Effect of Vitamin D Supplementation on Moderate to Severe Bronchial Asthma

Objective

To define the therapeutic role of vitamin D in children with moderate to severe bronchial asthma as an adjunct to standard treatment.

Methods

Hundred asthmatic children of either sex, attending the respiratory and asthma clinic were enroled in the study. Diagnosis was made on the basis of history and clinical examination. Randomization was done using sealed opaque envelop method. In addition to the treatment as per GINA guidelines, one group received oral vitamin D3 (Cholecalciferol) 60,000 IU per month for 6 mo and the other group received placebo powder in the form of glucose sachet with a double blinded design. Monthly follow up of every patient was done and during every visit change in severity, level of control, Peak expiratory flow rate (PEFR), steroid dosage, number of exacerbations and number of emergency visits were assessed.

Results

Monthly doses of 60,000 IU vitamin D significantly reduced the number of exacerbations as compared to placebo (p?=?0.011). PEFR significantly increased in the treatment group (p?=?0.000). Monthly doses of vitamin D significantly reduced the requirement of steroids (p?=?0.013) and emergency visits (p?=?0.015). Control of asthma was achieved earlier in patients who received monthly vitamin D. Vitamin D significantly reduced the level of severity of asthma patients over 6 mo of treatment (p?=?0.016).

Conclusions

Vitamin D has a definite role in the management of moderate to severe persistent bronchial asthma as an adjunct to standard treatment.     

Long-term quality of life of patients treated in paediatric intensive care unit

The changes in long-term quality of life (QOL) of children treated in paediatric intensive care unit (PICU) were investigated in relation to their QOL before critical illness together with the influence of underlying chronic health condition and severity of illness estimated by Paediatric Index of Mortality 2 on the long-term outcome. This study included 189 children treated in PICU and 179 children from outpatient clinics as controls. QOL was evaluated according to the Royal Alexandra Hospital for Children Measure of Function (RAHC MOF). The long-term QOL in 70 % of children treated in PICU was good, although there was a significant diminution of QOL in children treated in PICU in comparison with their preadmission scores and with the children from outpatient clinics who served as controls (p?<?0.001). Severity of illness had a significant impact on children’s QOL (p?=?0.016) 6 months after treatment in PICU. Twenty-four months after discharge, the RAHC MOF score was still decreased in 19 % of children treated in PICU, and in significantly more patients with a chronic health condition (CHC) treated in PICU, than in their peers from outpatient clinics (p?=?0.029). Reduced QOL was significantly more frequent in children with neurodevelopmental disability than in children without CHC 24 months after discharge from PICU (p?=?0.013). Conclusion: Acute illness has a significant impact both on children with and without CHC after treatment in PICU 6 months after discharge. Twenty-four months after discharge, comorbidity was identified as the decisive factor for diminished QOL in children after PICU treatment.     

An assessment of iron overload in children treated for cancer and nonmalignant hematologic disorders

Our goal was to assess the natural fate of iron overload (IO) following transfusions of packed red blood cells (PRBCs) in children treated for cancer and nonmalignant disorders according to the intensity level of their treatment. Sixty-six children were followed up from February 2010 to March 2013. The transfusion burden was compared between three treatment intensity groups assigned according to the Intensity of Treatment Rating Scale 3.0 (ITR-3). IO was assessed by serial measurements of serum ferritin (SF) (n?=?66) and quantification of tissue iron by magnetic resonance imaging (MRI) (n?=?12). Of the children studied, 36 % (24/66) received moderately intensive treatment (level 2), 21 % (14/66) received very intensive treatment (level 3), and 42 % (28/66) received the most intensive treatment (level 4). The number of PRBC (p?=?0.016), the total transfused volume (p?=?0.026), and transfused volume adjusted to body weight (p?=?0.004) were significantly higher in the level 4 group. By the median follow-up time of 35.5 months (range 8–133), 21–29 % of patients (including level 2 and level 3 children) had SF >1,000 μg/l 1 year after cessation of transfusions. The slowest decrease of SF was observed in the level 4 group. Initial MRI examination demonstrated either mild or moderate IO in the liver and spleen. Repetitive MRI showed significant improvement in relaxation time between the initial and follow-up MRI performances in the liver (5.9 vs. 8.6 ms, p?=?0.03) and the spleen (4.3 vs. 8.8 ms, p?=?0.03). Conclusion: IO diminished over time, but in the level 4 patients, it was detectable for years after cessation of transfusions.     

