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1.
Pediatr Surg Int ; 40(1): 210, 2024 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-39052072

RESUMO

Omphalocele and gastroschisis are the most common types of abdominal wall defects. Comprehensive local experience helps parents to make decisions on the pregnancy and foresee the disease journey. A retrospective review of abdominal wall defect patients in all three pediatric surgical centers in Hong Kong between January 2003 and February 2023 was conducted. All patients consecutively diagnosed with omphalocele and gastroschisis were included, excluding other forms. Data of demographics and short- and long-term outcome parameters were collected. A total of 99 cases were reviewed and 85 patients met the inclusion criteria. Diagnoses include omphalocele major (n = 49, 57.6%), omphalocele minor (n = 22, 25.9%) and gastroschisis (n = 14, 16.5%), with mean gestational age 37 weeks (SD 2.2) and birth weight 2.7 kg (SD 0.6). Omphalocele is most commonly associated with cardiovascular (n = 28, 39.4%) and chromosomal defects (n = 11, 15.5%). Surgical procedures including primary repair (n = 38, 53.5%), staged closure (n = 30, 42.3%) with average 8.6 days (SD 4.7) of silo reduction, and conservative management (n = 3, 4.2%) were performed. The mortality rate was 14.1% (n = 10) and the complication rate was 36.6% (n = 26). The majority of patients had normal intellectual development (92.5%) and growth (79.2%) on the latest follow-up. For gastroschisis, one patient (7.1%) had intestinal atresia. Surgical procedures included primary repair (n = 9, 64.3%) and staged closure (n = 5, 35.7%) with average 8 days (SD 3.5) of silo reduction. Complication rate was 21.4% (n = 3), with one mortality (7.1%). All patients had normal intellectual development and growth. The mean follow-up time of this series is 76.9 months (SD 62.9). Most abdominal wall defects in our series were managed surgically with a good overall survival rate and long-term outcome. This information is essential during antenatal and postnatal counseling for parents.


Assuntos
Gastrosquise , Hérnia Umbilical , Humanos , Gastrosquise/cirurgia , Gastrosquise/complicações , Gastrosquise/diagnóstico , Hérnia Umbilical/cirurgia , Estudos Retrospectivos , Feminino , Masculino , Recém-Nascido , Hong Kong/epidemiologia , Resultado do Tratamento
2.
FASEB J ; 33(4): 5143-5152, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30624964

RESUMO

The level of microRNA (miR)-431 was found to be markedly up-regulated in intestinal tissue of necrotizing enterocolitis (NEC). The objective of this study was to identify the target gene of miR-431 and to investigate the role of the miR-431-FOXA1 axis in the pathophysiology of NEC. The target gene of miR-431 was identified by in silico target prediction bioinformatics, luciferase assay, and Western blotting. Effects of miR-431 on downstream expression signals, cell proliferation, and apoptosis were investigated by overexpression in Caco-2 cells upon stimulation by LPS or lipoteichoic acid (LTA). FOXA1 was identified as the target gene of miR-431. Overexpression of miR-431 in Caco-2 cells significantly inhibited FOXA1, ESRRG, and HNF4A and activated IL-6, LGR5, NFKB2, PLA2G2A, PRKCZ, and TNF. IL-8 and - 10 were enhanced when costimulated with LPS or LTA. These potential downstream genes were also significantly dysregulated in primary NEC tissues compared with surgical-control tissues. Overexpression of miR-431 significantly decreased proliferation and increased apoptosis of Caco-2 cells. A proposed network of miR-431-FOXA1 interaction with LPS and LTA receptors demonstrates dysregulation of transcription factors, inflammatory mediators, epithelium tight junction regulators, and cell proliferation and apoptosis signals. The miR-431-FOXA1 axis could in part be responsible for the intensification of the inflammatory response in NEC tissues and contribute to the proinflammatory pathophysiology.-Wu, Y. Z., Chan, K. Y. Y., Leung, K. T., Lam, H. S., Tam, Y. H., Lee, K. H., Li, K., Ng, P. C. Dysregulation of miR-431 and target gene FOXA1 in intestinal tissues of infants with necrotizing enterocolitis.


