The validity of a scoring system in predicting intravenous immunoglobulin treatment failure in children with kawasaki disease

Background: Between 10 and 20 of children with Kawasaki disease (KD) will not respond to intravenous immunoglobulin (IVIG) treatment, and are prone to coronary abnormalities. A variety of predicting scoring systems, including the Kobayashi system, have been proposed, but have not yet been evaluated using Iranian patients. Objectives: To evaluate the Kobayashi scoring system with regard to predicting response to IVIG treatment in Iranian children. Patients and Methods: All patients who received a final diagnosis of KD at Aliasghar children�s hospital between 1982 and 2013, and who met the inclusion criteria, were enrolled in this retrospective cohort study. We excluded patients with missing data, abnormal echocardiographic finding on admission, late admission, atypical or afebrile cases, and those who had received an insufficient amount of IVIG. We compared demographic and echocardiographic data before IVIG, and within 7 days of treatment, as well as C reactive protein (CRP), sodium, aspartate aminotransferase, platelet levels, neutrophil percentage, age of patients, and duration of fever before IVIG administration, in treatment responders and non-responders. Results: Of the 141 cases, 97 patients met the criteria and were enrolled. Of these, 19 (19.6) did not respond to IVIG. A total of 61.8 of patients were male, and the mean patient age was 36.9 months (SD = 32.1 months). Echocardiographic evaluation revealed early coronary involvement in 15.3 of patients, and coronary abnormalities were diagnosed in 10 of patients within the first 10 days of presentation and concurrent with their IVIG treatment. A between-groups comparison of quantitative CRP, absolute neutrophil count, and platelet count showed that platelet count alone was significantly higher in nonresponders (P = 0.04). With regard to items of Kobayashi scoring system, data were present for just 41 cases, but a significant difference between the two groups was shown, with the treatment-refractory group having a significantly higher score (P = 0.002). Receiver-operating characteristic curve analysis revealed that the optimum cut-off point for our population would be 2, which makes the sensitivity of the test equal to 75, with a specificity of 60. Conclusions: This preliminary study showed that patients with KD and a high Kobayashi score are at greater risk of being unresponsive to IVIG treatment. Further studies, preferably multicenter evaluations, are required in order to understand the exact application of various scoring systems in the management of people with KD in Iran. © 2016, Pediartric Infections Research Center

Predictors of left ventricular performance after valve replacement in children and adolescents with chronic aortic regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction 4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs. © Springer Science+Business Media, Inc. 2005

The Nomogram of Clitoral Length and Width in Iranian Term and Preterm Neonates

Background and Objectives: Clitoromegaly is an important parameter in the evaluation of ambiguous genitalia in neonates, but the normative data for clitoral size in newborns have racial/ethnic differences. The present study aimed to determine clitoral length (CL) and clitoral width (CW) values and establish cutoff measurement to define clitoromegaly in both term and preterm Iranian neonates for the first time. Methods: A total number of 580 female newborn infants delivered at 28�42 weeks of gestation were enrolled in the study, and their CL and CW were measured on the first 72 h of birth. Data about birth weight (BW), body length (BL), and head circumference (HC) of newborns; mothers' age; and gestational age (GA) were recorded, too. Results were presented as mean ± standard deviation (SD) for quantitative variables and were summarized by frequency (percentage) for categorical variables. Backward stepwise regression analysis was used for prediction of CL and CW. Results: Among 580 Iranian female newborns studied, 187 were term neonates and the other 393 newborns were preterm. Mean ± SD values of CL were 6.11 ± 0.39 mm in term infants and 5.45 ± 0.64 mm in preterm infants (P < 0.001). Mean ± SD values of CW were 4.22 ± 0.43 in term infants and 3.68 ± 0.53 in preterm infants (P < 0.001). Regression analysis showed that CL was correlated with GA considered by last menstrual period, BL, BW, and HC; and CW was associated with GA, BL, and BW. Conclusion: This study suggests normative values (mean + 1, 2, and 3 SD) of CL and CW according to GA, which can be used as a reference for Middle East's newborns, especially Iranian newborn babies. © Copyright © 2020 Alaei, Rohani, Norouzi, Hematian Boroujeni, Tafreshi, Salehiniya and Soheilipour

