Iranian Journal of Pediatrics
Volume:22 Issue: 2, 2012

Pathophysiology of pulmonary arterial hypertension is based on three basic mechanisms: thrombotic pulmonary vascular lesions, vasoconstriction and vascular remodeling. Platelets are related to all of these mechanisms by their aggregation, production, storage and release of several mediators. The role of platelets is more prominent in some types of pulmonary arterial hypertension, including those which are secondary to inflammatory and infectious diseases, hemoglobinopathies, essential thrombocythemia, drugs, thromboembolism, and cardiac surgery. Most pulmonary antihypertensive drugs have a negative effect on platelets. In this review, the mechanisms of platelets association with pulmonary arterial hypertension, those types of pulmonary arterial hypertension with greatest platelet contribution to their pathophysiology, and the effects of pulmonary antihypertensive drugs on platelets are summarized.

Comparing the effect of topical human milk application and dry cord care on cord separation time. This research was a randomized clinical trial study on 130 singleton and mature newborns. Newborns were placed randomly in groups of topical application of human milk and dry cord care. The umbilical separation time was compared in the two groups. Data was analyzed by SPSS software. Independent Samples t-Test, χ2, Fisher were used in this study. Median time of cord separation in human milk application group (150.95±28.68 hours) was significantly shorter than dry cord care group (180.93±37.42 hours) (P<0.001). Topical application of human milk on the remaining part of the cord reduces the cord separation time and it can be used as an easy, cheap and non invasive way for cord care.

Comparing the effect of topical human milk application and dry cord care on cord separation time. This research was a randomized clinical trial study on 130 singleton and mature newborns. Newborns were placed randomly in groups of topical application of human milk and dry cord care. The umbilical separation time was compared in the two groups. Data was analyzed by SPSS software. Independent Samples t-Test, χ2, Fisher were used in this study. Median time of cord separation in human milk application group (150.95±28.68 hours) was significantly shorter than dry cord care group (180.93±37.42 hours) (P<0.001). Topical application of human milk on the remaining part of the cord reduces the cord separation time and it can be used as an easy, cheap and non invasive way for cord care.

Improved survival of preterm infants, beneficial effects of trophic feeding and limited data on timing management of enteral feeding for very low birth weight preterm infants requires more researches to determine the exact starting time and increased volumes. This study aims to compare early (<48 h) versus late (>72h) trophic feeding with respect to important neonatal outcomes. In a cohort study from September 2007 to October 2008, a total of 170 preterm infants (1000-1500 gram, 26-31 weeks) consisting of 125 who received trophic feeding enterally within the first 48 hours of birth (early group) and 45 fed enterally after 72 h0urs (late group), without major congenital birth defects and severe asphyxia entered the study. Bolus feeding was started in both groups at 1-2 cc/kg every 4-6 hours of human milk or preterm infant formula and was advanced 1-2 cc/kg/day if tolerated along with parenteral nutrition. Feeding intolerance, possibility of necrotizing entrocolitis (NEC), episodes of sepsis, body weight, length of NICU stay, and duration of parenteral nutrition were assessed serially. There were no statistically significant differences in the clinical and maternal characteristics of infants in the two groups. The time to gain birth weight (13.75±5.21 vs 20.53±6.31 (P<0.001)), duration of parenteral nutrition (9.26±4.572 days vs 14.11±6.415 days (P<0.001)), hospital stay (12.14±8.612 vs 21.11±1.156 (P<0.001)) were significantly shorter in early compared to late feeding group; none of the two groups experienced a high incidence of late onset sepsis (P=0.73). There was 1 case of confirmed NEC in every group. The benefits of early trophic feeding shown by this study strongly support its use for the preterm infants without adding to complications.

