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Vol 12, No 2
Vol 12, No 2 March 2016 ISSN 1708-8569
 
Clinical image
Correspondence
Personal viewpoint
Review articles
Meta-analysis
Original articles
Case reports
   
Clinical image:
Not just a capillary hemangioma
  Mehmet Kenan Kanburoglu
 
A four-day-old female infant with jaundice was admitted to our hospital. On her examination, a capillary hemangioma with a pigmented nevus and a Mongolian spot was noticed (Fig. 1). Ultrasound examination revealed spinal dysraphism at L2, L3 and L4 vertebrae (Fig. 2).
 
Spina bifida results from incomplete fusion of vertebral arches and manifests in various degrees of severity. Occult spinal dysraphism, however, can be suspected in the asymptomatic neonate when cutaneous stigmata, such as hemangiomas, lipomas, hairy patches, pigmented nevi or subcutaneous masses are seen over the lumbosacral spine.[1] Two or more congenital midline skin lesions are considered as the strongest markers of occult spinal dysraphism.[2] The present case shows that such simple lesions should be taken seriously if they occur simultaneously. On the other hand, Mongolian spots, sacral pits and dimples do not indicate a high risk of occult spinal dysraphism.[1]
 
Clinical examination should focus on neurological symptoms, urological disturbances and orthopedic deformities. Early diagnosis of occult defects can allow appropriate surgical intervention and prevent neurological deficits.
  [Abstract] [Full Text] [PDF]  
Correspondence:
An approximation technique for primary anastomosis (Gazi method) in selected cases of long gap esophageal atresia
  Ramazan Karabulut, Zafer Turkyilmaz, Kaan Sonmez
 
We read with great interest the article by Reismann et al about "Partial gastric pull-up (PGP) in the treatment of patients with longgap esophageal atresia (LGEA)." This article states that "The incidence of stenosis and gastro-esophageal refl ux (GER) after PGP is considerably high, even compared with alternative surgical techniques for the treatment of LGEA and with their high complication rates. PGP cannot yet be generally recommended for the treatment of LGEA."[1]
 
One of the challenges in evaluating the literature for the surgical treatment of long-gap esophageal atresia (LGEA) is the lack of a uniformly accepted defi nition for the entity. Some used a cutoff of a 2 cm gap to defi ne a long gap,[1] while others used 3 cm or more.[2] Practically, LGEA is one in which the ends of the esophagus cannot be brought together for a primary anastomosis without employing alternative techniques. Staged elongation of the esophagus in cases of LGEA has been effective in achieving esophageal length and preserving the native esophagus.[3] Internal (Foker) and external (Kimura) techniques have been described along with many modifi cations of each.[2,4] In a Kimura series, esophageal gaps of 2 to 7 vertebral bodies were treated with 1 to 5 revisions of esophagostomy for anastomosis. All patients developed strictures requiring multiple dilations and 11 of 12 patients required fundoplication for gastroesophageal reflux. Seven of the 12 patients have been able to eat normally.[5] In a Foker series, successful esophageal anastomosis required 2-15 thoracotomies in 52 patients.[6] A review article reported a mean complication rate of 11%-60%, an anastomotic leakage rate of 80%-83%, and an esophageal stricture rate of 83%-100% .[3] Reismann et al[1] reported a series of 9 cases of LGEA (including 7 cases of pure atresia). These patients but 2 underwent initially gastrostomy and delayed PGP for LGEA. The median distance between the upper and lower esophageal segment under tension was 3 vertebral bodies (range: 1-6). Leakage as early complication occurred in 3 patients, stenosis in 7, and GER as a late complication in 5.[1]
 
In our article "An approximation technique for primary anastomosis (Gazi Method) in selected cases of long gap esophageal atresia", we added fi ve patients with LGEA.[7] The fi ve patients were in type C and the gap length at the beginning of elongation varied from 3 to 5 cm. Dissection and anastomosis were carried out following an extrapleural right thoracotomy. The upper esophageal pouch was dissected up to the cervical inlet. The lower esophageal pouch was dissected as far down as the diaphragm. An eight French feeding tube was placed from the mouth and passed through the two pouches to the stomach. After this, both pouches were grabbed approximately 1 cm away from the cut ends, holding whole of the esophageal walls with tissue forceps and were approximated to each other under tension so that they contacted each other. The esophageal parts were held in this position until completion of the anastomosis using interrupted, 5-0 vicryl sutures (Gazi method). After all of the sutures were tied, the forceps were released so that the tension distributed evenly to all of the sutures and tissue holding the anastomosis. To minimize disruptive anastomotic forces, all infants with esophageal atresia were postoperatively kept paralyzed, sedated and mechanically ventilated for 2 to 5 days. In our series, 60% of our patients developed minor anastomotic leak and only 2 patients were given medication for GER. One patient required dilatation for anastomotic stricture. One patient with VACTERL syndrome died from cardiac failure at the 6th month. Its theoretical basis is that excellent intramural blood circulation in both upper and lower ends permits an excessive dissection, that esophageal lengthening could be achieved by intraoperative traction, and that an esophageal anastomosis could be constructed under considerable tension with associated minor complications if the tension is distributed evenly on the sutures. It is very important that the forceps holding and approximating the two esophageal ends to be released only after placing and tying the sutures. Advantages of our lengthening and approximation technique include no requirement of repeated thoracotomy, use of native esophagus, and short hospital stay. It is suitable for cases of esophageal gap between 3-5 cm.
 
