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1.
Eur J Pediatr ; 182(3): 1281-1288, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36631690

RESUMO

Tissue Doppler echocardiography (TDI) is a convenient method to detect cardiac dysfunction in the infants of diabetic mothers (IDMs). Umbilical cord serum insulin-like growth factor-1 (IGF-1) is known to be higher in IDMs. We aimed to determine whether there is a relation between diastolic functions examined by TDI and cord serum IGF-1 levels of IDMs. Cord serum IGF-1 levels of 32 IDMs and 22 healthy infants were measured. The cardiac functions of the infants were evaluated by M-Mode and TDI. For statistical analysis, Mann-Whitney U and Spearman correlation tests were used for continuous variables, and the chi-square test was used for categorical variables. The cord serum IGF-1 levels of the IDMs were higher (p = 0.000). The left ventricle (LV) e', LVa', LV e'/a', and LV Tei index, indicating left ventricular diastolic dysfunction in IDMs, were detected (LV e' p = 0.016; LV a' p = 0.003; LV e'/ a' p = 0.000; LV Tei index p = 0.023). IDMs' cord serum IGF-1 levels were found weakly correlated with the interventricular septum (IVS) and left ventricle posterior wall (LVPW) thicknesses in M-Mode and LV e' and LV e'/a' in TDI (IVS r = 0.357, p = 0.008; LVPW r = 0.289, p = 0.034; LV e' r = 0.297, p = 0.029; LV e'/ a' r = 0.031, p = 0.014). CONCLUSION: To our knowledge, this is the first study to examine the relationship between cord serum IGF-1 levels and diastolic functions of IDMs assessed by TDI. A weak correlation was found between IGF-1 levels and IVS and LVPW thicknesses in M-Mode and LV e' and LV e'/a' parameters in TDI, revealing diastolic dysfunction in IDMs. WHAT IS KNOWN: • The umbilical cord blood serum IGF-1 level of IDMs is higher than in infants of healthy mothers. • Diastolic dysfunction is a well-studied and frequently observed consequence in IDMs. WHAT IS NEW: • This is the first study to examine the relationship between cord serum IGF-1 levels and diastolic functions of IDMs assessed by TDI. • A weak correlation was detected between the median cord serum IGF-1 level of IDMs and the median values of IVS, LVPW, LV e', LV a', LV e'/a' ratio.


Assuntos
Diabetes Mellitus , Disfunção Ventricular Esquerda , Feminino , Humanos , Lactente , Mães , Fator de Crescimento Insulin-Like I , Ecocardiografia Doppler , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Diástole
2.
Am J Perinatol ; 39(4): 369-372, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-32882742

RESUMO

OBJECTIVE: The aim of the study is to determine the most accurate length and position of umbilical venous catheter (UVC). STUDY DESIGN: This prospective study included premature infants who were admitted to the neonatal intensive care unit with inserted UVC between January 1, 2014 and December 31, 2015. The length of UVC was calculated according to the Shukla formula [(3 × birth weight + 9)/2 + 1] and the catheter was inserted under sterile conditions. After the insertion, umbilical catheter was first evaluated through chest X-ray and then with echocardiography to confirm its position. Catheters seen on the chest X-ray at the level of T9-T10 vertebrae were classified as "accurate position," those seen above T9 vertebra as "high position," and the catheters identified below T10 vertebra were classified as "low position." RESULTS: A total of 68 infants smaller than 36 weeks of gestation were included in the study. In echocardiographic evaluation, 80% of the cases identified as in the "accurate position," 100% of the cases classified as in a "high position," and 33% of the cases defined as in a "low position" on the chest X-rays were found to be intracardiac. In our study, length of the catheter calculated according to the Shukla formula was intracardiac in 88.2% of premature infants. CONCLUSION: Radiography alone is not sufficient for the determination of adequate position of umbilical catheter, especially in premature infants. Specialists practicing in neonatal intensive care units could improve themselves and evaluate UVC with echocardiography, making this a routine part of clinical practice. Echocardiography-guided fixation of the catheter will reduce the complications related to catheter malposition. KEY POINTS: · Shukla formula is commonly used to calculate the adequate length of UVC.. · Chest X-ray is the most widely used modality for locating the tip of UVC.. · Echocardiography can be conveniently used for the determination of adequate position of UVC..