Clinical outcome of parapneumonic empyema in children treated according to a standardized medical treatment

Treatment of parapneumonic empyema (PE) consists of intravenous antibiotics and, in case of large effusions and persisting fever, pleural chest drain (±intrapleural fibrinolytics) or video-assisted surgical intervention. We standardized the treatment for PE in our tertiary care center choosing a first-step nonsurgical approach. The aim was to evaluate the need for surgery and to collect data on disease course, outcome, and microbiology. For all children treated for PE between 2006 and 2013, data were prospectively collected concerning treatment, length of stay, duration of fever, complications, and causative agent. Of 132 children treated for PE, 20 % needed surgical intervention. Analyzed per year, the need for surgery decreased from almost 40 % in 2007 to 0 % in 2010 again increasing to 40 % although this did not reach statistical significance (p?=?0.115). Median duration of “in-hospital fever” was 5 days (IQR, 3–8). The duration of fever correlated with pleural LDH (r?=?0.324; p?=?0.002) and pleural glucose (r?=??0.248; p?=?0.021) and was inversely correlated with pleural pH (r?=??0.249; p?=?0.046). Based on pleural PCR data, 85 % of PE were caused by Streptococcus pneumoniae (40 % serotype 1). Conclusion: After introduction of a standardized primary medical approach (chest drain?±?fibrinolysis) for PE in our institution, the need for surgical rescue interventions overall remained at 20 %, which is higher than in some other reports. Difference in microbiology or disease severity could not be proven.     

Effect of Multisensory Stimulation on Neuromotor Development in Preterm Infants

Objective

To investigate the effect of Auditory, Tactile, Visual and Vestibular stimulus (ATVV) on neuromotor development in preterm infants.

Methods

Fifty preterm infants born at 28–36 wk with a birth weight ranging from 1,000–2,000 g were recruited for the study. They were block randomized into a control group (n?=?25) and study group (n?=?25). New Ballard score was used for the baseline measurement of neuromaturity in both groups. In neonatal intensive care unit (NICU), the study group received multisensory stimulation for 12 min per session, 5 sessions per wk along with routine NICU care either from 33 wk corrected gestational age for infants born at 28–32 wk or from 48 h of birth for infants born at 33–36 wk until discharge from the hospital. The control group received the routine NICU care. At term age the preterm infants were assessed using Infant Neurological International Battery (INFANIB) and the groups were compared using independent t test.

Results

The multisensory stimulated infants showed higher neuromotor score (p?=?0.001) compared to the control group. The french angle components of INFANIB including heel to ear (p?=?0.016) and popliteal angle (p?=?0.001) were statistically significant between the groups.

Conclusions

Multisensory stimulation appears to have a beneficial effect on the tonal maturation in preterm infants. However, further studies are warranted to investigate the long-term effects of multisensory stimulation on neurodevelopmental outcome in preterm infants.     

Primary ciliary dyskinesia presentation in 60 children according to ciliary ultrastructure

Primary ciliary dyskinesia (PCD) is an inherited disease related to ciliary dysfunction, with heterogeneity in clinical presentation and in ciliary ultrastructural defect. Our study intended to determine if there are phenotypic differences in patients with PCD based on ciliary ultrastructural abnormality. In this retrospective study carried out among 60 children with a definitive diagnosis of PCD, we analyzed clinical, radiological, and functional features at diagnosis and at last recorded visit, according to cilia defect (absence of dynein arms: DAD group, n?=?36; abnormalities of the central complex: CCA group, n?=?24). Onset of respiratory symptoms occurred later in the CCA than in the DAD group (9.5 versus 0.5 months, p?=?0.03). Situs inversus was only observed in the DAD group, while respiratory disease in siblings were more frequent in the CCA group (p?=?0.003). At diagnosis, clinical presentation was more severe in the CCA group: frequency of respiratory tract infections (p?=?0.008), rhinosinusitis (p?=?0.02), otitis complications (p?=?0.0001), bilateral bronchiectasis (p?=?0.04), and number of hypoxemic patients (p?=?0.03). Pulmonary function remained stable in both groups, but outcome was better in the CCA than in the DAD group: less antibiotic therapy and hypoxemic patients (p?=?0.004). In conclusion, our results underlined the relationship between the severity of clinical presentation and the ultrastructural ciliary defect.     