Assuntos
Enterocolite Necrosante/metabolismo , Fator 3-alfa Nuclear de Hepatócito/metabolismo , MicroRNAs/metabolismo , Apoptose/efeitos dos fármacos , Apoptose/genética , Western Blotting , Células CACO-2 , Proliferação de Células/efeitos dos fármacos , Proliferação de Células/genética , Enterocolite Necrosante/genética , Citometria de Fluxo , Células HEK293 , Fator 3-alfa Nuclear de Hepatócito/genética , Humanos , Mucosa Intestinal/metabolismo , Lipopolissacarídeos/farmacologia , MicroRNAs/genética , Plasmídeos/genética , Reação em Cadeia da Polimerase em Tempo Real , Ácidos Teicoicos/farmacologia
3.
Pediatr Surg Int ; 34(1): 47-53, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29075908

RESUMO

BACKGROUND/PURPOSE: Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. METHODS: This is a retrospective multicenter (n = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed. The extent of disease involvement and bowel resection was retrieved by reviewing the operative records as well as histopathological reports of the resected specimens. Clinical assessment was performed according to the criteria of a seven-itemed bowel function score (BFS) (maximum score = 20). Manometric assessment was performed with anorectal manometry. RESULTS: The study period started from 2003 to 45 patients were studied with median age at assessment = 52.0 months and operation = 3.0 months. The disease involvement was categorized into upper sigmoid-descending colon (DC) (n = 8), sigmoid colon (SC) (n = 12), upper rectum (UR) (n = 14) and lower rectum (LR) (n = 11) according to the level of normal biopsy result. There was no significant difference in the age of assessment between the four groups. The median BFSs in the DC, SC, UR and LR were 13, 15, 17 and 17, respectively (p = 0.01). Nine patients from the DC and SC groups reported soiling for more than twice per week. Sub-group analysis comparing patients with and without the entire sigmoid colon resected revealed worse functional outcomes in terms of the incidence of soiling (40.7 vs 22.2%, p = 0.05) and the BFS (14 vs 18, p = 0.04) in the former group. Anorectal manometry did not reveal any significant difference between the four groups, but a higher proportion of patients in the UR and LR groups appeared to have a normal sphincter resting pressure (DC vs SC vs UR vs LR = 62.5 vs 75.0 vs 85.7 vs 80.0%, p = 0.10). CONCLUSION: Patients with short segment HSCR are not equal at all. HSCR patients with aganglionosis limited to the rectum without the need of removing the entire sigmoid colon have a better bowel control and overall functional score. Less bowel loss and colonic dissection maybe the underlying reasons. Although future studies with a larger sample size and a longer follow-up period are required to validate the results of this study, it has provided a new insight to the current understanding of short segment disease in HSCR.


Assuntos
Doença de Hirschsprung/cirurgia , Criança , Pré-Escolar , Colo Sigmoide/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório , Feminino , Humanos , Masculino , Manometria , Avaliação de Resultados da Assistência ao Paciente , Prognóstico , Reto/cirurgia , Estudos Retrospectivos
4.
J Pediatr ; 177: 78-83.e3, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27480200

RESUMO

OBJECTIVES: To assess preferences of health care workers (HCWs) and parents of term and preterm infants to adverse health outcomes, and how perceived surgical mortality influences decision-making. STUDY DESIGN: A total of 536 participants (157 HCWs, 201 parents of term infants, and 178 parents of preterm infants) were recruited to take part in a structured interview. Preferences related to treatment of a critically ill preterm infant with necrotizing enterocolitis were measured by health state rank permutation analysis and pivotal risk analysis. Between-group and subgroup comparisons were performed. RESULTS: HCWs rank adverse health states less favorably than parents of preterm infants, consistently ranking 2 of the most adverse health states worse than death. Pivotal risk values of HCWs for all health states were consistently the lowest of the 3 groups. High operative mortality was associated uniformly with reduction in pivotal risks for all groups both in favorable and adverse health states. Subgroup analyses revealed significant discrepancies in preferences between fathers and mothers as well as doctors and nurses. Regular religious practice was significantly associated with increased pivotal risks in parental subgroups. CONCLUSIONS: As discrepancies in health state preferences existed between subgroups (ie, doctors vs nurses, mothers vs fathers) and perceived operative mortality consistently biased parental and HCW health state preferences, we recommend that HCWs should first identify differences regarding patient management before interviewing the parents together. HCWs should be aware of inadvertently biasing parents when discussing the risks and outcomes of surgery in conjunction with the overall long-term prognosis of the underlying condition.


