Pediatric cataracts

Congenital and acquired pediatric cataracts are recognized by many as being among the visually devastating pathologies of children. They remain one of the leading causes of legal blindness for children in under developed countries world wide. Diagnosed in approximately 400–500 patients per year in the United States alone, their presence requires agressive management with concurrent amblyopia therapy. Although most pediatric opacities are idiopathic, systemic etiologies which include genetic inheritance (Fabry's disease, Lowe's syndrome, Conradi's syndrome) metabolic disturbances (Galactosemia, Hypocalcemia, hypoglycemia, retinitis pigmentosa), infectious diseases (maternal syphilis, varicella virus, herpes virus, cytomegalovirus), toxic substances and trauma must be investigated. We reviewed the literature for pertinent information on the differential diagnosis of congenital and acquired cataracts in the pediatric population. The modalities and techniques of the pediatric examination are presented along with a discussion of the maladies, most recent advances in surgical intervention, optical or contact lens correction and amblyopia therapy associated with pediatric cataracts.     

血清NT-proBNP水平在小儿心源性与非心源性呼吸困难诊断鉴别的价值

目的：探究血清B 型利钠肽前体（NT-proBNP）水平在小儿心源性与非心源性呼吸困难诊断鉴别的价值。方法：本研究于 2013 年2月~2015年2 月期间,选择我院收治的急性呼吸困难患儿72 例为研究对象,根据临床诊断将其分为心源性呼吸困难组 （39例）和非心源呼吸困难组（33 例）。采用电化学发光免疫分析法（ECLIA）测量两组患儿血清NT-proBNP 水平,并采用受试者工 作特征曲线（ROC）曲线评估血清NT-proBNP 水平在小儿心源性与非心源性呼吸困难诊断鉴别中的价值。结果：心源性呼吸困难 组患儿血清NT-proBNP水平为(253.23± 39.38)ng/L,明显高于非心源性呼吸困难组患儿血清NT-proBNP水平（76.39± 17.39） ng/L（t=23.882,P<0.05）。小儿血清NT-proBNP 水平诊断心源性呼吸困难和非心源性呼吸困难的曲线下面积为0.914,曲线下面积 的95%的置信区间为（0.861,0.967）,当血清NT-proBNP水平为118.34ng/L 时,约登指数取最大值为0.923,敏感度为97.43% （37/39）、特异度为94.87%（32/33）。结论：NT-proBNP 作为一个特异性指标可用于小儿心源性呼吸困难与非心源性呼吸困难的鉴 别诊断。     

History of the Department of Pediatrics Yale University School of Medicine.

The history of pediatrics at the Yale University School of Medicine can be divided into eight historical eras. The "Paleohistorical Era" included colonial figures such as Governor John Winthrop and Hezekiah Beardsley who wrote about children''s disease in colonial times. Eli Ives, Professor of the Diseases of Children at Yale Medical School gave the first systematic pediatric course in America in the first half of the nineteenth century. During the second era, from 1830-1920, the New Haven Hospital was opened. An affiliation between Yale University and the New Haven Hospital led to the formal establishment of clinical departments including pediatrics in the early 20th century. Six eras coinciding with successive pediatric chairman have led the department to its present respected position in American pediatrics. The department''s 75th anniversary in 1996 is an occasion to recognize many of the department''s accomplishments and leaders over the years. It is also a time to reaffirm the mission of the department: to the health needs of the children of Connecticut and beyond, to the advancement of scientific knowledge of infants and children and their diseases, and to the training and educational of the pediatric clinicians, educators and investigators of the future.     

儿科抗癫痫药物应用研究进展

儿童癫痫为小儿神经科的常见疾病,临床表现以抽搐为主。近年来,随着医疗技术的发展,以及人们对儿童癫痫的重视,国内外文献对儿科癫痫的治疗报道越来越多,目前,药物治疗仍然是抗癫痫的首选方法,除了运用新型抗癫痫药物外,也有采用中药治疗癫痫的报道,现就近年来儿童癫痫的药物治疗研究作一综述。     

The Association between Cold Spells and Pediatric Outpatient Visits for Asthma in Shanghai, China

Background

Asthma is a serious global health problem. However, few studies have investigated the relationship between cold spells and pediatric outpatient visits for asthma.

