Background
The age at Kasai portoenterostomy (KPE) was reported to correlate with the prognosis of patients with biliary atresia (BA) and that a late KPE is bounded to be failure. Herewith, we ...reported the outcome of patients receiving KPE after day 70 of life. In addition, the prognostic indicators were evaluated.
Materials and Methods
This was a retrospective analysis and all BA patients receiving KPE after day 70 of life in a tertiary centre between 1980 and 2018 were evaluated.
Results
A total of 164 KPE procedures were performed during the study period and 62 cases were done after day 70 of life which were included in this study. The median follow up period of these patients was 10.6 years (range: 4.5 to 41.5 years). Thirty-nine patients (62.9%) patients were able to achieve jaundice clearance at 6 months after KPE. The NLS rate was 53.2% (
n
= 33) as recorded at the time of writing. There was no statistical difference in the age at KPE between native liver survivors and patients requiring liver transplant. For complications among the native liver survivors (
n
= 33), portal hypertension and recurrent cholangitis were found in 63.6% and 30.3% of these patients. There was also no significant difference in the age at KPE between those who developed portal hypertension and recurrent cholangitis (
p
= 0.451 and
p
= 0.173 respectively). Regarding the prognostic indicators in predicting NLS, pre-KPE bilirubin, alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) were significantly higher among patients requiring liver transplant (
p
= 0.012, =0.011 and =0.017 respectively). The bilirubin level at 6 months after KPE was also higher among patients who required liver transplant (
p
= 0.016).
Conclusion
More than half of the BA patients can survive for 10 years with their native liver despite KPE was performed after day 70 of life. However, they have a higher chance to develop BA-related complications. The level of pre-KPE bilirubin and ductal enzymes as well as post-KPE bilirubin are prognostic indicators to predict NLS.
The primary pathology of Hirschsprung's disease (HSCR, colon aganglionosis) is the absence of ganglia in variable lengths of the hindgut, resulting in functional obstruction. HSCR is attributed to a ...failure of migration of the enteric ganglion precursors along the developing gut. RET is a key regulator of the development of the enteric nervous system (ENS) and the major HSCR-causing gene. Yet the reduced penetrance of RET DNA HSCR-associated variants together with the phenotypic variability suggest the involvement of additional genes in the disease. Through a genome-wide association study, we uncovered a ∼350 kb HSCR-associated region encompassing part of the neuregulin-1 gene (NRG1). To identify the causal NRG1 variants contributing to HSCR, we genotyped 243 SNPs variants on 343 ethnic Chinese HSCR patients and 359 controls. Genotype analysis coupled with imputation narrowed down the HSCR-associated region to 21 kb, with four of the most associated SNPs (rs10088313, rs10094655, rs4624987, and rs3884552) mapping to the NRG1 promoter. We investigated whether there was correlation between the genotype at the rs10088313 locus and the amount of NRG1 expressed in human gut tissues (40 patients and 21 controls) and found differences in expression as a function of genotype. We also found significant differences in NRG1 expression levels between diseased and control individuals bearing the same rs10088313 risk genotype. This indicates that the effects of NRG1 common variants are likely to depend on other alleles or epigenetic factors present in the patients and would account for the variability in the genetic predisposition to HSCR.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Introduction
Tracheobronchial obstruction, although uncommon in the pediatric age group, remains a challenging problem. We review the long-term outcome of endoscopic metallic stenting in infants with ...tracheobronchial obstruction.
Materials and methods
Medical records of all pediatric surgical patients who underwent tracheobronchial metallic stenting in our center were reviewed retrospectively from 1996 to 2014. Patients’ demographic data, including etiology, associated anomalies and nature of obstruction were reviewed. Outcome measures include complications such as re-stenosis, granulation tissue, stent migration, fractured stent, maximal tracheal diameter achieved, weaning of ventilator and growth at interval follow-up.
Results
Twelve balloon-expandable metallic stents were placed in the trachea (
n
= 10) and/or bronchi (
n
= 2) of 5 patients with a median age of 13 months (range 5–30 months). Etiology of the airway obstruction included congenital tracheal stenosis (
n
= 4), giant cervical and superior mediastinal lymphatic malformation with tracheobronchomalacia (
n
= 1). Seven complications were reported (3 patients developed granulation tissue, 2 patients had re-stenosis, 1 stent migrated, 1 stent fractured). All patients survived and were in good condition with a median follow-up of 16 years (range 11–18 years). Three patients weaned off ventilator and oxygen.
Conclusions
Endoscopic stenting with metallic stent has satisfactory long-term outcome in treating infants with tracheobronchial obstruction.
Biliary atresia: East versus west Chung, Patrick Ho Yu; Zheng, Shan; Tam, Paul Kwong Hang
Seminars in pediatric surgery,
August 2020, 2020-08-00, 20200801, Letnik:
29, Številka:
4
Journal Article
Recenzirano
Biliary atresia (BA) is a common cause of surgical jaundice during the neonatal period. It is currently considered as a spectrum of diseases with a common final pathology characterized by ...obliteration of the extrahepatic biliary tract and the absence of normally branching intrahepatic ducts. Though it is a global disease that can be found in all ethnicities there are some clear differences between BA arising in the East and the West. This is likely to be related to different genetic, environmental and cultural factors. BA is more frequently found in Far Eastern infants (both Chinese and Japanese) though the syndromic associations are much less common. Many Eastern countries have national screening programmes not seen in the West possibly due to debate over its cost effectiveness in countries where incidence is low. Kasai portoenterostomy (KPE) is considered as the primary treatment of BA but its outcome still remains unsatisfactory across the region. Given the complexity of BA, it is unlikely that strategic advances could be made by the sole effort of individual countries and we believe that collaboration between the East and West is the way forward.
