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Vol 14, No 2
Vol 14, No 2 April 2018 ISSN 1708-8569
Review articles
Original articles
Clinical images
The status quo and challenges of genetic diagnosis in children with steroid-resistant nephrotic syndrome
  Yan-Yan Jin, Bing-Yu Feng, Jian-Hua Mao
  [Abstract] [Full Text] [PDF]  
Review articles:
Surgical chylothorax in neonates: management and outcomes
  Karina Miura Costa, Amulya Kumar Sax
Background: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates.
Data sources: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS).
Results: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23.4%), esophageal atresia (n = 5, 4.7%) and CDH + extralobar sequestration (n = 1, 0.9%). Medium-chain triglycerides (MCT) was the initial treatment in 52 neonates (48.6%), prednisolone + MCT in one (0.9%), total parenteral nutrition in 51 patients (47.7%), and three patients (2.8%) did not require any treatment. Octreotide and somatostatin were used as second or third line treatment in 25 neonates (23.4%), and 15 neonates (14%) underwent 17 surgeries, including thoracic duct ligation (TDL) (n = 9); pleurodesis (n = 3) (2 patients required TDL); TDL + pleurodesis (n = 2), and TDL + placement of hemostat (n = 1). Complications due to the chylothorax were reported in 27 neonates (25.2%): hypoalbuminemia + hyponatremia (n = 18), hypoalbuminemia (n = 4), hypoalbuminemia with cutaneous flushing as colateral effect of somatostatin (n = 1), loose stool after somatostatin use (n = 1), pneumonia (n = 1), congestive heart failure + hypernatremia (n = 1), and hyponatremia (n = 1). There were 21 deaths (19.6%) and median LOS was 53.4 days (30每93.1 days).
Conclusions: Conservative management is appropriate as initial treatment for neonatal postsurgical chylothorax. Octreotide and somatostatin are safe in neonates and surgical approach should be considered in prolonged leaks.
  [Abstract] [Full Text] [PDF]  
Staphylococcal-scalded skin syndrome: evaluation, diagnosis, and management
  Alexander K. C. Leung, Benjamin Barankin, Kin Fon Leong
Background: Staphylococcal-scalded skin syndrome (SSSS), also known as Ritter disease, is a potentially life-threatening disorder and a pediatric emergency. Early diagnosis and treatment is imperative to reduce the morbidity and mortality of this condition. The purpose of this article is to familiarize physicians with the evaluation, diagnosis, and treatment of SSSS.
Data sources: A PubMed search was completed in Clinical Queries using the key terms ※Staphylococcal scalded skin syndrome§ and ※Ritter disease§.
Results: SSSS is caused by toxigenic strains of Staphylococcus aureus. Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation. The diagnosis is mainly clinical, based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance especially in friction zones, periorificial scabs/crusting, positive Nikolsky sign, and absence of mucosal involvement. Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin, oxacillin, or flucloxacillin is essential until cultures are available to guide therapy. Clarithromycin or cefuroxime may be used should the patient have penicillin allergy. If the patient is not improving, critically ill, or in communities where the prevalence of methicillin-resistant S. aureus is high, vancomycin should be used.
Conclusion: A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.
  [Abstract] [Full Text] [PDF]  
Calcineurin inhibitors and nephrotoxicity in children
  Fei Liu, Jian-Hua Mao
Background: Calcineurin inhibitors (CNIs) are commonly given to transplant recipients of kidneys and other solid organs and to patients with immune disorders, such as steroid-resistant nephrotic syndrome, steroid-dependent nephrotic syndrome, and frequent relapse nephrotic syndrome. Although CNIs remain the most effective available immunosuppressant agent, there is clinical concern regarding possible long-term nephrotoxicity. This concern is especially significant in children who have a longer life expectancy and greater growth rate.
Data sources: In this review, we analyzed the literatures to identify original articles that examined use of CNIs in children who received organ transplantation and nephropathy to assess the available evidence of their nephrotoxicity. PubMed, Elsevier, and Tompson ISI Web of Knowledge were searched for identifying relevant papers.