Objective

To examine the association between cold spells and pediatric outpatient visits for asthma in Shanghai, China.

Methods

We collected daily data on pediatric outpatient visits for asthma, mean temperature, relative humidity, and ozone from Shanghai between 1 January 2007 and 31 December 2009. We defined cold spells as four or more consecutive days with temperature below the 5^th percentile of temperature during 2007–2009. We used a Poisson regression model to examine the impact of temperature on pediatric outpatient visits for asthma in cold seasons during 2007 and 2009. We examined the effect of cold spells on asthma compared with non-cold spell days.

Results

There was a significant relationship between cold temperatures and pediatric outpatient visits for asthma. The cold effects on children''s asthma were observed at different lags. The lower the temperatures, the higher the risk for asthma attacks among children.

Conclusion

Cold temperatures, particularly cold spells, significantly increase the risk of pediatric outpatient visits for asthma. The findings suggest that asthma children need to be better protected from cold effects in winter.     

Why did 12 infants die? Winnipeg's endless inquest seeks answers

Canada''s longest running inquest will wrap up in Winnipeg in September. Although the judge conducting it will be answering many questions, one of the key ones is the most simple: Should a province the size of Manitoba operate a program as sophisticated as pediatric cardiac surgery?     

Heat Shock Protein-Derived T-Cell Epitopes Contribute to Autoimmune Inflammation in Pediatric Crohn's Disease

Pediatric Crohn''s disease is a chronic auto inflammatory bowel disorder affecting children under the age of 17 years. A putative etiopathogenesis of Crohn''s disease (CD) is associated with disregulation of immune response to antigens commonly present in the gut microenvironment. Heat shock proteins (HSP) have been identified as ubiquitous antigens with the ability to modulate inflammatory responses associated with several autoimmune diseases. The present study tested the contribution of immune responses to HSP in the amplification of autoimmune inflammation in chronically inflamed mucosa of pediatric CD patients. Colonic biopsies obtained from normal and CD mucosa were stimulated with pairs of Pan HLA-DR binder HSP60-derived peptides (human/bacterial homologues). The modulation of RNA and protein levels of induced proinflammatory cytokines were measured. We identified two epitopes capable of sustaining proinflammatory responses, specifically TNF〈 and IFN© induction, in the inflamed intestinal mucosa in CD patients. The responses correlated positively with clinical and histological measurements of disease activity, thus suggesting a contribution of immune responses to HSP in pediatric CD site-specific mucosal inflammation.     

Pediatric Health-Related Quality of Life: A Structural Equation Modeling Approach

Objectives

One of the most referenced theoretical frameworks to measure Health Related Quality of Life (HRQoL) is the Wilson and Cleary framework. With some adaptions this framework has been validated in the adult population, but has not been tested in pediatric populations. Our goal was to empirically investigate it in children.

Methods

The contributory factors to Health Related Quality of Life that we included were symptom status (presence of chronic disease or hospitalizations), functional status (developmental status), developmental aspects of the individual (social-emotional) behavior, and characteristics of the social environment (socioeconomic status and area of education). Structural equation modeling was used to assess the measurement structure of the model in 214 German children (3–5 years old) participating in a follow-up study that investigates pediatric health outcomes.

Results

Model fit was χ2 = 5.5; df = 6; p = 0.48; SRMR  = 0.01. The variance explained of Health Related Quality of Life was 15%. Health Related Quality of Life was affected by the area education (i.e. where kindergartens were located) and development status. Developmental status was affected by the area of education, socioeconomic status and individual behavior. Symptoms did not affect the model.

