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Vol 3, No 4
Vol 3, No 4 November 2007 ISSN 1708-8569
 
Review articles
Original articles
Case report
   
Review articles:
Clinical use of bisphosphonates in children
  Laura Somalo, Fernando Santos
  Author Affiliations: Department of Pediatrics, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain (Somalo L, Santos F); Department of Medicine, Universidad de Oviedo, Oviedo, Asturias, Spain (Santos F); Instituto Universitario de Oncolog¨ªa del Principado de Asturias, Oviedo, Asturias, Spain (Santos F)

Corresponding Author: Fernando Santos, MD, PhD, Pediatria. Facultad de Medicina. c/ Julian Claveria 6, 33006 Oviedo, Asturias, Spain (Tel: +34985102728; Fax: +34985103585; Email: fsantos@uniovi.es)

Background: Bisphosphonates are being increasingly and successfully used to prevent bone fractures and treat bone pain in children with severe osteoporosis from different origins. The purpose of this article is to update the clinical use of bisphosphonates in pediatric patients and to present the authors' experience with the intravenous administration of pamidronate in osteopenic children.  

Data sources: PubMed database was used to collect publications reporting the utilization of bisphosphonates in children. The medical records of five pediatric patients treated in our unit with bisphosphonates were also reviewed.  

Results: A growing experience is accumulating with the cyclical intravenous administration of pamidronate in children with osteogenesis imperfecta. Bisphosphonates may also be useful in the prevention and treatment of vascular calcification in patients with chronic renal failure, although no data on children in this clinical setting are available.  

Conclusions: Intravenous bisphosphonates are well tolerated, even in infants and small children, and represent a promising therapeutic tool to prevent the development of bone fractures and to improve the well-being of osteoporotic children. A number of questions about the precise clinical indications for bisphosphonates' administration, the duration of the treatment, the best way to monitor its effectiveness and to early detect toxic effects remain to be answered. 

Key words: bisphosphonates; pamidronate; osteoporosis; osteogenesis imperfecta; calcification; chronic renal failure; hypercalcemia; bone diseases; kidney diseases

                   World J Pediatr 2007;3(4):245-253

  [Abstract] [Full Text] [PDF]  
Relationship of
  Tomasz Karski, Jaroslaw Kalakucki, Jacek Karski
  Tomasz Karski, Jaroslaw Kalakucki, Jacek Karski

Lublin, Poland

Author Affiliations: Department of Pediatric Orthopedics and Rehabilitation, Skubiszewski Medical University of Lublin, Poland; University Pediatric Hospital, Chodźki St. 220-093 Lublin, Poland (Karski T, Kalakucki J, Karski J)

Corresponding Author: Tomasz Karski, MD, PhD, Department of Pediatric Orthopedics and Rehabilitation, Skubiszewski Medical University of Lublin, Poland; University Pediatric Hospital, Chodźki St. 220-093 Lublin, Poland (Tel/Fax: 0048 81/741 56 53; Email: tkarski@dsk.lublin.pl)

Background: In this article, we describe early clinical symptoms of "syndrome of contractures" (Mau) in newborns and children as well as its relations to the so-called idiopathic scoliosis.

Data sources: Two different groups of children were analyzed. The first group consisted of 60 children. They were first examined in newborn and infancy period with hip dysplasia, torticollis or deformities of feet or others. They were examined again at the age of 5-7 years in 1996. All the 60 examined children, who were found primary symptoms of "syndrome of contractures" in histories, showed first symptoms of the so-called idiopathic scoliosis later at the age of 5-7 years. The other group in our research included 123 newborns born in the period of 1998-2000. In this group we analyzed with emphasis on the mothers' conditions during the pregnancy period, the children's conditions in infancy period, and their connection with symptoms of "syndrome of contractures".

Results: "syndrome of contractures" could explain the predominance of female patients with scoliosis, sides of curves (lumbar¡ªleft convex, thoracic¡ªright convex). What we see "left sided syndrome of contractures" mostly can explain the clinical picture of so-called idiopathic scoliosis: the side of curves¡ªlumbar left, thoracic right, side of the rib hump (right side of thorax), progression in the acceleration period of growth, and the sensibility to new rehabilitation exercises.