Results: Clinical research supports the presence of CNI-related nephrotoxicity. However, some researchers have questioned the prevalence and seriousness of chronic CNIs nephrotoxicity, especially because the pathological lesions typically associated with long-term CNI use are nonspecific. Many researchers have focused on early markers of CNI nephrotoxicity, and the methods that may help prevent and manage nephrotoxicity.
Conclusions: Future research should focus on investigating early markers of CNI nephrotoxicity and strategies for improved immunosuppressant therapy, and developing alternative treatments. CNI-mediated nephrotoxicity should always be taken seriously in clinic.
  [Abstract] [Full Text] [PDF]  
Original articles:
Indications and results of renal biopsy in children: a 36-year experience
  Luisa Santangelo, Giuseppe Stefano Netti, Paolo Giordano, Vincenza Carbone, Marida Martino, Diletta Domenica Torres, Michele Rossini, Anna Maria Di Palma, Loreto Gesualdo, Mario Giordano
Background: This study was conducted to investigate retrospectively the indications for renal biopsy (RB) in native kidneys and to analyze pathological findings in a single tertiary pediatric hospital in Southern Italy for the last 36 years.
Methods: All patients who underwent RB at our hospital from 1979 to 2014 were included. All renal tissue specimens were studied under light and immunofluorescent microscopy, while electron microscopy was performed only for specific clinical indications.
Results: The study group included 213 patients (female 43.2%) who underwent 225 percutaneous native kidney biopsies. Median age was 10.4 years (range 0.6每24 years). The most frequent indication for RB was nephrotic syndrome (44.4%), followed by proteinuria (27.6%), asymptomatic hematuria (17.3%) and acute kidney injury (9.8%). Gross hematuria appeared after biopsy in less than 5% of the patients, but none of them needed blood transfusion. Adequate renal tissue sample was obtained in 95.5% of the renal biopsies. Primary glomerulonephritis (GN) was the most common finding (61.4%), followed by secondary GN (21.4%), tubulointerstitial diseases (3.7%) and hereditary nephropathy (2.8%), while in 10.7% of the cases, normal renal tissues were found. According to histopathological diagnosis, the most common causes of primary GN were IgA nephropathy (20.9%), followed by minimal change disease (18.1%) and focal segmental glomerulosclerosis (11.6%).
Conclusions: The epidemiology of glomerular disease in our single-center cohort is similar to that shown in other national and international reports. Moreover, our study shows that percutaneous ultrasound-guided RB is a safe, reliable and effective technique in children.
  [Abstract] [Full Text] [PDF]  
Urinary neutrophil gelatinase-associated lipocalin (NGAL) and serum cystatin C measurements for early diagnosis of acute kidney injury in children admitted to PICU
  Jameela Abdulaziz Kari, Mohamed Ahmed Shalaby, Kholoud Sofyani, Ahmad Saleh Sanad, Albaraa Fuad Ossra, Rayan Smeer Halabi, Maha Hassan Aljuhani, Wael Mohammad Toffaha, Feras Aymen Moria, Samar Sabry, Hanan AbdelAziz Ahmed, Khalid Abdulaziz Alhasan, Sara Sharief, Osama Safdar
Background: Acute kidney injury (AKI) is common in critically ill children with significant mortality and morbidity. Serum creatinine is an insensitive and late biomarker compared to newly proposed AKI biomarkers.
Methods: Prospective study in pediatric intensive care unit (PICU) over three months to compare between serum cystatin-C (s-Cys-C) and urinary neutrophil gelatinase-associated lipocalin (uNGAL) as AKI biomarkers at multiple time points with pediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE) classification in diagnosing AKI.