Clinical efficacy of gabexate mesilate for acute pancreatitis in children

Children with acute pancreatitis have been treated by fasting and parenteral nutritional support, and to date, the efficacy of drugs for acute pancreatitis in children is unclear. Gabexate mesilate is a synthetic serine protease inhibitor used to prevent or treat acute pancreatitis in adult patients. The purpose of this study was to evaluate the clinical efficacy of gabexate for acute pancreatitis in children. Fifty-three children hospitalized with acute pancreatitis between 2004 and 2012 were divided between a gabexate-treated group (n?=?26) and a control group without gabexate infusion (n?=?27). The severity of acute pancreatitis was graded according to Balthazar scoring of computed tomography images. All subjects had a Balthazar score of <4 without pancreatic necrosis or organ failure. The median age of the patients was 11.8 years (range, 18 months–17 years). The durations of hospitalization, abdominal pain, and parenteral nutrition in the gabexate-treated group were significantly shorter than in control subjects (P?=?0.032, P?=?0.000, and P?=?0.016, respectively). Serum levels of amylase and lipase were significantly lower in gabexate-infused children than in control subjects on day 7 (median amylase, 81 vs. 137 IU/L, P?=?0.001; median lipase, 273 vs. 523 IU/L, P?=?0.031). Conclusion: The present study showed that gabexate infusion has some clinical benefits for acute pancreatitis in children. The clinical application of gabexate for managing acute pancreatitis in children may be appropriate beyond conventional therapy.     

Bronchopulmonary dysplasia and neurodevelopmental outcome in extremely preterm neonates

We tested the hypothesis that the use of supplemental oxygen (sO₂) at discharge from the NICU in extremely preterm neonates is associated with a greater risk of neurodevelopmental impairment (NDI) at 18 months corrected gestational age (CGA) than the risk of NDI of those neonates discharged in room air. Four hundred twenty-four charts were retrospectively reviewed from infants born at <27 weeks and transferred to Nationwide Children’s Hospital from December 1, 2004 to June 14, 2010. Use of sO₂ was evaluated on day of life (dol) 28, at 36 weeks post-menstrual age (PMA), and at discharge. Logistic regression was used to identify postnatal risk factors associated with sO₂ at discharge and NDI. At dol 28, 96 % of surviving patients received sO₂, and therefore had bronchopulmonary dysplasia (BPD) by definition from a National Institutes of Child Health and Human Development workshop. At 36 weeks PMA, 89 % continued on sO₂ (moderate/severe BPD), and at discharge, 74 % continued on sO₂. When factors associated with NDI were examined, the need for mechanical ventilation ≥28 days (adjOR?=?3.21, p?=?0.01), grade III–IV intraventricular hemorrhage (IVH) (adjOR?=?4.61, p?<?0.01), and discharge at >43 weeks PMA (adjOR?=?2.12, p?=?0.04) were the strongest predictors of NDI at 18 months CGA. There was no difference in Bayley Scales of Infant Development, third edition composite scores between patients with no/mild BPD and patients with moderate/severe BPD (cognitive p?=?0.60, communication p?=?0.53, motor p?=?0.19) or those scores between patients on and off oxygen at discharge (cognitive p?=?0.58, communication p?=?0.70, motor p?=?0.62). Conclusions: The need for sO₂ at discharge is not associated with an increased risk of NDI in these patients. The strongest predictors of poor neurodevelopmental outcome in this population were prolonged positive pressure support, grade III–IV IVH, and discharge at >43 weeks PMA.     

Iron Stores in Low and Normal Birth Weight Infants at Birth and in Early Infancy

Serum ferritin levels of low birth weight (LBW; BW?<?2,500 g) and normal birth weight (NBW; BW?≥?2,500 g) infants were evaluated at birth and at 3 mo using electrochemiluminescence immunoassay. At birth, levels were 318.6 (31.0–829.5) ng/mL in LBW (n?=?217) and 366.2 (122.4–858.5) ng/mL in NBW infants (n?=?116; p?<?0.01), with 1.4 % of LBW and none of the NBW infants having levels <12 ng/mL (p?=?0.20). At follow up, levels were 66.9 (4.5–567.7) ng/mL in LBW (n?=?126) and 126.2 (6.8–553.7) ng/mL in NBW infants (n?=?76; p?=?0.27), with 11.9 % of LBW and 11.8 % of NBW infants having levels <12 ng/mL (p?=?0.80).     

