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Rationale, Aims, and Objectives: Waist circumference (WC) and waist-to-height ratio (WHtR) are superior surrogate markers of central obesity than body mass index. However, WC is not measured routinely in paediatric clinics. The objective of this study was to implement measurement of WC during routine assessment of children in an ambulatory outpatient clinic setting and subsequent dissemination of cardiometabolic risk counselling in children with central obesity (defined as WHtR ≥0.5)., Method: Prospective cohort of patients aged 6 to 20 years. Study period was divided into three phases: baseline (3 months), process improvement (2 months), and implementation (6 months). Define-Measure-Analyse-Improve-Control (DMAIC) strategy was applied. Measurement of WC was implemented as a component of the physical examination in patients. Outcome measures were (1) improvement in frequency of WC measurement and (2) utilization of WHtR in cardiometabolic risk counselling., Results: Waist circumference was not measured in any patient during baseline phase (n = 551). During process improvement phase, of the total 347 patients, WC was measured in 35% vs target of 30%. In the implementation phase, WC was measured in 37% patients (365 out of 964). Of these 365 patients, 175 (48%) had elevated WHtR, and 73% of them (n = 128) were counselled about their increased cardiometabolic risk., Conclusions: Application of an evidence-based DMAIC protocol led to significant improvement in assessment for central obesity in an ambulatory clinic practice and appropriate counselling regarding cardiometabolic risk reduction in children and adolescents with central obesity over an 8-month period. Meticulous planning and execution, frequent reinforcement, and integrating feedback from the involved multi-disciplinary team were important factors in successful implementation of this quality improvement project., (© 2018 John Wiley & Sons, Ltd.)

Wolff-Parkinson-White (WPW) syndrome is defined by the presence of a short PR interval, delta waves on the electrocardiogram (EKG), and symptomatic tachycardia. The condition is rare but can be life-threatening if not recognized in a timely manner. The WPW pattern on EKG can mask ischemic changes and may also increase the risk of arrhythmia and subsequent mortality. Our case describes the conversion of an underlying WPW pattern into WPW syndrome in the scenario of an acute myocardial infarction (AMI)., Competing Interests: The authors have declared that no competing interests exist.

Mycoplasma pneumoniae is one of the most common causes of community-acquired pneumonia in adults. Mycoplasma pneumoniae pneumonia (MPP) presents with pulmonary and extrapulmonary manifestations. Pneumothorax is a rare MPP complication in children. But, we reported a case of MPP with hydropneumothorax in an adult. The association of MPP, hydropneumothorax, and empyema is extremely rare.

Anion gap metabolic acidosis (AGMA) occurs when an anion gap exists along with metabolic acidosis, most commonly due to diabetic ketoacidosis (DKA) and lactic acidosis (LA). Isolated starvation ketoacidosis (ISK) is one of the rare causes of AGMA; however, it usually presents with a mild disturbance in pH. We report a rare case of a 45-year-old female with previously diagnosed squamous cell cancer (SCC) of the larynx. She presented to the emergency department complaining of difficulty in breathing following laryngectomy and tracheostomy for SCC. Her laboratory results on admission were consistent for isolated starvation ketoacidosis and the patient responded quickly to the appropriate treatment., Competing Interests: The authors have declared that no competing interests exist.

Multiple supernumerary teeth are one of the most common developmental anomalies in humans. They could be associated with prolonged retention of deciduous teeth, displacement or rotation of the adjacent teeth, crowding, aberrations in the root morphology (dilacerations), and failure in eruption or impaction of the succedaneous teeth. This article highlights 2 cases of non-syndromic mandibular twin supernumerary teeth in young female clients with a chief complaint of spacing between the teeth and over-retained deciduous teeth. A diligent clinical and radiographic monitoring is warranted considering the early innocuous effects and delayed adverse effects of this condition., Competing Interests: The authors have declared no conflicts of interest., (Copyright © 2019, CDHA | ACHD.)