Parenting confidence with regards to caring for their infants is crucial for the healthy adaptation to parenthood and the development of positive parent-infant relationships. The postpartum period is a tremendous transitional time for parents, so their unique needs should be considered. This study explored parenting confidence and needs in parents when their newborns are discharged from hospital, and explored the best predictors of parenting confidence and needs. A cross-sectional design with a questionnaire survey was used in this study. The questionnaire included three parts: Demographic, Parenting Needs and Parenting Confidence Questionnaire. We survey a convenience sample of 96 parents from a postnatal ward and a neonatal intermediate care unit of the medical central hospital in Taichung, Taiwan. The mean age of the subjects was 32 years and 67.7% of the subjects’ education level was college or above. Approximately one half of the subjects was multiparous, vaginal delivery and had planned pregnancy. The mean gestational age and birth weight of the newborns was 37.7 weeks and 2902 g, respectively. Parents who had a planned pregnancy (t=2.1, P=0.04) or preterm infants (t=2.0, P=0.046) and those whose infants were delivered by cesarean section (t=2.2, P=0.03) had higher parenting needs. In addition, parents of low birth weight infants had higher parenting needs (r=-0.23, P=0.02). Regarding parenting confidence, multipara parents perceived higher confidence than primipara parents (t=2.9, P=0.005). Needs in psychosocial support were significantly correlated with parenting confidence (r=0.21, P<0.05). The stepwise multiple regression analysis showed that parity and needs in psychosocial support predict parenting confidence of 13.8% variance. The findings of this study help care providers to identify parents with low parenting confidence at an early postpartum stage. Health care teams should provide appropriate psychosocial support and health education based on parents needs.

The objective of this study was assessment of hospital costs of 211 preterm babies admitted to NICU in a 12-month period. Preterm babies with gestational age 28-37 GW hospitalized in Dr. L. Kırdar Kartal Research and Training Hospital NICU between November 1st, 2006 to October 31st, 2007 were included in this retrospective study. The financial records of the babies were plotted and investigational, interventional, consumable costs, drugs and ancillary costs were determined. The average daily cost of a preterm has been determined. Group I and II consisted of babies with gestational ages 37-33 GW and 32-28 GWs respectively. The length of stay, ventilation duration and costs of each group were compared. The mean birth weight was 1689±497 gr. The mean length of hospital stay was 13.6±13.4 days. Hundred and four (49,5%) patients were found to be ventilated. The median ventilation day was 3 days. We found a statistically significant relation between length of hospital stay, ventilation duration, presence of intervention, RDS, sepsis and hospital costs. The mean total hospitalization cost and the daily cost of a preterm was determined as $4187 and $303 respectively. The highest intensive care costs of preterm neonates were found to be paid for interventional procedures, followed by NICU personnel salary and ancillary costs. Between two groups statistically significant difference was found for length of stay, duration of ventilation, interventional and consumable costs (P=0.014, P=0.019, P=0,001, P=0.03 respectively). Strategies for prevention of prematurity and early weaning from mechanical ventilation may shorten length of hospital stay leading to decreased NICU costs.

The current study was conducted to compare the opinions of mothers and nurses on the importance and availability of nursing support for parents with premature infants hospitalized in NICU. There is no overall picture of the nursing support for parents with hospitalized premature infants in Iran. Nurses, providing care in NICU, must view parent as an essential partner in care. But what is the situation? Assessing the viewpoints of both parents and nurses regarding the importance and availability of nursing support can elucidate this situation. A comparative descriptive design was used. The population consisted of all mothers with hospitalized premature newborns in NICU (n=300) and all NICU nurses (n=32) in three teaching hospitals in Tabriz (Iran), in 2007. Data was collected through the NPST questionnaire. Data analysis was done with SPSS. Mann-Whitney test showed that there were significant differences between nurses and mothers on four subscales of nursing support. Generally, mothers scored every subscale lower than nurses who cared for them and their children. On the other hand, it could be said that NICU nurses claimed more importance for the subscales and rated the provided support higher in comparison with mothers who did not think so. The opinions of the nurses and mothers toward the availability and importance of the nursing supports in NICU were different. This study provides nurses with concrete information in order to better understand parents’ need for support and try to meet their expectations, resulting in improved nursing care in neonatal intensive care units.