Ramazan Karabulut, Zafer Turkyilmaz, Kaan Sonmez Department of Pediatric Surgery, Gazi University Medical Faculty, 06500, Ankara, Turkey Email: karabulutr@yahoo.com
 
References
1 Reismann M, Granholm T, Ehr¨¦n H. Partial gastric pull-up in the treatment of patients with long-gap esophageal atresia. World J Pediatr 2015;11:267-271.
2 von Allmen D, Wijnen RM. Bridging the gap in the repair of long-gap esophageal stresia: still questions on diagnostics and treatment. Eur J Pediatr Surg 2015;25:312-317.
3 Foker JE, Linden BC, Boyle EM Jr, Marquardt C. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997;226:533-541, discussion 541-543.
4 Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 1994;29:566-568.
5 Kimura K, Nishijima E, Tsugawa C, Collins DL, Lazar EL, Stylianos S, et al. Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients. J Pediatr Surg 2001;36:1725- 1727.
6 Bairdain S, Hamilton TE, Smithers CJ, Manfredi M, Ngo P, Gallagher D, et al. Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery. J Pediatr Surg 2015;50:933-937.
7 Karabulut R, Turkyilmaz Z, Sonmez K, Ozbayoglu A, Basaklar AC. An approximation technique for primary anastomosis (Gazi Method) in selected cases of long gap esophageal atresia. Prensa Med Argent 2014;100:1.
 
doi: 10.1007/s12519-016-0010-5
  [Abstract] [Full Text] [PDF]  
Correspondence
  Marc Reismann
 
We are very grateful to Karabulut et al for their interest in our paper reporting the method of partial gastric pull-up (PGP) in the treatment of long-gap esophageal atresia (LGEA).
We agree to the view that the central problem in treatment of esophageal atresia is at present lack of generally accepted defi nitions, for instance, the defi nition of LGEA as mentioned in their letter. In our study, we used a practical approach to define this entity by incapability of conventional primary anastomosis due to insufficient length of esophageal segments.[1] The measurement of the distances in vertebral bodies has a pure descriptive character. Karabulut et al acknowledged this type of defi nition.
Further, they demonstrated that all other techniques for treatment of LGEA are also associated with a significant number of complications like anastomotic leakage and esophageal stenosis/stricture. For postoperative stenosis, we used a generous definition that is essentially based on radiological, endoscopic but not on clinical findings.[1] Another more practical and frequently used defi nition is given by clinical symptoms revealing anastomotic stenosis, like swallowing difficulties, recurrent respiratory problems or foreign body obstruction.[2] Our definition does not exclude the possibility that patients without severe clinical problems are treated for anastomotic stricture. Thus, the comparability of our results regarding this type of complication remains elusive.
In our study, we were not able to show convincingly better treatment results after PGP compared with other already established methods like jejunal interposition, colon interposition and gastric pull-up. Thus, we have been very reluctant with the conclusions drawn. However, our results do not exclude the possibility of comparable results regarding the outcome after PGP. There is a clear lack of comparative studies.
The published Gazi method for the treatment of LGEA can be seen as a modification of the method of PGP by further reducing the tension on the esophageal segments using forceps.[3] It is well conceivable that this modification represents an effective extension of our approach, leading to a reduction of the complication rate. On the other hand, the tension within the partial pullup presented by us has been considerably low in all the reported cases. A study comparing the Gazi method with pure limited dissection in the method presented by us could appropriately answer this question.
 
Marc Reismann Department of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska Hospital, Stockholm, Sweden Email: marc.reismann@charite.de
 
References
1 Reismann M, Granholm T, Ehr¨¦n H. Partial gastric pull-up in the treatment of patients with long-gap esophageal atresia. World J Pediatr 2015;11:267-271.
2 Lydia Serhal, Fr¨¦ d¨¦ ric Gottrand, Rony Sfeir, Dominique Guimber, Patrick Devos, Michel Bonnevalle, et al. Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations. J Ped Surg 2010;45:1459-1462.
3 Karabulut R, Turkyilmaz Z, Sonmez K, Ozbayoglu A, Basaklar AC. An approximation technique for primary anastomosis (Gazi Method) in selected cases of long gap esophageal atresia. Prensa Med Argent 2014;100:1. doi: 10.1007/s12519-016-0011-4   
  [Abstract] [Full Text] [PDF]  
Personal viewpoint:
Stop tarnishing steroid and Chinese medicine
  Kam Lun E. Hon, Vivian Wing Yan Lee, Ting Fan Leung
 
 
In August 2014, the local media in Hong Kong reports a case of illegal prescription of the topical corticosteroid (CS) triamcinolone acetonide to a child with eczema by a Chinese medicine (CM) practitioner.[1] The eminent CM practitioner is depicted as a "divine doctor for childbirth", and illegal prescription or sale of CS as a class I poison considered a criminal act. In November 2011, another eminent CM practitioner, also known as "holy hand for eczema" was charged for causing Cushing syndrome in a number of children with eczema who had unknowingly received CS whilst receiving acupuncture therapy.[2] In 2006, a complementary and alternative medicine (CAM) practitioner was charged for processing and prescribing CS.[3] The CAM practitioner, also known as "Father of Naturopathy", allegedly discouraged patients from using legally prescribed CS for treatment of common atopic diseases.
 
Like asthma and allergic rhinitis, childhood eczema is a common atopic disease which is associated with chronicity and impaired quality of life of the patients and their families.[4,5] Management generally includes patient education, avoidance of triggers, regular use of emollient and appropriate use of topical CS.[6,7] Nevertheless, myths and non-adherence on various therapeutic aspects prevail and management of this disease remains suboptimal.[8] CS is the firstline treatment during disease flare.[6] Steroid phobia, however, has largely restricted the usefulness and efficacy of therapeutic CS.[9-13] In Hong Kong, many parents would seek alternative and folklore treatment of unproven efficacy.[14] Paradoxically, some of these alternative treatments may contain potent CS and unknowingly used by steroid phobic parents.[8,15]
 
Eminent CM practitioners are especially tempted to prescribe CS to preserve their reputation that CM is efficacious in the treatment of any disease. It may be difficult to convince anxious steroid phobic parents to be cautious in the use of often adulterated proprietary CM. Nevertheless, the media has an important role not to directly disgrace CM and indirectly tarnish CS usage. Scientifi cally, CM is an important branch of medicine, and topical or systemic CS is a very important class of immunomodulating and anti-inflammatory medication. Steroids are inexpensive and efficacious armamentariums. They are often lifesaving in management of such severe diseases as life-threatening asthma, anaphylaxis, systemic inflammatory disorders and even childhood malignancies (such as leukemia and solid brain tumors). In Hong Kong, both CM and CS have important roles in disease management. The media should understand that the culprit is not CM or CS but rather the irresponsible practitioners. Tarnishing CS usage certainly does not help with the already evil image of CS among steroid phobic parents. Western and CM practitioners and the media should work together to promote correct public health education on this important class of medicine. Money is not evil but the love of money is the root of all evil. CS is defi nitely not an evil.
 