Background and methods
Iatrogenic diaphragmatic hernia in childhood is rare, with only scanty case reports available in the literature. It is a potentially life-threatening condition resulting in ...intestinal obstruction and bowel strangulation. In this study, we retrospectively reviewed four patients with iatrogenic diaphragmatic hernia following right hepatic trisectionectomy with caudate lobectomy and liver transplantation in a tertiary referral centre. The patient characteristics, clinical presentation and risk factors were assessed.
Conclusions
Iatrogenic diaphragmatic hernia should be considered in differential diagnosis in paediatric patients presenting with intestinal obstruction or respiratory distress after hepatic resection or liver transplantation for early diagnosis and management.
Background: We explored the feasibility of creating BA-like organoids by treating human liver organoids with Polyinosinic:Polycytidylic acid (Poly I:C). Methods: Organoids were developed from the ...liver parenchyma collected during Kasai portoenterostomy (BA) and surgery for other liver disorders (non-BA). The non-BA organoids were co-cultured with poly I:C (40 µg/mL). The organoid morphology from both samples was compared on day 17. RNA-sequencing was performed to examine the transcriptomic differences. Results: Non-BA liver organoids developed into well-expanded spherical organoids with a single-cell layer of epithelial cells and a single vacuole inside. After poly I:C treatment, the majority of these organoids developed into an aberrant morphology with a high index of similarity to BA organoids which are multi-vacuoled and/or unexpanded. RNA-sequencing analysis revealed that 19 inflammatory genes were commonly expressed in both groups. Conditional cluster analysis revealed several genes (SOCS6, SOCS6.1, ARAF, CAMK2G, GNA1C, ITGA2, PRKACA, PTEN) that are involved in immune-mediated signaling pathway had a distinct pattern of expression in the poly I:C treated organoids. This resembled the expression pattern in BA organoids (p < 0.05). Conclusions: Poly I:C treated human liver organoids exhibit morphology and genetic signature highly compatible to organoids developed from BA liver samples. They are potential research materials to study immune-mediated inflammation in BA.
Background
To evaluate factors affecting length of stay (LOS) after choledochal cyst resection in paediatric patients.
Methods
This was a retrospective study on patients operated between 2004 and ...2021. Associations between clinical factors and LOS were evaluated by bivariate analysis, multiple regression, and equivalence test.
Results
Sixty-two patients were included. Twenty-four underwent hepaticoduodenostomy as biliary reconstruction. Five suffered from major complications. The median (25th–75th percentile) operation time was 279 (182–378) min. Median LOS, time to enteral feeding, and time to abdominal drain removal were 8(6–10), 2(1–3), and 5(4–7) days, respectively. Seven factors were found significantly associated with a shorter LOS in bivariate analysis and were included in multiple regression. It revealed that early abdominal drain removal (
p
< 0.001), early enteral feeding (
p
= 0.042), and the absence of major complications (
p
< 0.001) were significantly associated with shorter LOS. Equivalence test suggested that age and preoperative cholangitis had no practical effect on LOS.
Conclusions
Early enteral feeding, early drain removal, and avoidance of major complications are associated with a shorter LOS.
Introduction
Data on laparoscopic Nissen fundoplication for gastro-oesophageal reflux disease (GERD) in infants remain limited. We describe our experience with this operation in children and in ...particular, infants younger than 12 months old.
Materials and methods
Medical records of all paediatric patients who had laparoscopic fundoplication done for GERD from 1998 to 2013 were reviewed. Patients were divided into two groups based on age: group I: 0–12 months, and group II >12 months. Data on indications, patient’s demographics, operative time, blood loss, conversions, complications, recurrences and duration of hospitalization were studied.
Results
A total of 86 patients were reviewed (group I,
n
= 21; group II,
n
= 65). While the mean age and body weight for group I were 8 ± 2.99 months and 6 ± 1.96 kg, the values for group II were 98 ± 65 months and 18 ± 9.6 kg, respectively. All patients had concurrent laparoscopic gastrostomy for feeding difficulties. The mean operative time was shorter in group I (157 ± 55 vs 169 ± 52 min,
p
= 0.66). Both groups had minimal blood loss only. The surgical outcomes in both groups were comparable in terms of recurrence (0 vs 3 %,
p
= 0.105) and complications (9.5 vs 6 %,
p
= 0.275). The median follow-up duration for group I and group II was 23 and 40 months, respectively. Sixteen (76 %) patients in group I and 45 (67 %) patients in group II did not develop pneumonia post fundoplication.
Conclusion
Laparoscopic Nissen fundoplication can be safely performed in infants with outcomes comparable to older patients and a shorter operative duration. Low recurrence rate (up to 3 %) is shown by our long term follow-up data. We recommend laparoscopic Nissen fundoplication to be considered in managing infants with GERD.
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