Subclinical Left Ventricular Dysfunction in Children and Adolescence With Thalassemia Intermedia.

Background: Cardiac complications are important causes of morbidity in patients with thalassemia intermedia (TI). We aimed to assess left ventricular (LV) function, using new tissue Doppler imaging (TDI) indices, in order to diagnose early ventricular impairment in asymptomatic children and adolescence with the TI. Materials and Methods: We investigated possible differences in echocardiographic systolic and diastolic parameters between a population of 28 asymptomatic patients (mean age, 13.6 ± 5.7 years) and 35 age-matched healthy control members. All of them underwent 2-D, pulsed Doppler, and tissue Doppler echocardiographic studies for the assessment of the LV mass, Trans-mitral velocities, mitral annular systolic and diastolic velocities, myocardial performance index (MPI), and myocardial acceleration during isovolumic contraction (IVA). The cardiac iron load was estimated by magnetic resonance imaging T2*. Results: Left ventricular hypertrophy (LVH) was found in 13 (46.4%) patients. We found significantly reduced TDI-derived peak systolic myocardial velocity (s') in patients, whereas no significant difference was identified between the patients and control group members when the IVA was compared. The ratio of peak mitral inflow velocity to annular early diastolic velocity (E/e') of the mitral valve as an index of the diastolic function was significantly higher in patients (9 ± 1 vs. 6 ± 1, p < 0.05). Choosing a TDI-derived MPI > 0.33 as a cutoff point, the global LV dysfunction was detected with a sensitivity of 78% and a specificity of 80%. The patients with LVH significantly exhibited higher values of TDI-MPI and lower values of s' velocity and IVA when compared against the subjects with normal LV mass. Conclusion: Subtle LV systolic and diastolic dysfunction develops early in young patients with the TI who have normal cardiac iron concentration. Moreover, LV remodeling as a main cardiac adaptive response plays a principal role in developing myocardial impairment.

The Nomogram of Penile Length and Circumference in Iranian Term and Preterm Neonates.

BACKGROUND AND OBJECTIVES: The normal length of penis in preterm and term neonates is different among different nations, and is affected by various factors. The present study aimed to determine stretched penile length (SPL) values and cutoff level of micropenis in term and preterm Iranian neonates, for the first time. MATERIALS AND METHODS: All male neonates born in two general hospitals of Tehran (Akbarabadi, and Rasoul hospitals), center of Iran, with gestational age of 28-42 weeks were included and their SPL and penile circumference (PC) were examined on the first 3 days after birth by the same physician. Birth weight (BW), and height, gestational age, type of delivery, mother's age, twin/multiple pregnancy, drug, and medical history of mother during pregnancy were recorded and cutoff levels of two variables were calculated based on the collected variables for different gestational ages. RESULTS: Among a total of 587 neonates, 203 neonates were born term and 384 preterm. Mean ± SD of neonates' BW were 2,682.51 ± 739.30 (850-4800) gr. Mean ± SD of their SPL was 22.48 ± 3.34 mm; 25.92 ± 1.54 mm in term and 20.66 ± 2.50 mm in preterm infants (P = 0.001). Mean ± SD of PC was 6.71 ± 1.31 mm; 8.14 ± 0.48 in term and 5.96 ± 0.92 in preterm infants (P = 0.001). SPL and PC were significantly correlated with type of delivery, number of parity, gestational age, BW, and crown-heel length, head circumference (P < 0.001). CONCLUSION: This study suggested that SPL in male neonates was 22.48 mm and PC was 6.71 mm, both correlated with gestational age and BW. Due to the ethnical variety of this cutoff points and lack of an appropriate study in Iran, these cutoff points can be used by all physicians as a reference for Iranian newborns (term and preterm), in order to prevent misdiagnosis of micropenis and genital disorders.

The prevalence of hypertension in children with renal scars.

BACKGROUND: Hypertension (HTN) is a late outcome of congenital or acquired renal scar. We used ambulatory blood pressure to assess the early blood pressure abnormalities in children with history of urinary tract infection with various degrees of renal scars. METHODS: Between 2009 and 2011, 60 (45 females, 15 males) children aged 5-15 years and height equal or more than 120 cm with previous history of febrile urinary tract infection were entered into the study. All children went on 24-hour ambulatory blood pressure monitoring (24-H ABPM). Updated classification of 24-H ABPM was used to interpret the results. RESULTS: Masked hypertension was detected in 5% of cases, hypertension in 8.4%, and white coat hypertension in 11.7%. Pre-hypertension was seen in 23.3% of children. There was significant correlation between abnormal blood pressure and the severity of renal parenchymal scar (r=0.39, P value=0.004), vesicoureteral reflux (r= 0.34, P value=0.009), microalbuminuria (r= 0.39, P value=0.004), and carotid intima media thickness (r=0.41, P value=0.006). CONCLUSIONS: This study revealed the utility of 24-H ABPM in early detection of hypertension and pre-hypertension in children with severe renal scars and past history of urinary tract infection.

Carotid artery function in children with idiopathic nephrotic syndrome.