Results: Forty children were recruited. Of these 40 children, 22 developed AKI according to pRIFLE criteria. There was no significant difference between AKI and non-AKI in age (P = 0.29). Post cardiac surgery, renal insult was the main cause of AKI (27.3%). There was a twofold increased risk of incident AKI in those patients with high baseline uNGAL at PICU admission and almost a fourfold increased risk in patients with high baseline s-Cys-C at PICU admission. uNGAL levels were highly predictive of AKI during the follow-up period [area under the curve (AUC) = 0.76, 95% confidence interval (CI) 0.61每0.92]. The cutoff point with the highest correctly classified proportion was 223 ng/mL (≡ 12 centiles) which correctly predict 80.0% patients with AKI, with a corresponding sensitivity of 72.7% and a specificity of 89.9%. AUC for s-Cys-C was 0.86 (95% CI 0.75每0.97), and the highest correctly classified proportion was 1009 米g/L (≡ 13 centiles); 75% of patients with AKI, with a corresponding sensitivity of 63.6% and a specificity of 88.9%.
Conclusion: uNGAL and s-Cys-C predicts AKI early in critically ill children.
  [Abstract] [Full Text] [PDF]  
Plasma gelsolin level predicts acute kidney injury after cardiopulmonary bypass in infants and young children
  Shan-Shan Shi, Xiao-Jie Yue, Dong-Yan Zhao, Jia-Jie Fan, Jian-Guo Xu, Xi-Wang Liu, Bao-Li Cheng, Xiang-Ming Fang, Jie Fan, Qiang Shu
Background: Acute kidney injury (AKI) after cardiopulmonary bypass (CPB) is a common complication especially in pediatric population. Plasma gelsolin (pGSN) is an anti-inflammatory factor through binding with actin and pro-inflammatory cytokines in circulation. Decrease in pGSN has been reported in some pathologic conditions. The purpose of the study was to determine the alterations of pGSN level in infants and young children after CPB and the role of pGSN as a predictor for the morbidity and severity of post-CPB AKI.
Methods: Sixty-seven infants and young children at age ≒ 3 years old undergoing CPB were prospectively enrolled. PGSN levels were measured during peri-operative period with enzyme-linked immuno-sorbent assay and normalized with plasma total protein concentration. Other clinical characteristics of the patients were also recorded.
Results: In patients developing AKI, the normalized pGSN (pGSNN) levels significantly decreased at 6 h post-operation and remained low for 24 h post-operation as compared to the patients with non-AKI. PGSNN at 6 h post-operation combining with CPB time presents an excellent predictive value for AKI.
Conclusions: Decreased pGSNN identifies post-CPB AKI in the patients ≒ 3 years old, and is associated with adverse clinical outcomes. The findings suggest that circulating GSN in post-CPB patients may have beneficial effects on diminishing inflammatory responses.
  [Abstract] [Full Text] [PDF]  
Establishment of age- and gender-specific pediatric reference intervals for liver function tests in healthy Han children
  Xin Li, Di Wang, Chun Yang, Qi Zhou, Suo-Lang Zhuoga, Li-Qiang Wang, Han-Xin Yao, Qin Zhang, Qing Ai, Chen-Xi Yang, Jian-Cheng Xu
Background: The development and growth of children influence values of liver function tests. This study aims to establish age- and gender-specific pediatric reference intervals of liver function among Han children in Changchun, China.
Methods: A total of 1394 healthy Han children, aged 2每14 years, were recruited from communities and schools with informed parental consent in Changchun. The levels of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), 污-glutamyltransferase (GGT), alkaline phosphatase (ALP), total protein (TP), albumin (ALB), total bilirubin (TBIL) and direct bilirubin (DBIL) were measured on Hitachi 7600-210 automatic biochemical analyzer. The age- and gender-specific reference intervals were partitioned using Harris and Boyd*s test and calculated using nonparametric rank method. The pediatric reference intervals were validated in five representative hospitals located in different areas in Changchun.
Results: All the analytes required some levels of age partitioning. Proteins (TP, ALB) and bilirubins (TBIL, DBIL) required no gender partitioning. In contrast, considerable gender partitioning was required for serum ALT, AST, GGT, and ALP. TP, TBIL, and DBIL showed steady increases, and AST showed apparent decreases over time, whereas ALT, GGT, ALP, and ALB demonstrated complex trends of change. ALT and GGT increased sharply in males from 11 to 14 years old. However, ALP declined in females from 13 to 14 years. All five laboratories passed the validation of reference intervals.
