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Vol 3, No 1
Vol 3, No 1 February 2007 ISSN 1708-8569
 
Editorial
Review article
Original article
Clinical summary
Case report
   
Editorial:
Molecular diagnosis of neurogenetic diseases of children in China: the current status and the future
  Xi-Ru Wu, Nanbert Zhong
 

 

  [Abstract] [Full Text] [PDF]  
Review article:
Podocyte and hereditary nephrotic syndromes
  Marie Claire Gubler
 

Background: In the past few years, genetic studies of familial steroid-resistant nephrotic syndrome and generation of murine models of these diseases have resulted in tremendous progress in the understanding of the physiology and pathology of podocyte

Data sources: Based on recent original publications and the experience with the disease of our group, we review the different genetic forms of autosomal recessive and dominant nephrotic syndrome, and  indicate the possible, cellular and functional consequences of the gene defects.

Results: The major role of the glomerular slit diaphragm in the formation and the maintenance of the glomerular ultrafiltration barrier has been demonstrated. This knowledge has improved the diagnosis and management of steroid-resistant nephrotic syndromes, especially in children. Familial nephrotic syndrome is characterized by clinical and genetic heterogeneneity, but most histological lesions are non-specific.

Conclusions: The recognition of the genetic origin of the disease and, if possible, the detection of the resultant mutation are useful clinically. They allow to avoid unnecessary and aggressive immunosuppressive treatments, to predict the absence of recurrence after transplantation, and to provide prenatal diagnosis to families at risk.

Key words: podocyte; hereditary nephrotic syndromes; NPHS1; NPHS2; WT1

  [Abstract] [Full Text] [PDF]  
Neonatal necrotizing enterocolitis: clinical aspects, experimental models and pathogenesis
  Wei Hsueh, Isabelle G. De Plaen, Michael S. Caplan, Xiao-Wu Qu, Xiao-Di Tan,
 

Backgroud: Necrotizing enterocolitis (NEC), a disease of premature infants, is a leading cause of morbidity and mortality in neonatal intensive care units. Its known predisposing factors include prematurity, enteral feeding, and infection, but the pathogenesis is still obscure.

Data sources and Results: Several animal models of NEC have been established in adult and neonatal rats and mice. Endogenous mediators, especially platelet-activating factor (PAF), play a pivotal role in NEC. Injection of PAF results in intestinal necrosis, and PAF antagonists prevent the bowel injury induced by endotoxin, hypoxia, or tumor necrosis factor-α (TNF) in adult rats. PAF antagonists or PAF-acetylhydrolase, the enzyme degrading PAF, also ameliorate the pathology of the neonatal model induced by hypoxia and enteral feeding. Human patients with NEC have elevated levels of plasma PAF and decreased PAF-acetylhydrolase. However, to exert its injurious action, PAF requires the presence of commensal bacteria in the intestine. The initial event in our models of NEC is probably polymorphonuclear leukocytes (PMN) activation and adhesion to venules in the intestine, probably in response to local PAF release. Other molecules and mediators taking part in the process include NF-κB, TNF, chemokines (especially MIP-2/CXCL2), and the complement system, leading to a local inflammatory reaction. Subsequent release of vasoconstrictors and the consequent mesenteric vasoconstriction result in splanchnic ischemia and reperfusion. Toxins from commensal bacteria (e.g., endotoxin) enter the intestinal tissue during local mucosal barrier breakdown, and synergize with PAF and TNF to amplify the inflammation. Reactive oxygen species produced by the activated leukocytes and by intestinal epithelial xanthine oxidase may be the final effector for tissue injury. Protective mechanisms include nitric oxide produced by the constitutive (mainly neuronal) nitric oxide synthase (which maintains intestinal perfusion and the integrity of the mucosal barrier), and the innate immune response, enhanced by indigenous probiotics such as Bifidobacteria and Lactobacilli.

Conclusions: The eventual development of severe bowel necrosis depends on the balance between the injurious and the protective mechanisms. The injurious includes various pro-inflammatory molecules such as PAF, leukotrienes, TNF, chemokines, the complement system, vasoconstrictors, adhesion molecules, xanthine oxidase, reactive oxygen species, NF-κB and products of commensal bacteria such as lipopolysaccharide. The protective mechanisms include neuronal nitric oxide synthase, PAF acetylhydrolase, and probiotics.