Assuntos
Cateterismo Venoso Central , Cateterismo Periférico , Cateteres , Cateteres de Demora , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Estudos Prospectivos , Veias Umbilicais/diagnóstico por imagem
3.
Fetal Pediatr Pathol ; 41(4): 616-626, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34280066

RESUMO

AimPentraxin-3, high sensitive CRP (HsCRP) and adropin were investigated in cord blood of infants of mothers with gestational diabetes mellitus (IDM) to evaluate the exposure of fetus to inflammation and whether there is any correlation with clinical findings.MethodsForty IDM and forty three infants whose mother did not have diabetes were included in this prospective study. Adropin, pentraxin-3 and HsCRP levels were measured in the cord blood samples. Echocardiographic measurements were performed in the first three days of life.ResultsAdropin and pentraxine-3 levels were significantly lower and HsCRP levels were significantly higher in IDM group. Echocardiographic measurements of myocardial hypertrophy were negatively correlated with adropin.ConclusionAlterations in these markers in IDM supports the hypothesis of in utero fetal exposure to inflammation caused by gestational diabetes mellitus. Potentially, cord blood adropin might be used as a predictor for complications of diabetes.


Assuntos
Diabetes Gestacional , Gravidez em Diabéticas , Biomarcadores , Proteína C-Reativa , Feminino , Sangue Fetal , Humanos , Inflamação , Mães , Gravidez , Estudos Prospectivos
4.
Am J Perinatol ; 36(11): 1205-1210, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30583298

RESUMO

OBJECTIVE: The present study aimed to assess the global oxidant and antioxidant status in infants born to preeclamptic mothers and their correlation with cardiac functions. STUDY DESIGN: We compared 40 infants born to preeclamptic mothers with 40 premature infants born to normotensive mothers. We assessed the relationship between echocardiographic measurements and total antioxidant capacity (TAC) and total oxidant status (TOS) values. RESULTS: In the study group, TAC, TOS, and oxidative stress index (OSI) levels were significantly higher in the cord blood (p = 0.03, 0.04, and 0.039, respectively) than in the control group. We did not observe any correlation between echocardiographic measurements and TAC, TOS, and OSI levels in infants born to preeclamptic mothers. CONCLUSION: Compared with the control group, despite higher TAC levels in infants born to preeclamptic mothers, concurrent elevated OSI levels reveal that the oxidant-antioxidant balance is disturbed in favor of oxidants. Furthermore, the findings of this study suggest that echocardiographic parameters are unaffected by the oxidant status.


Assuntos
Sangue Fetal/metabolismo , Coração/fisiologia , Recém-Nascido Prematuro/fisiologia , Estresse Oxidativo , Pré-Eclâmpsia , Estudos de Casos e Controles , Ecocardiografia , Feminino , Sangue Fetal/química , Coração/anatomia & histologia , Coração/diagnóstico por imagem , Humanos , Recém-Nascido , Recém-Nascido Prematuro/sangue , Recém-Nascido Prematuro/metabolismo , Magnésio/sangue , Masculino , Gravidez , Estudos Prospectivos , Ultrassonografia Doppler
6.
Artigo em Inglês | MEDLINE | ID: mdl-28630369

RESUMO

Primary cardiomyopathy is one of the most common inherited cardiac diseases and harbors significant phenotypic and genetic heterogeneity. Because of this, genetic testing has become standard in treatment of this disease group. Indeed, in recent years, next-generation DNA sequencing has found broad applications in medicine, both as a routine diagnostic tool for genetic disorders and as a high-throughput discovery tool for identifying novel disease-causing genes. We describe a male infant with primary dilated cardiomyopathy who was diagnosed using intrauterine echocardiography and found to progress to hypertrophic cardiomyopathy after birth. This proband was born to a nonconsanguineous family with a past history of a male fetus that died because of cardiac abnormalities at 30 wk of gestation. Using whole-exome sequencing, a novel homozygous frameshift mutation (c.2018delC; p.Gln675SerfsX30) in ALPK3 was identified and confirmed with Sanger sequencing. Heterozygous family members were normal with echocardiographic examination. To date, only two studies have reported homozygous pathogenic variants of ALPK3, with a total of seven affected individuals with cardiomyopathy from four unrelated consanguineous families. We include a discussion of the patient's phenotypic features and a review of relevant literature findings.