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目的观察槐杞黄颗粒辅助治疗原发性肾病综合征(PNS)患儿对淋巴细胞亚群、免疫球蛋白及感染次数的影响。方法 2008-01-01—2009-10-31中国医科大学附属盛京医院小儿肾脏风湿免疫科将住院的101例PNS患儿随机分为A组(62例)和B组(39例),选取正常体检儿童22名为正常对照组。A组采用糖皮质激素联合槐杞黄颗粒治疗,B组单独服用激素。分别于治疗前和治疗3个月、6个月检测患儿的淋巴细胞亚群、免疫球蛋白及记录感染次数、肾病复发次数和药物副反应。结果 (1)A、B组治疗前,CD8+高于正常对照组(P<0.05),CD4+、CD4+/CD8+和NK细胞均明显降低于正常对照组(P<0.05),IgA与IgG均低于正常对照组(P<0.05)。(2)治疗3个月,A组(39例)与B组(30例)之间CD8+细胞差异无统计学意义,两组均明显高于正常对照组,差异有统计学意义(P<0.01);B组CD4+及CD4+/CD8+显著低于A组和正常对照组(P<0.05);A组与B组NK细胞均高于治疗前,B组低于同期A组(P<0.05);IgG明显升高但组间差异无统计学意义。(3)治疗6个月,A组(23例)、B组(9例)间各指标均差异无统计学意义,均低于正常对照组(P<0.01)。(4)感染次数,A组发生上呼吸道感染6例次,肺炎2例次,肾病综合征复发4例次。B组发生上呼吸道感染10例次,肺炎3例次,泌尿系感染2例次,肾病综合征复发6例次。1例患儿服槐杞黄颗粒后出现较严重腹泻(排除感染性腹泻)。结论 PNS患儿治疗前细胞、体液免疫功能降低、紊乱;槐杞黄颗粒辅助治疗过程中,可能通过提高NK细胞和T辅助淋巴细胞活性,减少感染并有减少肾病综合征复发的趋势,且副反应轻微。     

Chloral Hydrate,Chloral Hydrate - Promethazine and Chloral Hydrate -Hydroxyzine Efficacy in Electroencephalography Sedation

Objective

To compare efficacy and safety of chloral hydrate (CH), chloral hydrate and promethazine (CH + P) and chloral hydrate and hydroxyzine (CH + H) in electroencephalography (EEG) sedation.

Methods

In a parallel single-blinded randomized clinical trial, ninety 1–7 y-old uncooperative kids who were referred to Pediatric Neurology Clinic of Shahid Sadoughi University, Yazd, Iran from April through August 2012, were randomly assigned to receive 40 mg/kg of chloral hydrate or 40 mg/kg of chloral hydrate and 1 mg/kg of promethazine or 40 mg/kg of chloral hydrate and 2 mg/kg of hydroxyzine. The primary endpoint was efficacy in sufficient sedation (obtaining four Ramsay sedation score) and successful completion of EEG. Secondary endpoint was clinical adverse events.

Results

Thirty nine girls (43.3 %) and 51 boys (56.7 %) with mean age of 3.34?±?1.47 y were assessed. Sufficient sedation and completion of EEG were achieved in 70 % (N?=?21) of chloral hydrate group, in 83.3 % (N?=?25) of CH + H group and in 96.7 % (N?=?29) of CH + P group (p?=?0.02). Mild clinical adverse events including vomiting [16.7 % (N?=?5) in CH, 6.7 % (N?=?2) in CH + P, 6.7 % (N?=?2) in CH + H], agitation in 3.3 % of CH + P (N?=?1) group and mild transient hypotension in 3.3 % of CH + H (N?=?1) group occurred. Safety of these three sedation regimens was not statistically significant different (p?=?0.14).

Conclusions

Combination of chloral hydrate—antihistamines can be used as the most effective and safe sedation regimen in drug induced sleep electroencephalography of kids.     