Conclusions: Newborns with clinical signs of "syndrome of contractures" require further spine examinations at the age of 3-6 years to detect the danger of oncoming scoliosis and to introduce rehabilitation management in the program of the "causal neo-prophylaxis".

Key words: "syndrome of contractures"; so-called idiopathic scoliosis; etiology; abduction contracture of the right hip

                                                                                                          World J Pediatr 2007;3(4):254-259

  [Abstract] [Full Text] [PDF]  
Original articles:
Focal segmental glomerulosclerosis: a single center study of over two decades
  James C. M. Chan
  Author Affiliations: University of Vermont, The Barbara Bush Children's Hospital, Maine Medical Center, Portland, Maine, USA (Chan JCM)

Corresponding Author: James C. M. Chan, MD, University of Vermont; The Barbara Bush Children's Hospital, Maine Medical Center, 22 Bramhall Street, Portland, Maine 04102-3175, USA (Tel: 1-207-662-2439; Fax: 1-207-662-6272; Email: chanj@mmc.org)

Background: By sharing our quarter century of care on children with kidney biopsy-proven focal segmental glomerulosclerosis (FSGS), we hope to advance early recognition of this condition with its changing demographics in recent years and examine promising treatment modalities to slow its rate of progression. 

Methods: Children fitting entry and exclusion criteria, who presented to our regional nephrology center, were enrolled into this retrospective study. The same criteria for kidney biopsies were uniformly applied under the same nephrologists in over 25 years of practice. Patients exited the study at the initiation of dialysis/transplantation or death. Kidney survival rate was determined by the Kaplan-Meier method.

Results: In 43 children (21 females and 22 males) enrolled, 15 were white and 28 black. The peak age at presentation was 10 to 15 years. Hypertension and heavy proteinuria were frequently seen as presenting features. Almost half of the patients showed serum creatinine concentrations minimally elevated above normal, indicating relatively early diagnosis of FSGS in the study population. Treatment consisted of one or more of the following medications: oral alternative-day prednisone, angiotensin converting enzyme inhibitors or angiotensin receptor blockers, vitamin E, and intravenous methylprednisolone with cyclophosphamide or cyclosporine A.

Conclusions: In this study, with the early interest in FSGS, an opportunity became available to use the different treatment options over more than 2 decades. With the present combined therapy, the kidney survival was no better than the therapeutic regimen described earlier by others. Therefore attention must be focused on the efficacy and side effects of treatments which have been called into question: cyclophosphamide and intravenous methylprednisolone. We recommend a modified treatment regimen, which is cost effective and with minimal side effects.

Key words: focal segmental glomerulosclerosis; African-American children; natural history; therapy; outcome

                                                                                                                       World J Pediatr 2007;3(4):260-26
  [Abstract] [Full Text] [PDF]  
Role of parents in determining children's physical activity
  Joan R Griffith, Jody L Clasey, Jason T King, Starr Gantz, Richard J Kryscio,
  Author Affiliations: Department of Pediatrics (Griffith JR, Gantz S, Bada HS); Department of Kinesiology and Health Promotion (Clasey JL, King JT); Department of Statistics (Kryscio RJ), University of Kentucky, Lexington, Kentucky, USA

Corresponding Author: Joan R. Griffith, MD, Harvard Medical School, 164 Longwood Avenue, Room 318, Boston, MA 02215, USA (Tel: 617-432-2278; Fax: 617-432-3834; Email: joanrgriffith@earthlink.net)

Background: Parents' physical activity was reported to influence children's physical activity. We hypothesized that parents of 10- to 14-year-old children could influence their children's level of physical activity in ways other than providing logistical support.

Methods: Clinical observational study was conducted in a General Clinical Research Center. Normal weight (body mass index [BMI] ¡Ü85th percentile) and at-risk of overweight (BMI >85th percentile) children aged 10 to 14 years were recruited. Sports and leisure indices were derived from the results of Baecke physical activity questionnaires administered to parent-child dyads. Children's habitual activity was determined by a 7-day pedometer log.

