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Vol 9, No 3
Vol 9, No 3 August 2013 ISSN 1708-8569
 
Review articles
Systematic review
Original articles
Brief reports
Case report
   
Review articles:
Mesenchymal stem cells in the treatment of pediatric diseases
  Guo-Ping Zheng, Meng-Hua Ge, Qiang Shu, Mauricio Rojas, Jianguo Xu
 

Mesenchymal stem cells in the treatment of pediatric diseases

Guo-Ping Zheng, Meng-Hua Ge, Qiang Shu, Mauricio Rojas, Jianguo Xu

Shaoxing, China

Author Affiliations: Shaoxing Second Hospital, Zhejiang University, Shaoxing, China (Zheng GP, Ge MH, Xu J); Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Shu Q); Center for Interstitial Lung Disease, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA (Rojas M); Investigational Drug Service, Emory University, Atlanta, GA, USA (Xu J)

Corresponding Author: Jianguo Xu, PhD, Department of Immunotherapy, Shaoxing Second Hospital, Zhejiang University, 123 Yan An Road, Shaoxing 312000, China (Tel: 86-575-88053995; Email: jxu5@emory.edu)

doi: 10.1007/s12519-013-0425-1

Background: In recent years, the incredible interests in mesenchymal stem cells have boosted the expectations of both patients and physicians. Unlike embryonic stem cells, neither their procurement nor their use is deemed controversial. Moreover, their immunomodulatory capacity coupled with low immunogenicity has opened up their allogenic use, consequently broadening the possibilities for their application. In May 2012, Canadian health regulators approved Prochymal, the first mesenchymal stem cells-based drug, for acute graft-versus-host diseases in children who have failed to respond to steroid treatment. The aim of this article is to review the recent advances in mesenchymal stem cells for pediatric diseases.

Data sources: A literature review was performed on PubMed from 1966 to 2013 using the MeSH terms "mesenchymal stem cells", "clinical trials" and "children". Additional articles were identified by a hand search of the references list in the initial search.

Results: The following categories are described: general properties, mechanisms of action, graft-versus-host diseases, cardiovascular diseases, liver diseases, inflammatory bowel diseases, osteoarticular diseases, autoimmune diseases, type 1 diabetes, and lung diseases.

Conclusions: Mesenchymal stem cells, owing to their availability, immunomodulatory properties, low immunogenicity, and therapeutic potential, have become one of the most attractive options for the treatment of a wide range of diseases. It is expected to see more and more clinical trials and applications of mesenchymal stem cells for pediatric diseases in the near future.

Key words: children; diseases; graft-versus-host; mesenchymal stem cells

World J Pediatr 2013;9(3):197-211

  [Abstract] [Full Text] [PDF]  
Treatment of children with chronic viral hepatitis: what is available and what is in store
  Pietro Vajro, Claudio Veropalumbo, Sergio Maddaluno, Mariacarolina Salerno, Giancarlo Parenti, Claudio Pignata
 

Treatment of children with chronic viral hepatitis: what is available and what is in store

Pietro Vajro, Claudio Veropalumbo, Sergio Maddaluno, Mariacarolina Salerno, Giancarlo Parenti, Claudio Pignata

Baronissi, Italy

Author Affiliations: Department of Medicine and Surgery, University of Salerno, Baronissi (Salerno), Italy (Vajro P); Department of Translational Medical Sciences, Pediatrics, University of Naples "Federico II", Naples, Italy (Veropalumbo C, Maddaluno S, Salerno M, Parenti G, Pignata C)

Corresponding Author: Pietro Vajro, Chair of Pediatrics, Department of Medicine and Surgery, University of Salerno, Via Allende 84081, Baronissi (Salerno), Italy (Tel: +39-089-672409; Email: pvajro@unisa.it)

doi: 10.1007/s12519-013-0426-0

Background: At present, therapy of children with chronic hepatitis B and C is still based on few drugs, all burdened by a series of side-effects, unsatisfactory serum conversion rates, and/or drug-resistance. Moreover, selection of subjects to treat with conventional therapies is not univocal, especially during the pediatric age when the disease course is often mild with significant spontaneous seroconversion rate. Our review deals with pros and cons points when a physician decides to design a drug therapy for a child with chronic viral hepatitis, and different possible therapeutic opportunities.

