masculino , Monitoreo Fisiológico/métodos , Embarazo , Diagnóstico Prenatal/métodos , Rabdomioma/diagnóstico , Rabdomioma/diagnóstico por imagen , Esclerosis Tuberosa/diagnóstico , Esclerosis Tuberosa/genética
14.
Fetal Pediatr Pathol ; 29(5): 330-7, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-20704479

RESUMEN

Turner syndrome is associated with a higher frequency of heart defects detected prenatally when compared to postnatal reports. The most common heart defects detected prenatally are hypoplastic left heart syndrome and coarctation of the aorta. We report a case involving a fetus at 16 gestational weeks with a septated cystic hygroma located on the neck and head, an interventricular septal mass, a hypoplastic left ventricle due to aortic stenosis, mitral stenosis, and a hypoplastic aortic arch with a karyotype of mos 45, X, [47 cells]/47, XXX [3 cells]. The autopsy findings confirmed our prenatal diagnosis with a final diagnosis of Turner syndrome and congenital cardiac vascular malformation.


Asunto(s)
Hamartoma/diagnóstico , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Diagnóstico Prenatal , Síndrome de Turner/diagnóstico , Aborto Inducido , Autopsia , Femenino , Feto/anomalías , Edad Gestacional , Hamartoma/patología , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/patología , Cariotipificación , Embarazo , Síndrome de Turner/patología
15.
Anatol J Cardiol ; 24(2): 97-106, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32749248

RESUMEN

OBJECTIVE: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. The available risk scores to predict intravenous immunoglobulin (IVIG) resistance and CAA were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. This study aimed to evaluate the ability of 5 risk scoring systems to predict IVIG resistance and CAA in Turkey patients with KD. METHODS: Patients with KD were retrospectively evaluated with clinical, laboratory, and echocardiographic findings. Data analyses were performed in 5 scoring systems (Harada, Kobayashi, Egami, Formosa, and Sano). RESULTS: A total of 259 patients (Male: Female, 1.7) were treated for KD in our hospital. The mean age of diagnosis in patients with KD, CAA, and IVIG resistance were 3.31, 2.19, and 2.06, respectively. CAA development and IVIG resistance were seen in 11.6% and 12.3% of cases, respectively. IVIG resistance was detected in 35.6% of patients with CAA. In our study, 5 risk scoring systems were applied to our patients. ROC analysis results were found highest in Kobayashi scoring system for IVIG resistance (AUC, 0.864) and in Harada scoring system for CAA development (AUC, 0.727). CONCLUSION: Harada score was significant in predicting CAA risk, and Kobayashi score was significant in predicting the risk of developing IVIG resistance. It is necessary to determine more specific and sensitive risk scores that increase the risk of IVIG resistance and the development of CAA in Turkey.


Asunto(s)
Aneurisma Coronario/diagnóstico , Síndrome Mucocutáneo Linfonodular/diagnóstico , Índice de Severidad de la Enfermedad , Pueblo Asiatico , Niño , Preescolar , Aneurisma Coronario/etnología , Resistencia a Medicamentos , Femenino , Humanos , Inmunoglobulinas Intravenosas , Lactante , Masculino , Síndrome Mucocutáneo Linfonodular/etnología , Valor Predictivo de las Pruebas , Curva ROC , Estudios Retrospectivos , Medición de Riesgo , Turquía
16.
Pediatr Int ; 51(5): 670-7, 2009 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-19419502

RESUMEN

BACKGROUND: The aim of the present study was to determine prenatal follow up and clinical outcome in fetuses born with cystic hygroma. METHODS: A series of 64 cystic hygroma patients, who were diagnosed in the first and the second trimester of pregnancy, was enrolled. Associated structural abnormalities, karyotype analysis and pregnancy outcome were studied. Survivors were followed for their fetal outcome and prognosis. RESULTS: There were 64 new cases of cystic hygroma in 8524 subjects screened (0.75%). Thirty-nine (60.9%) were of non-septated and 25 (39.1%) were of septated cystic hygroma. Chromosomal abnormalities were present in 25 (39.1%). The most common abnormality in non-septated cystic hygroma was trisomy 21 (10, 27.8%), and that in septated cystic hygroma was Turner syndrome (5, 23.8%). Associated structural malformations are common in cystic hygroma and overall survival was poor. Nine of the present infants were live-born and were subsequently followed up. Two had cardiac pathology and died after cardiac operation, two others were diagnosed with axillary cystic hygroma, had an excellent prognosis and responded well to treatment, and another two had cranial findings with mild neurological sequel. Only three cases had, at birth and in the follow-up period, no complications. CONCLUSION: Cystic hygroma is highly correlated with adverse perinatal outcome. Prenatal diagnosis and invasive procedures are vital for counselling with close follow-up after delivery for appropriate medical support. A multidisciplinary approach is strictly recommended in live-born children.