Results: Of 109 subjects enrolled, 48% were normal weight and 52% were at-risk of overweight. A significantly higher proportion of normal weight children (67%) versus at-risk of overweight children (49%; P=0.02, adjusted intrafamily correlation) reported patterning their physical activity after that of an adult. The mother's physical activity was more beneficial than the father's in influencing children to begin and continue physical activity.

Conclusions: The influence of parents (predominantly mothers) on their children's physical activity extends beyond enrolling children in activities and providing them with transportation to and from those activities. These findings suggest that primary prevention and intervention strategies for those at-risk of overweight children should be directed at increasing not only children's physical activity but also that of their parents, especially mothers.

Key words: exercise; at-risk of overweight; pediatric; physical activity

World J Pediatr 2007;3(4):265-270

  [Abstract] [Full Text] [PDF]  
Prevalence of fragile X syndrome among school-age Egyptian males
  Nagwa A Meguid, Ehab R Abdel-Raouf, Ahmed A Dardir, Mostafa K El Awady
  Author Affiliations: Department of Research on Children with Special Needs, National Research Center, Cairo, Egypt (Meguid NA, Abdel-Raouf ER, Dardir AA); Department of Biomedical Technology, National Research Center, Cairo, Egypt (El Awady MK)

Corresponding Author: Nagwa A Meguid, Department of Research on Children with Special Needs, National Research Center, Tahrir St., Cairo, Egypt (Tel: +20 12 331 6372; Fax: +20 2 337 0931; +20 2 760 1877; Email: meguidna@yahoo.com)

Background: Fragile-X syndrome (FXS) is the most common inherited form of intellectual disability.  Population-based studies have suggested that the prevalence of the full mutation ranges from 1/3717 to 1/8918 Caucasian males in the general population. The present study is the output of a project aimed at identifying the prevalence rate of fragile-X males in Egypt. A two-step selection with questionnaire and photography was done by trained health visitors and social workers. 

Methods: A total of 20 500 males were screened. The original work involved inhabitants of the three different large governorates: Cairo (Shobra and Rod El-Farag districts), Giza (Dokki district) and Suez (Suez district). The participants included students of primary and high schools. According to school records, parent reports, and clinical examinations, 130 males were selected and subjected to molecular analysis after informed consent was obtained from care givers.

Results: The prevalence of FXS mutation among Egyptian males was 0.9 per 1000. Moreover, it was 6.4% among mentally subnormal males.

Conclusions: The high prevalence of FXS necessitates special education for affected children. There is currently no cure for FXS, however, an individualized treatment plan, beginning during preschool years, can help affected children to reach their full potential.

Key words: Egypt; fragile X syndrome; males; prevalence

             World J Pediatr 2007;3(4):271-275

  [Abstract] [Full Text] [PDF]  
Expression of APAF1 gene during 3T3-L1 preadipocyte differentiation and regulative role of tumor necrosis factor-alpha
  Hong-Qi Fan, Xi-Rong Guo, Rong-Hua Chen, Yu-Hui Ni, Bin Wang, Min Zhang,
  Author Affiliations: Nanjing Medical University Affiliated Nanjing Maternal & Children Health Hospital, Nanjing 210004, China (Fan HQ, Guo XR, Zhang M); Institute of Pediatrics, Nanjing Medical University, Nanjing 210029, China (Fan HQ, Guo XR, Chen RH, Ni YH, Wang B, Liu F, Gu N, Qiu J)

Corresponding Author: Xi-Rong Guo, PhD, Nanjing Medical University Affiliated Nanjing Maternal & Children Health Hospital, Nanjing 210004, China; Institute of Pediatrics, Nanjing Medical University, Nanjing 210029, China (Tel: 86-25-8686-2996; Email: xrguo@njmu.edu.cn)

Background: The size and number of adipocytes are believed to play a key role in the pathophysiology of obesity. The former mainly refers to the differentiation of adipocytes and the latter principally refers to the proliferation and apoptosis of adipocytes. Our cDNA microarray assay had, compared to normal rats, suggested that apoptotic protease activating factor 1 (APAF1) gene expression level was lower in the adipose tissue of diet-induced obese (DIO) rats. This study was undertaken to investigate the changes of APAF1 gene expression during the differentiation of 3T3-L1 preadipocytes (0-10 days), and to analyze the regulative role of tumor necrosis factor-alpha (TNF-¦Á) on APAF1 gene expression in matured 3T3-L1 adipocytes.