Methods: A literature search was performed through PubMed. The newest articles, reviews, systematic reviews, and guidelines were included in this review.

Results: The management of children with viral hepatitis is still controversial over whom and when to treat and the use of drug(s). Novel therapeutic strategies have been evaluated only in clinical and preclinical trials involving, for instance, "therapeutic" vaccines. The data on safety and effectiveness of new drugs are also reviewed.

Conclusion: The results of reported studies confirmed that at least some of the new drugs, with greater efficacy and/or minor side-effects, will be used clinically.

Key words: children; hepatitis B; hepatitis C; therapy

World J Pediatr 2013;9(3):212-220

  [Abstract] [Full Text] [PDF]  
Systematic review:
Beta-blockers versus corticosteroids in the treatment of infantile hemangioma: an evidence-based systematic review
  Shi-Qiong Xu, Ren-Bing Jia, Wei Zhang, Huang Zhu, Sheng-Fang Ge, Xian-Qun Fan
 

Beta-blockers versus corticosteroids in the treatment of infantile hemangioma: an evidence-based systematic review

Shi-Qiong Xu, Ren-Bing Jia, Wei Zhang, Huang Zhu, Sheng-Fang Ge, Xian-Qun Fan

Shanghai, China

Author Affiliations: Department of Ophthalmology, Ninth People's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200011, China (Xu SQ, Jia RB, Ge SF, Fan XQ); Department of Emergency, Changzheng Hospital, the Second Military Medical University, Shanghai 200003, China (Zhang W); Department of Ophthalmology, Xinhua Hospital Affiliated to School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China (Zhu H)

Corresponding Author: Xian-Qun Fan, MD, PhD, Department of Ophthalmology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, No. 639 Zhi Zao Ju Road, Shanghai 200011, China (Tel: 86-21-23271699; Email: fanxq@sh163.net)

doi: 10.1007/s12519-013-0427-z

Background: The efficacy and safety of beta-blockers versus corticosteroids in the treatment of infantile hemangiomas (IHs) is controversial. This study aimed to summarize evidence described in the literature and to assess the quality of studies involving beta-blockers and corticosteroids for the treatment of cutaneous IHs.

Methods: Comparative studies were collected from 15 online electronic databases, including OVID Medline, PubMed, ISI Web of Science, CENTRAL, CNKI, ChiCTR, JPCTR, CTRIndia, IranCTR, SLCTR, ISRCTRN, NLCTR, GCTR, ANCTR, ClinicalTrial.gov, and associated references. Studies without a control group were excluded, and the remaining studies were assessed by two reviewers independently using the Downs & Black scale for reported quality. The main areas assessed in the included studies were volume changes, overall improvement in appearance, eye function, and adverse events.

Results: Ten comparative studies were included with a total of 419 children. A meta-analysis was not performed due to the considerable heterogeneity across studies. Some evidence showed that beta-blockers are superior to steroids in reducing volume and improving the overall appearance of IHs, such as lightening of the color and flattening of the surface. Conclusions regarding improved eye function and adverse events were divided, and no consensus has been reached on the superiority of one treatment over another. No episodes of severe-onset asthma, hypotension, or bradycardia occurred in the beta-blocker treatment due to the rigorous exclusion of patients with contraindications.

Conclusions: Available studies indicate that beta-blockers are an alternative option to corticosteroids for IH treatment with respect to volume shrinkage and improvement in appearance. No evidence has shown a significant difference in improved eye function and adverse events between beta-blockers and corticosteroids in the treatment of IH; indeed, there is a lack of well-designed, high-quality randomized control trials.