Assuntos
Atitude do Pessoal de Saúde , Tomada de Decisões , Pais/psicologia , Procedimentos Cirúrgicos Operatórios/psicologia , Feminino , Hong Kong , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Fatores de Risco , Nascimento a Termo
5.
Surg Endosc ; 30(12): 5494-5499, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27129540

RESUMO

BACKGROUND: The aim of this review was to study the characteristics and the outcome of children who underwent laparoscopic management of the antenatally detected choledochal cyst. METHODS: A retrospective review was conducted for all children who underwent excision of antenatally detected choledochal cyst and hepaticojejunostomy from 2005 to 2015. RESULTS: Fourteen patients (11 females and 3 males) were included in this study. The mean diameter of the cyst was 5.0 cm (range 2-12 cm). The mean age at operation was 3.5 months (range 11 days to 9 months). The mean body weight was 6.0 kg (range 3.9-10.0 kg). Five patients were symptomatic before operation. Laparoscopic excision of choledochal cyst was successful in all cases. The distal end of common bile duct (CBD) was cauterized in all but two cases. Conversion to open hepaticojejunostomy was required in two children. There was no intraoperative complication. Two patients had postoperative fever. One patient had minor bile leak that resolved on conservative management. The mean operative time was 286 min (range 200-390 min). The median hospital stay was 8 days (range 6-25 days). At a median follow-up of 46 months (range 6-118 months), all patients were freed from cholangitis or intestinal obstruction. Symptomatic patients had earlier operation (mean, 1.7 vs. 4.5 months p = 0.012) and were associated with postnatal increase in cyst size (p = 0.023) but were not associated with increased risk of complication or conversion (p = 1.000). CONCLUSIONS: Laparoscopic excision of choledochal cyst and hepaticojejunostomy could be safety performed in patients with the cyst detected antenatally. The distal CBD was usually small and stenotic. Symptomatic patients had earlier operation with no increase in morbidity.


Assuntos
Procedimentos Cirúrgicos do Sistema Biliar/métodos , Cisto do Colédoco/cirurgia , Laparoscopia , Diagnóstico Pré-Natal , Anastomose Cirúrgica , Cisto do Colédoco/diagnóstico , Feminino , Seguimentos , Ducto Hepático Comum/cirurgia , Humanos , Lactente , Recém-Nascido , Jejuno/cirurgia , Masculino , Gravidez , Estudos Retrospectivos , Resultado do Tratamento
6.
Pediatr Surg Int ; 32(11): 1087-1092, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27473011

RESUMO

PURPOSE: To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique. METHODS: We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra. RESULTS: There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture. CONCLUSIONS: Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.


Assuntos
Hipospadia/cirurgia , Uretra/cirurgia , Criança , Pré-Escolar , Humanos , Lactente , Masculino , Estudos Retrospectivos , Retalhos Cirúrgicos , Resultado do Tratamento
7.
Ann Surg ; 260(6): 1128-37, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24368664

RESUMO

OBJECTIVE: To provide a comprehensive database of gene regulation and compare differentially regulated molecular networks in human tissues of necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP). BACKGROUND: Both NEC and SIP are devastating surgical emergencies associated with high morbidity and mortality in preterm infants. Their pathophysiology and molecular mechanisms remain unclear. METHODS: Differential whole genome microarray analysis was performed on intestinal tissues collected from NEC (n = 15) and SIP (n = 12) infants and compared with tissues collected from surgical-control patients with noninflammatory intestinal conditions (n = 14). Validation of 52 target gene expressions was performed by quantitative polymerase chain reaction. Regulatory networks of significantly affected genes were constructed according to functional pathways. RESULTS: Extensive and significant changes of gene expression were observed in NEC tissues, which comprised multiple pathways of angiogenesis, arginine metabolism, cell adhesion and chemotaxis, extracellular matrix remodeling, hypoxia and oxidative stress, inflammation, and muscle contraction. These dysregulated genes could be networked downstream of key receptors, TLR2, TLR4, and TREM1, and mediated via NF-κB, AP-1, and HIF1A transcription factor pathways, indicating predominant microbial and inflammatory involvement. In contrast, SIP tissues exhibited much milder and less diversified expressional changes, with target genes significantly associated with G-protein-mediated muscle contraction and extracellular matrix remodeling. CONCLUSIONS: The molecular evidence suggests that NEC and SIP are likely 2 different diseases caused by distinct etiology and pathophysiology. This first comprehensive database on differential gene expression profiles of human NEC and SIP tissues could lead to development of disease-specific diagnostic and prognostic biomarkers and new therapeutic strategies for improving outcomes.


