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1.
Turk Arch Pediatr ; 58(5): 473-479, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37534463

RESUMO

OBJECTIVE: Congenital heart defects occur in approximately 50% of children with Down syndrome and they contribute considerably to morbidity and mortality. The aim of this study is to investigate the prevalence, classification, and survival of congenital heart defects in Down syndrome. MATERIALS AND METHODS: About 1731 Down syndrome patients who underwent echocardiography between 1986 and 2022 were evaluated. The median follow-up duration was 8.7 years (range 1-35.8 years). Congenital heart defect was grouped as cyanotic and acyanotic. RESULTS: Among the 1731 patients, 52.1% had congenital heart defects. Congenital heart defect was significantly more common in females than males. The most common cardiac defect was ventricular septal defect (35%), followed by atrial septal defect (31.8%), atrioventricular septal defect (23.4%), tetralogy of Fallot (5%), and patent ductus arteriosus (3.6%). In the follow-up, 43.2% of atrial septal defect, 17.8% of ventricular septal defect, and a total of 20% of congenital heart defects were closed spontaneously. About 34.4% of congenital heart defect was corrected by cardiac surgery/intervention. Five-year survival rate was 97.4% in patients without congenital heart defects, whereas it was 95.6% in mild congenital heart defects and 86.1% in moderate to severe congenital heart defects. There was no relationship between consanguinity, parental age, maternal disease, folic acid supplementation before/during pregnancy, gestational age, birth weight, and congenital heart defects. Neuromotor development was similar in patients with and without congenital heart defects. CONCLUSION: We demonstrated that almost half of the patients had congenital heart defects; ventricular septal defect was the most common congenital heart defect type. This study is valuable in terms of the largest single-center study describing the classification, prognostic factors, and survival of Down syndrome patients with congenital heart defect from Turkey.

2.
Cardiol Young ; 33(11): 2203-2208, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36606531

RESUMO

AIM: Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. While cardiac T2* MRI is the gold standard in determining cardiac iron accumulation, it is not always feasible, which has led to the search for new biomarkers. Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major. MATERIALS AND METHOD: Forty-one patients aged 11-21 years and 41 age-, gender-, body mass index-matched healthy controls were included. Serum growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were compared between the patients and controls. Additionally, the relations of these biomarkers with cardiac and liver T2 * MRI were investigated in the patients. RESULTS: In the patients, growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were higher than healthy controls (p < 0.001, p = 0.025, p < 0.001, respectively). There were no significant correlations of growth differentiation factor-15 and N-terminal pro-B-type natriuretic peptide levels with both cardiac and liver T2 * MRI measurements. While there was no significant correlation of serum galectin-3 with cardiac T2 * MRI measurements, a negative correlation was found with liver T2 * MRI measurements (p = 0.040, rho = -0.325). CONCLUSION: All three biomarkers investigated in this study failed to predict myocardial iron accumulation in asymptomatic children with beta-thalassemia major. However, a weak relation between serum galectin-3 level and hepatic iron accumulation was demonstrated.


Assuntos
Sobrecarga de Ferro , Talassemia beta , Humanos , Criança , Talassemia beta/complicações , Peptídeo Natriurético Encefálico , Galectina 3 , Sobrecarga de Ferro/diagnóstico , Sobrecarga de Ferro/etiologia , Miocárdio , Imageamento por Ressonância Magnética , Fígado , Biomarcadores , Ferro , Fatores de Diferenciação de Crescimento
3.
Turk Arch Pediatr ; 57(6): 661-667, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36314959