References
1 Apple Daily, 2014. http://hk.apple.nextmedia.com/news/art/20140827/18846011 (accessed October 15, 2014).
2 Oriental News, 2011. http://m.on.cc/nc/hknews/20111112/20111112205315lc.html (accessed October 15, 2014).
3 Apple Daily, 2006. http://hk.apple.nextmedia.com/template/apple/art_main.php?iss_id=20061202&sec_id=4104&art_id=6581414 (accessed October 15, 2014).
4 Foley P, Zuo Y, Plunkett A, Marks R. The frequency of common skin conditions in preschool-age children in Australia: atopic dermatitis. Arch Dermatol 2001;137:293-300.
5 Leung R, Wong G, Lau J, Ho A, Chan JK, Choy D, et al. Prevalence of asthma and allergy in Hong Kong schoolchildren: an ISAAC study. Eur Respir J 1997;10:354-360.
6 Leung AK, Hon KL, Robson WL. Atopic dermatitis. Adv Pediatr 2007;54:241-273.
7 Hon KL, Leung TF, Kam WY, Lam MC, Fok TF, Ng PC. Dietary restriction and supplementation in children with atopic eczema. Clin Exp Dermatol 2006;31:187-191.
8 Hon KL, Leung TF, Yau HC, Chan T. Paradoxical use of oral and topical steroids in steroid-phobic patients resorting to traditional Chinese medicines. World J Pediatr 2012;8:263-267.
9 Patterson R, Walker CL, Greenberger PA, Sheridan EP. Prednisonephobia. Allergy Proc 1989;10:423-428.
10 Charman C, Williams H. Outcome measures of disease severity in atopic eczema. Arch Dermatol 2000;136:763-769.
11 Hon KL, Kam WY, Leung TF, Lam MC, Wong KY, Lee KC, et al. Steroid fears in children with eczema. Acta Paediatr 2006;95:1451-1455.
12 Aubert-Wastiaux H, Moret L, Le Rhun A, Fontenoy AM, Nguyen JM, Leux C, et al. Topical corticosteroid phobia in atopic dermatitis: a study of its nature, origins and frequency. Br J Dermatol 2011;165:808-814.
13 Kojima R, Fujiwara T, Matsuda A, Narita M, Matsubara O, Nonoyama S, et al. Factors associated with steroid phobia in caregivers of children with atopic dermatitis. Pediatr Dermatol 2013;30:29-35.
14 Hon KL, Leung AK. Powerful proprietary Chinese medicine for eczema? Clin Exp Dermatol 2010;35:e14-e15.
15 Hon KL, Burd A. 999 abuse: do mothers know what they are using? J Dermatolog Treat 2008;19:241-245.
  [Abstract] [Full Text] [PDF]  
Review articles:
Rectal biopsy for Hirschsprung's disease: a review of techniques, pathology, and complications
  Eleanor Dorothy Muise, Robert Anthony Cowles
 
Hirschsprung's disease (HD) is one of the most common congenital anomalies of colorectal function, affecting approximately 1 in 5000 live births, with a 4:1 male predominance. HD is characterized by aganglionosis that is most often limited to the rectosigmoid, but can extend proximally along the colon and, in rare instances, reach into the small intestine. A clinical history of delayed passage of meconium beyond 48 hours after birth, physical exam findings of abdominal distention and vomiting, and a contrast enema demonstrating a transition zone are highly suggestive of HD.
Data sources:
We searched databases including PubMed, Google Scholar, and Scopus for the following key words: Hirschsprung's disease, rectal biopsy, pathology, ganglion cell, nerve trunk hypertrophy, pediatric constipation, and selected publications written in English that were relevant to the scope of this review.
Results:
Based on the data presented in the literature, we reviewed 1) biopsy techniques for the diagnosis of Hirschsprung's disease, addressed inadequate biopsies, and complications from rectal biopsy, and 2) pathologic and histologic interpretation of biopsy specimens for the diagnosis of Hirschsprung's disease.
Conclusion:
A well-executed rectal biopsy with expert pathologic evaluation of the specimen remains the gold standard for the diagnosis of Hirschsprung's disease and is the subject of this review.
 
Key words: ganglion cell; Hirschsprung's disease; nerve trunk hypertrophy; pediatric constipation; rectal biopsy
  [Abstract] [Full Text] [PDF]  
Immune neutropenias of infancy and childhood
  Piero Farruggia
 
Background:
Anti-neutrophil antibodies are a wellrecognized cause of neutropenia, producing a potential increase in risk of infection: in the majority of patients antibodies react against antigens located on the IgG Fc receptor type 3b (FcRIIIb), but other target antigens have been identified.
Data sources:
In this review the most important papers of auto and alloimmune neutropenias of infancy and childhood were analyzed. PubMed, Google Scholar and Thompson ISI Web of Knowledge were searched for identifying relevant papers.
Results:
Primary autoimmune neutropenia of infancy is mostly a benign condition with self-limited course, whereas isolated alloimmune neonatal neutropenia or secondary autoimmune neutropenia may be occasionally complicated by severe infections.
Conclusion:
Granulocyte colony stimulating factor is an effective therapy for patients affected by all types of autoimmune and alloimmune neutropenia, even though most of them do not need any therapy.  
 