Conclusions: There were apparent age or gender variations of the reference intervals for liver function. When establishing pediatric reference intervals, partitioning according to age and gender is necessary.
  [Abstract] [Full Text] [PDF]  
Evaluating the predictive factors of resting energy expenditure and validating predictive equations for Chinese obese children
  Lin Zhang, Ran Chen, Rong Li, Meng-Ying Chen, Rong Huang, Xiao-Nan Li
Background: To study the predictive factors of resting energy expenditure (REE) and evaluate the accuracy of predicted equations with indirect calorimeter (IC) in Chinese school-age children, particularly for the obese population.
Methods: Recruited children were from the department of child healthcare in Nanjing children*s hospital during July 2014每September 2015. Anthropometric parameters and body composition were measured by bioelectrical impedance. Measured REE was assessed by IC. Predicted REE was estimated using ten published equations.
Results: 248 children aged 7每13 years were recruited, including 148 obese [body mass index standard deviation score (BMISDS) = 2.48 ㊣ 0.91] and 100 non-obese (BMISDS = − 0.96 ㊣ 1.08). The unit mass of REE (REE/kg) in obese group (29.06 ㊣ 5.74) was lower than that in non-obese group (37.51 ㊣ 6.56). The stepwise regression showed that age, BMISDS and fat-free mass (FFM) had a major impact on REE/kg as the regression equation: Y = 54.41 − 1.36 ℅ X1 − 2.25 ℅ X2 − 0.16 ℅ X3 (Y REE/kg, X1 age, X2 BMISDS, X3 FFM; R = 0.633, R2 = 0.401, P < 0.01). The accuracy of predicted REE in obese subjects was 62.16% by the new predictive equations.
Conclusions: The REE/kg in obese children was lower and closely correlated with age, BMISDS and FFM. It is necessary to validate the new predictive equation in a larger sample to estimate energy requirements, particularly for children with obesity.
  [Abstract] [Full Text] [PDF]  
Prevalence of obesity in Italian adolescents: does the use of different growth charts make the difference?
  Luca De Carli, Elena Spada, Silvano Milani, Gian Pasquale Ganzit, Lucia Ghizzoni, Melissa Raia, Alessandra Coscia, Enrico Bertino, Simona Bo
Background: Since populations are becoming increasingly multi-ethnic, the use of local or international charts is a matter of debate. This study aimed to evaluate how the choice of cut-off thresholds affected prevalence of underweight (UW), overweight (OW), obesity (OB) in 1200 11每12-year Italian adolescents, and how their somatic growth depended on parental origin.
Methods: The height, weight and body mass index were expressed as standard deviation score (SDS) using Italian (ISPED-2006) and UK (UK-1990) charts. The classification of UW/OW/OB was computed with the IOTF international cut-offs, and thresholds were identified as centiles corresponding to BMI values of 18.5/25.0/30.0 kg/m2 at 18-year in ISPED-2006 or UK-1990 references.
Results: About 30% participants had non-Italian parents, above all from North-Africa and Romania. Referring to the UK-1990 charts, all groups showed negative mean SDS for height, and positive SDS for weight and BMI. Referring to the ISPED-2006 charts, all mean SDS were negative. Percentage of UW individuals was higher in accordance with ISPED-2006 than with UK-1990 charts, whereas percentages of OW/OB were higher with UK-1990 than ISPED-2006 charts. The results obtained using IOFT cut-off s were similar to UK-1990 cut-off s. These results were due to the different shape of age-dependent cutoff centiles. Independently by the parental origin, the percentages of adolescents classified as OW/OB were closer to the expected values using the ISPED-2006 then the UK-1990 cut-off s. The results suggested the use of the Italian references for adolescents with immigrant parents.
Conclusion: The use of local charts seems more appropriate at least in Italian adolescents in the age range studied.