RESUMO

OBJECTIVE: Transcatheter secundum type atrial septal defect closure is an alternative to surgical closure in many cases when conditions are appropriate. In this study, the demographic data and follow-up results of patients with secundum atrial septal defect undergoing transcatheter closure were discussed. MATERIALS AND METHODS: Data of patients who underwent transcatheter closure of secundum atrial septal defect between 2004 and 2017 were investigated retrospectively. Gender, age at intervention, defect size, procedure duration, fluoroscopy time, periprocedural complications, residual shunt existence, and early and mid-term follow-up results were collected. RESULTS: A total of 179 patients [41% males; 10% adults, median age: 8.1 years (1.3-58.6); weight: 28 kg (11-90)] were admitted to catheterization for atrial septal defect closure and their median atrial septal defect size was 13 mm (6-30); 74 (41%) patients had a large atrial septal defect (≥12 mm). Suitable defects for closure were observed in 165 of 179 patients. The procedural success rate was 95.7%. No death was observed; however, minor complications occurred in 3 patients during the procedure (1.6%). The rate of residual shunt after 1 year was 1.3%, and all shunts were mild. After a median follow-up of 2.8 years (range, 6 months to 13.6 years), delayed major complications such as death, cardiac erosion, and infective endocarditis were not experienced. The delayed minor complication was supraventricular extrasystole in 1 patient. CONCLUSION: Transcatheter atrial septal defect closure is safe in children and adults with a minimal rate of periprocedural and delayed complications. It has a favorable early and mid-term outcome in our study, especially with no death or major complications.

4.
Echocardiography ; 39(10): 1307-1315, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36126339

RESUMO

AIM: This study aimed to evaluate the role of real-time three-dimensional (four-dimensional) and speckle tracking echocardiography for early detection of left ventricular systolic dysfunction and also for the relationship between myocardial deformation parameters and myocardial iron load which is measured by cardiac magnetic resonance relaxation time T2* values in asymptomatic children with beta-thalassemia major. MATERIAL AND METHODS: This multicenter cross-sectional study included 40 patients (mean age 15.4 ± 2.9, 42.1% male) and 40 healthy children whose age, gender, and body mass index-matched with patients. Each participant underwent conventional echocardiography and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strains; twist; and torsion were measured by real-time three-dimensional and speckle tracking echocardiography. Cardiac magnetic resonance imaging T2* was measured in patients. RESULTS: Left ventricular global longitudinal, circumferential, and radial strains were decreased despite preserved global ventricular function in patients compared to healthy children (p = p = .029, p = p < .001, p = .003, respectively). There were no statistically significant differences between patients with T2* ≥ 20 ms and patients with T2* < 20 ms for all echocardiographic parameters. Also, there were no significant correlations between all echocardiographic parameters and T2* values in all patients, those with T2* ≥ 20 ms, and T2* < 20 ms. CONCLUSION: We found that even in asymptomatic children with beta-thalassemia major, left ventricular longitudinal, circumferential and, radial functions were impaired by real-time three-dimensional (four-dimensional) and speckle tracking echocardiography. This novel echocardiographic method might be an important tool for detecting subclinical left ventricular systolic dysfunction irrespective of T2* values.


Assuntos
Ecocardiografia Tridimensional , Disfunção Ventricular Esquerda , Talassemia beta , Criança , Humanos , Masculino , Feminino , Função Ventricular Esquerda , Talassemia beta/complicações , Volume Sistólico , Estudos Transversais , Ecocardiografia/métodos , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/diagnóstico por imagem , Ecocardiografia Tridimensional/métodos
5.
Eur J Med Genet ; 63(4): 103819, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31778856

RESUMO

Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by lipoatrophy affecting the face, limbs and trunk, acromegaloid features, hepatomegaly, hypertriglyceridemia, and insulin resistance. The aim of this study is to evaluate the long-term follow-up findings including gastrointestinal and cardiac manifestations of the patients with CGL1 and CGL4, caused by mutations in the AGPAT2 and CAVIN1 genes, respectively. Two patients aged 2 and 9 years with the same biallelic CAVIN1 mutation and five patients aged between 6 months and 11 years 4 months with AGPAT2 mutations have been followed up for 3-9 years. The patients were between 7 and 20 years of age at their last examination. One of the two patients with CGL4 had congenital pyloric stenosis. The other patient with CGL4 have developed recurrent duodenal perforations which have not been reported in CGL patients previously. The pathological examination of duodenal specimens revealed increased subserosal fibrous tissue and absent submucosal adipose tissue. None of the five CGL1 patients had gastrointestinal problems. Two patients with CGL4 developed hypertrophic cardiomyopathy (HCMP) and severe cardiac arrhythmia, only one patient with CGL1 had HCMP. Hyperinsulinemia was detected in one patient with CGL4 and three patients with CGL1, these three CGL1 patients also had acanthosis nigricans. Hepatic steatosis was detected in one patient with CGL4 and two patients with CGL1 by ultrasonography. In conclusion, these findings suggest that CGL4 patients should also be carefully followed up for gastrointestinal and cardiac manifestations.