Assuntos
Cardiomiopatia Hipertrófica/genética , Proteínas Musculares/genética , Proteínas Quinases/genética , Cardiomiopatias/genética , Pré-Escolar , Ecocardiografia , Exoma , Mutação da Fase de Leitura , Predisposição Genética para Doença , Testes Genéticos , Coração/fisiopatologia , Homozigoto , Humanos , Masculino , Proteínas Musculares/metabolismo , Mutação , Linhagem , Proteínas Quinases/metabolismo
7.
Arch. argent. pediatr ; 115(1): 50-57, feb. 2017. tab
Artigo em Inglês, Espanhol | LILACS | ID: biblio-1038346

RESUMO

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


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 tand Mann-Whitney U" tests. Results: Thestudy 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.


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Pais , Apolipoproteínas/sangue , Triglicerídeos/sangue , Doença da Artéria Coronariana , Lipoproteína(a)/sangue , Aterosclerose/sangue , Lipoproteínas LDL/sangue , Estudos Prospectivos , Fatores de Risco
8.
Arch Argent Pediatr ; 115(1): 50-54, 2017 02 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-28097840

RESUMO

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


Assuntos
Apolipoproteínas/sangue , Aterosclerose/sangue , Doença da Artéria Coronariana , Lipoproteína(a)/sangue , Lipoproteínas LDL/sangue , Pais , Triglicerídeos/sangue , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco
9.
Case Rep Pediatr ; 2016: 7070316, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27525147

RESUMO

We report an infant with tetralogy of fallot (TOF) who was born at 35 weeks of gestation and of 1700 g birth weight and presented with persistent retinopathy of prematurity (ROP) at 6 months of age. Follow-up ophthalmic examinations were done at 2, 3, and 4 weeks of age. A demarcation line in Zone II was noticed on the first ocular examination done at 4 weeks of postnatal age. At 6 months of postnatal age, the infant still had an avascular peripheral retina with the demarcation line in Zone II. Even though this index subject did not have any typical risk factors for ROP, TOF seems to be the probable reason for developing as well as persistence of avascular retina.

10.
J Matern Fetal Neonatal Med ; 29(23): 3786-9, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26820601

RESUMO

OBJECTIVE: The aim of this study is to evaluate whether the platelet mass in the first 24 h of life is effective on closure of patent ductus arteriosus (PDA) or not. STUDY DESIGN: Preterm infants with a gestational age of < 32 weeks, hospitalized at a tertiary neonatal intensive care unit (NICU) and requiring medical treatment (intravenous or oral ibuprofen) for hemodinamically significant PDA (hsPDA) were enrolled in this study. The patients were divided into two groups after first course of pharmacologic treatment according to closure of PDA (Group 1: PDA closure, Group 2: PDA without closure). Groups were compared in terms of demographics findings, morbidities, platelet measurements like counts, mean platelet volume (MPV) and platelet mass (platelet count × mean platelet volume). RESULTS: The study included 77 preterm newborns in Group 1, and 30 preterms in Group 2. There were no differences in birth weight, gestational age, gender and maternal risk factors between the study groups. The mean platelet count in the first postnatal blood count was in Group 1: 211.3 ± 89.2 × 10(3)/mm(3) and in Group 2: 216.5 ± 26 × 10(3)/mm(3), respectively (p = 0.783). The mean platelet volumes (MPV) were similar in both groups (p = 0.535). No statistically significant difference between platelet mass values was detected (Group 1: 1811 ± 884 fl/nl, Group 2: 1868 ± 717 fl/nl) (p = 0.753). CONCLUSION: Our data suggest that platelet count, MPV and platelet mass did not affect the closure of hsPDA with ibuprofen.


Assuntos
Inibidores de Ciclo-Oxigenase/uso terapêutico , Permeabilidade do Canal Arterial/tratamento farmacológico , Ibuprofeno/uso terapêutico , Recém-Nascido Prematuro/sangue , Volume Plaquetário Médio , Permeabilidade do Canal Arterial/sangue , Feminino , Humanos , Recém-Nascido , Masculino , Contagem de Plaquetas , Distribuição Aleatória , Estudos Retrospectivos , Estatísticas não Paramétricas
11.
Pediatr Cardiol ; 37(1): 131-4, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26260095