Key words: immune; infancy; neutropenia
  [Abstract] [Full Text] [PDF]  
The etiology of congenital nephrotic syndrome: current status and challenges
  Jing-Jing Wang, Jian-Hua Mao
 
Background:
Congenital nephrotic syndrome (CNS), defined as heavy proteinuria, hypoalbuminemia, hyperlipidemia and edema presenting in the first 0-3 months of life, may be caused by congenital syphilis, toxoplasmosis, or congenital viral infections (such as cytomegalovirus). However, the majority of CNS cases are caused by monogenic defects of structural proteins that form the glomerular fi ltration barrier in the kidneys. Since 1998, an increasing number of genetic defects have been identifi ed for their involvements in the pathogenesis of CNS, including NPHS1, NPHS2, WT1, PLCE1, and LAMB2.
Data sources:
We searched databases such as PubMed, Elsevier and Wanfang with the following key words: congenital nephrotic syndrome, proteinuria, infants, neonate, congenital infection, mechanism and treatment; and we selected those publications written in English that we judged to be relevant to the topic of this review.
Results:
Based on the data present in the literature, we reviewed the following topics: 1) Infection associated CNS including congenital syphilis, congenital toxoplasmosis, and congenital cytomegalovirus infection; 2) genetic CNS including mutation of NPHS1 (Nephrin), NPHS2 (Podocin), WT1, LAMB2 (Laminin-¦Â2), PLCE1 (NPHS3); 3) Other forms of CNS including maternal systemic lupus erythematosus, mercury poisoning, renal vein thrombosis, neonatal alloimmunization against neutral endopeptidase.
Conclusions:
At present, the main challenge in CNS is to identify the cause of disease for individual patients. To make a definitive diagnosis, with the exclusion of infection-related CNS and maternal-associated disorders, pathology, family history, inheritance mode, and other accompanying congenital malformations are sometimes, but not always, useful indicators for diagnosing genetic CNS. Next-generation sequencing would be a more effective method for diagnosing genetic CNS in some patients, however, there are still some challenges with next-generation sequencing that need to be resolved in the future.
 
Key words: congenital infection; congenital nephrotic syndrome; mono-genetic mutation; next-generation sequencing
  [Abstract] [Full Text] [PDF]  
Meta-analysis:
Clinical significance of FABP2 expression in newborns with necrotizing enterocolitis
  Yu Liu, Liang-Fu Jiang, Rong-Peng Zhang, Wen-Tong Zhang
 
Background:
This meta-analysis aimed to determine the role of human fatty acid binding protein 2 (FABP2) expression in the diagnosis of necrotizing enterocolitis (NEC) of newborns.
Data sources:
Eligible studies for further statistical analysis were identified from various databases including PubMed, Expert Medica Database, Web of Science, Cochrane Library, Google Scholar, China BioMedicine and China National Knowledge Infrastructure. Random effects model was used, and summary standardized mean difference (SMD) with its 95% confi dence interval (CI) was calculated to assess the association of FABP2 expression and NEC.
Results:
Ten articles which included 572 infants (262 infants with NEC and 310 healthy controls) were included in the current meta-analysis. FABP2 showed a positive relationship with NEC of newborns (SMD=2.88, 95% CI=2.09-3.67, P<0.001). And FABP2 expression was higher in patients with advanced stage of NEC (stage III or stage II+III) than in those with early stage of NEC (stage I) (SMD=-0.48, 95% CI=-0.87 to -0.09, P=0.015). Ethnicity-stratifi ed analysis yielded signifi cantly different estimates with a high FABP2 expression in NEC in both Caucasians (SMD=3.16, 95% CI=1.90-4.43, P<0.001) and Asians (SMD=2.57, 95% CI=1.50-3.64, P<0.001). Samplebased subgroup analysis showed that FABP2 expression was positively correlated with neonatal NEC in both urinary- and blood-sample subgroups (all P<0.05).
Conclusion: The results prove that the high FABP2 expression is related to the damage to intestinal cells, which may be a possible early detection marker identifying neonatal NEC.
 
Key words: fatty acid binding protein 2; meta-analysis; necrotizing enterocolitis of newborn; protein expression
  [Abstract] [Full Text] [PDF]  
Original articles:
Effect of zinc supplementation on infants with severe pneumonia
  Xiao Yuan, Su-Yun Qian, Zheng Li, Zhe-Zhe Zhang
 
Background:
Pneumonia is a common respiratory infectious disease in infancy. Previous work shows controversial results on the benefit of zinc supplementation in patients with pneumonia. We conducted this study to investigate serum zinc status amongst infants with severe pneumonia and the clinical impact that zinc supplementation has on those patients with low serum zinc levels.
Methods:
This study design was a non-blinded prospective randomized controlled trial. The study is approved by the Ethics Committees of Beijing Children's Hospital. A total of 96 infants diagnosed with severe pneumonia and hospitalized in the pediatric intensive care unit between November 2011 and January 2012 were enrolled. Enrolled patients were divided into low serum zinc and normal serum zinc group. The low serum zinc group was randomized into treatment and control groups. Only the treatment group received zinc supplementation within 48-72 hours after hospitalization.
Results:
The prevalence of zinc defi ciency on admission was 76.0%. The low zinc level was most apparent in infants between 1 and 3 months of age. The serum zinc level increased in the zinc treatment group and returned to a normal level (median, 53.20 ¦Ìmol/L) on day 12¡À2. There was no statistical difference in the pediatric critic illness score, lung injury score, length of hospital stay, and duration of mechanical ventilation between the zinc treatment group and control group.
Conclusions:
Zinc deficiency is common in infants with severe pneumonia. Normalization of zinc levels with zinc supplementation did not improve clinical outcomes of infants with pneumonia.
 
