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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.
Pediatr Surg Int ; 38(7): 1013-1018, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35523886

RESUMO

OBJECTIVE: Liver transient elastography (TE) using FibroScan® has gained popularity as a non-invasive technique to assess hepatic fibrosis by measuring liver stiffness. This study focused on biliary atresia patients post Kasai operation for more than 10 years to prospectively correlate the hepatic fibrosis score to the biochemical changes of liver fibrosis and clinical development of portal hypertensive complications. METHODS: TE was performed in 37 patients who had biliary atresia post Kasai operation done at median age of 60 days. Biochemical indices of liver fibrosis including aspartate aminotransferase/platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score based on age, platelet count, alanine aminotransferase and aspartate aminotransferase level were calculated at the time of TE. Platelet count, spleen size, varices, ascites and hepatic encephalopathy were evaluated as clinical markers of portal hypertension. RESULTS: There were 22 female and 15 male with TE done at median age of 17.0 years. Median FibroScan® fibrosis score was 11.4. Fibrosis score of 6.8 kilopascal (kPa) was taken as the upper reference limit of normal. Nine patients (24%) had normal fibrosis score. Score above or equal to 6.8 kPa was significantly associated with lower platelet level (p = 0.001), higher INR (p = 0.043), higher APRI (p = 0.021), higher FIB-4 score (p = 0.013), and larger splenic diameter (p = 0.004). Higher FibroScan® fibrosis score was also significantly associated with portal hypertensive complications (p = 0.001). CONCLUSIONS: The FibroScan® fibrosis score correlated well with the biochemical changes of liver fibrosis and development of portal hypertensive complications clinically. Screening of portal hypertensive complications such as varices is recommended for patients with raised fibrosis score upon long-term follow-up. LEVEL OF EVIDENCE: Level III, retrospective comparative study.


Assuntos
Atresia Biliar , Técnicas de Imagem por Elasticidade , Fígado , Varizes , Adolescente , Aspartato Aminotransferases/metabolismo , Atresia Biliar/complicações , Atresia Biliar/diagnóstico por imagem , Atresia Biliar/cirurgia , Biomarcadores/análise , Feminino , Seguimentos , Humanos , Fígado/diagnóstico por imagem , Fígado/patologia , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico por imagem , Masculino , Estudos Retrospectivos , Varizes/etiologia , Varizes/patologia
3.
J Pediatr Hematol Oncol ; 43(8): e1191-e1193, 2021 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-34001798

RESUMO

Germline adenomatous polyposis coli (APC) gene mutation is a cancer-predisposing condition commonly presenting as familial adenomatous polyposis. We describe a patient first diagnosed at the age of 3 years with metastatic hepatoblastoma. With a positive family history, germline testing confirmed maternally inherited APC mutation (p.Thr899Ansfs*13). The patient was subsequently diagnosed at 8 years with colonic adenocarcinoma in the absence of macroscopic polyposis. Total colectomy with adjuvant chemotherapy was delivered and the patient remained disease-free for 5 years since the second diagnosis. This report demonstrates the importance of considering germline APC mutation in children with hepatoblastoma, who may benefit from the early institution of colonoscopic surveillance.


Assuntos
Adenocarcinoma/patologia , Proteína da Polipose Adenomatosa do Colo/genética , Polipose Adenomatosa do Colo/patologia , Neoplasias do Colo/patologia , Mutação em Linhagem Germinativa , Hepatoblastoma/patologia , Neoplasias Hepáticas/patologia , Adenocarcinoma/etiologia , Adenocarcinoma/terapia , Polipose Adenomatosa do Colo/etiologia , Polipose Adenomatosa do Colo/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia Adjuvante , Criança , Pré-Escolar , Colectomia , Neoplasias do Colo/etiologia , Neoplasias do Colo/terapia , Terapia Combinada , Feminino , Hepatoblastoma/genética , Hepatoblastoma/terapia , Humanos , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/terapia , Prognóstico
4.
Hum Mol Genet ; 27(2): 351-358, 2018 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-29177441