Background: Individuals with congenital adrenal hyperplasia (CAH) require glucocorticoid therapy to replace cortisol and to control androgen excess. We sought to evaluate the effects of glucocorticoid therapy on cardiovascular and metabolic outcomes in individuals with CAH., Methods: We searched bibliographical databases through January 2016 for studies evaluating cardiovascular risk factors in individuals with CAH treated with glucocorticoids compared with controls without CAH. We used a random-effects model to synthesize quantitative data., Results: We included 20 observational studies (14 longitudinal, six cross-sectional) with a moderate to high risk of bias. The average dose of glucocorticoids (in hydrocortisone equivalents) was 9 to 26.5 mg/m2/d. In the meta-analysis (416 patients), compared with controls without CAH, individuals with CAH had increased systolic blood pressure [weighted mean difference (WMD), 4.44 mm Hg; 95% CI, 3.26 to 5.63 mm Hg], diastolic blood pressure (WMD, 2.35 mm Hg; 95% CI, 0.49 to 4.20 mm Hg), homeostatic model assessment of insulin resistance (WMD, 0.49; 95% CI, 0.02 to 0.96), and carotid intima thickness (WMD, 0.08 mm; 95% CI, 0.01 to 0.15 mm). No statistically significant differences were noted in fasting blood glucose, insulin level, glucose, or insulin level after 2-hour glucose load or serum lipids. Data on cardiac events were sparse, and most of the literature focused on surrogate outcomes., Conclusion: Individuals with CAH demonstrate a high prevalence of cardiovascular and metabolic risk factors. The current evidence relies on surrogate outcomes. Long-term prospective studies are warranted to assess strategies for reducing cardiovascular risk in individuals with CAH.

Background: Females with congenital adrenal hyperplasia (CAH) and atypical genitalia often undergo complex surgeries; however, their outcomes remain largely uncertain., Methods: We searched several databases through 8 March 2016 for studies evaluating genital reconstructive surgery in females with CAH. Reviewers working independently and in duplicate selected and appraised the studies., Results: We included 29 observational studies (1178 patients, mean age at surgery, 2.7 ± 4.7 years; mostly classic CAH). After an average follow-up of 10.3 years, most patients who had undergone surgery had a female gender identity (88.7%) and were heterosexual (76.2%). Females who underwent surgery reported a sexual function score of 25.13 using the Female Sexual Function Index (maximum score, 36). Many patients continued to complain of substantial impairment of sensitivity in the clitoris, vaginal penetration difficulties, and low intercourse frequency. Most patients were sexually active, although only 48% reported comfortable intercourse. Most patients (79.4%) and treating health care professionals (71.8%) were satisfied with the surgical outcomes. Vaginal stenosis was common (27%), and other surgical complications, such as fistulas, urinary incontinence, and urinary tract infections, were less common. Data on quality of life were sparse and inconclusive., Conclusion: The long-term follow-up of females with CAH who had undergone urogenital reconstructive surgery shows variable sexual function. Most patients were sexually active and satisfied with the surgical outcomes; however, some patients still complained of impairment in sexual experience and satisfaction. The certainty in the available evidence is very low.

Background: There is a lack of consensus on the cardiometabolic consequences of mild subclinical hypothyroidism (SCH) among children. The objective of the current study was to compare lipid profiles in children with mild SCH with those of euthyroid children., Study Design: Retrospective medical record review., Patients: Children (ages 2-18 years) who had undergone simultaneous measurement of TSH, free thyroxine (T4) and lipids. Lipids in children with mild SCH (TSH 5-<10 mIU/L and normal free T4, n = 228) were compared with those in euthyroid children (n = 1215)., Results: TSH level was positively associated with total cholesterol and nonhigh density lipoprotein (non-HDL) cholesterol [β 0.05(0.03-0.08), P < .0001 and β 0.05(0.03-0.08), P < .0001, respectively]. Total cholesterol was significantly higher in children and adolescents with mild SCH compared with euthyroid children (4.43 ± 1.14 mmol/L vs 4.2 ± 0.85 mmol/L, P = .0005). Similarly, non-HDL cholesterol level was also higher in children with mild SCH relative to euthyroid children (3.08 ± 1.14 mmol/L vs 2.91 ± 0.8 mmol/L, P = .001). The adjusted odds ratio of having elevated total cholesterol and elevated non-HDL cholesterol was greater in children with mild SCH compared with euthyroid children (OR 1.88, 95% CI; 1.28-2.73; P = .001 and 1.72, 95% CI 1.2-2.5; P = .003, respectively). The presence of thyroid autoimmunity was not associated with higher rates of dyslipidaemia., Conclusions: Mild SCH in children and adolescents was associated with higher rates of elevated total cholesterol and elevated non-HDL cholesterol. Randomized placebo controlled studies are warranted to determine if treatment of mild SCH in children leads to improvement in lipid profile., (© 2018 John Wiley & Sons Ltd.)