Key words: infant; pneumonia; supplementation; zinc
  [Abstract] [Full Text] [PDF]  
Determinants of moderate to vigorous physical activity and obesity in children: a structural equation modeling analysis
  Daniel Chi-Shing Yeung, Xin Yuan, Stanley Sai-Chuen Hui, Shingairai Aliifi na Feresu
 
Background:
The determinants of physical activity (PA) and body fatness in Chinese adolescents are rarely examined. This study aimed to investigate the effect of attitude toward PA, screen time, parents' socioeconomic status (SES), and exercise habit on PA and body fatness among Chinese children by using structural equation modeling (SEM) analysis.
Methods:
Data obtained from the second Community Fitness Survey in Hong Kong were utilized, in which students from one secondary school of each of the 18 districts of Hong Kong were recruited. A total of 2517 questionnaires with physical fitness items were successfully distributed to students aged 13-19 years in these districts. Families' SES, parents' exercise habit, children's intention to participate in PA, amount of moderate to vigorous PA (MVPA), screen time, children's attitude toward PA, and children's body fat percentage were measured and analyzed with SEM. The structural equation model was composed of a measurement model and a structural model. The model was tested with Mplus 6. The Chi-square test, root mean square error of approximation, comparative fi t index, and Tucker-Lewis index were calculated to evaluate model fit. The model was then modifi ed based on the model fi t indices.
Results:
Children's intention to participate in PA was a strong predictor of their engagement in MVPA. Parents' exercise habit had both direct and indirect (via attitude) effects on their children's intention to participate in PA. Screen time was not a predictor of body composition. Children's intention to participate in PA directly affected their body composition. Children's attitude toward PA, parents' exercise habit, and SES had significant effects on the children's intention to participate in PA. Furthermore, obesity had a negative effect on the children's attitude toward PA.
Conclusions:
To promote MVPA and prevent obesity in Chinese children of Hong Kong, it is important to design intervention that enhances children's intention and attitude in PA, as well as parent's exercise habits. Tailormade programs that take SES into consideration are also essential. Further studies are necessary to extend the results and test the model in other metropolitan areas in China.
 
Key words: adolescent obesity; behavior; childhood obesity; physical activity; socioeconomic factors
  [Abstract] [Full Text] [PDF]  
Effects of Bifidobacterium supplementation on intestinal microbiota composition and the immune response in healthy infants
  Bing-Bing Wu, Yi Yang, Xiu Xu, Wei-Ping Wang
 
Background:
Intestinal microbiotas are thought to be the most important source of maturational stimuli to the development of the immune system. However, few studies have focused on the development of T helper (Th) 1 immune response and antibody response to vaccinations in healthy infants, especially in a large cohort. Through this randomized, double-blind control trial, we investigated the effects of Bifidobacterium longum BB536 (BB536) supplementation on intestinal microbiota composition and the immune response in term infants.
Methods:
In total, 300 healthy newborns were recruited, randomized and fed formula either supplemented with BB536 or with no supplementation. Stool samples were analyzed at months 2, 4 and 11. The representative cytokine for Th1 [interferon-¦Ã (IFN-¦Ã)] and Th2 [interleukin-4 (IL-4)] secretion cells were measured using enzyme-linked immunospot assay at 4 and 7 months of age. The antibody response to vaccines was measured at months 7 and 11.
Results:
A total of 264 infants completed the study. The amount of bifidobacteria and the bifidobacteria/ Enterobacteriaceae ratio (B/E) were signifi cantly higher in the BB536 supplementation group at months 2 and 4. The number of IFN-¦Ã secretion cells and the ratio of IFN-¦Ã/IL-4 secretion cells were increased in the BB536 supplementation group at 7 months. Moreover, the higher value of B/E in the early stages seems to be related to the increased Th1 response. No difference was observed between groups in the antibody response after vaccination.
Conclusions:
BB536 has positive effects on establishing a healthy intestinal microbiota early in life, and it also plays an important role in improving the Th1 immune response.
 
Key words: intestinal microbiota; probiotics supplementation; term infants; T helper 1/T helper 2 balance; vaccination
  [Abstract] [Full Text] [PDF]  
Detection of respiratory viruses and bacteria in children using a twenty-two target reverse-transcription real-time PCR (RT-qPCR) panel
  Chelsey Ellis, Amita Misir, Charles Hui, Mona Jabbour, Nicholas Barrowman, Jonathan Langill, Jennifer Bowes, Robert Slinger
 
Background:
Rapid detection of the wide range of viruses and bacteria that cause respiratory infection in children is important for patient care and antibiotic stewardship. We therefore designed and evaluated a ready to- use 22 target respiratory infection reverse-transcription real-time polymerase chain reaction (RT-qPCR) panel to determine if this would improve detection of these agents at our pediatric hospital.
Methods:
RT-qPCR assays for twenty-two target organisms were dried-down in individual wells of 96 well plates and saved at room temperature. Targets included 18 respiratory viruses and 4 bacteria. After automated nucleic acid extraction of nasopharyngeal aspirate (NPA) samples, rapid qPCR was performed. RT-qPCR results were compared with those obtained by the testing methods used at our hospital laboratories.
Results:
One hundred fi fty-nine pediatric NPA samples were tested with the RT-qPCR panel. One or more respiratory pathogens were detected in 132/159 (83%) samples. This was significantly higher than the detection rate of standard methods (94/159, 59%) (P<0.001). This difference was mainly due to improved RT-qPCR detection of rhinoviruses, parainfl uenza viruses, bocavirus, and coronaviruses. The panel internal control assay performance remained stable at room temperature storage over a two-month testing period.
Conclusions:
The RT-qPCR panel was able to identify pathogens in a high proportion of respiratory samples. The panel detected more positive specimens than the methods in use at our hospital. The pre-made panel format was easy to use and rapid, with results available in approximately 90 minutes. We now plan to determine if use of this panel improves patient care and antibiotic stewardship.
 