Evaluation by N-terminal Prohormone of Brain Natriuretic Peptide Concentrations and Ross Scoring of the Efficacy of Digoxin in the Treatment of Heart Failure Secondary to Congenital Heart Disease With Left-to-Right Shunts

This study aimed to evaluate the effectiveness of digoxin in children with heart failure secondary to left-to-right shunt lesions and normal left ventricular systolic function. The study registered 37 such patients (ages 10 days to 24 months, groups 1 and 2) and used 20 healthy children as a control group (group 3). Left ventricular systolic function, as assessed by conventional echocardiography, was normal in all the subjects. Congestive heart failure was diagnosed by clinical evaluation and modified Ross scoring. Plasma N-terminal prohormone of brain natriuretic peptide (NT-proBNP) concentrations and complete blood counts were assessed in all the children. Group 1 was treated with digoxin, enalapril, and furosemide and group 2 with enalapril and furosemide. Approximately 1 month after starting treatment, the patients were reevaluated by physical and echocardiographic examinations, modified Ross scoring, plasma NT-proBNP concentrations, and complete blood counts. The pre- and posttreatment Ross scores of group 1 (p = 0.377) and group 2 (p = 0.616) did not differ significantly. The NT-proBNP values in both groups decreased after treatment (p = 0.0001). The pre- and posttreatment NT-proBNP values did not differ significantly in group 1 (p = 0.094)) and group 2 (p = 0.372). The pretreatment NT-proBNP values in groups 1 and 2 (p = 0.0001) were significantly higher than in the control group (p = 0.003). A smaller difference was observed between posttreatment NT-proBNP values in group 1 and the control group (p = 0.045). We found no significant difference between the posttreatment NT-proBNP values of group 2 and those of the control group (p = 0.271). The study showed that both treatments currently used to treat heart failure secondary to congenital heart disease with left-to-right shunts and preserved left ventricular systolic function are effective and do not differ significantly. Thus, digoxin does not provide any extra benefit in the treatment of such patients.     

Low serum 25-hydroxyvitamin D levels and bronchiolitis severity in Spanish infants

This cross-sectional study was performed to examine the prevalence of hypovitaminosis D in infants with acute bronchiolitis compared with control subjects and to evaluate the relationship between serum 25-hydroxyvitamin D (25(OH) D) and the severity of bronchiolitis. Serum 25(OH) D levels were measured by radioimmunoassay in 48 infants with acute bronchiolitis (2.5?±?2.0 months) and in 30 healthy infants (3.2?±?2.3 months). 25(OH) D levels (ng/ml) in children with acute bronchiolitis were significantly lower than in the control group (median 29.9 ng/ml (interquartile range (IQR) 21.4–37.5) versus median 38.2 ng/ml ((IQR 26.1–48.1), p?=?0.022), mainly in infants with moderate–severe bronchiolitis (median 29.8 ng/ml, IQR 19.2–35.9). The prevalence of hypovitaminosis D was remarkably greater among infants with bronchiolitis than in control subjects (52.1 versus 26.6 %). A significant inverse correlation was found between serum 25-hydroxyvitamin D levels and disease severity (rho?=??0.457, p?<?0.001). Conclusion: The prevalence of hypovitaminosis D is high in Spanish infants with bronchiolitis. The severity of acute bronchiolitis increases with a decline in serum 25 (OH) D level.     

Post-transplant food allergy in children is associated with liver and not with renal transplantation: A monocentric comparative study

Food allergy is increasingly reported after paediatric liver transplantation. The underlying physiopathological mechanism remains incompletely understood. Therefore, we aimed to determine the incidence, clinical presentation, possible risk factors, and prognosis of post-transplant food allergy in children currently followed after liver and renal transplantation. The study population consists of 49 liver and 21 renal transplant patients transplanted between the age of 22 months and 15 years. Data were collected retrospectively from medical records and via a doctor’s questionnaire taken from the parents in a monocentric setting. Post-transplant food allergy has developed in 13 liver transplant patients and in none of the renal transplant recipients. Within the liver transplant group, median age at liver transplantation is significantly lower in the food-allergic (10 months) versus non-food-allergic group (3.3 years; p?=?0.002). The use of tacrolimus as primary maintenance immunosuppression is associated with food allergy (p?=?0.032) and mean donor age is significantly lower in the food-allergic group (p?=?0.009). Compared to the renal transplant group, median age at transplantation is significantly lower in the liver patients (p?<?0.001). No significant differences are found in primary immunosuppressive regimens between renal and liver transplant patients. Conclusion: Post-transplant food allergy is an important clinical problem in children after liver transplantation which does not affect renal transplant patients despite similar immunosuppressive regimens. Within the group of liver transplant recipients, tacrolimus use, young age at time of transplant and younger donor age were associated with the development of food allergy.     