Assuntos
Aciltransferases/genética , Lipodistrofia Generalizada Congênita , Proteínas de Ligação a RNA/genética , Adolescente , Adulto , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/genética , Criança , Pré-Escolar , Duodeno/patologia , Fígado Gorduroso/etiologia , Fígado Gorduroso/genética , Feminino , Humanos , Hiperinsulinismo/etiologia , Hiperinsulinismo/genética , Lipodistrofia Generalizada Congênita/complicações , Lipodistrofia Generalizada Congênita/genética , Masculino , Mutação , Estenose Pilórica/etiologia , Estenose Pilórica/genética , Adulto Jovem
6.
Turk Pediatri Ars ; 54(3): 139-140, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31619924
7.
Turk Pediatri Ars ; 53(2): 124-128, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30116135

RESUMO

Catecholaminergic polymorphic ventricular tachycardia is a rhythm disorder that develops due to genetic reasons in the absence of structural cardiac abnormalities. Ventricular tachycardia, ventricular fibrillation, cardiac arrest, and death may occur. Two-year-old patient presented to the Emergency Department with sudden cardiac arrest. He had syncope attacks after playing with his brother and he was followed up by the pediatric neurology and cardiology clinics. Cardiopulmonary resuscitation was performed, and he was then transferred to the Intensive Care Unit because of hypotension; dobutamine and norepinephrine treatment was started. After treatment, ventricular tachycardia, ventricular fibrillation, and cardiac arrest developed. Dobutamine and noradrenaline was stopped immediately and amiodarone was started. A genetic test revealed heterozygote missense mutation (c.9110G>A(p.Gly3037Asp)) in exon 64 of the RYR2 gene, which is compatible with catecholaminergic polymorphic ventricular tachycardia. This mutation has been reported in the literature for the first time. This case is presented with the purpose of highlighting catecholaminergic polymorphic ventricular tachycardia.

8.
Pediatr Cardiol ; 39(8): 1547-1553, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29980825

RESUMO

We evaluated the natural course of congenital aortic valvular stenosis (AVS) and factors affecting AVS progression during long-term follow-up with echocardiography. Medical records of 388 patients with AVS were reviewed; patients with concomitant lesions other than aortic regurgitation (AR) were excluded. Trivial AVS was defined as a transvalvular Doppler peak systolic instantaneous gradient of < 25 mmHg; mild stenosis, 25-49 mmHg; moderate stenosis, 50-75 mmHg; and severe stenosis, > 75 mmHg. Median age of the patients was 3 years (range 0 day to 21 years), and 287 (74%) were male. A total of 355 patients were followed with medical treatment alone for a median of 4.6 years (range 1 month to 20.6 years), and the degree of AVS increased in 75 (21%) patients. The risk of AVS progression was higher when AVS was diagnosed in neonates (OR 4.29, CI 1.81-10.18, p = 0.001) and infants (OR 3.79, CI 2.21-6.49, p = 0.001). After the infancy period, bicuspid valve morphology increased AVS progression risk (OR 2.4, CI 1.2-4.6, p = 0.034). Patients with moderate AVS were more likely to have AVS progression (OR 2.59, CI 1.3-5.1, p = 0.006). Bicuspid valve morphology increased risk of AR development/progression (OR 1.77, CI 1.1-2.7, p = 0.017). The patients with mild and moderate AVS were more likely to have AR development/progression (p = 0.001). The risk of AR development/progression was higher in patients with AVS progression (OR 2.25, CI 1.33-3.81, p = 0.002). Newborn babies and infants should be followed more frequently than older patients according to disease severity. Bicuspid aortic valve morphology and moderate stenosis are risk factors for the progression of AVS and AR.