Key words: bacterial infections; pediatric; polymerase chain reaction; respiratory infectious diseases; viral infections
  [Abstract] [Full Text] [PDF]  
Population-based frequency of surfactant dysfunction mutations in a native Chinese cohort
  Yu-Jun Chen, Jennifer Anne Wambach, Kelcey DePass, Daniel James Wegner, Shao-Ke Chen, Qun-Yuan Zhang, Hillary Heins, Francis Sessions Cole, Aaron Hamvas
 
Background:
Rare mutations in surfactant-associated genes contribute to neonatal respiratory distress syndrome. The frequency of mutations in these genes in the Chinese population is unknown.
Methods:
We obtained blood spots from the Guangxi Neonatal Screening Center in Nanning, China that included Han (n=443) and Zhuang (n=313) ethnic groups. We resequenced all exons of the surfactant proteins-B (SFTPB), -C (SFTPC), and the ATP-binding cassette member A3 (ABCA3) genes and compared the frequencies of 5 common and all rare variants.
Results:
We found minor differences in the frequencies of the common variants in the Han and Zhuang cohorts. We did not find any rare mutations in SFTPB or SFTPC, but we found three ABCA3 mutations in the Han [minor allele frequency (MAF)=0.003] and 7 in the Zhuang (MAF=0.011) cohorts (P=0.10). The ABCA3 mutations were unique to each cohort; five were novel. The collapsed carrier rate of rare ABCA3 mutations in the Han and Zhuang populations combined was 1.3%, which is signifi cantly lower than that in the United States (P<0.001).
Conclusions:
The population-based frequency of mutations in ABCA3 in south China newborns is signifi cantly lower than that in United States. The contribution of these rare ABCA3 mutations to disease burden in the south China population is still unknown.
 
Key words: genetic epidemiology; human population genetics; neonatal respiratory distress syndrome; pulmonary surfactant
  [Abstract] [Full Text] [PDF]  
Effect of long-term Helicobacter pylori infection on growth of children: a cohort study
  Celebi Kocaoglu, Ahmet Ozel, Mustafa Cayci, Ece Selma Solak
 
The effects of Helicobacter pylori (H. pylori) infection on growth are a controversial issue. We investigated the effects of long-term H. pylori infection on height and weight in children.
Methods:
A total of 200 children of 7-18 years old suffering from dyspeptic complaints were classified into two groups: H. pylori positive and negative groups, respectively. Whether the infection was impoved was followed up while performing urea breath test, and according to exposure time to the infection, the children were further divided into group 1 (¡Ü1.5 months), group 2 (>1.5-¡Ü6 months) and group 3 (>6 months). Antropometric measurements were obtained and repeated every six months.
Results:
Mean growth velocity scores in the H. pylori positive and negative groups were 0.49¡À3.85 [95% confidence interval (CI): -0.21-1.18] and 1.98¡À4.42 (95% CI: 1-2.96), respectively. The difference between both groups was statistically significant (P=0.012). Mean growth velocity scores in groups 1, 2 and 3 were 0.96¡À3.84, 0.16¡À4.51 and -0.85¡À3.09, respectively. Mean growth velocity scores of group 3 were significantly lower than those of groups 0 and 1 (P=0.005 and P=0.041). The mean weight scores in group 3 were similar to those in group 2, but the scores in group 3 were signifi cantly lower than those in group 1 (-1.75¡À1.05, -1.21¡À1.37 and -0.88¡À1.49, respectively).
Conclusion:
As the duration of exposure is prolonged in children with H. pylori infection, the negative effect of the infection on both height and weight is evident.
 
Key words: antropometry; failure to thrive; growth velocity; Helicobacter pylori
  [Abstract] [Full Text] [PDF]  
Predictive scores for mortality in full-term infants with necrotizing enterocolitis: experience of a tertiary hospital in Southwest China
  Yu Zhang, Ji-Kun Ma, Hong Wei, Xiao-Wen Li, Lu-Quan Li, Jia-Lin Yu
 
Background:
Although many risk factors for mortality of necrotizing enterocolitis (NEC) were investigated, most of them were obtained from preterm infants, and few works focused on the prognostic risk factors in fullterm infants. This study aimed to identify risk factors and develop a prediction score model for mortality in fullterm neonates with NEC.
Methods:
The risk factors were analyzed retrospectively by bivariate and multivariate logistic regression analysis in 153 full-term neonates with NEC, who were hospitalized in Children's Hospital of Chongqing Medical University from 2000 to 2013. A prediction score model was developed according to the regression coeffi cients of risk factors.
Results:
The mortality of the infants was 19.6% (30/153). The non-survivors had a younger age of diagnosis and advanced stage of NEC (P<0.05). They had a higher prevalence of respiratory failure, intestinal perforation, peritonitis and other complications, compared with the survivors (P<0.05). On the day of diagnosis, the nonsurvivors were more likely to have abnormal laboratory indicators than survivors (P<0.05). Age at diagnosis [odds ratio (OR)=0.91, 95% confidence interval (CI)=0.836- 0.99], respiratory failure (OR=2.76, 95% CI=1.10-6.92), and peritonitis (OR=26.36, 95% CI=7.52-173.92) had significant independent contributions to death. A score model predicting death was developed, and the area under the receiver operating characteristic curve was 0.869 (95% CI=0.803-0.935). All infants with scores ¡Ý8 died.
Conclusions:
Younger age at diagnosis, peritonitis, and respiratory failure might be risk factors for the mortality of full-term infants with NEC. Infants with a predictive score of 8 were at high risk for death.
 