Key words: adverse events; beta-blockers; eye functions; infantile hemangioma; steroids

World J Pediatr 2013;9(3):221-229

  [Abstract] [Full Text] [PDF]  
Original articles:
Relationships between age of puberty onset and height at age 18 years in girls and boys
  Mitra Yousefi, Wilfried Karmaus, Hongmei Zhang, Graham Roberts, Sharon Matthews, Bernie Clayton, Syed Hasan Arshad
 

 

Relationships between age of puberty onset and height at age 18 years in girls and boys

Mitra Yousefi, Wilfried Karmaus, Hongmei Zhang, Graham Roberts, Sharon Matthews, Bernie Clayton, Syed Hasan Arshad

Columbia, SC, USA

Author Affiliations: University of South Carolina, Columbia, SC, USA (Yousefi M, Karmaus W, Zhang H); The David Hide Asthma and Allergy Research Center, St Mary's Hospital, Newport, Isle of Wight, UK (Roberts G, Matthews S, Clayton B, Arshad SH); Faculty of Medicine, University of Southampton, Southampton, UK (Roberts G, Arshad SH)

Corresponding Author: Wilfried Karmaus, +800 Sumter Street Suite 304, Columbia, SC 29201, USA (Tel: (803) 777-9814; Fax: (803) 777-2524; Email: karmaus@sc.edu)

doi: 10.1007/s12519-013-0399-z

Background: Changes during puberty may influence final adult height. Height is related to multiple health conditions, including lung function. We investigated the association between the age of onset of five puberty events and height at age 18 years, analyzing boys and girls separately.

Methods: Of 1456 children recruited into the Isle of Wight birth cohort (1989-1990), 1313 were followed up at age 18 years. Height was measured, and age of pubertal onset was collected at age 18 years. Cluster analysis was performed on the five puberty events in boys and girls and linear regression was applied with the clusters predicting height at age 18 years. Individual linear regression analyses assessed the age of onset of each pubertal event as a potential predictor for height at age 18 years.

Results: Of the 1313 children followed up at age 18 years, 653 were males and 660 were females. All puberty variables had high internal consistency. In girls, earlier age of menarche, breast development, and growth spurt were related to shorter height. In boys, earlier age of growth spurt and slower progression through puberty were related to taller height at age 18 years.

Conclusions: Given that boys and girls may have opposing associations between pubertal timing and adult height and that height is an important predictor of lung function, the effect of pubertal timing on respiratory health should be explored.

Key words: height; puberty onset; respiratory health

World J Pediatr 2013;9(3):230-238

  [Abstract] [Full Text] [PDF]  
B-type natriuretic peptide and N-terminal pro-BNP in the acute phase of Kawasaki disease
  Satoru Iwashima, Takamichi Ishikawa
 

 

B-type natriuretic peptide and N-terminal pro-BNP in the acute phase of Kawasaki disease

Satoru Iwashima, Takamichi Ishikawa

Hamamatsu, Japan

Author Affiliations: Department of Pediatrics, Hamamatsu University School of Medicine, Hamamatsu, Japan (Iwashima S, Ishikawa T)

Corresponding Author: Satoru Iwashima, MD, Department of Pediatrics, Hamamatsu University School of Medicine, Handayama 1-20-1, Hamamatsu City 431-3192, Japan (Tel: +81-53-435-2312; Fax: +81-53-435-2311; Email: iwashima@hama-med.ac.jp)

doi: 10.1007/s12519-013-0402-8

Background: This study was undertaken to identify factors correlating with plasma levels of B-type natriuretic peptide (BNP) and its N-terminal portion (NT-pro BNP) in the acute phase of Kawasaki disease (KD).

Methods: This study included 91 patients with KD treated at a hospital affiliated to Hamamatsu University School of Medicine between October 2003 and June 2011. We quantified BNP and NT-pro BNP in the acute phase. The BNP level was expressed as the NT-pro BNP level using the formula NT-pro BNP=9.080BNP0.923. We sought relationships between NT-pro BNP values and different clinical and laboratory data in the acute phase of KD.

Results: Of the 91 patients, 14 failed to respond to the initial intravenous immunoglobulins therapy. NT-pro BNP levels were significantly higher in these non-responders than in the responders (1689.31168.8 pg/dL vs. 844.41276.3 pg/dL, P<0.001). Seventeen patients developed coronary artery lesions, but this was not associated with NT-proBNP levels. NT-pro BNP was positively correlated with CRP (r=0.421, P<0.001) and negatively correlated with the hematocrit (r=-0.206, P=0.050), Na value (r=-0.214, P=0.041) and albumin level (r=-0.345, P<0.001). Stepwise multiple linear regression analysis with NT-pro BNP as a dependent variable revealed significant correlations with CRP and albumin (beta=0.345, P=0.001; beta=-0.225, P=0.027).