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. © 2018 Soheilipour, Rohani, Dehkordi, Isa Tafreshi, Mohagheghi, Zaheriani, Jesmi and Salehiniya

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. © 2018 Soheilipour, Rohani, Dehkordi, Isa Tafreshi, Mohagheghi, Zaheriani, Jesmi and Salehiniya

Evaluation of combined left ventricular function using the myocardial performance index in children with chronic kidney disease

Background: Myocardial dysfunction that complicates the initial stages of chronic kidney disease (CKD) has not been yet fully characterized in young patients. We aimed to assess the clinical usefulness of myocardial performance index obtained by pulsed-wave Doppler method (PWD-MPI) in predicting early disturbances of global left ventricular (LV) function in children with CKD stages 2-4. In addition, we evaluated the clinical utility of tissue Doppler imaging (TDI) as a tool for calculating MPI in comparison with the conventional method. Methods: Standard echocardiography was performed in 34 patients aged 3-18 years and for 35 age-matched healthy control subjects. PWD-MPI was calculated from Doppler spectra of mitral inflow and LV outflow. To obtain TDI-MPI, time intervals were measured from mitral annulus. Results: The mean values of both PWD-MPI and TDI-MPI of the patients were significantly different from those of the control subjects. Using receiver operating characteristics curve analysis, TDI-MPI yielded a better predictive discrimination for separating patients with versus those without myocardial dysfunction than PWD-MPI. Using a PWD-MPI >0.36 as the cutoff value, myocardial dysfunction was found with a sensitivity of 64.7 and specificity of 97. The sensitivity and specificity of TDI-MPI >0.34 in identification of LV dysfunction were 91 and 82, respectively. TDI-MPI was correlated with that measured by PWD (P < 0.004, r = 0.57). Conclusions: Subtle abnormalities of LV function develop early when renal insufficiency is mild to moderate. MPI, measuring by PWD and TDI, are appropriate indicators of overall LV function in young patients with CKD. (Echocardiography 2011;28:97-103) 2010, Wiley Periodicals, Inc

The frequency of congenital long QT syndrome based on new formula in children with sensori-neural hearing loss

Introduction: Long QT syndrome (LQTS) is a repolarization cardiac disorder that can lead to syncope, cardiac arrest and sudden death in apparently healthy individuals. The congenital type can be accompanied with congenital sensory-neural deafness (Jervell-Lang-Nielsen syndrome). Although there are limited studies assessed the frequency of LQTS in these children in developed countries, regarding introducing the new formula, it is necessary to re-evaluate the frequency of this syndrome. Materials and Methods: This cross-sectional and descriptive study was done on 203 patients with congenital sensory-neural hearing loss (SNHL) that had cochlear implant surgery in Baqiyatallah cochlear implant center from 2008 to 2012. Corrected QT was calculated with this formula: QTC = QT + 1.75 (heart rate-60) Patients with QTC > 460 ms, were categorized in four groups: Long QT: QT > 460, Borderline: 440 470 and very markedly: >500. Also, cardiac arrhythmias or arrest were evaluated in patients during cochlear implant surgery and in the postoperative recovery period. Result: Prevalence of LQTS in patients was 12.32 (25 patients). Prevalence of markedly long QT and very markedly long QT were 8.87 (18 patients) and 2.46 (5 patients) respectively. The prevalence of borderline group was 14.29 (29 patients). None of the patients during or after surgery were affected by cardiac arrhythmias or arrest. Conclusion: This study showed higher prevalence of LQTS in patients with SNHL than the normal population, and we suggest that all patients with congenital deafness should be screen for LQTS