Incomplete clinical manifestation as a risk factor for coronary artery abnormalities in Kawasaki disease: a meta-analysis

Incomplete Kawasaki disease (KD) comprises a large proportion of the total number of cases. Although it has the potential of delaying diagnosis, it is not conclusive whether an incomplete presentation is a risk factor for coronary artery abnormalities (CAAs). We performed a meta-analysis to establish the risk of CAA in 20 studies including 4,504 cases and 32,519 controls, and the risk of giant aneurysm in two studies including 5,390 cases and 37,648 controls. The pooled results indicated that incomplete KD was associated with an increased risk of CAA [odds ratio (OR)?=?1.447, 95 % confidence interval (CI)?=?1.158–1.808, p?=?0.001]. Subgroup analyses demonstrated higher associations in patients younger than 12 months (OR?=?2.023, 95 % CI?=?1.252–3.271, p?=?0.004), Asians and Indians (OR?=?1.57, 95 % CI?=?1.234–1.999, p?<?0.001 and OR?=?7.088, 95 % CI?=?1.640–30.631, p?=?0.009, respectively). Subgroup analysis according to the period of patient enrollment before and after 2004 showed increased association of incomplete KD with CAA only among studies with patients enrolled after 2004 (OR?=?1.969, 95 % CI?=?1.240–3.127, p?=?0.004). In conclusion, incomplete KD seems to be associated with an increased risk of CAA, and this is more prominent in patients younger than 12 months, Asians and Indians.     

Long-term body composition and metabolic changes in HIV-infected children switched from stavudine to tenofovir and from protease inhibitors to efavirenz

This is an 8-year cohort study of 24 HIV-infected patients aged 5–17 years to assess body composition and metabolic changes after switching from lamivudine + stavudine (d4T) + protease inhibitors (PI) to lamivudine + tenofovir (TDF) + efavirenz (EFV). Body composition (dual-energy X-ray absorptiometry) and cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, triglycerides, glucose and insulin were measured annually. Linear mixed models and generalized linear mixed models were used to evaluate time changes of the outcome of interest. Body mass index increased linearly by 0.3 kg/m²/year (p?<?0.001); waist circumference increased non-linearly from 68 to 74 cm (p?=?0.004 for the linear term and p?=?0.04 for the quadratic term). Percent body fat, percent trunk fat and percent bone mineral content increased linearly by 0.6 %/year (p?=?0.005), 1.2 %/year (p?<?0.001) and 0.02 %/year (p?=?0.04), respectively. Percent arm fat remained stable (p?=?0.5), and percent leg fat decreased linearly by 1.2 %/year (p?<?0.001). The probability of low HDL was 0.2 % at baseline and remained stable during the study. The probability of high triglycerides was 3 % at baseline and increased linearly to become 11 % at the 8th year of follow-up (p?=?ns). The probability of high glucose was 1 % for the whole study duration. Conclusions: patients, after switching from d4T to TDF and from PI to EFV, show most of the changes in anthropometry and body composition associated with normal growth and no frankly pathological change in metabolic parameters.     

Relation between biomarkers and clinical severity in patients with Smith–Lemli–Opitz syndrome

Smith–Lemli–Opitz syndrome (SLOS), a multiple congenital anomaly with severe mental retardation, is caused by decreased activity of 7-dehydrocholesterol reductase. Fifteen Hungarian patients were diagnosed with SLOS on the basis of clinical symptoms, serum cholesterol, 7-dehydrocholesterol, and molecular genetic testing. Their age at the time of diagnosis in mild SLOS (n?=?4, clinical score <20) was 0.5–18 years, cholesterol was 2.37?±?0.8 mmol/L, and 7DHC was 0.38?±?0.14 mmol/L. In the group of typical SLOS (n?=?7, score 20–50), the diagnosis was set up earlier (age of 0.1–7 years); t-cholesterol was 1.47?±?0.7 mmol/L, and 7DHC was 0.53?±?0.20 mmol/L. Patients with severe SLOS (n?=?4, clinical score?>?50) died as newborns and had the lowest t-cholesterol (0.66?±?0.27 mmol/L), and 7DHC was 0.47?±?0.14 mmol/L. Correlation coefficient with clinical severity was 0.74 for initial t-cholesterol and 0.669 for Cho/7DHC. Statistically significant difference was between the initial t-cholesterol of mild and severe SLOS (p?=?0.01), and between the Cho/7DHC ratios of groups (p?=?0.004). In severe SLOS, the percentage of α-lipoprotein was significantly lower than in typical (p?=?0.003) and mild SLOS (p?=?0.004). Although serum albumin, total bilirubin, and hemostasis parameters remained in the reference range during cholesterol supplementation (n?=?10) combined with statin therapy (n?=?9), increase of aspartate aminotransferase and alanine aminotransferase in 50 % of the patients probably refers to a reversible alteration of liver function; therefore, statin therapy was suspended. Conclusion: life expectancy is fundamentally determined by the initial t-cholesterol, but dehydrocholesterol and α-lipoprotein have prognostic value. Accumulation of hepatotoxic DHC may inhibit the synthesis of α-lipoproteins, decreasing the reverse cholesterol transport. During statin therapy, we suggest monitoring of lipid parameters and liver function.     