Conclusions

The goodness of fit and the overall variance explained were good. However, the results between children'' and adults'' tests differed and denote a conceptual gap between adult and children measures. Indeed, there is a lot of variety in pediatric Health Related Quality of Life measures, which represents a lack of a common definition of pediatric Health Related Quality of Life. We recommend that researchers invest time in the development of pediatric Health Related Quality of Life theory and theory based evaluations.     

The Influence of Parenting Change on Pediatric Weight Control

Objective: Parenting style was examined as a predictor of weight loss maintenance in behavioral family‐based pediatric obesity treatment. Research Methods and Procedures: Fifty obese children who participated in a behavioral family‐based pediatric obesity treatment were studied. Hierarchical regression tested the incremental effect of baseline parenting and parenting during treatment on children's percentage overweight change over 12 months, beyond demographics and adherence to targeted behaviors. Results: Children's percentage overweight significantly decreased at 6 (?16.3) and 12 (?11.1) months. Adherence to program goals significantly increased variance accounted for in the regression model by 10.8%, whereas adding baseline father acceptance and change in father acceptance accounted for another 20.5%. The overall model accounted for 40.6% of the variance in pediatric weight control. ANOVA showed significantly greater percentage overweight decrease from baseline for youth with fathers who increased their acceptance vs. those who decreased acceptance at 6 (?19.8 vs. ?14.6) and 12 (?17.4 vs. ?8.1) months. Discussion: Youth who perceive an increase in father acceptance after treatment had better changes in percentage overweight over 12 months than youth with lower ratings of father acceptance. Future directions include examining how other parenting dimensions impact pediatric obesity treatment outcome and how parental acceptance can be enhanced to improve child weight control.     

Brain-Derived Neurotrophic Factor and Interleukin-6 Levels in the Serum and Cerebrospinal Fluid of Children with Viral Infection-Induced Encephalopathy

We investigated changes in the brain-derived neurotrophic factor (BDNF) and interleukin (IL)-6 levels in pediatric patients with central nervous system (CNS) infections, particularly viral infection-induced encephalopathy. Over a 5-year study period, 24 children hospitalized with encephalopathy were grouped based on their acute encephalopathy type (the excitotoxicity, cytokine storm, and metabolic error types). Children without CNS infections served as controls. In serum and cerebrospinal fluid (CSF) samples, BDNF and IL-6 levels were increased in all encephalopathy groups, and significant increases were noted in the influenza-associated and cytokine storm encephalopathy groups. Children with sequelae showed higher BDNF and IL-6 levels than those without sequelae. In pediatric patients, changes in serum and CSF BDNF and IL-6 levels may serve as a prognostic index of CNS infections, particularly for the diagnosis of encephalopathy and differentiation of encephalopathy types.     

Impact of Prior Traumatic Life Events on Parental Early Stage Reactions following a Child's Cancer

Background

In pediatric oncology, effective clinic–based management of acute and long–term distress in families calls for investigation of determinants of parents'' psychological response to the child''s cancer. We examined the relationship between parents'' prior exposure to traumatic life events (TLE) and the occurrence of posttraumatic stress symptoms (PTSS) following their child''s cancer diagnosis. Factors mediating the TLE–PTSS relationship were analyzed.

Methodology

The study comprised 169 parents (97 mothers, 72 fathers) of 103 cancer diagnosed children (median age: 5,9 years; range 0.1–19.7 years). Thirty five parents were of immigrant origin (20.7%). Prior TLE were collated using a standardized questionnaire, PTSS was assessed using the Impact of Events–Revised (IES–R) questionnaire covering intrusion, avoidance and hyperarousal symptoms. The predictive significance of prior TLE on PTSS was tested in adjusted regression models.

Results

Mothers demonstrated more severe PTSS across all symptom dimensions. TLE were associated with significantly increased hyperarousal symptoms. Parents'' gender, age and immigrant status did not significantly influence the TLE–PTSS relationship.

Conclusions

Prior traumatic life–events aggravate posttraumatic hyperarousal symptoms. In clinic–based psychological care of parents of high–risk pediatric patients, attention needs to be paid to life history, and to heightened vulnerability to PTSS associated with female gender.     