Methods: 3T3-L1 preadipocytes were cultured in vitro and differentiated into matured adipocytes by insulin, 3-isobutyl-1-methyxanthine (MIX), and dexamethasone (DEX). Oil red O dyeing was used to identify the differentiation of 3T3-L1 preadipocytes. To further evaluate the linkage between the APAF1 gene and 3T3-L1 cells differentiation/de-differentiation, TNF-¦Á which can promote the matured adipocytes to de-differentiation was incubated with 3T3-L1 matured adipocytes. TNF-alpha (1.0 ng/ml) was added into the culture medium of differentiated 3T3-L1 cells (day 10) for various periods (2, 6, 12, 24 hours). Total RNA and protein of these cells were then extracted. The level of APAF1 gene mRNA was evaluated by RT-PCR. Western blot was used to detect the protein level of APAF1.

Results: During the differentiation of 3T3-L1 preadipocytes, the down-regulation of APAF1 gene expression was significant (P<0.05). TNF-¦Á treatment significantly up-regulated the expression of the APAF1 gene in adipocytes in a time-dependent way.

Conclusions: The APAF1 gene may play a role in the differentiation of preadipocytes and TNF-¦Á related adipocytes and this finding highlights that the APAF1 gene has a potential role in the pathophysiology of obesity.

Key words: APAF1; preadipocyte; differentiation; TNF-¦Á                                      

World J Pediatr 2007;3(4):276-281

  [Abstract] [Full Text] [PDF]  
Atopy in young children with asthma
  Li Xiang, Qi Zhang, Kun-Ling Shen
  Author Affiliations: Center of Asthma, Beijing Children's Hospital Affiliated to Capital University of Medical Sciences, Beijing 100045, China (Xiang L, Zhang Q, Shen KL)

Corresponding Author: Kun-Ling Shen, MD, Center of Asthma, Beijing Children's Hospital Affiliated to Capital University of Medical Sciences, Beijing 100045, China (Tel: 86-10-68028401 ext 2894; Fax: 86-10-68011503; Email: kunling_shen@hotmail.com)

Background: A close relationship between outcome in patients with early-onset asthma and atopy has been reported. Allergic sensitization in early life seems to be an important risk factor for subsequent persistent asthma during childhood and adulthood. It might be valuable to evaluate atopy in patients with early-onset asthma in order to predict prognosis and take early intervention.

Methods: Clinical data of 62 asthmatic children under 4 years of age were collected. The atopy status of each patient was determined by both personal allergic history (eczema and/or allergic rhinitis) and specific diagnosis of allergens (screening tests of fx5E, mx2 and Phadiatop conducted by fluoroenzyme-immunometric assay using the UniCAP100 system). The total serum IgE level was also measured. Logistic regression was used to analyze the effect of clinical characteristics on allergic sensitization.

Results: There were 74.2% children who reported personal history of atopy and 33.9% reported parents' history of atopy based on the clinical data. The positive rates of fx5E, mx2, and Phadiatop were 40.3%, 14.5%, and 14.5% respectively. The total rate of allergic sensitization was 46.8% and the rate of sensitization to inhalant allergens was 24.2%. The allergic history of parent(s), the sensitization to food allergen, the age of first wheezing attack, and the total serum IgE level were main factors influencing sensitization to inhalant allergens.

Conclusion: The asthmatic history of parent(s), the sensitization to food allergens, the age of first wheezing attack above 2 years and the significantly higher total serum IgE level may increase the possibility of sensitization to inhalant allergens in asthmatic children under 4 years of age.

Key words: asthma; atopy; children

                 World J Pediatr 2007;3(4):282-285

  [Abstract] [Full Text] [PDF]  
Diagnosis of tracheal bronchus in children
  Yue-Jie Zheng, Ji-Kui Deng, Dao-Zhen Zhang, Yun-Geng Gen
  Author Affiliations: Department of Respiratory Diseases, Shenzhen Children's Hospital, Shenzhen 518026, China (Zheng YJ, Deng JK, Zhang DZ, Gen YG)

Corresponding Author: Yue-Jie Zheng, Shenzhen Children's Hospital, Shenzhen 518026, China (Tel: 86-755-83936199; Fax: 86-755-83936148; Email: yuejiez@sina.com).