RESUMO

The cloaca is an embryonic cavity that is divided into the urogenital sinus and rectum upon differentiation of the cloacal epithelium triggered by tissue-specific transcription factors including CDX2. Defective differentiation leads to persistent cloaca in humans (PC), a phenotype recapitulated in Cdx2 mutant mice. PC is linked to hypo/hyper-vitaminosis A. Although no gene has ever been identified, there is a strong evidence for a genetic contribution to PC. We applied whole-exome sequencing and copy-number-variants analyses to 21 PC patients and their unaffected parents. The damaging p.Cys132* and p.Arg237His de novo CDX2 variants were identified in two patients. These variants altered the expression of CYP26A1, a direct CDX2 target encoding the major retinoic acid (RA)-degrading enzyme. Other RA genes, including the RA-receptor alpha, were also mutated. Genes governing the development of cloaca-derived structures were recurrently mutated and over-represented in the basement-membrane components set (q-value < 1.65 × 10-6). Joint analysis of the patients' profile highlighted the extracellular matrix-receptor interaction pathway (MsigDBID: M7098, FDR: q-value < 7.16 × 10-9). This is the first evidence that PC is genetic, with genes involved in the RA metabolism at the lead. Given the CDX2 de novo variants and the role of RA, our observations could potentiate preventive measures. For the first time, a gene recapitulating PC in mouse models is found mutated in humans.


Assuntos
Fator de Transcrição CDX2/genética , Fator de Transcrição CDX2/metabolismo , Anormalidades Urogenitais/genética , Povo Asiático/genética , Diferenciação Celular/genética , Cloaca/embriologia , Variações do Número de Cópias de DNA , Família , Feminino , Proteínas de Homeodomínio/genética , Humanos , Masculino , Mutação , Fenótipo , Anormalidades Urogenitais/metabolismo , Sequenciamento do Exoma
5.
J Pediatr ; 224: 30-36, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32565097

RESUMO

OBJECTIVES: To compare the clinical and laboratory features of severe acute respiratory syndrome 2003 (SARS) and coronavirus disease 2019 (COVID-19) in 2 Chinese pediatric cohorts, given that the causative pathogens and are biologically similar. STUDY DESIGN: This is a cross-sectional study reviewing pediatric patients with SARS (n = 43) and COVID-19 (n = 244) who were admitted to the Princess Margaret Hospital in Hong Kong and Wuhan Children's Hospital in Wuhan, respectively. Demographics, hospital length of stay, and clinical and laboratory features were compared. RESULTS: Overall, 97.7% of patients with SARS and 85.2% of patients with COVID-19 had epidemiologic associations with known cases. Significantly more patients with SARS developed fever, chills, myalgia, malaise, coryza, sore throat, sputum production, nausea, headache, and dizziness than patients with COVID-19. No patients with SARS were asymptomatic at the time of admission, whereas 29.1% and 20.9% of patients with COVID-19 were asymptomatic on admission and throughout their hospital stay, respectively. More patients with SARS required oxygen supplementation than patients with COVID-19 (18.6 vs 4.7%; P = .004). Only 1.6% of patients with COVID-19 and 2.3% of patients with SARS required mechanical ventilation. Leukopenia (37.2% vs 18.6%; P = .008), lymphopenia (95.4% vs 32.6%; P < .01), and thrombocytopenia (41.9% vs 3.8%; P < .001) were significantly more common in patients with SARS than in patients with COVID-19. The duration between positive and negative nasopharyngeal aspirate and the length in hospital stay were similar in patients with COVID-19, regardless of whether they were asymptomatic or symptomatic, suggesting a similar duration of viral shedding. CONCLUSIONS: Children with COVID-19 were less symptomatic and had more favorable hematologic findings than children with SARS.