Assuntos
Estenose da Valva Aórtica/diagnóstico por imagem , Ecocardiografia Doppler em Cores , Adolescente , Adulto , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/patologia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/congênito , Estenose da Valva Aórtica/fisiopatologia , Doença da Válvula Aórtica Bicúspide , Criança , Pré-Escolar , Progressão da Doença , Feminino , Seguimentos , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Adulto Jovem
9.
Pediatr Cardiol ; 39(8): 1554-1560, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29923134

RESUMO

The two main modalities used for congenital aortic valvular stenosis (AVS) treatment are balloon aortic valve dilatation (BAD) and surgical aortic valvuloplasty (SAV). This study evaluates residual and recurrent stenosis, aortic regurgitation (AR) development/progression, reintervention rates, and the risk factors associated with this end point in patients with non-critical congenital AVS who underwent BAD or SAV after up to 18 years of follow-up. From 1990 to 2017, 70 consecutive interventions were performed in patients with AVS, and 61 were included in this study (33 BADs and 28 SAVs). There were no significant differences in age, sex distribution, PSIG, and AR frequency between the BAD and SAV groups. Bicuspid valve morphology was more common in the BAD group than the SAV group. There was no statistically significant difference between PSIGs and AR development or progression after intervention at the immediate postoperative echocardiography of patients who underwent BAD or SAV (p = 0.82 vs. p = 0.29). Patients were followed 6.9 ± 5.1 years after intervention. The follow-up period in the SAV group was longer than that of the BAD group (9.5 ± 5.4 vs. 5.5 ± 4.4 years, p = 0.003). There was no statistically significant difference in the last echocardiographic PSIG between patients who underwent SAV or BAD (51.1 ± 33.5 vs. 57.3 ± 35.1, p = 0.659). Freedom from reintervention was 81.3% at 5 years and 57.5% at 10 years in the BAD group and 95.5% at 5 years and 81.8% at 10 years in the SAV group, respectively (p = 0.044). There was no difference in postprocedural immediate PSIG and last PSIG at follow-up and the development/progression of AR between patients who were treated with BAD versus SAV. However, long-term results of SAV were superior to those of BAD, with a somewhat prolonged reintervention interval.


Assuntos
Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Valvuloplastia com Balão/métodos , Adolescente , Insuficiência da Valva Aórtica/epidemiologia , Insuficiência da Valva Aórtica/etiologia , Valvuloplastia com Balão/efeitos adversos , Criança , Pré-Escolar , Progressão da Doença , Ecocardiografia/métodos , Feminino , Seguimentos , Humanos , Masculino , Recidiva , Reoperação/estatística & dados numéricos , Fatores de Risco , Taxa de Sobrevida , Substituição da Valva Aórtica Transcateter/estatística & dados numéricos , Resultado do Tratamento
10.
Transplantation ; 102(3): 484-492, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-28926375

RESUMO

BACKGROUND: The early impact of renal transplantation on subclinical cardiovascular measures in pediatric patients has not been widely investigated. This analysis is performed for pediatric patients participating in the prospective cardiovascular comorbidity in children with chronic kidney disease study and focuses on the early effects of renal replacement therapy (RRT) modality on cardiovascular comorbidity in patients receiving a preemptive transplant or started on dialysis. METHODS: We compared measures indicating subclinical cardiovascular organ damage (aortal pulse wave velocity, carotid intima media thickness, left ventricular mass index) and evaluated cardiovascular risk factors in 166 pediatric patients before and 6 to 18 months after start of RRT (n = 76 transplantation, n = 90 dialysis). RESULTS: RRT modality had a significant impact on the change in arterial structure and function: compared to dialysis treatment, transplantation was independently associated with decreases in pulse wave velocity (ß = -0.67; P < 0.001) and intima media thickness (ß = -0.40; P = 0.008). Independent of RRT modality, an increase in pulse wave velocity was associated with an increase in diastolic blood pressure (ß = 0.31; P < 0.001). Increasing intima media thickness was associated with a larger increase in body mass index (ß = 0.26; P = 0.003) and the use of antihypertensive agents after RRT (ß = 0.41; P = 0.007). Changes in left ventricular mass index were associated with changes in systolic blood pressure (ß = 1.47; P = 0.01). CONCLUSIONS: In comparison with initiating dialysis, preemptive transplantation prevented further deterioration of the subclinical vascular organ damage early after transplantation. Classic cardiovascular risk factors, such as hypertension and obesity are of major importance for the development of cardiovascular organ damage after renal transplantation.