Assuntos
Enterocolite Necrosante/genética , Proteínas da Matriz Extracelular/genética , Regulação da Expressão Gênica no Desenvolvimento , Estudo de Associação Genômica Ampla/métodos , Mucosa Intestinal/metabolismo , Perfuração Intestinal/genética , RNA Mensageiro/genética , Enterocolite Necrosante/metabolismo , Proteínas da Matriz Extracelular/biossíntese , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/genética , Doenças do Prematuro/metabolismo , Perfuração Intestinal/metabolismo , Masculino , Reação em Cadeia da Polimerase , Estudos Retrospectivos
8.
Clin Gastroenterol Hepatol ; 12(10): 1759-62.e1, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24534547

RESUMO

Abuse of ketamine leads to liver injury. We investigated the histopathologic and radiologic features of ketamine abusers with significant liver injury in a cross-sectional survey of 297 consecutive chronic abusers of ketamine with urinary tract dysfunction. Liver biopsy and magnetic resonance cholangiopancreatography were performed in patients with liver injury (concentrations of bilirubin, alkaline phosphatase, and/or alanine aminotransferase >2-fold the upper limit of normal). The prevalence of liver injury was 9.8% (all cases cholestatic). Bile duct injury was observed in all 7 patients assessed by liver biopsy. Two patients had bridging fibrosis despite their young age. Three of 6 patients who underwent magnetic resonance cholangiopancreatography examination were found to have prominent or dilated common bile ducts without obstructions or extrinsic compressions. Ketamine abuse therefore appears to lead to common bile duct dilatation, microscopic bile duct injury, and even significant liver fibrosis.


Assuntos
Doença Hepática Induzida por Substâncias e Drogas/epidemiologia , Ketamina/efeitos adversos , Transtornos Relacionados ao Uso de Substâncias/complicações , Adulto , Ductos Biliares/patologia , Biópsia , Colangiopancreatografia por Ressonância Magnética , Estudos Transversais , Feminino , Histocitoquímica , Humanos , Fígado/diagnóstico por imagem , Fígado/patologia , Masculino , Prevalência , Radiografia , Adulto Jovem
9.
BJU Int ; 114(5): 754-60, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24552244

RESUMO

OBJECTIVE: To describe a service delivery model and report the baseline characteristics of patients investigated by a non-invasive approach for ketamine-associated uropathy. PATIENTS AND METHODS: This was a cross-sectional study in a prospective cohort of patients who attended their first visit and underwent non-invasive investigations at a dedicated centre to treat ketamine-associated uropathy in Hong Kong from December 2011 to July 2013. Data on demographics, illicit ketamine use, symptoms scores and voiding function parameters at baseline were prospectively collected. Differences between active abusers and ex-abusers, and risk factors for the most symptomatic group were investigated by univariate and multivariate analysis. RESULTS: In all, 318 patients completed the non-invasive assessment at their first visit and were eligible for inclusion. In all, 174 were female and the mean (sd) age of the entire cohort was 24.4 (3.1) years. Patients had used ketamine for a mean (sd) period of 81 (36) months. The mean (sd) ketamine use per week was 18.5 (15.8) g. In all, 214 patients were active abusers while 104 were ex-abusers but had persistent lower urinary tract symptoms. The mean (sd) voided volume, bladder capacity, and bladder emptying efficiency were 111.5 (110) mL, 152.5 (126) mL and 73.3 (26.9)%, respectively. The ex-abusers had a lower symptom score (19.3 vs 24.1; P < 0.001), a larger voided volume (126 vs 85 mL; P < 0.001), and a larger bladder capacity (204.8 vs 126.7 mL; P < 0.001) compared with active abusers. Multivariate analysis found female gender was associated with a higher symptom score (odds ratio [OR] 2.39; 95% confidence interval [CI] 1.35-4.23; P = 0.003) and a smaller voided volume (OR 1.9; 95% CI 1.1-3.3; P = 0.02). Ketamine taken (g/week) was another risk factor for a higher symptom score (OR 1.03; 95% CI 1.01-1.05; P = 0.002). Status of ex-abuser was the only protective factor associated with fewer symptoms, larger voided volume and bladder capacity. CONCLUSIONS: An effective service model for recruiting patients with ketamine-associated uropathy is possible. With such a service model as a platform, further prospective studies are warranted to investigate the appropriate choice of treatment for this new clinical entity.