Key words: necrotizing enterocolitis; neonate; predictive score model; prognosis
  [Abstract] [Full Text] [PDF]  
Diagnosis and management of fulminant Wilson's disease: a single center's experience
  Yi Tian, Guo-Zhong Gong, Xu Yang, Feng Peng
 
Background: Medical therapy is rarely effective in patients with fulminant Wilson's disease (FWD). Liver transplantation is limited by the lack of donor liver in most patients with FWD at the time of diagnosis. New Wilson's index, model for end-stage liver disease (MELD) and Child-Pugh score are useful tools for decisionmaking of liver transplantation; however, none of them is an independent decisive tool. It is worthwhile to explore a more effective and practical therapeutic strategy and reevaluate the prediction systems for patients with FWD.
Methods: Nine patients with FWD associated with hemolytic crisis and fulminant hepatic failure (FHF) were investigated. The clinical presentation, prognostic score and medical therapies of the patients were analyzed.
Results: In 7 of the 9 patients with FWD who received the comprehensive therapy of corticosteroid, copperchelating agent (dimercaptopropansulfonate sodium) and therapeutic plasma exchange (TPE), 6 patients recovered from FHF. The remaining one had been improved through the comprehensive therapy but died of septicemia 51 days later. Two patients with spontaneous bacterial peritonitis (SBP) died from liver failure in three or five hospital days without plasma exchange or chelating therapy. All of the 9 patients with FWD presented with acute hepatic failure, severe jaundice and mild to severe hemolytic anemia. No marked difference in the incidence of severe hemolytic anemia was detected between the survival and deceased groups. However, the incidence and the degree of hepatic encephalopathy (HE) in the non-survival group were higher than those in the survival group. Unlike the deceased group, the survival group had no complications induced by bacterial infection. Compared to new Wilson's index, Child-Pugh score and MELD score, the variation of prothrombin activity (PTA) between the survival and deceased groups was more evident.
Conclusions: For patients with FWD, the episode of severe hepatic encephalopathy or/and spontaneous bacterial peritonitis indicates worse prognosis, and PTA is a recommendable predictor. An emergent liver transplantation should be considered for patients whose PTA is below 20%, or for those with severe HE or/ and SBP. The comprehensive therapy of corticosteroid, copper-chelating agent and TPE is effective for patients without SBP and whose PTA is higher than 20%.
 
Key words: decoppering; dimercaptopropansulfonate sodium; fulminant hepatic failure; therapeutic plasma exchange; Wilson's disease
  [Abstract] [Full Text] [PDF]  
Thyroid dysfunction and developmental anomalies in first degree relatives of children with thyroid dysgenesis
  Lakshminarasimhan Sindhuja, Devi Dayal, Kushaljit Singh Sodhi, Naresh Sachdeva, Anish Bhattacharya
 
Background:
Familial clustering in patients with permanent congenital hypothyroidism (CH) caused by thyroid dysgenesis (TD) has been reported in developed countries. There is no information on familial TD from developing countries.
Methods:
A total of 312 first degree relatives belonging to 80 families of children with TD (group 1) and 40 families of age-matched normal children (group 2) were screened by thyroid ultrasonography, serum total thyroxine (T4) and thyroid stimulating hormone (TSH).
Results:
Thyroid scintigraphy revealed agenesis in 78.7% of the patients, ectopic gland in 15%, and hypoplasia in 6.2%. The mean thyroid volumes were similar in parents and siblings of both groups. Eight (10.6%) mothers in group 1 were identified to have thyroid hypoplasia as compared with none in group 2 (P=0.03). Serum TSH was significantly higher in group 1 than in group 2 (P=0.004). Sixteen (7.8%) subjects (6 mothers, 5 fathers, and 5 siblings) in group 1 were found to have subclinical hypothyroidism as compared to none in group 2 (P<0.05). Four families were identified to have thyroid developmental anomalies and abnormal thyroid functions accounting for 5% of cases of familial TD in our cohort.
Conclusions:
Thyroid developmental anomalies and thyroid function abnormalities are more frequent in first degree relatives of children with TD as compared with a control population. These findings suggest that possibly there is a genetic component of TD in Indian patients.
 
Key words: congenital hypothyroidism; familial occurrence; first degree relatives; thyroid developmental anomalies; thyroid dysgenesis
  [Abstract] [Full Text] [PDF]  
Genetic analysis of Italian patients with congenital tufting enteropathy
  Maria d'Apolito, Daniela Pisanelli, Flavio Faletra, Ida Giardino, Maddalena Gigante, Massimo Pettoello-Mantovani, Olivier Goulet, Paolo Gasparini, Angelo Campanozzi
 
Background:
Congenital tufting enteropathy (CTE), an inherited autosomal recessive rare disease, is a severe diarrhea of infancy which is clinically characterized by absence of inflammation and presence of intestinal villous atrophy. Mutations in the EpCAM gene were identified to cause CTE. Recent cases of syndromic tufting enteropathy harboring the SPINT2 (19q13.2) mutation were described.
Methods:
Four CTE Italian patients were clinically and immunohistochemically characterized. Direct DNA sequencing of EpCAM and SPINT2 genes was performed.
Results:
All patients were of Italian origin. Three different mutations were detected (p.Asp219Metfs*15, Tyr186Phefs*6 and p.Ile146Asn) in the EpCAM gene; one of them is novel (p.Ile146Asn). Two patients (P1 and P2) showed compound heterozygosity revealing two mutations in separate alleles. A third patient (P3) was heterozygous for only one novel EpCAM missense mutation (p.Ile146Asn). In a syndromic patient (P4), no deleterious EpCAM mutation was found. Additional SPINT2 mutational analysis was performed. P4 showed a homozygous SPINT2 mutation (p.Y163C). No SPINT2 mutation was found in P3. CLDN7 was also evaluated as a candidate gene by mutational screening in P3 but no mutation was identified.
Conclusions:
This study presented a molecular characterization of CTE Italian patients, and identified three mutations in the EpCAM gene and one in the SPINT2 gene. One of EpCAM mutations was novel, therefore increasing the mutational spectrum of allelic variants of the EpCAM gene. Molecular analysis of the SPINT2 gene also allowed us to identify a SPINT2 substitution mutation (c.488A>G) recently found to be associated with syndromic CTE subjects.
 
Key words: diarrhea; gastroenterology; gene mutation
  [Abstract] [Full Text] [PDF]  
Crescentic glomerulonephritis in children: a single centre experience
  Sare G¨¹lfem Özl¨¹, Aysun Çaltık, Özlem Aydoğ, Mehmet B¨¹lb¨¹l, G¨¹lay Demircin, Evrim Çakıcı, Nil¨¹fer Arda, Ayşe Öner
 
Background:
Crescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function.
Methods:
We evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively.
Results:
The mean age of the patients was 130.86¡À33.77 months. The mean duration of symptoms prior to diagnosis was 26¡À12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fi brous crescents (P=0.015).
Conclusion:
The outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.
 