Assuntos
Doenças Cardiovasculares/etiologia , Falência Renal Crônica/terapia , Terapia de Substituição Renal , Adolescente , Velocidade do Fluxo Sanguíneo , Espessura Intima-Media Carotídea , Criança , Comorbidade , Humanos , Falência Renal Crônica/complicações , Estudos Prospectivos
11.
Pediatr Cardiol ; 38(5): 915-921, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28401252

RESUMO

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40 days to 24.9 years (median, 1.8 years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p < 0.001). Defect size became small in 306 (16.8%) of patients with VSD at a median of 2.5 years. Aneurysmal transformation was detected in 32.9% (600), left ventricular-to-right atrial shunt in 9.7% (176), subaortic ridge in 2.6% (48) of 1823 patients who were followed. In 381 (20.9%) of the 1823 patients, the VSD had been closed by a surgical or transcatheter technique. Surgery is required in one-fifth of patients with subaortic ridge or aortic valve prolapse. In conclusion, isolated VSDs are usually benign abnormalities that tend to shrink and close spontaneously.


Assuntos
Comunicação Interventricular , Adolescente , Adulto , Prolapso da Valva Aórtica/complicações , Criança , Pré-Escolar , Ecocardiografia , Feminino , Comunicação Interventricular/classificação , Comunicação Interventricular/complicações , Comunicação Interventricular/fisiopatologia , Comunicação Interventricular/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Fluxo Sanguíneo Regional , Remissão Espontânea , Adulto Jovem
12.
Indian Heart J ; 68 Suppl 2: S233-S236, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27751300

RESUMO

For a newborn, surgical correction has been the primary treatment of native coarctation at most centers; however, there has been an increased use of balloon angioplasty (BA). The anterograde transvenous (AT) technique is another alternative way for coarctation (AoC) angioplasty in low weight patients with large ventricular septal defect (VSD). Four, 5-day-old to 7-month-old, infants weighing 2500, 2700, 2800, and 3400g, respectively presented to emergency unit (EU) with cyanosis, tachypnea, and loss of weight. Echocardiography demonstrated AoC and VSD. All four children were admitted to the EU with hemodynamic compromise and critically ill status. We used femoral vein for sheath and used VSD to enter left ventricle from right antegrade route, and performed BA without any complication. AT described in this report is another alternative way for coarctation angioplasty in patients with large VSD. We suggest that AT BA can be applied to small infants in situations where surgery might have been hazardous.


Assuntos
Anormalidades Múltiplas , Angioplastia com Balão/métodos , Coartação Aórtica/cirurgia , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Angiografia , Coartação Aórtica/diagnóstico , Ecocardiografia Doppler em Cores , Veia Femoral , Seguimentos , Comunicação Interventricular/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino
13.
Turk Pediatri Ars ; 51(3): 135-141, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27738397

RESUMO

AIM: To investigate ventricular arrhythmias in children with primary mitral valve prolapse and to evaluate its relation with QT length, QT dispersion, autonomic function tests and heart rate variability measurements. MATERIAL AND METHODS: Fourty two children with mitral valve prolapse and 32 healthy children were enrolled into the study. Twelve-lead electrocardiograms, autonomic function tests, echocardiography and 24-hour rhythm Holter tests were performed. Electrocardiograms were magnified digitally. The QT length was corrected according to heart rate. The patients were grouped according to the number of premature ventricular contractions and presence of complex ventricular arhythmia in the 24-hour rhythm Holter monitor test. Heart rate variability measurements were calculated automatically from the 24-hour rhythm Holter monitor test. Orthostatic hypotension and resting heart rate were used as autonomic function tests. RESULTS: The mean age was 13.9±3.3 years in the patient group and 14.6±3.1 years in the control group (p>0.05). Thirty four of the patients (81%) were female and eight (19%) were male. Twenty five of the control subjects (78%) were female and seven (22%) were male. The QT dispersion and heart rate corrected QT interval were found to be significantly increased in the children with primary mitral valve prolapse when compared with the control group (56±16 ms vs. 43±11 ms, p=0.001; 426±25 ms vs. 407±26 ms, p=0.002, respectively). In 24-hour rhythm Holter monitor tests, ventricular arrhythmias were found in 21 out of 42 patients (50%) and 6 out of 32 control subjects (18.8%) (p=0.006). QT dispersion was found to be significantly increased in patients with premature ventricular contractions ≥ 10/day and/or complex ventricular arrhythmias compared to the control group without ventricular premature beats (p=0.002). There was no significant difference in autonomic function tests and heart rate variability measurements between the patient and control groups. CONCLUSIONS: The noted increase in QT dispersion may be a useful indicator for the clinician in the evaluation of impending ventricular arrhythmias in children with primary mitral valve prolapse.