Key Message: Twelve meta-QTL for seed Fe and Zn concentration and/or content were identified from 87 QTL originating from seven population grown in sixteen field trials. These meta-QTL include 2 specific to iron, 2 specific to zinc and 8 that co-localize for iron and zinc concentrations and/or content. Common bean (Phaseolus vulgaris L.) is the most important legume for human consumption worldwide and it is an important source of microelements, especially iron and zinc. Bean biofortification breeding programs develop new varieties with high levels of Fe and Zn targeted for countries with human micronutrient deficiencies. Biofortification efforts thus far have relied on phenotypic selection of raw seed mineral concentrations in advanced generations. While numerous quantitative trait loci (QTL) studies have been conducted to identify genomic regions associated with increased Fe and Zn concentration in seeds, these results have yet to be employed for marker-assisted breeding. The objective of this study was to conduct a meta-analysis from seven QTL studies in Andean and Middle American intra- and inter-gene pool populations to identify the regions in the genome that control the Fe and Zn levels in seeds. Two meta-QTL specific to Fe and two meta-QTL specific to Zn were identified. Additionally, eight Meta QTL that co-localized for Fe and Zn concentration and/or content were identified across seven chromosomes. The Fe and Zn shared meta-QTL could be useful candidates for marker-assisted breeding to simultaneously increase seed Fe and Zn. The physical positions for 12 individual meta-QTL were identified and within five of the meta-QTL, candidate genes were identified from six gene families that have been associated with transport of iron and zinc in plants.

Background: Immature ovarian teratoma is very rare in childhood. We report on a 12-year-old girl with immature ovarian teratoma who presented initially with syndrome of inappropriate antidiuretic hormone., Case: A 12-year-old girl presented with acute abdomen and distention. Initial laboratory tests showed hyponatremia (sodium, 123 mmol/L), that did not respond to fluid management. Computed tomography imaging showed a 15 cm × 9 cm × 20 cm mass in the right ovary with multifocal internal fat, and dystrophic calcifications. She underwent exploratory laparotomy with a right salpingo-oophorectomy, omentectomy, and peritoneal stripping. The pathology revealed metastatic immature teratoma. Hyponatremia resolved soon after the surgery., Summary and Conclusion: Although a rare diagnosis, immature ovarian teratoma must be considered in a girl who presents with abdominal mass and hyponatremia., (Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

We present the frequency- and temperature-dependent dielectric response of Eu 1-x Ba x TiO 3 (0  ⩽  x  ⩽  0.5) in detail. Excluding grain boundary effects, four relaxation mechanisms were observed. Relaxation dynamics were observed to arise due to hopping conduction associated with defects, namely oxygen vacancies as well as Eu 3+ and Ti 3+ ions. Dielectric relaxation analysis led to the identification of Ti ions in two different environments with different relaxation rates in the overall EuTiO 3 perovskite structure. The emergence of another relaxation mechanism associated with ferroelectric order as a consequence of the formation of polar regions was also observed for higher Ba concentrations. The addition of Ba led to the identification of relaxation dynamics associated with hopping conduction between Eu ions, Ti ions (in the regions with and without oxygen vacancies) and with the formation of ferroelectric polar regions. Furthermore, the polydispersivity and relaxation times were extracted within the framework of the modified Debye model. Relaxation times have been observed to increase with a decrease in temperature while larger values of polydispersivity reveal a wide distribution of relaxation times due to the presence of lattice parameter and energy barrier distributions.

Severe obesity is associated with abnormal lipids and increased risk for cardiovascular disease. Obesity is a risk factor for vitamin D deficiency. We examined relationship between 25-hydroxy vitamin D (25(OH)D) concentrations and lipids in children with severe obesity. Medical records of 376 children were reviewed. Linear regression models and logistic regression were used to examine the relationship between 25(OH)D and lipids after adjustment for age, gender, season of blood draw, body mass index (BMI) z-score, and BMI % of 95th percentile. Two-hundred sixty-three out of 376 children (70%) had 25(OH)D concentrations < 30 ng/mL. Concentrations of 25(OH)D were positively correlated with those of high-density lipoprotein cholesterol (HDL-C) (r² = 0.08, r = 0.22, β = 0.16, 95% CI = 0.05-0.27, p = 0.004). HDL-C was lower in children with 25(OH)D < 30 ng/mL (n = 263) compared to those with 25(OH)D ≥ 30 ng/mL (n = 113) (41.3 ± 10.2 vs. 46.4 ± 12 mg/dL, p < 0.0001). Children with 25(OH)D concentrations < 30 ng/mL had greater adjusted odds of low HDL-C (<40 mg/dL) compared with those with 25(OH)D ≥ 30 ng/mL (47.9% vs. 29.2%, OR 2.15 (1.33-3.51), p = 0.0019). Total cholesterol and non-HDL-C were not correlated with 25(OH)D concentrations. Vitamin D deficiency is highly prevalent in children with severe obesity. Prospective clinical trials are warranted to determine if vitamin D supplementation can improve HDL-C and potentially decrease risk for cardiovascular disease in children with obesity., Competing Interests: The authors declare no conflict of interest