Assuntos
Infecções por Coronavirus/epidemiologia , Pneumonia Viral/epidemiologia , Síndrome Respiratória Aguda Grave/epidemiologia , Adolescente , Infecções Assintomáticas , Betacoronavirus , COVID-19 , Criança , Pré-Escolar , China/epidemiologia , Infecções por Coronavirus/diagnóstico , Estudos Transversais , Feminino , Hong Kong , Hospitalização , Humanos , Lactente , Tempo de Internação , Masculino , Pandemias , Pneumonia Viral/diagnóstico , Estudos Retrospectivos , Coronavírus Relacionado à Síndrome Respiratória Aguda Grave , SARS-CoV-2 , Síndrome Respiratória Aguda Grave/diagnóstico
6.
Surg Endosc ; 34(5): 2172-2177, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31342261

RESUMO

BACKGROUND: Choledochal cysts are congenital dilations of the biliary tree. Complete cyst excision and biliary-enteric reconstruction have been the standard operations. In our center, more than 95% of choledochal cyst excision is now performed laparoscopically. Majority of current studies describe laparoscopic-assisted reconstruction using Roux-en-Y hepaticojejunostomy (HJ). However, only a few have studied laparoscopic hepaticoduodenostomy (HD) as an alternative method of biliary-enteric reconstruction. In this study, we focused on comparing longer-term outcomes between laparoscopic HJ and HD reconstruction following choledochal cyst excision. METHODS: We performed retrospective analysis of 54 children who had undergone laparoscopic choledochal cyst excision and biliary-enteric reconstruction between October 2004 and April 2018. Short-term outcomes including operative time, complications such as anastomotic leakage and bleeding, and hospital stays were included. Long-term outcomes including contrast reflux into biliary tree, cholangitis, anastomotic strictures, and need of reoperation were analyzed. RESULTS: Of the 54 patients, 21 of them underwent laparoscopic HD and 33 underwent laparoscopic Roux-en-Y HJ anastomosis reconstruction. There were no significant differences in gestation, gender, age at operation, antenatal diagnosis, and Todani type of choledochal cyst between HD and HJ group. Operative time was significantly shortened in HD group (p = 0.001). Median time to enteral feeding was 3 days in both groups. Median intensive care unit (p = 0.001) and hospital stay (p = 0.019) were significantly shorter in HD group. There was no perioperative mortality. There was no significant difference in anastomotic leakage requiring reoperation (p = 0.743). There were no significant differences in long-term outcomes including anastomotic stricture (p = 0.097), cholangitis (p = 0.061), symptoms of recurrent abdominal pain or gastritis (p = 0.071), or need of reoperation (p = 0.326). All patients had normal postoperative serum bilirubin level. CONCLUSIONS: Laparoscopic excision of choledochal cyst with HD reconstruction is safe and feasible with better short-term outcomes and comparable long-term outcomes compared to Roux-en-Y HJ reconstruction.


Assuntos
Anastomose em-Y de Roux/métodos , Cisto do Colédoco/cirurgia , Duodenostomia/métodos , Jejunostomia/métodos , Laparoscopia/métodos , Fígado/cirurgia , Adolescente , Adulto , Idoso , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Criança , Cisto do Colédoco/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
7.
Pediatr Blood Cancer ; 66(1): e27482, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30270490

RESUMO

BACKGROUND AND AIM: We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS: Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS: Sixty patients were enrolled with median age at diagnosis of 1.1 years and median follow-up time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients. Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. CONCLUSIONS: Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Hepatoblastoma/mortalidade , Neoplasias Hepáticas/mortalidade , Transplante de Fígado/mortalidade , Recidiva Local de Neoplasia/mortalidade , Criança , Pré-Escolar , Terapia Combinada , Consenso , Feminino , Seguimentos , Hepatoblastoma/patologia , Hepatoblastoma/terapia , Hong Kong , Humanos , Lactente , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Estudos Longitudinais , Masculino , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Estudos Prospectivos , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
8.
Lancet ; 390(10099): 1072-1082, 2017 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-28901937

RESUMO

Recent developments in paediatric gastrointestinal surgery have focused on minimally invasive surgery, the accumulation of high-quality clinical evidence, and scientific research. The benefits of minimally invasive surgery for common disorders like appendicitis and hypertrophic pyloric stenosis are all supported by good clinical evidence. Although minimally invasive surgery has been extended to neonatal surgery, it is difficult to establish its role for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of these disorders. Advances in treatments for biliary atresia and necrotising enterocolitis have been achieved through specialisation, multidisciplinary management, and multicentre collaboration in research; similarly robust clinical evidence for other rare gastrointestinal disorders is needed. As more neonates with gastrointestinal diseases survive into adulthood, their long-term sequelae will also need evidence-based multidisciplinary care. Identifying cures for long-term problems of a complex developmental anomaly such as Hirschsprung's disease will rely on unravelling its pathogenesis through genetics and the development of stem-cell therapy.