RESUMO

Prostaglandin E1 is crucial for keeping the patent ductus arteriosus in critical congenital heart disease for the survival and palliation of particularly prematurely born babies until a cardiosurgical intervention is available. In this study, the side effects of prostaglandin E1 in newborns with critical congenital heart disease and clinical outcomes were evaluated. Thirty-five newborns diagnosed with critical congenital heart disease were treated with prostaglandin E1 between January 2012 and September 2014 at our hospital. Patient charts were examined for prostaglandin E1 side effects (metabolic, gastric outlet obstruction, apnea), clinical status, and prognosis. Acquired data were analyzed in the SPSS 20.0 program. Patients with birth weight under 2500 g needed more days of prostaglandin E1 infusion than ones with birthweight over 2500 g (P = 0.016). The ratio of patients with birth weight under 2500 g who received prostaglandin E1 longer than 7 days was higher than the patients with birth weight over 2500 g (P = 0.02). Eighteen side effects were encountered in 11 of 35 patients (31%). Of these side effects, 1 patient had 4, 4 patients had 2, and 6 patients had only 1 side effect. Discontinuation of the therapy was never needed. Prostaglandin E1 is an accepted therapy modality for survival and outcome in critical congenital heart disease in particularly low-birth-weight babies until a surgical intervention is available. Side effects are not less encountered but are almost always manageable, and discontinuation is not needed.


Assuntos
Cardiopatias Congênitas/tratamento farmacológico , Inibidores da Agregação Plaquetária/administração & dosagem , Alprostadil/administração & dosagem , Alprostadil/efeitos adversos , Peso ao Nascer , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Recém-Nascido , Infusões Intravenosas , Masculino , Cuidados Paliativos , Inibidores da Agregação Plaquetária/efeitos adversos , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo
12.
J Matern Fetal Neonatal Med ; 29(1): 135-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-25471090

RESUMO

OBJECTIVES: To compare the efficacy of oral and intravenous paracetamol for closure of hemodynamically significant patent ductus arteriosus (HSPDA) in very low birth weight (VLBW) preterm infants. METHODS: Eighteen VLBW infants with HSPDA treated with either intravenous (n = 10) or oral (n = 8) paracetamol at 60 mg/kg/d for three consecutive days were analysed retrospectively. Ductal closure rate and evaluation of liver function tests were the major outcomes. RESULTS: After two courses of treatment, HSPDA closure rate was higher in oral paracetamol group than that in the intravenous paracetamol group (88% versus 70%), but it was not statistically significant (p = 0.588). Liver function tests were normal after the treatment. CONCLUSION: Although it was not statistically significant, the cumulative closure rates were higher in oral paracetamol group than those in the intravenous group. Larger trials are needed to confirm these data.


Assuntos
Acetaminofen/administração & dosagem , Analgésicos não Narcóticos/administração & dosagem , Permeabilidade do Canal Arterial/tratamento farmacológico , Administração Intravenosa , Administração Oral , Feminino , Humanos , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Masculino , Estudos Retrospectivos
13.
Diabetes Res Clin Pract ; 109(1): 104-9, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25934526

RESUMO

Recently, oxidative stress was suggested to play a role in maternal and fetal complications of diabetic pregnancies. The aim of this study is to evaluate the global oxidant and antioxidant status in infants of diabetic mothers (IDM) via measurement of total antioxidant capacity (TAC) and total oxidant status (TOS) and to determine their association with the clinical and cardiac manifestations of gestational diabetes on infants. Forty five infants constituted the IDM group, 51 infants born to non diabetic mothers served as the control group. Umbilical cord blood was drawn from IDM and controls for TAC and TOS measurement. Echocardiographic measurements were performed in the first three days of life. Infants of diabetic mother had significantly higher TAC (p=0.024), TOS (p=0.03) and oxidative stress index (OSI, p=0.04) levels compared to controls. Hemoglobin values were correlated to TOS (r=0.310, p=0.03) and OSI (r=0.310, p=0.03). Maternal HbA1c values were also correlated to TOS (r=0.576, p=0.001) and OSI (r=0.606, p<0.001). Systolic and diastolic interventicular septum measurements, and left ventricular mass were also correlated with TOS (r=0.330, p=0.02; r=0.453, p=0.002; r=0.404, p=0.006, respectively) and OSI (r=0.330, p=0.02; r=0.300, p=0.04, r=0.300; p=0.04, respectively). Oxidant-antioxidant balance is disturbed in favor of oxidants in IDM despite compensatory increase in TAC. The degree of oxidative stress is related to the severity of myocardial and hematological involvement in IDM in the first days of life and maternal glycemic control.