Objectives: To determine prevalence of delirium in critically ill children and explore associated risk factors., Design: Multi-institutional point prevalence study., Setting: Twenty-five pediatric critical care units in the United States, the Netherlands, New Zealand, Australia, and Saudi Arabia., Patients: All children admitted to the pediatric critical care units on designated study days (n = 994)., Intervention: Children were screened for delirium using the Cornell Assessment of Pediatric Delirium by the bedside nurse. Demographic and treatment-related variables were collected., Measurements and Main Results: Primary study outcome measure was prevalence of delirium. In 159 children, a final determination of mental status could not be ascertained. Of the 835 remaining subjects, 25% screened positive for delirium, 13% were classified as comatose, and 62% were delirium-free and coma-free. Delirium prevalence rates varied significantly with reason for ICU admission, with highest delirium rates found in children admitted with an infectious or inflammatory disorder. For children who were in the PICU for 6 or more days, delirium prevalence rate was 38%. In a multivariate model, risk factors independently associated with development of delirium included age less than 2 years, mechanical ventilation, benzodiazepines, narcotics, use of physical restraints, and exposure to vasopressors and antiepileptics., Conclusions: Delirium is a prevalent complication of critical illness in children, with identifiable risk factors. Further multi-institutional, longitudinal studies are required to investigate effect of delirium on long-term outcomes and possible preventive and treatment measures. Universal delirium screening is practical and can be implemented in pediatric critical care units.

Poverty is one of the most important determinants of adverse health outcomes globally, a major cause of societal instability and one of the largest causes of lost human potential. Traditional approaches to measuring and targeting poverty rely heavily on census data, which in most low- and middle-income countries (LMICs) are unavailable or out-of-date. Alternate measures are needed to complement and update estimates between censuses. This study demonstrates how public and private data sources that are commonly available for LMICs can be used to provide novel insight into the spatial distribution of poverty. We evaluate the relative value of modelling three traditional poverty measures using aggregate data from mobile operators and widely available geospatial data. Taken together, models combining these data sources provide the best predictive power (highest r 2 = 0.78) and lowest error, but generally models employing mobile data only yield comparable results, offering the potential to measure poverty more frequently and at finer granularity. Stratifying models into urban and rural areas highlights the advantage of using mobile data in urban areas and different data in different contexts. The findings indicate the possibility to estimate and continually monitor poverty rates at high spatial resolution in countries with limited capacity to support traditional methods of data collection., (© 2017 The Authors.)

Background: Variability in the incidence and severity of bronchopulmonary dysplasia (BPD) among premature infants suggests that genetic susceptibility plays a role in pathogenesis. An assessment of copy number variants (CNV) in BPD subjects may help to identify loci that harbor genetic susceptibility factors., Methods: We conducted a retrospective analysis of clinical DNA microarray data from our institution. We identified 19 BPD subjects, and 2 controls groups (full-term and preterm) with no lung-related disease. We reanalyzed raw data from each of these subjects to identify recurrent CNV loci in BPD subjects., Results: We identified three loci (at 11q13.2, 16p13.3, and 22q11.23-q12.1) with recurrent CNV in BPD subjects. The frequency of these CNV was significantly higher in BPD subjects when compared with at least one control group. We interrogated 21 genes residing within the recurrent CNV regions for development-associated changes in expression. Fifteen genes demonstrated significant changes in expression between the pseudoglandular and canalicular stage in human lungs, a time commensurate with birth at highest risk for BPD. We also identified pathways represented by the genes present within the recurrent loci., Conclusion: These data identify novel loci that may harbor genes contributing to the genetic susceptibility of BPD.