Assuntos
Drogas Ilícitas/intoxicação , Ketamina/intoxicação , Dor Pélvica/induzido quimicamente , Transtornos Relacionados ao Uso de Substâncias/etiologia , Doenças Urológicas/induzido quimicamente , Adolescente , Adulto , Análise de Variância , Estudos Transversais , Feminino , Hong Kong/epidemiologia , Humanos , Masculino , Dor Pélvica/epidemiologia , Fatores de Risco , Transtornos Relacionados ao Uso de Substâncias/epidemiologia , Doenças Urológicas/epidemiologia , Adulto Jovem
10.
Pediatr Surg Int ; 30(6): 605-8, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24722760

RESUMO

PURPOSE: Apparently superior result was observed after open Kasai portoenterostomy in infant with biliary atresia. Our institute stopped performing laparoscopic portoenterostomy since 2007. We aimed to investigate the outcome after reintroduction of open portoenterostomy. METHODS: 27 non-syndromic infants underwent open Kasai portoenterostomy from 2007 to 2012. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 µmol/L within 6 month of portoenterostomy), the native liver survival at 2 years after the operation were reviewed. The results were retrospectively compared with all 16 infants who underwent laparoscopic Kasai portoenterostomy before 2007. RESULTS: All infants had type III biliary atresia. No statistical difference was observed regarding the age at operation and the pre-operative bilirubin level. The early clearance of jaundice rate was 81% (22/27) after open operation and was 50% (8/16) after laparoscopic operation (p = 0.03). At 2 years after the operation, the native liver survival was 81% (22/27) after open operation and was 50% (8/16) after laparoscopic operation (p = 0.03). CONCLUSION: Reintroduction of open Kasai portoenterostomy was associated with superior early clearance of jaundice rate and 2-year native liver survival rate.


Assuntos
Atresia Biliar/cirurgia , Laparoscopia/métodos , Portoenterostomia Hepática/métodos , Bilirrubina/análise , Biomarcadores/análise , Feminino , Humanos , Lactente , Masculino , Taxa de Sobrevida , Resultado do Tratamento
11.
Cureus ; 15(8): e43352, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37700992

RESUMO

Disorders of sex development (DSD) are caused by defects in the complex sexual differentiation cascade, resulting in discordance among an individual's genetic, gonadal, and genital sexes. It affects one in 4,500 live births. A wide spectrum of genital phenotypes can be found depending on the underlying pathogenic mechanism and the developmental stage that is affected. We herein report a newborn with female external genitalia but palpable gonads at labia majora with normal testicular function and structure, which is typical of complete androgen insensitivity syndrome (CAIS). The genetic study revealed 45,X/46,XY mosaicism and c.2081A>C missense androgen receptor gene mutation, indicating the likelihood of co-existing CAIS. This case demonstrated the importance of correlating genital phenotype and the underlying pathogenic mechanism, to provide appropriate management of DSD. Important considerations on managing the gonads about the risks of gonadal malignancies are also discussed.

12.
Pediatr Blood Cancer ; 59(7): 1302-4, 2012 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-22415906

RESUMO

Central venous catheterization is essential in the management of patients requiring long-term intravenous access. Various risks during central line insertion have been described in the medical literature, including the potentially life-threatening complication of iatrogenic arteriovenous fistula (AVF). We describe a novel case of carotid jugular AVF following implanted port (BardPort® by C.R. Bard, Inc.) insertion in a pediatric oncology patient who had suffered cerebral infarct due to thromboembolism. Pediatr Blood Cancer 2012; 59: 1302-1304. © 2012 Wiley Periodicals, Inc.