Conclusions: A high level of NT-pro BNP in acute phase KD is associated with systemic inflammatory responses and increased vascular permeability. The NT-pro BNP level is a useful marker to identify potential non-responders to IVIG among KD patients.

Key words: B-type natriuretic peptide; Kawasaki disease; myocarditis; NT pro BNP

World J Pediatr 2013;9(3):239-244

  [Abstract] [Full Text] [PDF]  
The differences in T and B cell subsets in thyroid of children with Graves' disease and Hashimoto's thyroiditis
  Iwona Ben-Skowronek, Leszek Szewczyk, Beata Kulik-Rechberger, Elzbieta Korobowicz
 

The differences in T and B cell subsets in thyroid of children with Graves' disease and Hashimoto's thyroiditis

Iwona Ben-Skowronek, Leszek Szewczyk, Beata Kulik-Rechberger, Elzbieta Korobowicz

Lublin, Poland

Author Affiliations: Department of Pediatric Endocrinology and Diabetology (Ben-Skowronek I, Szewczyk L), Department of Neonatology and Infant Pathology (Rechberger B), Department of Pathology (Korobowicz E), Medical University, Ul. Chodzki 2, 20-093 Lublin, Poland. This work was presented as the poster in the 35th Annual Meeting of European Thyroid Association.

Corresponding Author: Iwona Ben-Skowronek, Department of Pediatric Endocrinology and Diabetology, Medical University, Ul. Chodzki 2, 20-093 Lublin, Poland (Tel: +48 81 7185 440; Email: skowroneki@interia.pl)

doi: 10.1007/s12519-013-0398-0

Background: The differences between Graves' disease (GD) and Hashimoto's thyroiditis (HT) suggest that changes in the subsets of T cells may have an influence on the course of these reactions.

Methods: This study included 90 children: 30 with GD, 30 with HT, and 30 healthy children as controls. After thyroidectomy, standard histological examinations and immunohistochemical reactions were performed in paraffin specimens with monoclonal antibodies against T cell markers CD3, CD4, CD8 as well as against CD79 alpha B cells. Ultrathin sections were examined under a transmission electron microscope.

Results: Autoimmune reaction in GD consisted of an increased number of CD4+ T cells (3.174.27%) and plasma cells (22.898.61%) producing thyroid-stimulating hormone-receptors and stimulating thyrocytes to activity. The number of CD8+ T cells was increased in children with HT (20.540.68%) as compared with the controls (0.650.30%). The autoimmune reaction in the HT children showed antibody dependent cytotoxicity with a low number of CD4+ T cells and an increased number of CD8+ T cells   in the thyroid tissue in comparison with that in the GD children and the controls. Plasma cells (31.659.11%) in this situation produced the antibodies involved in cytotoxic reactions against thyrocytes.

Conclusions: Graves' disease is characterized by the increased number of CD4+ T cells and CD8+ T cells. Hashimoto's thyroiditis is characterized by the low number of CD4+ T cells and increased number of CD8+ T cells. CD8+ T cells have cytotoxic properties only in Hashimoto's thyroiditis.

Key words: autoimmunity; lymphocytes; thyroid disorders

World J Pediatr 2013;9(3):245-250

  [Abstract] [Full Text] [PDF]  
Association of serotonin transporter polymorphisms with responsiveness to adrenocorticotropic hormone in infantile spasm
  Xiu-Yu Shi, Li-Ping Zou, Guang Yang, Ying-Xue Ding, Bing He, Yan-Hong Sun, Fei-Yong Jia
 

 

Association of serotonin transporter polymorphisms with responsiveness to adrenocorticotropic hormone in infantile spasm

Xiu-Yu Shi, Li-Ping Zou, Guang Yang, Ying-Xue Ding, Bing He, Yan-Hong Sun, Fei-Yong Jia