The relationships between body fat distribution, lipid profile and blood pressure, have not been studied extensively in young population. This study was designed to evaluate the association between measures of adiposity and established cardiovascular risk factors in adolescent girls. A total of 477 adolescent girls aged 15 to 18 years were recruited from Mashhad high schools. Socio-demographic characteristics were assessed using a self-administered questionnaire. Anthropometric assessments, blood pressure measurement and biochemical assessment were performed. Total and regional fat mass were determined by bio-impedance analysis. Cardiovascular disease risk factors were assessed in relation to body fat measures with adjustment for confounder factors including age and family socioeconomic status. The prevalence of overweight and obesity was 14.6% and 3.4% respectively; 16% of study population had greater fat mass compared to its ideal distribution. The majority of cardiovascular risk factors, especially systolic and diastolic blood pressure, triglyceride concentration, CRP and fasting blood sugar were significantly higher in group with a high body fat when compared to those with normal and low values. All anthropometric indices showed significant correlation with fat mass, fat free mass, total and regional body fat percent (P<0.001). After adjustment for age and family socioeconomic status, a high fat mass especially, truncal fat, was positively associated with triglyceride and blood pressure. Adiposity, especially truncal adiposity, which can be assessed by simple measures such as Body Mass Index (BMI) and Waist Circumference (WC) may predispose adolescent girls for demonstration of metabolic abnormalities and consequently cardiovascular diseases

Enuresis is a health problem frequently encountered in childhood. This study was carried out in two socio-demographically different districts of the province of Istanbul, for the purpose of determining the relationship between the prevalence of primary nocturnal enuresis and certain demographic characteristics. The study design is a cross-sectional carried out on 420 children (5 to 13 yr old) through random sampling. The research was conducted at two health centers in two different districts in the province of Istanbul. Data was collected with a questionnaire created by the researchers. Diagnosis of enuresis considered nocturnal voiding twice a week for at least three consecutive months. Enuresis was a complaint expressed by 16.2% of the cases in the study group; 8.3% reported intermittent bedwetting. The data collected in the two districts pointed to a significant difference in terms of the frequency of enuresis in favor of the district where socio-demographic features were inferior (P<0.005). When family histories were explored in cases of children with enuresis, it was found that the mothers of 76.2% had the problem of enuresis while 14.9% had enuretic fathers. Thus statistically, the presence of enuresis in the family history was seen to have had a markedly significant impact on the occurrence of enuresis in the child (P<0.001). It was concluded that familial predisposition to the condition constituted a more significant risk factor for enuresis compared to socio-demographic or economic characteristics.

To explore CT findings in pediatric novel influenza A (H1N1)-associated pneumonia We examined the CT findings in a series of six children with influenza H1N1-associated pneumonia. In this series of cases, the predominant CT patterns were consolidations surrounded by ground glass opacities (GGOs) as well as isolated GGOs in all patients. Atelectasis was present in the right upper lobe (n=2) in three cases and pneumomediastinum in two. In this series of cases, there may be no imaging differences between pediatric and reported adult influenza H1N1 cases and other viral pneumonia cases even with CT. Pneumomediastinum and atelectasis, especially in the right upper lobe, may frequently present in influenza H1N1-associated pneumonia as well as in other pediatric respiratory diseases.

Human milk (HM) is the ideal food for all newborns and infants. Apart from various bioactive compounds, including cytokines, antibodies, hormones, vitamines, it also contains polyamines, such as spermine (Sp), spermidine (Spd) and putrescine (Put). The present study investigated polyamine metabolism in colostrum and mature human milk by measuring the polyamine oxidase (PAO) and diamine oxidase (DAO) enzyme activities, which are necessary for polyamine catabolism, as well as by determining the malondialdehyde (MDA) levels, the final product of polyamine biodegradation. The PAO, DAO activity and MDA levels were quantified in colostrum (1st and 2nd day) as well as in mature human milk, 30th day of lactation. We found the steady increase of PAO activity and steady decrease of DAO activity and MDA levels during first month of lactation. Since the products of PAO activity such as, amino aldehydes and hydrogen peroxide (H2O2) might have potential antimicrobial effects, promoting the oxidative stress, it is likely that human milk PAO throughout the lactation period, contributes to the protective effects of human milk.

This study aims at providing local reference values for blood pressure by height and determining distribution pattern of systolic and diastolic blood pressure in 6.5-11.5 elementary school children for the first time in Shiraz (Southern Iran). Height, systolic blood pressure (SBP) and diastolic blood pressure (DBP) were measured with standard methods in 2270 healthy school children (1174 boys, 1096 girls) who were selected by multi-stage random sampling in 2003-2004 academic years. We produced blood pressure percentiles by height percentiles using Healy-Rasbash-Yang method. The blood pressure (both systolic and diastolic) tends to increase with age, but after adjusting the measurements for height, no significant correlation was found between either systolic blood pressure or diastolic blood pressure and age (r=-0.03 and P=0.15 for systolic blood pressure, r=-0.005 and P=0.8 for diastolic blood pressure). Then systolic and diastolic blood pressure percentile values by age and height percentiles, and blood pressure smoothed centiles by height in 6.5-11.5 years school children were derived. Due to genetic, cultural and environmental differences among populations, it is suggested to use local blood pressure standards in Iran. We produced blood pressure percentiles by height instead of age because it seems that it would lead to better evaluation for real hypertensive diagnosis.