Background: Tracheal bronchus is defined as an abnormal bronchus that comes directly off the lateral wall of the trachea above the carina and goes toward the upper-lobe territory. This congenital anomaly may be asymptomatic or present with such symptoms as stridor, cough, wheezing, persistent or recurrent upper-lobe pneumonia, atelectasis or air trapping. This retrospective study was undertaken to summarize the characteristics of tracheal bronchus in children.

Methods: The clinical features of 7 patients with tracheal bronchus were retrospectively analyzed. Of these patients, 2 were initially diagnosed with tracheal bronchus by chest CT and later confirmed bronchoscopically, and 5 by bronchoscopic examination.

Results: Tracheal bronchus was detected in 7 (1.8%) of the 396 patients receiving bronchoscopy. It occurred at the right lateral wall of the trachea in all the 7 patients. Four patients had retained secretions in the tracheal bronchus, which may result in the symptoms of cough, wheezing, and tracheal or bronchial obstruction. Three patients presented with symptoms irrelevant to tracheal bronchus: 1 patient was complicated by laryngomalacia and tetralogy of Fallot, 1 by tracheomalacia, tracheal stenosis and ventricular septal defects, and 1 had mucus plugs in the left lower bronchus.

Conclusion: Tracheal bronchus is a common congenital anomaly in children receiving bronchoscopic examination. More than 50% patients have relevant symptoms. Bronchoscopy is definitely a diagnostic method for tracheal bronchus.

Key words: bronchus; trachea; child; bronchoscopy; abnormality

World J Pediatr 2007;3(4):286-289
  [Abstract] [Full Text] [PDF]  
Detection of minimal residual disease in pediatric patients with acute lymphoblastic leukemia and its prognostic significance
  Yan Bai, Hui Yu, Wen Lin, Dong-Feng Zhou, Yan Xiao, Xiao-Yan Wu,
  Yan Bai, Hui Yu, Wen Lin, Dong-Feng Zhou, Yan Xiao, Xiao-Yan Wu,

Run-Ming Jin, Hong-Bao Fei

Wuhan, China

Author Affiliations: Department of Pediatrics, Union Hospital, Huazhong University of Science and Technology, Wuhan 430022, China (Bai Y, Yu H, Lin W, Zhou DF, Xiao Y, Wu XY, Jin RM, Fei HB)

Corresponding Author: Yan Bai, MD, Department of Pediatrics, Union Hospital, Huazhong University of Science and Technology, Wuhan 430022, China (Tel: 86-27-85726002; Fax: 86-27-85726002; Email: swcathy32@yahoo.com.cn)

Background: At diagnosis, leukemic cell burden reaches 1012 in patients with acute lymphoblastic leukemia (ALL). When patients get the first complete remission (CR) after induction chemotherapy, less than 5% of lymphoblasts are found in bone marrow smear by light microscopy. But an amount of 109 or less residual leukemic cells may be left, thus the disease is known as minimal residual disease (MRD), which is proven to be responsible for clinical relapse. Detection and quantification of MRD in ALL patients after chemotherapy might improve therapeutic results and prognosis. 

Methods: Polymerase chain reaction (PCR) and nested-PCR were used to detect rearranged immunoglobulin heavy-chain (IgH) CDRIII gene and T lineage cell receptor (TCR) V¦Ä2D¦Ä3 gene for 71 ALL patients. Cerebral spinal fluid (CSF) samples from 67 patients at CR were also detected by the same methods. Leukemic clones were quantified by limiting dilution analysis.