Assuntos
Cardiomegalia/congênito , Cardiomegalia/epidemiologia , Diabetes Gestacional/epidemiologia , Mães , Estresse Oxidativo , Efeitos Tardios da Exposição Pré-Natal/epidemiologia , Adulto , Glicemia/análise , Estudos de Casos e Controles , Diabetes Gestacional/tratamento farmacológico , Feminino , Sangue Fetal/química , Humanos , Recém-Nascido , Masculino , Gravidez , Adulto Jovem
14.
J Pediatr Gastroenterol Nutr ; 60(2): 177-81, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25250684

RESUMO

BACKGROUND: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.


Assuntos
Cardiomiopatias/etiologia , Hipertensão Portal/complicações , Cirrose Hepática/complicações , Adolescente , Anti-Hipertensivos/uso terapêutico , Ascite/etiologia , Pressão Sanguínea , Cardiomiopatias/tratamento farmacológico , Cardiomiopatias/epidemiologia , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Diuréticos/uso terapêutico , Ecocardiografia , Eletrocardiografia , Feminino , Frequência Cardíaca , Humanos , Hipertensão Portal/tratamento farmacológico , Incidência , Masculino , Propranolol/uso terapêutico , Fatores de Risco , Índice de Gravidade de Doença , Espironolactona/uso terapêutico , Taquicardia/etiologia
15.
Pediatr Int ; 55(4): 508-12, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23910800

RESUMO

Reported herein is the case of a 2-year-old boy with Adams-Oliver syndrome who presented with dilated cardiomyopathy and complete atrioventricular block. The patient had aplasia cutis congenita with partial aplasia of the skull bones, and terminal transverse limb malformations characteristic of the disease. Although congenital cardiac malformations may be associated with the syndrome, dilated cardiomyopathy has not been previously reported to be associated with the syndrome.


Assuntos
Cardiomiopatia Dilatada/complicações , Displasia Ectodérmica/complicações , Eletrocardiografia , Bloqueio Cardíaco/complicações , Deformidades Congênitas dos Membros/complicações , Dermatoses do Couro Cabeludo/congênito , Cardiomiopatia Dilatada/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Ecocardiografia , Displasia Ectodérmica/diagnóstico , Bloqueio Cardíaco/diagnóstico , Humanos , Deformidades Congênitas dos Membros/diagnóstico , Masculino , Dermatoses do Couro Cabeludo/complicações , Dermatoses do Couro Cabeludo/diagnóstico
16.
Pathol Res Pract ; 209(5): 302-8, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23582365

RESUMO

We aimed to demonstrate the potential protective effects of pyrrolidine dithiocarbamate (PDTC) on monocrotaline (MCT)-induced pulmonary arterial hypertension (PAH). Adult male rats were randomly assigned to 4 groups: control group, MCT-treated rats only, MCT-injected rats treated with PDTC, and PDTC-treated rats only. Blood and tissue samples were collected after the sacrifice. Levels of malondialdehyde (MDA) were measured by using the thiobarbituric acid method. Total antioxidant status (TAS) was determined using a commercially available ImAnOx kit. A histopathological evaluation was accomplished by scoring the degree of severity. Endothelial damage of the main pulmonary artery was evaluated by immunohistochemical labeling of endothelial cells using anti-rat endothelial cell antigen 1 (RECA-1) antibody. MCT-induced right ventricular hypertrophy (RVH) was reduced significantly in the MCT+PDTC-treated group. MDA levels were significantly lowered in the MCT+PDTC-treated group. TAS was significantly higher in the MCT+PDTC-treated group when compared with the rats with PAH. Histopathological examination demonstrated that PDTC treatment reduced the development of inflammation, hemorrhage and congestion, and collagen deposition. In conclusion, PDTC attenuated PAH and protected pulmonary endothelium in rats administered MCT. These findings suggest that PDTC treatment may provide a new effective therapeutic approach in the treatment of PAH.