Beijing, China

Author Affiliations: Department of Pediatrics, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China (Shi XY, Zou LP, Yang G); Department of Pediatrics, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China (Ding YX); Division of Matrix Biology, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden (He B); Department of Pediatrics, Cangzhou Central Hospital, Cangzhou 061001, China (Sun YH); Department of Pediatrics, First Hospital of Jilin University, Changchun 130021, China (Jia FY)

Corresponding Author: Li-Ping Zou, MD, Department of Pediatrics, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China (Tel: +86-10-66939770; Fax: +86-10-66939770; Email: zouliping21@hotmail.com).

doi: 10.1007/s12519-013-0420-6

Background: Serotonin or 5-hydroxytryptamine (5-HT) is an important neurotransmitter in the central nervous system. The serotonin transporter (5-HTT) is a key regulator of the level of serotonergic neurotransmission. In the present study, the contribution of 5-HTT polymorphisms to the risk of infantile spasm (IS) and the responsiveness to adrenocorticotropic hormone (ACTH) were investigated.

Methods: Two functional polymorphisms, the 44-bp insertion-deletion polymorphism in the promoter region (5-HTTLPR) and the variable number tandem repeat in the second intron (5-HTTVNTR), were genotyped in a Chinese case-control study involving 112 patients with IS and 120 controls.

Results: Genotyping yielded valid data in 111 patients and 118 controls for 5-HTTLPR and 110 patients and 118 controls for 5-HTTVNTR. The polymorphisms were not found to have an allelic or genotypic association with IS. However, responsiveness to ACTH was higher in patients who were homozygous for L (91%) than in those with S/L (56%) or S/S (60%) (P=0.017). Haplotype analysis did not improve the observed association.

Conclusions: The results suggest that the 5-HTTLPR genotype may influence the responsiveness to ACTH. This interpretation deserves further study.

Key words: genetic predisposition; infantile spasms; polymorphisms; serotonin transporter

World J Pediatr 2013;9(3):251-255

  [Abstract] [Full Text] [PDF]  
Central adiposity and the risk of hypertension in Asian Indian girls
  Jyoti Ratan Ghosh, Arup Ratan Bandyopadhyay
 

 

Central adiposity and the risk of hypertension in Asian Indian girls

Jyoti Ratan Ghosh, Arup Ratan Bandyopadhyay

West Bengal, India

Author Affiliations: Department of Anthropology, Visva-Bharati University, Santiniketan-731235, West Bengal, India (Ghosh JR); Department of Anthropology, University of Calcutta, 35 Ballygunge Circular Road, Kolkata 700019, West Bengal, India (Bandyopadhyay AR)

Corresponding Author: Jyoti Ratan Ghosh, Department of Anthropology, Visva-Bharati University, Santiniketan-731235, West Bengal, India (Email: jrghosh@rediffmail.com or jrghosh@visva-bharati.ac.in)

doi: 10.1007/s12519-013-0424-2

Background: Elevated blood pressure during childhood is an established predictor of elevated blood pressure in adulthood. This study was undertaken to evaluate the relationship of general and central adiposity measures with hypertension and to find out the best adiposity measure in predicting hypertension.

Methods: A cross-sectional study was carried out in 197 girls aged 5 to 16 years. Anthropometric measurements included stature, weight, waist circumference (WC) and hip circumference. Body mass index (BMI, kg/m2), waist hip ratio (WHR) and conicity index (CI) were calculated subsequently. Systolic blood pressure (SBP) and diastolic bold pressure (DBP) were taken and hypertension was defined as age and stature adjusted SBP and/or DBP 95th percentile. Linear and logistic regression analysis was made to determine the relationship of adiposity measure with blood pressure and hypertension and to find out the best adiposity measure in predicting hypertension.

Results: Both WC [odds ratio (OR)=2.20, 95% confidence interval, 1.32-3.69] and CI (OR=1.85, 95% confidence interval, 1.14-3.0) were significantly associated with hypertension. However, there was no significant association in BMI and WHR with hypertension.

Conclusion: WC is the best adiposity measure in predicting hypertension in girls.