Assuntos
Gastroenterologia/tendências , Gastroenteropatias/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Apendicite/cirurgia , Atresia Biliar/cirurgia , Criança , Enterocolite Necrosante/cirurgia , Humanos , Recém-Nascido , Diagnóstico Pré-Natal
9.
Pediatr Surg Int ; 31(2): 149-53, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25433691

RESUMO

BACKGROUND: With the advent of laparoscopic surgery, more choledochal cysts are excised laparoscopically. In this study, we compared the outcomes from laparoscopic hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD) for biliary-enteric reconstruction. METHODS: A retrospective analysis of patients who had undergone laparoscopic choledochal cyst excision between February 2005 and January 2014 in a tertiary referral centre was performed. Demographics data, operative techniques and surgical outcomes were analysed according to the way of biliary-enteric reconstruction. RESULTS: A total of 31 patients were identified, 20 of whom underwent HJ and 11 underwent HD. There were no significant differences in terms of demographics. Median operative time was significantly shorter in HD group (211.0 ± 96.4 vs. 386.0 ± 90.4 min, p = 0.001). Although postoperative enteral feeding was initiated later in HD group (5.0 ± 0.8 vs. 4.0 ± 3.6 days, p = 0.036), postoperative stay in intensive care unit (ICU) (0.7 ± 1.0 vs. 2.4 ± 1.7 days, p = 0.007) and overall hospital stay (9.1 ± 1.0 vs. 14.4 ± 12.2 days, p = 0.157) favoured HD group. There was no perioperative mortality. Median follow-up duration was 24.0 (±11.0) months in HD group and 67.5 (±23.7) months in HJ group. One patient in HJ group had postoperative cholangitis related to anastomotic stricture whereas no cholangitis noted in HD group. In total, five patients in HJ group required second operation for complications and residual diseases whereas none in HD group required reoperation. CONCLUSIONS: Laparoscopic excision of choledochal cyst with hepaticoduodenostomy reconstruction is safe and feasible with shorter operative time, ICU stay and overall hospital stay. It is not inferior to HJ in terms of short-term postoperative outcomes.


Assuntos
Ductos Biliares Extra-Hepáticos/cirurgia , Cisto do Colédoco/cirurgia , Duodeno/cirurgia , Anastomose Cirúrgica , Criança , Pré-Escolar , Duodenostomia , Feminino , Ducto Hepático Comum/cirurgia , Humanos , Lactente , Laparoscopia , Masculino , Estudos Retrospectivos , Resultado do Tratamento
10.
Hong Kong Med J ; 21(6): 518-23, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26371157

RESUMO

OBJECTIVES: To review all paediatric patients with intussusception over the last 17 years. DESIGN: Retrospective case series. SETTING: A tertiary centre in Hong Kong. PATIENTS: Children who presented with intussusception from January 1997 to December 2014 were reviewed. MAIN OUTCOME MEASURES: The duration of symptoms, successful treatment modalities, complication rate, and length of hospital stay were studied. RESULTS: A total of 173 children (108 male, 65 female) presented to our hospital with intussusception during the study period. Their median age at presentation was 12.5 months (range, 2 months to 16 years) and the mean duration of symptoms was 2.3 (standard deviation, 1.8) days. Vomiting was the most common symptom (76.3%) followed by abdominal pain (46.2%), per rectal bleeding or red currant jelly stool (40.5%), and a palpable abdominal mass (39.3%). Overall, 160 patients proceeded to pneumatic or hydrostatic reduction, among whom 127 (79.4%) were successful. Three (1.9%) patients had bowel perforation during the procedure. Early recurrence of intussusception occurred in four (3.1%) patients with non-operative reduction. No recurrence was reported in the operative group. The presence of a palpable abdominal mass was a risk factor for operative treatment (relative risk=2.0; 95% confidence interval, 1.8-2.2). Analysis of our results suggested that duration of symptoms did not affect the success rate of non-operative reduction. CONCLUSIONS: Non-operative reduction has a high success rate and low complication rate, but the presence of a palpable abdominal mass is a risk factor for failure. Operative intervention should not be delayed in those patients who encounter difficult or doubtful non-operative reduction.