  [Abstract] [Full Text] [PDF]  
Outcome after early mobilization following hip reconstruction in children with developmental hip dysplasia and luxation
  Katharina Susanne Gather, Eva von Stillfried, Sebastien Hagmann, Sebastian M邦ller, Thomas Dreher
Background: Most orthopedic surgeons prefer spica cast immobilization in children for 4 to 12 weeks after surgical hip reconstruction in children with developmental hip dysplasia. This challenging treatment may be associated with complications. Studies are lacking that focus on early mobilization without casting for postoperative care after hip reconstruction.
Methods: Twenty-seven children (3.4㊣2.0 years), including 33 hips with developmental hip dysplasia (DDH) and dislocation of the hip (Tonnis grade 1 to 4), who underwent hip reconstruction (Dega acetabuloplasty, varisation-derotation osteotomy and facultative open reduction) were retrospectively included in this study. Postoperatively the patients were placed in an individual foam shell with 30 degrees of hip abduction, hip extension, and neutral rotation. Early mobilization physiotherapy was performed within the fi rst few days after the surgery under epidural anaesthesia. Full weight bearing was allowed after 3每4 weeks. All children received a clinical examination and radiographic evaluation before and after surgical intervention. The follow-up period was 12.3㊣2.9 months.
Results: On average, the postoperative acetabular index decreased significantly from 36.9 to 21.7 degrees and the centeredge angle increased from 9.9 to 28.6 degrees. All hips had reached Tonnis grade 1 at the time of the last follow-up. No complications such as dislocation of the bone wedge, avascular necrosis of the acetabulum or femur, lack of non-union, or nerve injury, were reported.
Conclusions: In this cohort study, hip reconstruction was successful according to clinical and radiographic outcome parameters after early mobilization without cast therapy. Early mobilization may be used as an alternative treatment option after hip reconstruction in DDH.
  [Abstract] [Full Text] [PDF]  
Pediatric appendicitis with appendicolith often presents with prolonged abdominal pain and a high risk of perforation
  Hee Mang Yoon, Jung Heon Kim, Jong Seung Lee, Jeong-Min Ryu, Dae Yeon Kim, Jeong-Yong Lee
Background: Appendicolith can cause appendiceal obstruction and acute appendicitis. Its high prevalence may be related to the high perforation rate in pediatric appendicitis. This study assessed the characteristics of appendicolith and its clinical significance in pediatric appendicitis.
Methods: A retrospective study was performed among children and adolescents younger than 17 years who were preoperatively diagnosed with appendicitis in the pediatric emergency department (ED). A total of 269 patients with a mean age of 9.98 ㊣ 3.37 years were enrolled. Clinical features and contrast-enhanced computed tomography findings were analyzed.
Results: Among the 269 patients, 147 (54.6%) had appendicoliths, with a mean maximal diameter of 5.21 ㊣ 2.34 mm. Compared to the no appendicolith group, the appendicolith group demonstrated more prolonged abdominal pain (≡ 48 hours) before the ED visit (23.1% vs. 11.5%; P = 0.013), clinical features of severe appendicitis (presence of fever, vomiting, positive urine ketone, and increased C-reactive protein), and higher rate of perforation (43.5% vs. 9.8%; P < 0.001). Multivariate risk factor analysis for perforated appendicitis in the appendicolith group revealed that maximal diameter of 5 mm or more in the appendicolith (adjusted odds ratio [aOR] 2.919; 95% CI 1.325每6.428, P = 0.008) and proximal collapse adjacent to the appendicolith (aOR 2.943; 95% CI 1.344每6.443, P = 0.007) were significant.
Conclusion: Pediatric appendicitis with appendicolith often presents with prolonged abdominal pain and severe clinical conditions with a high risk of perforation.
  [Abstract] [Full Text] [PDF]  
Does time taken to achieve jaundice-clearance influence survival of the native liver in post-Kasai biliary atresia?