14.
Turk Pediatri Ars ; 51(1): 1-7, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27103858

RESUMO

In the final Jones criteria, different diagnostic criteria were established for the diagnosis of acute rheumatic fever for low risk and moderate-high risk populations. Turkey was found to be compatible with moderate-high risk populations as a result of regional screenings performed in terms of acute rheumatic fever and rheumatic heart disease. The changes in the diagnostic criteria for low-risk populations include subclinical carditis found on echocardiogram as a major criterion in addition to carditis found clinically and a body temperature of 38.5°C and above as a minor criterion. In moderate-high risk populations including Turkey, subclinical carditis found on echocardiogram in addition to clinical carditis is used as a major criterion as a new amendment. In addition, aseptic monoarthritis and polyarthralgia are used as major criteria in addition to migratory arthritis and monoarhtralgia is used as a minor criterion among joint findings. However, differentiation of subclinical carditis from physiological valve regurgitation found in healthy individuals and exclusion of other diseases involving joints when aseptic monoarthritis and polyarthralgia are used as major criteria are very important. In addition, a body temperature of 38°C and above and an erythrocyte sedimentation rate of 30 mm/h and above have been accepted as minor criteria. The diagnostic criteria for the first attack have not been changed; three minor findings have been accepted in presence of previous sterptococcal infection in addition to the old cirteria for recurrent attacks. In the final Jones criteria, it has been recommended that patients who do not fully meet the diagnostic criteria of acute rheumatic fever should be treated as acute rheumatic fever if another diagnosis is not considered and should be followed up with benzathine penicilin prophylaxis for 12 months. It has been decided that these patients be evaluated 12 months later and a decision for continuation or discontinuation of prophylaxis should be made. In countries where the disease is prevalent, it is very important for physicians to make an accurate diagnosis of acute rheumatic fever with their own logic and assessment in addition to the criteria proposed.

15.
Clin Exp Nephrol ; 20(6): 926-933, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26822545

RESUMO

BACKGROUND: Cardiovascular disease (CVD) is an important complication of chronic kidney disease (CKD) in children. However, it is not well known when and how cardiovascular alterations start. METHODS: This cross-sectional, controlled study consisted of 25 patients and 28 healthy controls. 24-h ambulatory blood pressure monitoring, aortic pulse wave velocity (aPWV), carotid intima-media thickness (cIMT) and carotid distensibility (distensibility coefficient and ß stiffness index), and echocardiography were assessed to evaluate CVD. Routine biochemical parameters, fibroblast growth factor-23 (FGF23) and high sensitive C- reactive protein were measured to determine cardiovascular risk factors. RESULTS: Hypertension was found in 12 patients (48 %). Patients had higher FGF23 levels and aPWV-standard deviation score (SDS) as compared to the controls (p = 0.003 and p = 0.002, respectively). Aortic PWV-SDS was predicted by increased daytime systolic blood pressure load (ß = 0.512, p = 0.009, R 2 = 0.262). Neither cIMT nor distensibility differed between the groups; however, older age and high level of FGF23 were independent predictors of ß stiffness index in patients (ß = 0.507, p = 0.005, R 2 = 0.461 and ß = 0.502, p = 0.005, R 2 = 0.461, respectively). As compared to controls, patients had worse left ventricular diastolic function [lower E/A ratio p = 0.006) and increased left atrial dimension (p < 0.001)]. CONCLUSIONS: Cardiovascular deteriorations appear in children with stage-2 CKD. Good control of BP and decreasing the level of FGF23 may be useful to slow down the progression of cardiovascular complications.