Key words: necrotizing enterocolitis; platelet-activating factor; tumor necrosis factor; nuclear factor kappa B; chemokines; reactive oxygen species; nitric oxide synthesis; probiotics; intestines

  [Abstract] [Full Text] [PDF]  
Childhood gastrointestinal dysfunction and protection mechanism of intestinal mucosal barrier
  Mei Sun, Ming Ma
   

Childhood gastrointestinal dysfunction has been recognized as the triggering elements of multiple organ failure. The causes of gastrointestinal dysfunction include: injury of intestinal barrier, release of inflammatory mediators, translocation of intestinal bacteria, gastro-intestinal mucosal ischemia and anoxia injury caused by abnormal intestinal blood flow perfusion, and apoptosis of gastrointestinal epithelial cells. To prevent and treat this disease, the mechanism of gastrointestinal mucosal protection should be clarified in various aspects. Prostaglandins (PGs) and nitric oxide (NO) have a protective effect on gastrointestinal mucosa by inhibiting acid secretion, increasing mucus and bicarbonate ion secretion and by increasing mucosal blood flow as well as inhibiting mast cell and leukocyte adhesion. Many peptides participate in the process of gastrointestinal mucosal protection and injury repair differently with transforming growth factor, pancreatic secretory trypsin inhibitor (PSTI), glucagon-like peptides-2 (GLPs-2), basic fibroblast growth factor (bFGF), platelet-derived growth factor (PDGF), and epithelial growth factor (EGF). Phase II enzymes form a super-family of detoxification enzymes involving in the protection of cells from potentially harmful compounds and oxidation injury. In the trifoil peptide family, the stable structure of each peptide gives a characteristic three-loop shape and provides for exceptional resistance to proteolytic degradation. The peptides participate in mucosal repair by epithelial restitution and reconstitute the integrity of mucosa barrier. Intestinal trifoil factor (ITF) is also an endogenous peptide which could inhibit the apoptosis of epithelial cells through the NF-κB cascade regulation. Glutamine protects mucosa via three aspects: maintenance of mucosal barrier, regulation of the enteric immune function, and protection of mucosa from oxidization injury. Clinical manifestations of the disease and possible intervention approaches are also described.

Key words: gastrointestinal dysfunctions; children; pathogenesis; mucosal protection; clinical feature; diagnosis

  [Abstract] [Full Text] [PDF]  
Original article:
Survival rate of children with rhabdomyosarcoma and prognostic factors
  Khadijeh Arjmandi Rafsanjani, Parvaneh Vossough, Ali Bashardoust, Mohammad Faranoush
   

Background: Rhabdomyosarcoma is the most frequent tumor of soft tissue in children. The survival rate of the patients is significantly increased since the 1970s. This study was undertaken to evaluate the 5- and 10-year survival rates of patients with rhabdomyosarcoma in a single center in Iran. 

Methods: A total of 77 children with rhabdomyosarcoma up to 15 years old who had been treated at the Hematology and Oncology Department in Ali Asghar Children Hospital from 1993 to 2003 were evaluated for their age and gender, as well as histology, stage and primary site of the tumor at diagnosis.

Results: The mean age of the patients was 6.58 years (SD=4.02, median 6 years). In this series, 46 patients (59.7%) were male and 31 (40.3%) female, and the mean survival time of patients was 8 years (95% CI: 8-9). The 5-year survival rate was the highest in patients with localized tumor, stage I and II (82.25% and 86.88% respectively). The survival rate of patients with embryonal tumor was 86.8%, in those with primary orbital tumor was 94%, and in those with genitourinary tumor was 85.71%. The 5- and 10-year overall survival rates were 79.54% and 77.92%, respectively.

Conclusions: Children with rhabdomyosarcoma of lower stage, embryonal histology, and orbital and genitourinary primary sites had a better survival rate. Poor prognosis was associated with metastasis of the tumor at the time of presentation, alveolar histology (48%), and tumor of the extremities (58%). In our study, only the stage of the tumor was significantly different in the variables (P=0.0077) because of the small number of patients. Children who survived the first 5 years after diagnosis were found to have an excellent survival rate.