  Hideaki Nakajima, Hiroyuki Koga, Manabu Okawada, Hiroki Nakamura, Geoffrey James Lane, Atsuyuki Yamataka
Background: We reviewed the time taken for post-portoenterostomy (PE) biliary atresia (BA) patients to obtain jaundice每clearance (total bilirubin ≒ 1.2 mg/dL; JC) post-PE to determine if JC time (JCT) is prognostic for survival of the native liver (SNL).
Methods: The subjects were 66 BA patients treated with PE at our institute between 1989, the year when liver transplantation (LTx) became available in Japan, and 2014. JCT was used to create three groups (≒ 30 days: n = 14; 31每60 days: n = 31; ≡ 61 days: n = 21). Medical records were reviewed retrospectively to evaluate: age at onset of symptoms, duration of symptoms pre-PE, age and weight at PE, serum liver function tests, incidence of cholangitis, and micro-bile duct size at PE.
Results: Age at onset of symptoms, age and weight at PE, duration of symptoms pre-PE, and micro-bile duct size were similar for all patients in all three groups. JCT and SNL appeared to correlate because preoperative total bilirubin (7.1, 9.6, 10.2 mg/dL; P < 0.05) was significantly lower in the JCT ≒ 30 days group (P < 0.05) while there was a significant decrease in SNL (P < 0.03) and a significant increase in LTx (P < 0.01) in the JCT ≡ 61 days group. All LTx subjects who achieved JC were found to have developed cholangitis within 3 months of PE.
Conclusion: During the follow-up of post-PE subjects, longer JCT and cholangitis occurrence within 3 months of PE would appear to be negative prognostic factors for SNL while preoperative total bilirubin would appear to be a positive prognostic factor for SNL.
  [Abstract] [Full Text] [PDF]  
Eight novel mutations of CBS gene in nine Chinese patients with classical homocystinuria
  Dong-Xiao Li, Xi-Yuan Li, Hui Dong, Yu-Peng Liu, Yuan Ding, Jin-Qing Song, Ying Jin, Yao Zhang, Qiao Wang, Yan-Ling Yang
Background: Classical homocystinuria (homocysteinemia type 1, MIM# 236200) is a rare inherited disorder in Mainland China. This study aimed to identify mutations in the cystathionine 汕-synthase (CBS) gene which are associated with classical homocystinuria in nine Chinese patients.
Methods: Nine Chinese patients were diagnosed at the age of 5 years 4 months to 18 years by plasma total homocysteine and blood methionine determination. CBS gene analysis was performed for the patients and their families.
Results: All nine patients had significantly increased plasma total homocysteine (142每500 米mol/L vs. the normal range of 0每15 米mol/L) and blood methionine (144.3每500 米mol/L vs. the normal range of 0每50 米mol/L). None of the patients was pyridoxine responsive. Eleven mutations in CBS gene were identified in the nine patients. Eight mutations (IVS3+1G>A, p.Thr493fsX46, p.Thr236Asn, p.Leu230Gln, p.Lys72Ile, p.Ser201ProfsX36, p.Met337IlefsX115, and IVS14-1G>C) were novel. Three mutations (p.Arg125Gln, p.Thr257Met and p.Gly116Arg) had been previously reported.
Conclusions: In this study, eight novel mutations in CBS were identified in nine Chinese patients with classical homocystinuria. None of the hotspot mutations reported in other regions previously was found. These data indicated that Chinese maybe had different CBS mutation spectrum from other population. The identification of mutations not only confirms the diagnosis but also enables accurate genetic counselling and prenatal diagnosis for the fetuses of the families.
  [Abstract] [Full Text] [PDF]  
Clinical images:
A rare cause of chronic ataxia in childhood: ganglioneuroma
  Elif Acar Arslan, T邦lay Kamaşak, Bet邦l Diler Turgut, İsmail Saygın, Haluk Sarıhan, Ali Cansu
  [Abstract] [Full Text] [PDF]  
Diffuse lung disease associated with neurofibromatosis type-1 can also affect children
  Paolo Spinnato, Giancarlo Facchini, Alberto Bazzocchi, Ugo Albisinni
  [Abstract] [Full Text] [PDF]  
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