Assuntos
Fístula Arteriovenosa/etiologia , Artéria Carótida Primitiva , Cateterismo Venoso Central/efeitos adversos , Veias Jugulares , Antineoplásicos/administração & dosagem , Pré-Escolar , Feminino , Humanos
13.
J Paediatr Child Health ; 48(7): 600-3, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22404641

RESUMO

AIM: Oophorectomy performed in children is extremely uncommon. We aimed to investigate the disease pattern and the association between the underlying pathology and the clinical presentation among those patients who had their ovaries removed in their childhood. METHODS: A retrospective study was performed on 41 consecutive children who underwent oophorectomy in a tertiary referral centre in the period between June 1995 and May 2008. RESULTS: The median age was 11 years, ranged from 11 weeks to 15 years at the time of surgery. The primary presentations were acute lower abdominal pain (n= 20), progressive abdominal distension or abdominal mass (n= 13), chronic abdominal pain (n= 3), irregular menses (n= 1), antenatal diagnosis (n= 3) and incidental finding (n= 1). Ultrasound examination was performed in 31 patients and positive findings of ovarian pathology were found in all but one examination. Twenty cases of ovarian torsion were confirmed intra-operatively. Patients presenting with acute abdominal pain were more likely to have torsion than other presentations (P < 0.01). Non-neoplastic conditions and ovarian neoplasms were found in 11 and 30 patients, respectively. The most common neoplasm was mature teratoma (52%). Malignant neoplasms included immature teratoma (n= 3), dysgerminoma (n= 1), mixed dysgerminoma + yolk sac tumour (n= 2), yolk sac tumour (n= 2) and juvenile granulose cell tumour (n= 1). Malignant neoplasms were found to have more chronic presentation and less torsion than benign pathologies (P < 0.05). CONCLUSION: Although ovarian pathology is uncommon in children, a girl presenting with acute lower abdominal pain or progressive abdominal distension should raise the suspicion and prompt immediate investigation to rule out ovarian torsion or ovarian neoplasms.


Assuntos
Doenças Ovarianas/cirurgia , Ovariectomia/estatística & dados numéricos , Ovário/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Doenças Ovarianas/diagnóstico , Ovário/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
14.
Pediatr Surg Int ; 28(11): 1109-13, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22987040

RESUMO

PURPOSE: Laparoscopic Kasai portoenterostomy was reported to be a safe and feasible procedure in infant with biliary atresia. We aimed to investigate the long-term results after laparoscopic portoenterostomy as such data in the literature are lacking. METHODS: Sixteen infants underwent laparoscopic Kasai portoenterostomy from 2002 to 2006. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 µmol/L within 6 months of portoenterostomy), the native liver survival at 2 and 5 years after the operation were reviewed. The results were retrospectively compared with 16 consecutive infants who underwent open Kasai portoenterostomy before 2002. RESULTS: All infants had type III biliary atresia. The early clearance of jaundice rate at 6 months was 50 % (8/16) after laparoscopic operation and was 75 % (12/16) after open operation (p = 0.144). Two years after the operation, the native liver survival was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group (p = 0.076). Five years after the operation, the native liver survival rate was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group (p = 0.076). The jaundice-free native liver survival rate at 5 years was 50 % (8/16) in laparoscopic group and was 75 % (12/16) in the open group. In the laparoscopic group, all patients with early clearance of jaundice survived and remained jaundice freed 5 years after the operation. CONCLUSION: The 5-year native liver survival rate after laparoscopic portoenterostomy was 50 %. Apparently superior result was observed in the open group (81 %) although the figures did not reach statistical difference because of the small sample size. A larger scale study is required to draw a more meaningful conclusion.