Key words: rhabdomyosarcoma; survival rate; age; primary site; stage; pathology

                    World J Pediatr 2007;3(1):36-40

  [Abstract] [Full Text] [PDF]  
Construction of the right ventricular outflow tract in children with pulmonary atresia associated with ventricular septal defect
  Jing-Hao Zheng, Jin-Fen Liu, Zhi-Wei Xu, Zhao-Kang Su, Wen-Xiang Ding
   

Background: It is difficult to build up the right ventricular outflow tract for pediatric patients with pulmonary atresia complicated by ventricular septal defect (VSD). This study was undertaken to evaluate the surgical procedures for the reconstruction of the right ventricular outflow tract (RVOT) in infants and young children.

Methods: From June 1999 to December 2004, 81 patients with PA associated with VSD were treated by primary repair, palliative repair or staged repair. These patients were divided into 2 groups according to different surgical procedures. In group I (32 patients), 17 underwent biventricular primary repair and transannular patching with autologous pericardial flake. Group II (49 patients) underwent palliative repair including construction of the right ventricular outflow tract according to preoperative conditions. Fifteen patients underwent staged radical repair, during which transannular patching was performed with homovariant pericardial flake in 8 patients.

Results: Four patients (12.5%) died after primary radical repair. Three patients died (6.1%) after palliative repair. But no death occurred for stage II radical repair. Follow-up for 3 months to 2 years showed satisfactory results. The oxygen saturation (SatO2) was 79%-87% for the palliative repair group, and the McGoon index was increased from 0.4-0.7 to 1.1-1.6.

Conclusions: With the development of pulmonary artery and pulmonary artery confluence, RVOT construction by the palliative method includes transannular autologous pericardial patching, Gortex conduit, and pericardial rolling. By the radical procedure, the back wall of the traverse pulmonary artery trunk is anastomosed to the incision and homograft besides the transannular autologous pericardial patch.

Key words: congenital heart defect; cardiac surgical methods; pulmonary atresia; infants; young children

  [Abstract] [Full Text] [PDF]  
Surgical treatment of mediastinal neoplasm in children
  Xi Zhan, Wen-Dong Wang, Jian-Jun Wang
   

Background: Mediastinal tumors in children comprise a heterogeneous group of lesions from a range of embryonic origins. They may present as benign cysts or malignant lesions. Mediastinal tumors in children and adolescents result in significant morbidity and mortality. Lymphoma is more common in the anterior mediastinum and neurogenic tumors are more common in the posterior mediastinum. Surgery is an important measure used in the diagnosis and treatment of such lesions. This study was to evaluate the strategy of surgical treatment of mediastinal tumors in children. 

Methods: We retrospectively analyzed the clinical data from 61 children with mediastinal tumor treated in our department from 1994 to 2006.

Results: Forty-four patients had benign tumors (72.1%), and 17 malignant tumors (27.9%). Fifty patients were subjected to complete resection, 8 to partial resection, and 3 to biopsy. Thirty-nine patients were followed up for 3 years; the survival rate of patients with benign tumors was 100%, and that of patients with malignant tumors was 44%.

Conclusions: Thoracic computerized tomography (CT) scan is the first choice before operation; patients with benign mediastinal tumors may have good outcome after surgical treatment. Anaesthesia and perioperative management are of crucial importance.

Key words: mediastinal tumor; surgical treatment; children

  [Abstract] [Full Text] [PDF]  
Separation and identification of endothelial progenitor cells from rat peripheral blood
  Si-Lin Pan, Quan-Sheng Xing, Long Sun
   

Background: Kawasaki disease (KD) as the most commonly acquired heart disease in children worldwide is an acute systemic febrile illness with endothelial injury. The incidence of KD varies in different countries, but it is higher in Asian than in Western countries. Endothelial progenitor cells (EPCs) can differentiate into endothelial cells and serve as a potential therapeutic agent for KD. The present study aimed at exploring the simple procedures of isolation, cultivation and purification of EPCs from peripheral blood.