Assuntos
Enteropatias/complicações , Intussuscepção/complicações , Neoplasias Abdominais/etiologia , Neoplasias Abdominais/cirurgia , Dor Abdominal/etiologia , Adolescente , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Feminino , Hemorragia Gastrointestinal/etiologia , Hong Kong , Humanos , Lactente , Enteropatias/cirurgia , Perfuração Intestinal/etiologia , Intussuscepção/cirurgia , Tempo de Internação , Masculino , Recidiva , Estudos Retrospectivos , Fatores de Risco , Centros de Atenção Terciária , Resultado do Tratamento , Vômito/etiologia
12.
Hong Kong Med J ; 18(4): 340-2, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22865181

RESUMO

Foreign body aspiration is a potentially fatal condition. Yet, an accurate diagnosis may not be easy. Management is also challenging and requires a high level of expertise with proper instruments. In this article, we report our experience in the management of peanut aspiration in two young children by means of a ventilating bronchoscope.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Arachis , Brônquios , Corpos Estranhos/complicações , Feminino , Corpos Estranhos/terapia , Humanos , Lactente , Sons Respiratórios/etiologia
13.
EBioMedicine ; 74: 103689, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34781099

RESUMO

Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.


Assuntos
Ductos Biliares/citologia , Atresia Biliar/diagnóstico , Organoides/patologia , Animais , Atresia Biliar/patologia , Diferenciação Celular , Modelos Animais de Doenças , Células Epiteliais/citologia , Humanos
14.
Transl Pediatr ; 9(5): 702-706, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33209734

RESUMO

Thoracoamniotic shunt has been considered as a treatment for antenatal pleural effusion and complication is rare. In majority of cases, the shunt can be removed uneventfully. In this article, we reported a rare complication of shunt migration resulting in the need of thoracoscopic removal at newborn period. The patient born at 39+3 weeks of gestation suffered from antenatal chylothorax detected at 28 weeks and was managed by intrauterine thoracoamniotic shunt insertion. This was complicated by shunt displacement, which caused respiratory distress after birth requiring ventilatory support and progressive pleural effusion in this patient. To prevent further neonatal compromise, thoracoscopic removal of the retained shunt was done on day 7 of life followed by post-op chest drain insertion. Post-op condition was stable with resolution of respiratory distress, and the patient was discharge on post-op day 16. We would like to remind clinicians about this potential complication of thoracoaminotic shunt, which can pose a potential risk of severe neonatal compromise, and that it can be managed by minimal invasive surgery even in the newborn period.

15.
J Pediatr Surg ; 55(9): 1892-1896, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31759650

RESUMO

BACKGROUND: The objective of this study was to determine the most optimal timing of liver transplant (LT) for post-Kasai portoenterostomy (KPE) patients based on disease severity scores. METHODS: This was a retrospective study and the clinical data of all LT recipients aged <18 years (n = 89) with a history of KPE were analyzed. They were divided into three groups according to their PELD/MELD scores at the time of LT (A: <15; B: 15-25; C: >25). The effects of LT on the clinical outcomes and hospitalization status were analyzed. RESULTS: There were 33, 34 and 22 patients in group A, B and C, respectively. There was no significant difference in 3-year graft survival rate between the three groups but group C patients had the highest incidence of vascular or biliary complications (p = 0.022). Group C patients had a significantly lower hospital admission frequency (p = 0.036) and shorter hospital stay (p = 0.041) after LT when compared with their pre-LT status and with non-LT patients with similar disease severity scores. On the other hand, the hospitalization frequency and duration were similar in patients with the lowest disease severity score (group A) before, after and without LT. CONCLUSIONS: The benefit of LT was less obvious when the disease severity score is <15. A high complication rate was reported when LT was performed at a score > 25. Donor availability, the patient's general condition and parental wish should be considered during individual assessment. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level III.