Assuntos
Atresia Biliar/cirurgia , Laparoscopia , Portoenterostomia Hepática , Feminino , Humanos , Lactente , Fígado/fisiologia , Masculino , Estudos Retrospectivos , Fatores de Tempo
15.
J Pediatr Surg ; 57(7): 1398-1403, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34167801

RESUMO

BACKGROUND: Children affected by 45,X/46,XY mosaicism have a wide spectrum in their phenotypes. Although surgery has a role in management, there is no universally agreed strategy. METHODS: We conducted a retrospective review in a tertiary center on the surgical management and its long-term outcomes in children with the karyotype 45,X/46,XY or its variants over a 20-year study period. Patients were divided into 4 groups based on their phenotypes. RESULTS: 22 children(female=13, male=9) were included in the study, and were grouped as I)female phenotype(n = 11), II)ambiguous external genitalia(n = 2), III)undervirilized male with hypospadias(n = 6), and IV)normal male phenotype(n = 3). 19/22(86%) underwent gonadal surgeries(bilateral gonadectomy=15; unilateral gonadectomy=1; testicular biopsy=3) and  8/22(36%) underwent genitalia reconstructive surgeries(hypospadias repair=6; feminizing surgery=2). Gonadal germ cell tumors were detected in 55%, 50% and 50% of Group I, II and III respectively. 3/6 patients required reoperations for complications after hypospadias repairs. None of the patients had recurrence of germ cell tumors after gonadectomy. All the patients who had reached late adolescence or adulthood maintained their genders as their sex of rearing. CONCLUSIONS: Majority of 45,X/46,XY children benefit from timely gonadal surgery while genitalia reconstructive surgery can be considered in selected patients. Surgical management can be strategized by patients' phenotypes.


Assuntos
Transtornos do Desenvolvimento Sexual , Hipospadia , Neoplasias Embrionárias de Células Germinativas , Adulto , China/epidemiologia , Feminino , Humanos , Hipospadia/complicações , Hipospadia/genética , Hipospadia/cirurgia , Masculino , Mosaicismo , Fenótipo
16.
Front Pediatr ; 10: 1017722, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36389392

RESUMO

The management of children with concomitant ureteropelvic junction (UPJ) obstruction and calyceal stones remains challenging. The various treatment options available for pediatric nephrolithiasis may require multiple sessions, and the techniques by themselves are not designed for simultaneous correction of UPJ obstruction. Recently, success in combining robot-assisted laparoscopic pyeloplasty (RALP) and flexible endoscopy has been reported by multi-institutional studies to treat children with concomitant UPJ obstruction and renal stones. Given the paucity of technical details of this novel approach in the existing literature, we herein report our techniques to treat two girls aged 6 and 10 years who had concomitant UPJ obstruction and multiple stones in mid- and lower poles calyces. Three robotic ports were used without any assistant ports. A flexible endoscope, either a cystoscope or a single-use ureteroscope, was introduced via the undocked epigastric port to perform nephroscopy and stones removal after the renal pelvis was opened. The rest of the RALP was completed in the usual manner. Technical modifications were employed to facilitate the flexible endoscope to examine the entire calyceal system. Both patients underwent successful surgical procedures by the combined approach without any intra- or post-operative complications. Three and 14 stones were removed from each of the patients respectively. Postoperative investigations demonstrated successful correction of UPJ obstruction and complete stone clearance in both patients. A combined approach of RALP and flexible endoscopy is a safe and effective technique to treat concurrent UPJ obstruction and calyceal stones in children.

18.
J Pediatr Gastroenterol Nutr ; 52(4): 387-91, 2011 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21415670

RESUMO

OBJECTIVE: Pediatric Rome III criteria of functional dyspepsia (FD) has eliminated the mandatory use of upper endoscopy and recommended a symptom-based approach. In the absence of alarm symptoms, FD can be positively diagnosed in children having normal physical findings without exclusionary investigations. We aimed to investigate the effectiveness of Rome III guidelines to discriminate organic diseases from FD and to identify the predictors for positive endoscopic findings. PATIENTS AND METHODS: A prospective study was conducted on consecutive children fulfilling Rome III criteria of FD. Upper endoscopy was performed in all subjects, both with and without alarm features. RESULTS: Eighty consecutive children ages 7 to 15 were recruited. Nine (11.3%) had experienced alarm features. Five (6.3%) had organic diseases confirmed in upper endoscopy: duodenal ulcer (n = 2), duodenitis with erosion (n = 2), and gastritis with erosion (n = 1), 33.3% of children having alarm features had organic pathology, compared with 2.8% of those without (P < 0.01). A male predominance (80% vs 25.3%, P < 0.01), higher prevalence of alarm features (60% vs 8%, P < 0.01), and higher prevalence of Helicobacter pylori infection (80% vs 5.3%, P < 0.01) were found in children with organic diseases, compared with FD. Multivariate analysis identified H pylori infection (odds ratio 23.2; 95% confidence interval 1.5-333) and nocturnal pain (odds ratio 26.3; 95% confidence interval 1.2-500) to be independent predictors for positive endoscopic findings. CONCLUSIONS: Rome III recommendations of screening dyspeptic children for alarm features and investigation for H pylori are effective to identify children who have a higher likelihood of organic diseases and require upper endoscopy before making a diagnosis of FD.