Assuntos
Doenças Cardiovasculares/etiologia , Insuficiência Renal Crônica/complicações , Adolescente , Pressão Sanguínea , Espessura Intima-Media Carotídea , Criança , Estudos Transversais , Feminino , Fator de Crescimento de Fibroblastos 23 , Fatores de Crescimento de Fibroblastos/sangue , Humanos , Masculino , Análise de Onda de Pulso , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/fisiopatologia , Fatores de Risco
16.
Turk Pediatri Ars ; 50(1): 61-4, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26078698

RESUMO

Information about the effects of synthetic cannabinoids "bonzai" on the cardiovascular system is limited. In this article, two patients in whom different cardiological side effects were observed following use of synthetic cannabinoids 'bonzai' were presented. Our first patient who was a 16-year old boy presented to pediatric emergency department with severe chest pain which had started one hour before. On electrocardiographic examination, ST segment elevations greater than 3 mm were observed in DII, DIII, AVF and V5-V6 derivations which caused to suspicious myocardial infarction. Cardiac catheterization was performed and coronary angiographic findings were found to be normal. When the patient was questioned again, it was learned that he used synthetic cannabinoid 'bonzai'. The second patient who was aged 18 years and addicted to synthetic cannabinoid 'bonzai' was brought to our emergency department by his friends because of clouding of consciousness. Dopamine treatment was started in the patient whose cardiac apical beat reduced to 40/min and who was found to develop hypertension, because bradycardia worsened. However, the blood pressure increased rapidly and dopamine treatment was stopped. Heart rate and blood pressure monitoring was continued. Use of synthetic cannabinoids 'bonzai' should be kept in mind in patients who present to emergency departments with chest pain and complaints related with the cardiovascular system.

17.
Cardiol Young ; 24(3): 422-9, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23680583

RESUMO

BACKGROUND: Assessment of right ventricular function is a key point in the follow-up of operated patients with tetralogy of Fallot. Cardiac magnetic resonance assessment of right ventricular function is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. Myocardial performance index and isovolumic acceleration have recently been studied for the assessment of right ventricular function and are shown to be simple yet powerful tools for assessing patients with right ventricular dysfunction of various origins. METHODS: In this study, the integrity of myocardial performance index and isovolumic acceleration obtained by tissue Doppler imaging echocardiography to quantify right ventricular function was assessed in 31 patients operated for tetralogy of Fallot. Myocardial performance index and isovolumic acceleration measurements were compared with the parameters derived by cardiac magnetic resonance imaging. RESULTS: In this study, a significant correlation has not been detected between cardiac magnetic resonance-originated right ventricular ejection fraction, pulmonary regurgitation fraction and myocardial performance index, isovolumic acceleration obtained by tissue Doppler imaging echocardiography from the lateral tricuspid annulus of the right ventricle. CONCLUSION: We have concluded that when evaluated separately, myocardial performance index and isovolumic acceleration obtained from tissue Doppler imaging echocardiography can be used in the long-term follow-up of patients who have been operated for tetralogy of Fallot, but that they do not show correlation with cardiac magnetic resonance-originated right ventricle ejection fraction and pulmonary regurgitation fraction.


Assuntos
Técnicas de Imagem Cardíaca , Imageamento por Ressonância Magnética , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita , Adolescente , Criança , Estudos Transversais , Feminino , Coração/fisiologia , Humanos , Masculino
19.
Pediatr Hematol Oncol ; 30(7): 674-6, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23484777

RESUMO

Voriconazole is a triazole antifungal drug that is used to treat invasive fungal infections, especially aspergillus. Here, we report two children who had severe bradycardia associated with voriconazole at a dose of 12 mg/kg per day. Bradycardia resolved in 24 hours in both after decreasing the dose to 10 mg/kg per day. Heart rates were in normal limits on follow-up. Bradycardia may be a side effect of voriconazole treatment in children under immunosuppressive treatment. Heart rate should be monitored in patients receiving voriconazole and other triazole treatments.


Assuntos
Antifúngicos/efeitos adversos , Aspergilose/tratamento farmacológico , Bradicardia/congênito , Pirimidinas/efeitos adversos , Triazóis/efeitos adversos , Antifúngicos/administração & dosagem , Bradicardia/terapia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Pirimidinas/administração & dosagem , Fatores de Tempo , Triazóis/administração & dosagem , Voriconazol
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