Assuntos
Hepatopatias/classificação , Transplante de Fígado/métodos , Portoenterostomia Hepática/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Fígado/cirurgia , Hepatopatias/diagnóstico , Hepatopatias/cirurgia , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Índice de Gravidade de Doença , Tempo para o Tratamento
16.
Front Oncol ; 10: 570479, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33330043

RESUMO

BACKGROUND: Hepatocellular carcinoma (HCC) is a rare hepatic malignancy in children. Hepatitis B virus (HBV) infection is a key predisposing factor in endemic regions but its impact on outcome has not been studied. We aim to evaluate the prognostic implication of HBV seropositivity and role of cancer surveillance in children with HCC from East Asian populations with national HBV vaccination. METHODS: Review of population-based databases for patients (< 18 years old) diagnosed with HCC from 1993 to 2017 in two Southeast Asian regions with universal HBV vaccination (instituted since 1988 and 1987 in Hong Kong and Singapore, respectively). RESULTS: Thirty-nine patients were identified (Hong Kong, 28; Singapore, 11). Thirty were male; median age at diagnosis was 10.8 years (range, 0.98-16.6). Abdominal pain was the commonest presentation while five patients were diagnosed through surveillance for underlying condition. Alpha-fetoprotein was raised in 36 patients (mean, 500,598 ng/ml). Nineteen had bilobar involvement, among the patients in whom pretreatment extent of disease (PRETEXT) staging could retrospectively be assigned, 3 had stage I, 13 had stage II, 4 had stage III, and 11 had stage IV disease. Seventeen had distant metastasis. HBsAg was positive in 19 of 38 patients. Two patients had fibrolamellar HCC. Upfront management involved tumor resection in 16 (liver transplantation, 2), systemic chemotherapy in 21, interventional procedures in 6 [transarterial chemoembolization (TACE), 5, radiofrequency ablation (RFA), 1], and radiotherapy in 4 (selective internal radiation, 3, external beam radiation, 1). Five-year event-free survival (EFS) and overall survival (OS) were 15.4 ± 6.0 and 26.1 ± 7.2%, respectively. Patient's HBsAg positivity, metastatic disease and inability to undergo definitive resection represent poor prognostic factors in univariate and multivariable analyses. Patients diagnosed by surveillance had significantly better outcome. CONCLUSION: Pediatric HCC has poor outcome. HBV status remains relevant in the era of universal HBV vaccination. HBV carrier has inferior outcome and use of surveillance may mitigate disease course.

17.
Data Brief ; 31: 105953, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32685628

RESUMO

Haematological and immunological data of children with COVID-19 infection is lacking. Between 21st January and 20th March 2020, 244 children who were confirmed to have COVID-19 infection and admitted to the Wuhan Children's Hospital, China were retrospectively reviewed. 193 children were considered as symptomatic, which was defined as having either the presence of clinical symptoms or the presence of CT thorax abnormalities. Their haematological and immunological profiles, including complete blood counts, lymphocyte subsets (T, B and NK cell counts), immunoglobulin (Ig) profiles (IgG, IgA and IgM) and cytokine profiles were analysed and compared between the symptomatic and asymptomatic groups. The median values and the interquartile ranges were calculated. Comparison was made using the Mann-Whitney U test. Children with symptomatic COVID-19 infection had significantly lower haemoglobin levels, but higher absolute lymphocyte and monocyte counts, IgG and IgA levels, as well as interleukin 6 (IL-6), IL-10, tumour necrosis factor alpha and interferon gamma levels. The obtained data will be utilized for further studies in comparing children and adults with COVID-19 infections in other parts of the world and with different severity .

18.
Hong Kong Med J ; 15(2): 136-8, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19342740

RESUMO

Although Wilms' tumour is one of the most common solid malignancies in children, bilateral disease is rare. We report a child with bilateral Wilms' tumour who developed abdominal compartment syndrome after an open biopsy.