Results: IgH CDRIII rearrangements were found positive in 43 (82.7%) of 52 patients with B lineage ALL and 3 (15.8%) of 19 patients with T lineage ALL. TCR V¦Ä2D¦Ä3 positive rearrangements were present in 17 (32.7%) patients with B lineage ALL and 12 (63.2%) with T lineage ALL. MRD was quantified with the nested-PCR through limiting dilution of DNA samples from these rearrangement positive patients. Patients with MRD level above 2¡Á10-3 in the first CR were found to have a high relapse rate and this situation was also found in patients with a slow decrease or a high level of MRD. Whereas patients with undetectable or detected less than 2¡Á10-5 level of MRD in the first CR had a good prognosis. Of the 67 CSF samples, 9 were positive for IgH CDRIII rearrangement and 4 positive for TCR V¦Ä2D¦Ä3 rearrangement; 5 of them eventually developed central nervous system leukemia.

Conclusions: The relationship between MRD detection and outcome is significant for ALL patients. Early quantification of leukemic cells after chemotherapy may be an effective strategy for predicting ou tcome and hence individualizing ALL treatment in childhood.

Key words: minimal residual disease; acute lymphoblastic leukemia; polymerase chain reaction; immunoglobulin heavy-chain; T lineage cell receptor; gene rearrangement

 

World J Pediatr 2007;3(4):290-294

  [Abstract] [Full Text] [PDF]  
Massage and motion training for growth and development of infants
  Jin Jing, Xiu-Hong Li, Ling-Ying Feng, Qing Wu, Zi-Cai Wang, Shu-Ping Zeng,
  Author Affiliations: Faculty of Maternal and Child Health, Public Health School, Zhongshan University, Guangzhou 510080, China (Jin J, Li XH); Children's Hospital of Fudan University, Shanghai 200032, China (Feng LY); Foshan Maternity and Child Health Hospital, Foshan 528000, China (Wu Q); The Sixth People Hospital of Shanghai, Shanghai 200233, China (Wang ZC); Zhuhai Maternity and Child Health Hospital, Zhuhai 519000, China (Zeng SP); Shanghai Changning Maternity and Infant Health Institute, Shanghai  200052, China (Gu YP); Guangdong Women's and Children's Hospital and Health Institute, Guangzhou 510000, China (Cai XM)

Corresponding Author: Jin Jing, PhD, Faculty of Maternal and Child Health, Public Health School, Zhongshan University, Guangzhou 510080, China (Tel: 86-20-87333526; Fax: 86-20-87330446; Email: jingjin@mail.sysu.edu.cn)

Background: Massage and motion training are considered to promote physical and mental health of infants. This study was conducted to elucidate their effect on the early development of normal infants. 

Methods: Infants were randomly assigned to two groups according to their age: 0-month group and 6-month group. Growth and development of all infants were evaluated regularly. For the 0-month group, 90 infants were included in the experimental group and 90 in the control group. After one year, the number of infants decreased to 54 in the experimental group and 62 in the control group because of the lost to follow up. For the 6-month group, the number of infants in the experimental group was 62, and that in the control group was 52. After half a year, the number of infants in the experimental group was 52, and that in the control group was 39 because of lost to follow up. Infants in the experimental group received massage and motion training. Growth and development of all infants were evaluated regularly and compared between the two groups.

Results: Infants in the experimental group who received massage and motion training in the newborn period had a higher level in length and body weight than those in the control group in the first 6 months of life. At age of 6 months, the developmental quotient (DQ) of the experimental group was significantly higher than that of the control group. At age of one year, the DQ of the experimental group was significantly higher than that of the control group. For the infants receiving motion training beginning from 6-month old, the body length and weight of infants in the experimental group were slightly higher than those of infants in the control group at age of 12 months. Significant difference was found in the body length. Also the DQ of adaptive behavior and language was significantly higher than that of infants in the control group.

Conclusions: Massage and motion training can significantly promote the physical and intelligent develop-ment of infants beginning from birth or at age of 6 months.