Hepatic dysfunction is common in dengue infection and the degree of liver dysfunction in children varies from mild injury with elevation of transaminases to severe injury with jaundice. This study was undertaken to asses the spectrum of hepatic involvement in dengue infection. 110 children with serologically positive dengue fever aged between 2 months - 14 years were studied for their hepatic functions both clinically and biochemically after excluding malaria, enteric fever, Hepatitis A and Hepatitis B with relevant investigations. All cases were grouped into DF (Dengue fever), DHF (Dengue hemorrhagic fever) and DSS (Dengue shock syndrome) according to WHO criteria. The spectrum of hepatic manifestations included hepatomegaly (79%), hepatic tenderness (56%), jaundice (4.5%), raised levels of aspartate transaminase (AST)(93 %), alanine transaminase (ALT)(78%), alkaline phosphatase (AP) (57%), prolonged prothrombin time (PT) (20%), reduced levels of serum albumin (66%) and abnormal abdomen ultrasound (65%). Hepatic dysfunction was observed more in DHF and DSS group compared to DF group. About 17.27% of children had >10 fold increase in the liver enzymes. There was no correlation between the degree of hepatic enlargement or hepatic tenderness with the abnormalities of liver functions. Any child with fever, jaundice and tender hepatomegaly in geographical areas where dengue is endemic, the diagnosis of dengue infection should be strongly considered

This study was performed to determine the relationship between urinary nitrite results and bacterial resistance to antimicrobial drugs in urinary tract infection of children. In a cross-section study 119 children younger than 12 years with urinary tract infection were evaluated in Qazvin children's hospital. Patients were divided into negative and positive nitrite groups depending on urinary nitrite test result. Rates of antibiotic resistance in the two groups were compared. Sixty seven patients were in the negative nitrite group and 52 in the positive nitrite group. Resistance rates to ceftriaxone, trimethoprim sulfamethoxazole, ampicillin, gentamicin, amikacin, nalidixic acid, cephalothin and nitrofurantoin in the nitrite negative group were 7.5%, 31.3%, 50.7%, 11.9%, 9%, 3%, 14.9% and 11.9%, respectively. These values in the nitrite positive group were 21.2%, 28.8%, 63.5%, 7.7%, 5.8%, 1.9%, 9.6%, and 3.8%, respectively (P>0.05). This study showed that there is no correlation between urinary nitrite results and bacterial resistance to antimicrobial drugs. Therefore, it seems that physicians should not adjust antibiotic therapy for UTI based on nitrite results.

An appropriate treatment of acute viral bronchiolitis can reduce the symptoms,hospitalization duration and exorbitant costs which is imposed on the families and insurance organizations. This study was conducted to determine the efficacy of epinephrine in comparison with salbutamol in the treatment of the disease. Forty infants aged one month to 2 years with acute bronchiolitis in Amin and Al-Zahra hospitals, during 2008, were enrolled in this study. The participants were randomized in two treatment groups to receive epinephrine 0.1 ml/kg or salbutamol 0.15 mg kg. Three doses of each medication were prescribed at intervals of 20 minutes and continued every 10 minutes after the third dose. The patients in both groups were monitored and rated by RDAI, number of the hospitalized days in the hospital, level of oxygen saturation and vital signs. Mean hospitalization duration was 3.3±1.1 and 3±0.9 in the patients receiving salbutamol and epinephrine, respectively (P=0.03). There was a significant difference in assessing RDAI index between the two groups (P=0.03). There were no differences in SPO2, PR, or RR variables in the studied intervals in both groups (P>0.05). Regarding the effect of epinephrine on reduction of hospitalization duration and the RDAI index in patients with acute bronchiolitis, it seems that using epinephrine instead of salbutamol could be more effective in the management of the disease.