Key words: anthropometry; girls; hypertension; waist circumference

World J Pediatr 2013;9(3):256-260

  [Abstract] [Full Text] [PDF]  
Psycho-behavioral changes in children with type 1 diabetes mellitus
  Xiao-Ping Zheng, Shuo-Hui Chen
 

 

Psycho-behavioral changes in children with type 1 diabetes mellitus

Xiao-Ping Zheng, Shuo-Hui Chen

Hangzhou, China

Author Affiliations: Department of Endocrinology, Children's Hospital, Zhejiang University School of Medicine and Key Laboratory of Reproductive Genetics, Zhejiang University, Ministry of Education, Hangzhou, China (Zheng XP, Chen SH)

Corresponding Author: Shuo-Hui Chen, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86-13588887709; Fax: +86-571-87033296; Email: sh89925@sina.com)

doi: 10.1007/s12519-013-0428-y

Background: Type 1 diabetes mellitus (T1DM) is the most common type of diabetes in children. This study aimed to investigate psycho-behavioral changes in Chinese children with T1DM and to provide some advices for nurses, parents and other persons.

Methods: Forty-five patients with T1DM (26 boys and 19 girls with a mean age of 10.403.01 years) were enrolled. According to the glycosylated hemoglobin levels recommended by the American Diabetes Association, the patients were subdivided into a well-controlled group and a poorly-controlled group. Fifty-three healthy children served as a control group. Psycho-behavioral changes were investigated by using Achenbach's Child Behavior Check List.

Results: Compared with the control group, the patients with T1DM had significantly higher mean scores for withdrawal, anxiety/depression, attention problems, delinquent behavior, aggressive behavior, externalizing problems, and internalizing problems (P<0.017). Moreover, the mean scores for somatic complaints in the poorly-controlled subgroup were significantly higher than those in the well-controlled subgroup (t=3.582, P=0.001). Compared with the control group, the well-controlled subgroup had higher scores for withdrawal, anxiety/depression, and internalizing problems (P<0.017). But the poorly-controlled subgroup had higher scores for withdrawal, somatic complaint, anxiety/depression, delinquent behavior, aggressive behavior, externalizing and internalizing problems (P<0.017).

Conclusions: Children with T1DM may have some psycho-behavioral problems. Timely nursing interventions must be conducted to solve these problems.

Key words: behavior; Child Behavior Check List; psychology; type 1 diabetes mellitus

World J Pediatr 2013;9(3):261-265

  [Abstract] [Full Text] [PDF]  
Hepatic CYP3A expression and activity in low birth weight developing female rats
  Zhi-Wei Zhu, Shao-Qing Ni, Xiu-Min Wang, Jue Wang, Su Zeng, Zheng-Yan Zhao
 

 

Hepatic CYP3A expression and activity in low birth weight developing female rats

Zhi-Wei Zhu, Shao-Qing Ni, Xiu-Min Wang, Jue Wang, Su Zeng, Zheng-Yan Zhao

Hangzhou, China

Author Affiliations: Department of Children's Health and Care (Zhu ZW, Zhao ZY), Department of Clinical Pharmacology, the National Clinical Trial Institute (Ni SQ, Wang J), Department of Endocrinology (Wang XM), Children's Hospital of Zhejiang University School of Medicine and Zhejiang Key Laboratory for Diagnosis and Therapy of Neonatal Diseases, Hangzhou 310003, China; Department of Pharmaceutical Analysis and Drug Metabolism, College of Pharmaceutical Sciences, Zhejiang University, Hangzhou 310058, China (Zeng S)

Corresponding Author: Su Zeng, Department of Pharmaceutical Analysis and Drug Metabolism, College of Pharmaceutical Sciences, Zhejiang University, No. 388 Yu Hang Tang Road, Hangzhou 310058, China (Tel: +86 571 88208407; Email: nsqshc@163.com)

doi: 10.1007/s12519-013-0429-x

Background: We aimed to investigate the effects of low birth weight (LBW) on the hepatic expression of cytochrome P-450 3A (CYP3A) in developing female rats.