Assuntos
Síndromes Compartimentais/etiologia , Neoplasias Renais/patologia , Veia Cava Inferior/patologia , Tumor de Wilms/patologia , Abdome , Humanos , Lactente , Neoplasias Renais/complicações , Masculino , Tumor de Wilms/complicações
19.
Pediatr Surg Int ; 25(9): 763-6, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19652983

RESUMO

INTRODUCTION: Primary spontaneous pneumothorax is a condition that carries significant morbidities and mortalities if not managed properly. Thoracotomy with bullectomy has been the treatment of choice for persistent air leak or recurrence after initial chest drain insertion. With the advancement in minimal invasive surgery, the thoracoscopic approach can dramatically reduce the complications of open thoracotomy. We review our experience in managing spontaneous pneumothorax in children using thoracoscopy. MATERIALS AND METHODS: The medical records of all patients who were discharged with the diagnosis of spontaneous pneumothorax from 1997 to 2007 were reviewed. The demographic data and management were noted. For those patients who underwent thoracoscopic surgery, the intra-operative findings, post-operative outcomes and complications were compared. RESULTS: A total of 15 patients with spontaneous pneumothorax were identified. They all received chest drain insertion as the primary treatment modality. Nine patients, with mean age 16.1 +/- 0.9 years, failed the initial management and subsequently received thoracoscopic surgery with a mean interval of 7.6 +/- 2.5 days after initial chest drain insertion. Among these patients, bullae were found in seven patients (two patients had more than one bulla). The bullae were excised with mean operative time being 63.9 +/- 25.2 min. No conversion to thoracotomy was reported. All patients received paracetamol as pain control and there was no associated complication. No recurrence was found at follow-up till now. CONCLUSION: This study confirms that the presence of bulla is commonly seen in patients with spontaneous pneumothorax who fail initial management. Thoracoscopic bullectomy, a safe and effective operation, should be offered to this group of patients.


Assuntos
Vesícula/cirurgia , Pneumotórax/cirurgia , Toracoscopia , Adolescente , Vesícula/patologia , Tubos Torácicos , Feminino , Humanos , Masculino , Pleurodese , Estudos Retrospectivos
20.
J Pediatr Surg ; 54(2): 255-257, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30497821

RESUMO

OBJECTIVE: Helicobacter pylori infection is common among Asians. However, evidence in the recent years has demonstrated a decrease in the prevalence of H. pylori infection among children and adults worldwide. Our aim was to update its prevalence in symptomatic children in our locality in the recent 12 years and compared to the results of our previous review published in 2005. METHODS: A retrospective review was carried out between 2005 and 2017. All children who presented with dyspepsia or gastrointestinal bleeding and underwent oesophagogastroduodenoscopy with antral biopsy taken were included. Patient demographics, endoscopic, or histological diagnosis and the H. pylori status were recorded. MAIN RESULTS: A total of 602 patients were included. There was a statistically significant decreasing trend of H. pylori infection rate between 2005 and 2017 (p = 0.003). The overall infection rate from this study was 12.8%, compared to 25.6% from our previous review. Overall failure of eradication with first-line antibiotic therapy has increased to 29.3% from 10% in our previous review. CONCLUSION: There was a decrease in the prevalence of H. pylori infection among symptomatic children for the recent 12 years, comparing to our previous data from 2005. We hypothesize that the reduction in prevalence of H. pylori infection among adults and the decrease in the practice of sharing chopsticks during meals have led to a decrease in transmission of the bacteria among family members in Hong Kong. However, the failure of eradication with first line treatment was higher, possibly due to the increase in antibiotics usage and resistance. LEVEL OF EVIDENCE: III.


Assuntos
Infecções por Helicobacter/epidemiologia , Infecções por Helicobacter/patologia , Helicobacter pylori , Antro Pilórico/patologia , Adolescente , Antibacterianos/uso terapêutico , Biópsia , Criança , Pré-Escolar , Dispepsia/microbiologia , Feminino , Infecções por Helicobacter/tratamento farmacológico , Hong Kong/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Prevalência , Antro Pilórico/microbiologia , Estudos Retrospectivos , Centros de Atenção Terciária
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