BACKGROUND: Nephrotic patients are prone to atherosclerosis in consequence of frequent exposures to hyperlipidemia, hypertension, and immunosuppressive drugs. OBJECTIVES: We studied the carotid parameters as early indicators of atherosclerosis in children with nephrotic syndrome. METHODS: Between 2008 and 2011, 51 children with history of nephrotic syndrome enrolled in the study. The inclusion criteria were: idiopathic nephrotic syndrome with normal serum complement, at least one year after initiation of disease, glomerular filtration rate more than 20 mL/min/1.73 m², age over two years old at the time of study. Seventy-five healthy sex-age-matched children considered as a control group. Carotid function parameters and left ventricular mass index were studied in nephrotic children. RESULTS: Steroid sensitive, resistant, and dependent nephrotic syndrome included one-third each. The mean carotid intima-media thickness (mm) in nephrotic children was 0.42 (±.14) while the mean cIMT in controls was 0.37 (±.08) (p-value <.05). After log transformation, General Linear Multivariate analysis revealed significant difference of carotid intima-media thickness in nephrotic patients (p-value <.001). Subsequently, the factor that influenced on cIMT was duration of disease (P<.05). One-half of nephrotic children who had echocardiography, showed left ventricular hypertrophy. It was correlated with carotid stiffness and systolic hypertension (P<.05). CONCLUSIONS: Carotid intima-media thickness was thicker in nephrotic children. Carotid parameters were influenced by duration of disease and hypertension.

Carotid Artery Function in Children with Idiopathic Nephrotic Syndrome

Background: Nephrotic patients are prone to atherosclerosis in consequence of frequent exposures to hyperlipidemia, hypertension, and immunosuppressive drugs. Objectives: We studied the carotid parameters as early indicators of atherosclerosis in children with nephrotic syndrome. Methods: Between 2008 and 2011, 51 children with history of nephrotic syndrome enrolled in the study. The inclusion criteria were: idiopathic nephrotic syndrome with normal serum complement, at least one year after initiation of disease, glomerular filtration rate more than 20mL/min/1.73m2, age over two years old at the time of study. Seventy-five healthy sex-age-matched children considered as a control group. Carotid function parameters and left ventricular mass index were studied in nephrotic children. Results: Steroid sensitive, resistant, and dependent nephrotic syndrome included one-third each. The mean carotid intima-media thickness (mm) in nephrotic children was 0.42 (±.14) while the mean cIMT in controls was 0.37 (±.08) (p-value <.05). After log transformation, General Linear Multivariate analysis revealed significant difference of carotid intima-media thickness in nephrotic patients (p-value <.001). Subsequently, the factor that influenced on cIMT was duration of disease (P<.05). One-half of nephrotic children who had echocardiography, showed left ventricular hypertrophy. It was correlated with carotid stiffness and systolic hypertension (P<.05). Conclusions: Carotid intima-media thickness was thicker in nephrotic children. Carotid parameters were influenced by duration of disease and hypertension (AU)

Antecedentes: Los pacientes con síndrome nefrótico son propensos a sufrir aterosclerosis como consecuencia de las frecuentes exposiciones a medicamentos para la hiperlipidemia, la hipertensión e inmunodepresores. Objetivos: Hemos estudiado los parámetros de la carótida como indicadores tempranos de aterosclerosis en niños con síndrome nefrótico. Métodos: 51 niños con antecedentes de síndrome nefrótico participaron en el estudio entre 2008 y 2011. Los criterios de inclusión fueron: síndrome nefrótico idiopático con complemento sérico normal, al menos un año después del comienzo de la enfermedad, índice de filtración glomerular superior a 20 ml/min/1,73 m2, mayor de dos años de edad en el momento del estudio. Se tuvo en consideración a setenta y cinco niños del mismo sexo y edad como grupo de control. Se estudiaron los parámetros de la función carótida y el índice de masa ventricular izquierda en niños con síndrome nefrótico. Resultados: Síndrome nefrótico sensible a esteroides, resistente a esteroides y dependiente de esteroides a partes iguales. El grosor íntima-media carotídeo medio (mm) en niños con síndrome nefrótico fue de 0,42 (±0,14), mientras que la TMIR media en controles fue de 0,37 (±0,08) (valor p <0,05). Tras la transformación logarítmica, los análisis multivariables lineales generales revelaron una diferencia significativa de grosor íntima-media carotídeo en pacientes con síndrome nefrótico (valor p <0,001). Posteriormente, el factor que influyó sobre la TMIR fue la duración de la enfermedad (p <0,05). Conclusiones: La mitad de los niños con síndrome nefrótico a los que se les realizó una ecocardiografía presentó hipertrofia ventricular izquierda. Se correlacionó con la rigidez carotídea y la hipertensión sistólica (AU)

Ambulatory blood pressure monitoring for children with ß-thalassemia major: a preliminary report.

INTRODUCTION: Heart disease is one of the most common reasons of death in beta-thalassemia major. A few studies have been done in children about blood pressure changes. The aim of this study was to assess hemodynamic changes by ambulatory blood pressure monitoring (ABPM). MATERIALS AND METHODS: In this cross-sectional study, 30 patients with beta-thalassemia major aged 5 to 18 years old were evaluated with 24-hour ABPM. The exclusion criteria were an ejection Fraction less than 50% and a glomerular filtration rate less than 90 mL/min/1.73 m2. Hypertension was defined as a mean blood pressure index of 1 and greater with or without load blood pressure greater than 25%. Dipper status was defined as a 10% decrease in nighttime versus daytime mean arterial blood pressure. RESULTS: High blood pressure was detected in 16.7% of the patients. The whole-day ABPM showed hypertension in 6.7% of the children. During daytime measurements, systolic hypertension was seen in 3.3% (load 3.7%) and diastolic in 6.7% (load 3.3%). These figures for nighttime evaluation were 6.7% (load 3.3%) and 10.3% (load 6.9%), respectively. Nondipper status was detected in 56.7% of the children. There was no significant correlation between abnormal blood pressure and age, sex, body mass index, hemoglobin, number or rates of blood transfusion, or serum ferritin level. CONCLUSIONS: The ABPM may be a useful instrument for early detection of hemodynamic changes in children with beta-thalassemia major.