Assuntos
Dispepsia/etiologia , Gastroenteropatias/diagnóstico , Guias de Prática Clínica como Assunto , Adolescente , Criança , China/epidemiologia , Diagnóstico Diferencial , Técnicas de Diagnóstico do Sistema Digestório , Endoscopia Gastrointestinal , Feminino , Gastroenteropatias/epidemiologia , Gastroenteropatias/fisiopatologia , Humanos , Masculino , Ambulatório Hospitalar , Pediatria/métodos , Prevalência , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Sociedades Médicas
19.
Pediatr Surg Int ; 27(7): 671-4, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21286732

RESUMO

AIM: There were only a few reports on laparoscopic portoenterostomy for biliary atresia in children. We report our experience on laparoscopic Kasai operation for biliary atresia. MATERIAL AND METHODS: A retrospective review of all children who had biliary atresia underwent laparoscopic portoenterostomy from January 2002 to September 2006 were included and analyzed. RESULTS: Sixteen children (five boys and eleven girls) with a mean age of 66 days (range 47-106 days) at operation were included in this study. All patients had type III biliary atresia. There was no conversion to open procedure. The operative time ranged from 193 to 435 min (mean 292 min). At a median follow-up of 72 months (range 33-89 months), eight patients were free of jaundice with bilirubin level <20 µmol/L and did not require liver transplantation. Seven patients underwent liver transplantation. One patient who had persistent elevated bilirubin defaulted follow-up. One patient complicated with volvulus of intestine post-operatively. All patients survived except one patient who had combined liver and intestine transplantation. CONCLUSION: Laparoscopic portoenterostomy in children with biliary atresia is technically feasible. 50% of children who had type III biliary atresia were free of jaundice and did not require liver transplantation. A longer follow-up is required to assess the long-term outcome.


Assuntos
Atresia Biliar/cirurgia , Laparoscopia/métodos , Portoenterostomia Hepática/métodos , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
20.
Front Pediatr ; 9: 647139, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34195160

RESUMO

Objective: To investigate the outcomes of minimally invasive approach to infants with ureteropelvic junction (UPJ) obstruction by comparing the two surgical modalities of robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP). Methods: We conducted a retrospective review of all consecutive infants aged ≤12 months who underwent either LP or RALP in a single institution over the period of 2008-Jul 2020. We included primary pyeloplasty cases that were performed by or under the supervision of the same surgeon. Results: Forty-six infants (LP = 22; RALP = 24) were included with medians of age and body weight at 6 months (2-12months) and 8.0 kg (5.4-10 kg), respectively. There was no difference between the two groups in the patients' demographics and pre-operative characteristics. All infants underwent LP or RALP successfully without conversion to open surgery. None had intraoperative complications. Operative time (OT) was 242 min (SD = 59) in LP, compared with 225 min (SD = 39) of RALP (p = 0.25). Linear regression analysis showed a significant trend of decrease in OT with increasing case experience of RALP(p = 0.005). No difference was noted in the post-operative analgesic requirement. RALP was associated with a shorter hospital length of stay than LP (3 vs. 3.8 days; p = 0.009). 4/22(18%) LP and 3/24(13%) RALP developed post-operative complications (p = 0.59), mostly minor and stent-related. The success rates were 20/22 (91%) in LP and 23/24 (96%) in RALP (p = 0.49). Conclusions: Pyeloplasty by minimally invasive approach is safe and effective in the infant population. RALP may have superiority over LP in infants with its faster recovery and a more manageable learning curve to acquire the skills.

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