Methods: Pregnant rats were divided into two groups, a nourished group and an under-nourished group. The offspring of the nourished rats were defined as a normal weight, normal diet group (NN group). The offspring of the under-nourished rats were designated as a LBW, normal diet group (LN group). CYP3A mRNA expression, protein expression, protein localization and activity were determined.

Results: The CYP3A1 mRNA expression levels of the LN group on days 3, 21, and 56 were significantly higher than those of the same age in the NN group (P0.01). The mRNA expression of CYP3A2 in the LN group on day 21 was higher than in rats of the same age in the NN group (P<0.01). The staining intensity and frequency of CYP3A1-positive hepatocytes were significantly lower on days 7 and 21 in the LN group than those of rats of the same age in the NN group (P<0.05). The staining intensity and frequency of CYP3A2-positive hepatocytes on days 14 and 21 were higher in the LN group than those on the same days in the NN group  (P<0.05). The mean CYP3A activity of the LN group on day 3 was significantly higher than that of the NN group (P<0.001).

Conclusions: We found the effect of LBW on CYP3A activity was most prominent during the early days of life in rats. Investigators and clinicians should consider the effect of LBW on CYP3A in both pharmacokinetic study design and data interpretation, when prescribing drugs to LBW infants.

Key words: cytochrome P-450 3A; development; expression; low birth weight

World J Pediatr 2013;9(3):266-272

  [Abstract] [Full Text] [PDF]  
Brief reports:
Clinical features and mutation analysis of X-linked agammaglobulinemia in 20 Chinese patients
  Xian Qin, Li-Ping Jiang, Xue-Mei Tang, Mo Wang, En-Mei Liu, Xiao-Dong Zhao
 

Clinical features and mutation analysis of X-linked agammaglobulinemia in 20 Chinese patients

Xian Qin, Li-Ping Jiang, Xue-Mei Tang, Mo Wang, En-Mei Liu, Xiao-Dong Zhao

Chongqing, China

Author Affiliations: Division of Immunology, Children's Hospital of Chongqing Medical University; Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics, Committee of Science and Technology in Chongqing; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Chongqing 400014, China (Qin X, Jiang LP, Tang XM, Wang M, Liu EM, Zhao XD)

Corresponding Author: Xiao-Dong Zhao, MD, PhD, Division of Immunology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China (Tel: 86 23 6362 2554; Fax: 86 23 6360 2136; Email: zhaoxd530@yahoo.com.cn)

doi: 10.1007/s12519-013-0400-x

Background: X-linked agammagobulinemia (XLA) is a primary immunodeficiency caused by Bruton's tyrosine kinase (BTK) gene mutation. XLA patients have an extremely small amount of peripheral B cells and profound deficiency in all immunoglobulin isotypes. We analyzed the clinical, immunologic, and molecular characteristics of children with XLA in an attempt to improve the diagnosis and treatment of XLA in China.

Methods: Twenty children with XLA-compatible phenotypes from 18 unrelated families were enrolled in this study. The BTK gene was amplified and sequenced, followed by mutation analysis in these children and their female relatives.

Results: Eighteen different mutations of the BTK gene were identified in the 20 patients. Eleven mutations had been reported previously including eight missense mutations (c.994C>T, c.1987C>A, c.1885G>T, c.502T>C, c.1085C>T, c.1816C>T, c.214C>T, c.1912G>A) and three nonsense mutations (c.1267T>A, c.1793C>G, c.1618C>T). Seven novel mutations of the BTK gene were also presented and included five missense mutations (c.134T>A, c.1646T>A, c.1829C>G, c.711G>T, c.1235G>A), one splice-site mutation (c.523+1G>A) and one insertion mutation (c.1024-1025insTTGCTAAAGCAACTGCTAAAGCAAG). Eight out of 18 mutations of the BTK gene were located in the TK domain, 4 in the PH domain, 4 in the SH2 domain and 2 in the TH domain. Genetic study for carrier status was carried out in 18 families with definite BTK gene mutations. Nine carriers with BTK gene mutations were identified. Six families without carriers were detected, and 3 patients were not tested in this study.

Conclusion: Our results support that molecular genetic testing represents an important tool for early confirmed diagnosis of congenital agammaglobulinemia and may allow accurate carrier detection and prenatal diagnosis.