Methods: Five milliliters of peripheral blood was collected from the femoral artery of each Sprague-Dawley rat. Mononuclear cells were isolated by Ficoll density gradient centrifugation and cultured in a special medium, including vascular endothelial growth factor and basic fibroblast growth factor. After 6 days of continuous culture on 24-well fibronectin-coated plates, the cells expanded and differentiated into endothelial-like progenitor cells. The expression of Flk-1, von Willebrand factor (vWF), CD31, and CD34 was assessed. Surface lectin staining was performed using fluorescently labeled UEA-1 lectin at 10 µg/ml. Meanwhile, the ability for live cells to take up fluorescently labeled acetylated low-density lipoprotein (DiI-Ac-LDL) was assessed by incubation with DiI-Ac-LDL.

Results: The cells harvested by this procedure were CD31, CD34, Flk-1 positive and demonstrated double-positive fluorescence for LDL, and lectin-UEA-1.

Conclusion: Relatively purified EPCs can be obtained by certain procedures of isolation and culture. For its differentiating potential, EPCs act as an important source of mature endothelial cells (ECs).

Key words: peripheral blood; mononuclear cell; endothelial progenitor cells; cell culture

  [Abstract] [Full Text] [PDF]  
Insulin-like growth factors in lung development of neonatal rats and effect of dexamethasone andretinoic acid on their expression
  Han-Chu Liu, Li-Wen Chang, Zhi-Hui Rong, Hua-Ping Zhu, Qian-Sheng Zhang, Hong-Bing Chen, Wen-Bin Li
   

Background: The lung is prone to being attacked by intrinsic/extrinsic factors and lung injury is formed. With the increased survival rate of very low birth weight, extremely low birth weight infants, respiratory failure and mortality rate caused by lung development arrest and dysplasia have increased accordingly. This study was designed to investigate the role of insulin-like growth factors (IGFs) in lung development of neonatal rats and effect of dexamethasone (DEX) and retinoic acid (RA) on their expression. 

Methods: Eighty timed pregnant Sprague-Dawley rats were randomly divided into 4 groups (n=20): control group (group A), DEX treatment group 1 (group B), DEX treatment group 2 (group C), and RA treatment group (group D). Groups A, B and D were injected subcutaneously with normal saline, intraperitoneally with DEX and RA, respectively on gestational day 18-20; group C was injected subcutaneously with DEX on postnatal day 1-3. Lung tissues were collected at the following time points: gestational day 18, 20, 21 and postnatal day 1, 3, 5, 7, 10, 14, 21 respectively  for histopathological examination and expression level determinations of IGF-I, IGF-II polypeptides and mRNA.

Results: The highest expression level of IGF-I in groups A and D occurred on postnatal day 5-7. There was a positive correlation between IGF-I and alveolar development. The highest expression level of IGF-I in group B was observed on postnatal day 3. The expression level of IGF-I was much higher in group B than in group A from gestational day 18 to postnatal day 3 (P<0.01); but it was significantly lower at other time points (P<0.01). The expression level of IGF-I in group C was lower but it was higher in group D than that in group A at any time points (P<0.01). The peak level of IGF-II expression occurred on gestation day 18 and reduced to trace gradually. No differences were found among all groups in the level of IGF-II expression (P>0.05). RT-PCR showed that the trends of IGF-I, -II mRNA expression in the 4 groups were in parallel with those of their polypeptides.

Conclusions: IGF plays an important role in lung development of neonatal rats and there is a close link between DEX, RA and IGF-I, -II. DEX could restrain but RA could accelerate lung development by influencing the expression of IGF-I, -II.

Key words: dexamethasone; retinoic acid; lung development; insulin-like growth factor
  [Abstract] [Full Text] [PDF]  
Long-term effects of recurrent seizures on learning, behavior and anxiety: an experimental study in rats
  Xiu-Yu Shi, Ji-Wen Wang, Ge-Fei Lei, Ruo-Peng Sun
   

Background: Animal models have suggested that seizures in the developing brain cause less macroscopic structural damage than seizures in adulthood but accumulating evidence shows that recurrent seizures in infants are associated with a high incidence of cognitive deficits. In this study, we determined the effects of recurrent neonatal seizures on cognitive tasks using a set of behavioral paradigms when the animals were in adulthood. 

Methods: Recurrent seizures were induced by injecting rat pups with pilocarpine on postnatal day 2 (P2), P4 and P7. In adulthood (P52-P56), spatial learning was examined by the Morris water maze; activity level was assessed by an open field test; and anxiety was examined by the elevated plus maze.