Atlantoaxial dislocation and Down's syndrome.

The phenotypic features of Down''s syndrome are easily recognized and include characteristic facial features, hypotonia, ligament laxity, transverse palmar creases and mental subnormality. Associated manifestations and complications are also familiar and involve almost every organ system. Congenital heart defects, bowel malformations and a tendency to leukemia are common attendant problems. Less common, however, are defects of the skeletal system; in fact, the most recent edition of a standard pediatric textbook makes no mention of anomalies of the vertebral column. The purpose of this paper is to call attention to the association between Down''s syndrome and atlantoaxial dislocation, which in our patient resulted in quadriplegia and eventually death.     

Acute hepatic failure in children.

Many diseases may present as acute hepatic failure in the pediatric age group, including viral hepatitis A and B, adverse drug reactions, both toxic and "hepatitic," and inherited metabolic disorders such as tyrosinemia, alpha 1 antitrypsin deficiency, and Wilson''s disease. Management is primarily supportive, with care taken to anticipate the known complications of hepatic failure. Few "curative" therapies are known, although attempts at stimulating hepatic regeneration may be helpful.     

HRV Biofeedback for Pediatric Irritable Bowel Syndrome and Functional Abdominal Pain: A Clinical Replication Series

Irritable bowel syndrome (IBS) and Functional Abdominal Pain (FAP) are among the most commonly reported Functional Gastrointestinal Disorders. Both have been associated with varying autonomic dysregulation. Heart Rate Variability Biofeedback (HRVB) has recently begun to show efficacy in the treatment of both IBS and FAP. The purpose of this multiple clinical replication series was to analyze the clinical outcomes of utilizing HRVB in a clinical setting. Archival data of twenty-seven consecutive pediatric outpatients diagnosed with IBS or FAP who received HRVB were analyzed. Clinical outcomes were self-report and categorized as full or remission with patient satisfaction, or no improvement. Qualitative reports of patient experiences were also noted. Full remission was achieved by 69.2 % and partial remission was achieved by 30.8 % of IBS patients. Full remission was achieved by 63.6 % and partial remission was achieved by 36.4 % of FAP patients. No patients in either group did not improve to a level of patient satisfaction or >50 %. Patient’s commonly reported feeling validated in their discomfort as a result of psychophysiological education. Results suggest that HRVB is a promising intervention for pediatric outpatients with IBS or FAP. Randomized controlled trials are necessary to accurately determine clinical efficacy of HRVB in the treatment of IBS and FAP.     

Investigation of pediatric neck response and muscle activation in low-speed frontal impacts

Pediatric necks present different responses and injury patterns compared with those of adults in motor vehicle crashes (MVCs). To evaluate the effect of different muscle modeling methodologies, three muscle models were developed and simulated under low-speed frontal impact conditions with an average peak acceleration of 3g's. The muscle activation curve for the curve-guided model, the muscle segment was curved using guiding nodes, was further optimized based on experimental data. The pediatric neck model was also simulated under more severe frontal impact conditions with an average peak acceleration of 8g's. Simulation results revealed that the curve-guided model needed more muscle force than the straight-guided model, in which the muscle segment was straight with guiding nodes, and the curve-constrained model, in which the muscle segment was curved without guiding nodes and which imposes more constraints on the head and neck than the curve-guided model. The predicted head responses for the child finite element neck model were within or close to the experimental corridors of 3- and 8-g's frontal impacts. The neck injuries for a 10-year-old child commonly occurred at the interspinous ligament in the C7–T1 segment. The model could be used to analyze the responses and injuries of pediatric neck and head in low-speed frontal impacts.     

Acute rheumatic fever

While rheumatic fever is relatively uncommon except where there are poor and crowded living conditions, sporadic acute attacks continue to occur in a family or pediatric medical practice. The physician''s role in management of the sore throat in the diagnosis of suspected cases of rheumatic fever and in follow-up for continued prophylaxis is discussed. The frequency of admissions and presenting features of 159 patients with acute rheumatic fever is reviewed. Continued surveillance is required if we are to achieve a further reduction in attack rate and complications.     