Asunto(s)
Neoplasias de Cabeza y Cuello/diagnóstico , Neoplasias de Cabeza y Cuello/terapia , Comunicación Interdisciplinaria , Linfangioma Quístico/diagnóstico , Linfangioma Quístico/terapia , Manejo de Atención al Paciente/métodos , Femenino , Enfermedades Fetales/diagnóstico , Enfermedades Fetales/terapia , Estudios de Seguimiento , Humanos , Recién Nacido , Enfermedades del Recién Nacido/diagnóstico , Enfermedades del Recién Nacido/terapia , Masculino , Embarazo , Diagnóstico Prenatal , Pronóstico , Derivación y Consulta , Resultado del Tratamiento
17.
Am J Perinatol ; 25(7): 427-34, 2008 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-18720325

RESUMEN

The aim of this study was to examine prenatal diagnosis of double-outlet right ventricle (DORV)-associated anomalies and prognosis of each case. Medical records were reviewed of fetuses with DORV who had fetal echocardiography at our institution from 2002 to 2006. Pre- and postnatal diagnosis and outcome were compared and evaluated. Twenty-one fetuses were diagnosed with DORV. The pregnancy was terminated in seven cases. Three cases had chromosomal abnormalities; three cases, hypoplastic left ventricle; and one case, encephalocele. Accurate prenatal diagnosis of the ventricular septal defect, outflow obstruction, and great artery relationship was achieved in 14 of 16 cases (87.5%). Only 2 of 13 live-born cases survived beyond 6 months. The overall prognosis for fetuses with DORV is poor. DORV is found in fetuses with a huge spectrum of associated cardiac and extracardiac anomalies. Careful assessment by fetal echocardiography can determine important anatomic details with adequate correctness for precise counseling.


Asunto(s)
Ventrículo Derecho con Doble Salida/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/diagnóstico , Corazón Fetal/anomalías , Corazón Fetal/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/mortalidad , Ecocardiografía , Femenino , Enfermedades Fetales/diagnóstico por imagen , Enfermedades Fetales/mortalidad , Edad Gestacional , Humanos , Mortalidad Infantil , Recién Nacido , Embarazo , Resultado del Embarazo , Pronóstico , Estudios Retrospectivos , Ultrasonografía Prenatal
18.
Arch Argent Pediatr ; 116(1): e102-e105, 2018 Feb 01.
Artículo en Inglés, Español | MEDLINE | ID: mdl-29333832

RESUMEN

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.


El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.


Asunto(s)
Anomalías Múltiples/diagnóstico por imagen , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Arteria Pulmonar/anomalías , Arteria Pulmonar/diagnóstico por imagen , Ecocardiografía , Humanos , Hallazgos Incidentales , Lactante , Masculino
19.
Turk J Pediatr ; 60(4): 407-414, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30859765

RESUMEN

Aghdasi-Bornaun H, Kutluk G, Keskindemirci G, Öztarhan K, Dedeoglu R, Yilmaz N, Tosun Ö. Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission. Turk J Pediatr 2018; 60: 407-414. Heart Rate Variability (HRV) is one of the reliable and noninvasive parameters to evaluate autonomic control of the cardiovascular system in patients. The aim of our study was to assess autonomic function in pediatric patients with inflammatory bowel disease (IBD) in remission using Power Spectral Analysis of HRV. Autonomic cardiovascular function was evaluated by time and frequency-domain indexes of spontaneous heart rate in 36 IBD children patients in remission phase and 36 sex and age matched healthy controls. Twenty children with Ulcerative Colitis (UC) and 16 patients with Crohn`s disease (CD) were diagnosed according to their history, physical and laboratory examination, endoscopic, histopathological and radiological findings of upper and lower gastrointestinal system. Significant decrease was observed at HRV parameters in IBD patients when compared with control group. These differences was found in some of time domain parameters (NNmean, SDNNtotal, SDNNday) and frequency domain parameters (TP, LF,VLF) (p < 0.05). Also, there was a significant higher minimum heart rate ratio (p < 0.04) in patients in comparison to the control group. In the IBD group, there was a relative tendency for parasympathetic suppression and sympathetic predominance which reflects an autonomic dysfunction. This imbalance has a circadian rhythm and it is more obvious during the day. These observations may suggest a previously unrecognized role of chronic inflammation for autonomic modulation in IBD.


Asunto(s)
Sistema Nervioso Autónomo/fisiopatología , Frecuencia Cardíaca/fisiología , Enfermedades Inflamatorias del Intestino/fisiopatología , Adolescente , Estudios de Casos y Controles , Niño , Electrocardiografía Ambulatoria/métodos , Femenino , Humanos , Masculino
20.
Arch Argent Pediatr ; 115(1): 50-54, 2017 02 01.
Artículo en Inglés, Español | MEDLINE | ID: mdl-28097840

RESUMEN

BACKGROUND/AIM: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). POPULATION AND METHODS: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student t and Mann-Whitney U" tests. RESULTS: The study group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. CONCLUSION: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.


Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC


Asunto(s)
Apolipoproteínas/sangre , Aterosclerosis/sangre , Enfermedad de la Arteria Coronaria , Lipoproteína(a)/sangre , Lipoproteínas LDL/sangre , Padres , Triglicéridos/sangre , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de Riesgo
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