Key words: BTK gene; genotype-phenotype correlation; mutations; X-linked agammaglobulinemia

World J Pediatr 2013;9(3):273-277

  [Abstract] [Full Text] [PDF]  
The HHEX gene is not related to congenital heart disease in 296 Chinese patients
  Xiao-Peng Deng, Li-Xi Zhao, Bin-Bin Wang, Jing Wang, Long-Fei Cheng, Zhi Cheng, Pei-Su Suo, Hui Li, Xu Ma
 

The HHEX gene is not related to congenital heart disease in 296 Chinese patients

Xiao-Peng Deng, Li-Xi Zhao, Bin-Bin Wang, Jing Wang, Long-Fei Cheng, Zhi Cheng, Pei-Su Suo, Hui Li, Xu Ma

Shenyang and Beijing, China

Author Affiliations: Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, Shenyang 110004, China (Deng XP, Li H); Graduate School of Peking Union Medical College, Beijing 100730, China (Zhao LX, Wang BB, Wang J, Cheng LF, Cheng Z, Suo PS, Ma X); National Research Institute for Family Planning, Beijing 100081, China (Zhao LX, Wang BB, Wang J, Cheng LF, Cheng Z, Suo PS, Ma X); World Health Organization Collaborating Centre for Research in Human Reproduction, Beijing 100081, China (Ma X)

Corresponding Author: Hui Li, Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, No. 36 Sanhao Street, Heping District, Shenyang 110004, China (Tel: +86 024 83955179; Fax: +86 024 83955092; Email: FCKLIHUI@126.com)

doi: 10.1007/s12519-013-0430-4

Background: The hematopoietically expressed homeobox (HHEX) gene is an important determinant of mammalian heart development. This study aimed to identify the potential mutations of the gene in Chinese patients with congenital heart disease (CHD).

Methods: We collected 296 CHD patients and 200 controls, and classified the cardiac deformities. Then we conducted sequence analyses of the HHEX gene in those patients.

Results: In all the CHD patients, we did not find any causative mutations in the coding region of the HHEX gene.

Conclusion: To our knowledge, this is the first study to examine the HHEX gene in non-symptomatic CHD cases, and this has expanded our knowledge about its etiology.

Key words: congenital heart disease; genetics; HHEX; transcription factor

World J Pediatr 2013;9(3):278-280

  [Abstract] [Full Text] [PDF]  
Case report:
Successful rescue of a child with fall-related multi-organ injuries
  Zi-Hao Yang, Chen-Mei Zhang, Xiao-Fang Lou, Zhen-Jie Chen, Sheng Ye
 

Successful rescue of a child with fall-related multi-organ injuries

Zi-Hao Yang, Chen-Mei Zhang, Xiao-Fang Lou, Zhen-Jie Chen, Sheng Ye

Hangzhou, China

Author Affiliations: Department of Pediatric ICU, Children's Hospital of Zhejiang University School of Medicine and Zhejiang Laboratory for Diagnosis and Therapy of Neonatal Disease, Hangzhou 310003, China (Yang ZH, Zhang CM, Lou XF, Chen ZJ, Ye S)

Corresponding Author: Sheng Ye, Department of Pediatric ICU, Children's Hospital of Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86-13588074637; Fax: +86-571-87033296; Email: yeshengchina@126.com)

doi: 10.1007/s12519-013-0401-9

Background: Fall-related injury is a major cause of accidental death and is rarely reported in children. Even successful treatment of children with multi-organ injuries caused by fall from the extreme height has been rarely reported.

Methods: We herein report a 2-year-old girl who fell from the 10th floor, had multi-organ injuries, and was successfully rescued.

Results: The girl sustained multi-organ injuries, such as head injury, closed abdominal injury, as well as fractures of the left limbs, and successfully recovered after two surgeries and supportive treatment.

Conclusions: This case illustrates that a child who falls from extreme heights can occasionally be rescued. Early neuroprotective strategies help to improve neurological recovery.

Key words: child; fall-related injury; trauma

World J Pediatr 2013;9(3):281-283

  [Abstract] [Full Text] [PDF]  
   
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