Results: Compared with controls, rats with recurrent seizures had deficient spatial learning and a greater degree of anxiety.

Conclusions: Our findings indicate that recurrent seizures during the neonatal period result in life-long impairment of certain cognition.

Key words: epilepsy; development; pilocarpine; long-term effects; behavioral testing; water maze
  [Abstract] [Full Text] [PDF]  
Clinical summary:
Juvenile fibromyalgia syndrome: clinical experience at a university hospital
  Xiu-Feng Cheng, Yan Jin, Jun Tan
   

Background: Fibromyalgia syndrome (FMS) is one of the relatively common overlapping syndromes characterized by unexplained chronic pain and fatigue. The aim of this study was to evaluate the clinical and laboratory features of FMS in children.

Methods: The clinical data of 6 children with FMS treated at the Department of Rheumatology of People's Hospital Affiliated to Jiangsu University during the period of 1997-2004 were retrospectively analyzed.

Results: Abdominal pain was the first complaint in 5 of the 6 patients with FMS. All the 6 patients were misdiagnosed before rheumatological examination. Diffuse pain, fatigue, sleep disturbance, exacerbated pain caused by low temperature or physical exercise were the main complaints. The mean pain score was 8.8 and the mean initial tender point count was 13.7. Arthrodynia, joint swelling, abdominal pain, irritable bowel symptoms and urinary urgency were observed in 5 patients (83%). Dysmenorrhea was seen in 4 patients (67%), depression in 3 (50%), morning stiffness in 2 (33%), paresthesia in 2 (33%), and anxiety in 2 (33%). Laboratory examination showed normal results and the outcomes of treatment were satisfactory.

Conclusions: FMS, a common disease in children, needs more attention to prevent misdiagnosis.

Key words: fibromyalgia syndrome; children; clinical analysis
  [Abstract] [Full Text] [PDF]  
Case report:
Silver-Russell syndrome: a case report
  Bing-Ping Qiu, Chang-Hong Shi
   

Background: We present a case of an 8-year-old girl with a short stature. She had features of Silver-Russell syndrome (SRS) including asymmetrical body, maldevelopment of the fifth finger clinodactyly, small face, broad forehead, and delayed bone age. She had been diagnosed as having intrauterine growth retardation after birth, and feeding problems had been noted in her infancy. Psychomotor development was delayed.

Methods: The patient underwent physical examina-tion, X-ray examination, and laboratory examination. Treatment with recombinant human growth hormone (rhGH) and gonadotropin releasing hormone analogue (GnRHa) was given, and the patient was followed up.

Results: The imaging of the legs revealed that her left leg was 2 cm shorter and the circumference of the upper left leg was larger than the right one. During the follow-up for 2 years, her growth accelerated with mature breast and pubic hair. The levels of blood follicle stimulating hormone (FSH), luteinizing hormone (LH) and estradiol (E2) increased to the levels of adolescence.

Conclusions: This patient met the clinical criteria for SRS, and also with sexual precosity, she could be treated with rhGH and GnRHa, but the final height would not be ideal.

Key words: Silver-Russell syndrome; asymmetry; intrauterine growth retardation
  [Abstract] [Full Text] [PDF]  
Congenital absence of multiple ribs
  Hui-Jin Chen
   

Background: Congenital rib absence might be the absence of either single or several ribs or all ribs at one side. The sporadic cases were ever reported over years.

Methods: A full-term Chinese infant was noticed that both shoulders were asymmetric significantly with left sagged shoulder at the age of 6 months. Her left hand could not rise over her shoulder and her gripping power was a little bit poor. She presented a good mental and motor development without other deformity. Imaging examination was given to the infant  for diagnosis.

Results: The absence of left three ribs was found on her chest X-ray film. The results of three-dimensional CT reconstruction image confirmed the absence of left 1st-4th ribs, the overlapping of 5th-7th ribs on the left side and 2nd-3rd ribs on the right side.

Conclusions: Simple congenital absence of multiple ribs is confirmed based on her manifestation and imaging results.

Key words: infant; congenital rib absence; three-dimensional CT reconstruction

  [Abstract] [Full Text] [PDF]  
   
 
 
 
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