Assuntos
Antioxidantes/farmacologia , Hipertensão Pulmonar/prevenção & controle , Pirrolidinas/farmacologia , Tiocarbamatos/farmacologia , Animais , Modelos Animais de Doenças , Endotélio Vascular/efeitos dos fármacos , Endotélio Vascular/metabolismo , Endotélio Vascular/patologia , Eritrócitos/efeitos dos fármacos , Hipertensão Pulmonar Primária Familiar , Hematócrito , Hipertensão Pulmonar/induzido quimicamente , Hipertensão Pulmonar/patologia , Hipertrofia Ventricular Direita/tratamento farmacológico , Hipertrofia Ventricular Direita/metabolismo , Hipertrofia Ventricular Direita/patologia , Masculino , Malondialdeído/metabolismo , Monocrotalina/toxicidade , Ratos , Ratos Sprague-Dawley
17.
Eur J Pediatr ; 172(2): 269-72, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23011746

RESUMO

Poland syndrome has been reported to be associated with true dextrocardia, but not with true situs inversus. In this report, we describe the first patient with total situs inversus in medical literature and try to highlight the syndrome's probable etiology and pathogenetic mechanisms in utero.


Assuntos
Anormalidades Múltiplas , Dextrocardia/complicações , Síndrome de Poland/complicações , Situs Inversus/complicações , Criança , Dextrocardia/diagnóstico por imagem , Humanos , Masculino , Radiografia
19.
Adv Ther ; 25(8): 795-800, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18670742

RESUMO

INTRODUCTION: Atrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD. METHODS: Children with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0+/-3.0 years) and healthy controls (n=51; mean age, 7.6+/-2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography. RESULTS: Mean Pmax was found to be significantly higher in children with ASD compared with controls (95.2+/-10.8 vs 84.1+/-9.2 msec; P<0.001), and Pd before surgery was significantly higher compared with controls (47.4+/-12.0 vs 38.8+/-9.7 msec; P<0.001). Both P-wave indices were significantly decreased within the first year after surgical closure - the values decreased to those comparable to healthy controls (Pmax, 86.2+/-9.7 msec; Pd, 39.8+/-10.7 msec; P>0.05). CONCLUSION: Surgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.


Assuntos
Eletrocardiografia , Comunicação Interatrial/cirurgia , Criança , Feminino , Comunicação Interatrial/fisiopatologia , Humanos , Masculino
20.
Eur J Pediatr ; 167(11): 1321-6, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18668262

RESUMO

We aimed to evaluate the predictors of the severity of chronic rheumatic valvar disease. The long term follow-up records of 139 patients with chronic rheumatic carditis were reviewed. Children were followed-up on an outpatient basis for a period ranging from 1-16 years (5.0 +/- 3.7 years). Mitral regurgitation either isolated (51%, n=71) or combined with aortic regurgitation (AR) (49%, n=68) was observed in all cases of the initial attack of rheumatic carditis. AR at initial attack of the rheumatic carditis was found to be affected by gender (AR was more associated with males, p = 0.032), combined mitral and aortic regurgitation (CMAR), and presence of MR at initial attack (p = 0.000 and p = 0.012, respectively) with univariate analysis. The effect of CMAR on AR at initial attack was also significant by multivariate analysis (p = 0.000). CMAR, MR, and AR at initial attack had significant effects on CMAR at final evaluation (p = 0.000, p = 0.020, and p = 0.000, respectively) in univariate analysis. Multivariate analysis revealed the significant effects of CMAR and MR at initial attack on CMAR at final evaluation (p = 0.000 and p = 0.005, respectively). Univariate analysis showed that MR and AR at initial attack, and CMAR at final evaluation, had significant effects on MR at final evaluation (p = 0.000, p = 0.029, and p = 0.000, respectively). MR at initial attack and CMAR at final evaluation had significant effects on MR at final evaluation with multivariate analysis (p = 0.001 and p = 0.003, respectively). AR at final evaluation was affected by CMAR and AR at initial attack (p = 0.000 and p = 0.000, respectively), and CMAR and MR at final evaluation (p = 0.000 and p = 0.000, respectively) with both univariate and multivariate analysis. Mitral valve prolapsus was more common in patients with a longer duration (37.5%, 6 out of 16) than those with a shorter duration (11%, 14 out of 123) and the difference was significant (p = 0.020). In conclusion, the initial severity of valve involvement and the presence of CMAR at initial attack were found to be the best predictors for the severity of chronic rheumatic valvar disease in this study.


Assuntos
Cardiopatia Reumática/epidemiologia , Adolescente , Insuficiência da Valva Aórtica/epidemiologia , Criança , Pré-Escolar , Doença Crônica , Eletrocardiografia , Feminino , Seguimentos , Humanos , Masculino , Insuficiência da Valva Mitral/epidemiologia , Prolapso da Valva Mitral/epidemiologia , Estudos Prospectivos , Radiografia Torácica , Turquia/epidemiologia
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