Key words: glomerulonephritis; renal biopsy; renal failure
  [Abstract] [Full Text] [PDF]  
Long-term outcome of laparoscopic Nissen-Rossetti fundoplication versus Thal fundoplication in children with esophageal hiatal hernia: a retrospective report from two children's medical centers in Shanghai
  Ji-Meng Hu, Ming Hu, Ye-Ming Wu, Jun Wang, Zhi-Long Yan, Chi Zhang, Wei-Hua Pan, Hao Xia
 
Background:
This study was undertaken to investigate the intraoperative and postoperative complications, efficacy and outcome of two laparoscopic fundoplications for the treatment of esophageal hiatal hernia in children.
Methods:
To find a rational procedure, we performed a retrospective analysis of 136 children with esophageal hiatal hernia who underwent laparoscopic Nissen-Rossetti or Thal fundoplication at two children's hospitals in Shanghai over 13 years. The median follow-up time of the children was 42 months (range: 1-138 months). Their age varied from 1 month to 11 years (median: 18.6 months).
Results:
All the children underwent laparoscopic fundoplications (72 cases of Nissen-Rossetti and 60 cases of Thal fundoplication) and 4 children converted to open surgery. The mean age of the children at the time of operation was 1.6¡À1.9 years, and the mean weight was 9.1¡À5.6 kg. Gastroesophageal reflux was significantly more severe after a Thal fundoplication (P=0.003) and slight esophageal stenosis was significant after a Nissen-Rossetti fundoplication (P=0.02). The recurrent rate of hiatal hernia was 2.8% (2/72) after Nissen-Rossetti fundoplication in contrast to 5% (3/60) after Thal fundoplication. No death occurred after surgery.
Conclusions:
There was no statistical difference of recurrence between laparoscopic Nissen-Rossetti and Thal fundoplication in the long-term outcomes. The rate of slight dysphagia was higher in the Nissen-Rossetti group. The Thal group had a significantly higher recurrence rate of gastroesophageal reflux. There still exited learning curve for this procedure. The incidence rate of complications is signifi cantly related to the profi ciency of pediatric surgeon.
 
Key words: esophageal hiatal hernia; fundoplication; laparoscopy
  [Abstract] [Full Text] [PDF]  
Use of off-label nephrology-related drugs in hospitalized pediatric patients: a retrospective study
  Marchella Yasinta, Ruo-Chen Che, Cai-Yu Hu, Xiang-Hui Du, Gui-Xia Ding, Song-Ming Huang, Ying Chen, Ai-Hua Zhang
 
Background:
The information about the use of off-label drugs in pediatric nephrology is still lacking, which leads to increased adverse reactions and medical disputes. We retrospectively analyzed the use of off-label drugs in the in-patient ward of the nephrology department of Nanjing Children's Hospital, China in order to provide more complete information about the use of drugs for children.
Methods:
Proportional stratified random sampling was applied to select patients with renal diseases aged 1 month to 18 years, who were admitted to the hospital from October 1, 2012 to September 30, 2013. All nephrologyrelated drugs prescribed in the hospitalization period and take-home drugs prescribed on discharge were recorded and evaluated as off-label drugs or not from three different perspectives: person-time, prescription, and drug category.
Results:
From 385 person-times of patients with 1424 prescriptions, according to the ratio between off-label drugs and person-times, drug prescriptions, and drug products, the rates of off-label drugs were 40.78%, 16.64%, and 31.43%, respectively. Low-molecular-weight heparin, alfacalcidol and diltiazem were the most commonly used off-label drugs. Infants and younger children were the high-risk population of off-label drug use. The high rate off-label nephrology-related drug use in children was mainly related to lacking clinical research into drugs in children and the pace of drug label's revision, which cannot follow the development of medical science.
Conclusions:
Approximaely half of pediatric patients with renal diseases are usually prescribed with off-label nephrology-related drugs. Analyzing the off-label conditions from different perspectives may lead to various results. More clinical research into drugs for infants and younger children is needed so as to update drug descriptions.
 
Key words: in-patient; kidney; off-label drug; pediatrics
  [Abstract] [Full Text] [PDF]  
Case reports:
Obstructive uropathy and severe acute kidney injury from renal calculi due to adenine phosphoribosyltransferase deficiency
  Siew Le Chong, Yong Hong Ng
 
Background:
Adenine phosphoribosyltransferase (APRT) deficiency is an uncommon genetic cause of chronic kidney disease due to crystalline nephropathy.
Methods:
A case of a Chinese boy with APRT defi ciency presenting with severe acute kidney injury secondary to obstructive uropathy from multiple renal calculi was reviewed.
Results:
The patient underwent staged removal of the calculi. Infrared spectrometry of the renal calculi showed 2,8-dihydroxyadenine. APRT deficiency was confirmed with abolished APRT enzyme activity in red blood cells. He was started on allopurinol and low purine diet with complete resolution of the residual calculi.
Conclusion:
APRT defi ciency should be considered in patients with multiple radiolucent renal calculi.
 
Key words: 2,8-dihydroxyadenine; acute renal failure; adenine phosphoribosyltransferase defi ciency; urolithiasis
  [Abstract] [Full Text] [PDF]  
Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis
  Ahmet Celik, Sibel Tiryaki, Sukran Darcan, Orkan Ergun
 
Splenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad.
Methods:
We report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome.
Results:
The patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, "spleen-like" tissue attached to the gonad induced splenogonadal fusion, which was confi rmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face.
Conclusion:
Splenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities.
 
Key words: diagnostic laparoscopy; splenogonadal fusion; undescended testis
  [Abstract] [Full Text] [PDF]  
   
 
 
 
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