Key words: infant; massage; motion; growth; development

World J Pediatr 2007;3(4):295-299

  [Abstract] [Full Text] [PDF]  
Postoperative complications and their management after arterial switch operation in infants with transposition of great arteries
  Li-Xing Zhu, Lin-Hua Tan, Xiao-Jun He, Cai-Yun Zhang, Yan-Ping Yu, Ze-Wei Zhang
 

Author Affiliations: Surgical ICU, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Zhu LX, Tan LH, He XJ, Zhang CY, Yu YP, Zhang ZW)

Corresponding Author: Lin-Hua Tan, MD, Surgical ICU, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Tel: 86-571-87061007; Fax: 86-571-87033296; Email: linhua_tan@yahoo.com)

Background: Arterial switch operation (ASO) has been optimal for children with transposition of great arteries (TGA) in either simple or complex form with an excellent survival rate. This operation was introduced late in China, but there has been a decreasing mortality in recent years. Optimizing the postoperative management has been essential to improve the survival rate after ASO. This study summarizes the experience in the management of the postoperative complications after ASO.

Methods: Twenty-eight infants with TGA underwent ASO from January 2004 through December 2006. These patients aged 1-70 days (median, 6 days) had a body weight of 3.36¡À0.57 kg on average. Before operation, continuous intravenous infusion of prostaglanding E1 was routinely used to keep the ductus open with a SpO2 of 75%-90%. Ten patients required tracheal intubation and mechanical ventilation. Two patients underwent emergency ASO under general anesthesia and low-flow cardiopulmonary bypass or hypothermic circulatory arrest. All the 28 patients were further treated with modified ultrafiltration and delayed sternal closure after cessation of cardiopulmonary bypass. After ASO, they were on circulatory and respiratory support, with antibiotics, nutritional supplement in the ICU.

Results: The patients had a cardiopulmonary bypass time of 167¡À32 minutes and an aortic cross-clamping time of 101¡À24 minutes. Delayed sternal closure was carried out on 3.63¡À1.49 days after ASO. They had a mechanical ventilation of 5.89¡À3.02 days and an ICU stay of 10.12¡À3.25 days. There were 5 deaths after ASO with a mortality rate of 17.9%. Fifteen patients developed low cardiac output syndrome. In 12 patients presenting with cardiac arrhythmias, 9 had paroxysmal supraventricular tachycardia, 1 had frequent ventricular premature beats, 1 had ventricular tachycardia, and 1 had ventricular fibrillation. One patient suffered from pulmonary hypertensive crisis. Three patients with major bleeding and tamponade required emergency mediastinal exploration and finally survived. There were 6 patients with ventilator-associated pneumonia, 6 with delayed incision healing, and 1 with chylothorax.

Conclusions: The complications after ASO are common and complicated. Understanding of the physiological characteristics of infants and the pathophysiological changes, and optimizing postoperative treatment help to improve the survival rate after ASO.

Key words: transposition of great arteries; arterial switch operation; complication; infant

                                                                                                                    World J Pediatr 2007;3(4):300-304
  [Abstract] [Full Text] [PDF]  
Case report:
Portal venous gas and biliary sludge in infantile pyloric stenosis
  Aharon Klar, Eitan Gross, Raid Haj Yahya, Yaacov Akerman
  Jerusalem, Israel

Author Affiliations: Department of Pediatrics, Bikur Cholim General Hospital, Jerusalem, Israel (Klar A, Yahya RH); Department of Pediatric Surgery, Hadassah University Hospital, Jerusalem, Israel (Gross E); Ultra Sound Unit, Bikur Cholim General Hospital, Jerusalem, Israel (Akerman Y)

Corresponding Author: Aharon Klar, MD, Department of Pediatrics, Bikur Cholim General Hospital, 3-5 Strauss St., POB 492, Jerusalem, Israel (Tel: 972-2-6464214; Fax: 972-2-6464273; Email: aklar@inter.net.il)

 

Background: A 1-month-old infant with recurrent vomiting for 2 days was diagnosed with gas in the portal vein. This finding is usually regarded as an ominous sign. 

Methods: Physical examination, plain abdominal X-ray, abdominal ultrasonography and pyloromyotomy were performed.

Results: Hypertrophic pyloric stenosis, gas in the portal vein and sludge in the gallbladder were diagnosed.

Conclusions: The benign course of this patient and the patients reported previously shows that portal gas associated with hypertrophic pyloric stenosis preoperatively may not be an ominous sign and the definite surgical treatment should not be delayed.

Key words: portal vein; venous gas; pyloric stenosis

World J Pediatr 2007;3(4):305-307

  [Abstract] [Full Text] [PDF]  
   
 
 
 
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