Current Outcomes of Hypoplastic Left Heart Syndrome With Restrictive Atrial Septum: A Single-Center Experience

Advances in the management of hypoplastic left heart syndrome (HLHS) have resulted in improved survival. However, short and long-term mortality in patients with a restrictive atrial septum remains high. All neonates diagnosed with HLHS from 2003 to 2010 at our institution were evaluated. Patients who underwent atrial septostomy within the first 72 h conformed the restrictive atrial septum group (HLHS-RS). Patients with a non-restrictive communication (HLHS-NRS) formed the control group. Outcomes and survival status were determined from review of medical records. Of the 141 newborns diagnosed with HLHS, 20 (14 %) required intervention for a restrictive atrial septum. Procedural success was achieved in 17/20 (85 %) patients. Complications occurred in ten procedures, two of which were life threatening. No procedural deaths occurred. Overall median follow up was 35.5 months (0.4–104). Initial hospitalization survival was 16/20 (80 %) for the HLHS-RS group and 114/121(94 %) for the HLHS-NRS (p = 0.028). Twenty (14 %) patients were lost to follow up and 9 (6 %) underwent heart transplant. Overall survival was 10/16 (62 %) for HLHSRS patients and 77/95 (81 %) for HLHS-NRS (p = 0.1). Survival after initial discharge was 10/12 (83 %) for the HLHS-RS group and 77/88 (87 %) for the HLHS-NRS (p = 0.67). No predictors for HLHS-RS outcome were identified. Mortality at first-stage palliation in HLHS neonates with a restrictive atrial septum remains higher than in those with an unrestrictive communication. However, survival after initial hospital discharge is similar.     

Fate of Ventricular and Valve Performance Following Early Bidirectional Glenn Procedure After Norwood Operation Controlled for Hypoplastic Left Heart Syndome Anatomic Subtype

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS–MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS–MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7–4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS–MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.     

Effect of Oil Massage on Growth in Preterm Neonates Less than 1800 g: A Randomized Control Trial

Objective

To study the effect of oil massage on growth in preterm babies less than 1800 g.

Methods

This randomised controlled trial was conducted in Neonatal intensive care unit of a level II hospital. Neonates with birth weight?<?1800 g, gestation?<?35 wk and?<?48 h of age at enrolment were included in the studies. Eligible neonates were randomized to one of the two groups (a) Oil massage along with standard care of low birth weight (b) Standard care of low birth weight without massage. Weight, length and head circumference was measured in the two groups at 7 d intervals. Serum triglyceride levels were measured at enrolment and at completion of study. Primary outcome variable was weight gain at 28 d after enrolment.

Results

A total of forty-eight neonates were randomisd to either oil massage group (n?=?25) or standard care of low birth weight without massage group (n?=?23). Mean (SD) weight of babies in the two groups was 1466.4?±?226.8 g in oil massage group and 1416.6?±?229.9 g in the control group. At 28 d, weight gain in the oil massage group (476.76?±?47.9 g) was higher compared to the control group (334.96?±?46.4 g) (p?<?0.05). At 7 d, less weight loss (7.80?±?9.8 g) was observed in babies in oil massage group compared to control group (21.52?±?19.4 g) (p?=?0.003). However, there was no significant difference in serum triglycerides and other anthropometric parameters.

Conclusions

Oil application has a potential to improve weight gain and cause less weight loss in first 7 d in low birth weight neonates