ONC201 induces cell death in pediatric non-Hodgkin's lymphoma cells

ONC201/TIC10 is a small molecule initially discovered by its ability to coordinately induce and activate the TRAIL pathway selectively in tumor cells and has recently entered clinical trials in adult advanced cancers. The anti-tumor activity of ONC201 has previously been demonstrated in several preclinical models of cancer, including refractory solid tumors and a transgenic lymphoma mouse model. Based on the need for new safe and effective therapies in pediatric non-Hodgkin''s lymphoma (NHL) and the non-toxic preclinical profile of ONC201, we investigated the in vitro efficacy of ONC201 in non-Hodgkin''s lymphoma (NHL) cell lines to evaluate its therapeutic potential for this disease. ONC201 caused a dose-dependent reduction in the cell viability of NHL cell lines that resulted from induction of apoptosis. As expected from prior observations, induction of TRAIL and its receptor DR5 was also observed in these cell lines. Furthermore, dual induction of TRAIL and DR5 appeared to drive the observed apoptosis and TRAIL expression was correlated linearly with sub-G1 DNA content, suggesting its potential role as a biomarker of tumor response to ONC201-treated lymphoma cells. We further investigated combinations of ONC201 with approved chemotherapeutic agents used to treat lymphoma. ONC201 exhibited synergy in combination with the anti-metabolic agent cytarabine in vitro, in addition to cooperating with other therapies. Together these findings indicate that ONC201 is an effective TRAIL pathway-inducer as a monoagent that can be combined with chemotherapy to enhance therapeutic responses in pediatric NHL.     

Five Years' Experience with Intrauterine Transfusion

Five years'' experience with intrauterine transfusion involving 94 transfusions on 50 fetuses forms the basis of the paper. Twenty-three fetuses survived, which represents an overall salvage of 46%. Of 22 fetuses who received intrauterine transfusions before 28 weeks'' gestation, seven (31.9%) survived, which justifies the attempt. Of 28 fetuses who received intrauterine transfusions after 28 weeks'' gestation, 16 (57.1%) survived, which compares favourably with other series. A comparison of two different procedural techniques shows no statistically significant difference in ultimate results. Indications for amniocentesis are outlined and intrauterine transfusion was advised if the optical density difference fell in Liley''s zone III (or a very high zone II) and rose at a rate which anticipated a zone III reading prior to 32 weeks'' gestation. A pediatric assessment and therapeutic management of the 33 live births are presented. Twenty-eight babies received exchange transfusions. Five were excluded for reasons outlined in the text. Ten of the live-born died neonatally. The 23 survivors continue to thrive mentally and physically and follow-up continues.     

Readability of the written study information in pediatric research in France

Background

The aim was to evaluate the readability of research information leaflets (RIL) for minors asked to participate in biomedical research studies and to assess the factors influencing this readability.

Methods and Findings

All the pediatric protocols from three French pediatric clinical research units were included (N = 104). Three criteria were used to evaluate readability: length of the text, Flesch''s readability score and presence of illustrations. We compared the readability of RIL to texts specifically written for children (school textbooks, school exams or extracts from literary works). We assessed the effect of protocol characteristics on readability. The RIL had a median length of 608 words [350 words, 25^th percentile; 1005 words, 75^th percentile], corresponding to two pages. The readability of the RIL, with a median Flesch score of 40 [30; 47], was much poorer than that of pediatric reference texts, with a Flesch score of 67 [60; 73]. A small proportion of RIL (13/91; 14%) were illustrated. The RIL were longer (p<0.001), more readable (p<0.001) and more likely to be illustrated (p<0.009) for industrial than for institutional sponsors.

Conclusion

Researchers should routinely compute the reading ease of study information sheets and make greater efforts to improve the readability of written documents for potential participants.