The aim of this study was to investigate the prevalence of molar incisor hypomineralization (MIH) and its relationship with systemic conditions in a group of Iranian children. The study population comprised of 433 7-9 year olds, from four schools in Zahedan, Iran. Subjects were evaluated clinically by one examiner, and at a separate session, their mothers completed a coded medical history questionnaire. Hypo-mineralized molars and incisors were recorded based on DDE (developmental defects of enamel) index and DMFT (number of decayed, filled and missing teeth) was determined. Statistical analyses were performed using Chi-square and independent sample t-tests. Fifty-five (12.7%) children showed MIH. The overall mean number of affected teeth was 0.2. The mean value of DMFT in MIH children was greater than in normal children. Demarcated opacities were the most frequent (76%) enamel defect. Mother’s and child’s medical problems during prenatal, perinatal and post natal period were significantly remarkable in MIH children. The prevalence of MIH in a group of Iranian children was 12.7%. Prenatal, perinatal and post natal medical conditions were more prevalent in children affected by MIH.

Mongolian spots are benign skin markings at birth which fade and disappear as the child grows. Often persistent extensive Mongolian spots are associated with inborn error of metabolism. We report thirteen people of the single family manifested with extensive Mongolian spots showing autosomal dominant inheritance. A one day old female child, product of second degree consanguineous marriage, born by normal vaginal delivery with history of meconium stained amniotic fluid and birth asphyxia. On examination the child showed extensive bluish discoloration of the body involving trunk and extremities in both anterior and posterior aspects associated with bluish discoloration of the tongue. A detailed family history revealed most of the family members manifested with extensive bluish discoloration of the body soon after birth which faded in the first few years of life and completely disappeared by puberty. Thus it was diagnosed to be extensive Mongolian spots with an autosomal dominant inheritance. Knowledge about the natural history of extensive Mongolian spots, their inheritance and association with certain metabolic diseases mainly IEM and Mucopolysaccharidosis aids in the diagnosis and in order to improve the patient’s prognosis.

Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity. A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient’s various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established. Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases.

First and second-degree heart blocks are partly common rhythm disorders in thalassemic patients but complete heart block is a very rare complication of iron overload cardiomyopathy. This 15-year-old boy, a known case of major β-thalassemia was admitted to our emergency unit with dyspnea and cough because of decompensated heart failure. The electrocardiogram showed complete heart block with junctional escape rhythm. Interestingly, his previous electrocardiogram taken 2 months earlier, had some PVC and second degree, Mobitz type 1 (Wenckebach) heart block. After improvement of dyspnea and control of blood pressure in normal range, the patient was referred to ER. A dual-chamber permanent pacemaker was implanted and his symptoms improved, but he died 24 days after discharge from hospital. We present a rare case of complete heart block after a second-degree (Mobitz 1) heart block that was due to severe iron overload cardiomyopathy.

The pandemic influenza A/H1N1, spread through the world in 2009, producing a serious epidemic in Italy. Complications are generally limited to patients at the extremes of age (<6 months or >65 years) and those with comorbid medical illness. The most frequent complications of influenza involve the respiratory system. A 3-year-old boy with a recent history of upper respiratory tract infection developed a nephrotic syndrome. Together with prednisone, furosemide and albumin bolus, a therapy with oseltamivir was started since the nasopharyngeal swab resulted positive for influenza A/H1N1. Clinical conditions and laboratory findings progressively improved during hospitalization, becoming normal during a 2 month follow up. The possibility of a renal involvement after influenza A/H1N1 infection should be considered.

Triple X syndrome is a sex chromosomal aneuploidy condition characterized by taL stature, microcephaly, hypertelorism, congenital abnormalities, and motor and language delays. It is mainly derived from maternal nondisjunctional errors during meiosis. To highlight the clinical features and diagnosis of triple X syndrome, we present a rare phenotype of the syndrome. A 5.9 year-old girl was admitted to our hospital because of short stature. Both her height and weight were below the 3rd percentile compared to the normal peers. She was found with mild motor and speech delay. Laboratory investigation showed low level of IGF-1 and zinc, elevated estradiol level and normal result of arginine provocation test. Our data suggest that triple X syndrome should also be suspected in patients with short stature, elevated estradiol and low level of IGF-1, even with normal result of arginine provocation test.