Objective: To determine the prevalence and weight gain as a risk factor of hypertension (HTN) at three years of age and older among extremely low birth weight (ELBW) infants., Study Design: Retrospective cohort study of all ELBW infants born and followed up at our institution over 15 years., Results: 204 infants were followed up for [median (inter-quartile range)] 10.2 years (6.9-13.0). Their birth weight and gestational age were (mean±standard deviation) 758±149 g and 26.4±2.1 weeks respectively. At 3 years of age and older, the prevalence of HTN was 7.3% (15/204), and the prevalence of obesity was 13.2% (27/204). Obesity was more prevalent in children with than without HTN [33% (5/15) vs. 12% (22/189); p = 0.01]. In a regression analysis, body mass index (BMI) percentile at 3 years of age and older (p = 0.04) and increase in weight Z scores since birth (p = 0.008) were associated with a higher systolic blood pressure (SBP; R = 0.386, p <  0.001)., Conclusion: Obesity is prevalent in ELBW infants at three years of age and older, and it is significantly associated with hypertension. An increase in BMI percentile at ≥3 years of age, and the rate of weight gain since birth among ELBW infants are associated with an increase in SBP.

A 54 years old male presented with central chest pain for five hours in a local hospital, Comilla. He was diagnosed as a case of acute STEMI (Extensive Anterior) and was thrombolized with Streptokinase 1.5 million IU. His pain was relieved, ST segment was depressed by >50% after thrombolysis. While in hospital, he developed weakness of his left limbs with gradually deteriorating level of consciousness. A CT scan of brain showed haemorrhage in the right frontoparietal region. This is a rare case of haemorrhagic stroke after thrombolysis with streptokinase.

Effects of laparoscopic donor nephrectomy (LDN) on graft function, especially early post-transplant, have been controversial. To assess and compare early and late graft function in kidneys procured by open and laparoscopic methods, a retrospective observational study was carried out on 37 recipients-donors who underwent LDN after introduction of this technique in February 2007 at our center, a tertiary care nephrology referral center. Demographic, immunological and intraoperative variables as well as immunosuppressive protocols and number of human leukocyte antigen (HLA) mismatches were noted. Early graft function was assessed by serum creatinine on Days two, five, seven, 14 and 28 and at the time of discharge. Serum creatinine values at three months and at one year post-transplant were considered as the surrogates of late graft function. Data obtained were compared with the data from 33 randomly selected kidney transplants performed after January 2000 by the same surgical team, in whom open donor nephrectomy was used. Pearson's chi square test, Student's t test and Mann-Whitney U test were used for statistical analysis. Early graft function (serum creatinine on Day five 2.15 mg/dL vs 1.49 mg/dL, P = 0.027) was poorer in the LDN group. Late graft function as assessed by serum creatinine at three months (1.45 mg/dL vs 1.31 mg/dL, P = 0.335) and one year (1.56 mg/dL vs 1.34 mg/dL, P = 0.275) was equivalent in the two groups. Episodes of early acute graft dysfunction due to acute tubular necrosis were significantly higher in the LDN group (37.8% vs 12.1%, Z score 2.457, P = 0.014). Warm ischemia time was significantly prolonged in the LDN group (255 s vs 132.5 s, P = 0.002). LDN is associated with slower recovery of graft function and higher incidence of early acute graft dysfunction due to acute tubular necrosis. Late graft function at one year is however comparable.

In patients with myelodysplastic syndromes (MDS), chromosome anomalies are detected by conventional cytogenetic studies (CCS) and/or interphase fluorescence in situ hybridization (FISH) of bone marrow (BM) samples and provide prognostic and diagnostic information, which can direct therapy. Whether peripheral blood (PB) can be substituted for bone marrow in these cases and can provide the same information remains unknown. Concurrent BM and PB specimens collected from 100 patients with recently diagnosed MDS were studied using both CCS and FISH. While 68% of BM samples showed an abnormal karyotype by CCS, only 31% of PB samples were abnormal by CCS. In 12% of patients, FISH and CCS were discordant due to the inability of the FISH panel to detect all possible abnormalities. However, only one case (1%) had a cryptic abnormality detected by FISH. BM and PB FISH were discordant in 3% of cases, most likely due to the smaller clone size in PB vs. BM. While PB should not be substituted for BM at diagnosis, it is a viable alternative for monitoring patients using the appropriate FISH probe(s)., (Copyright © 2012 Elsevier Ltd. All rights reserved.)