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1.
Hiroki Nakamura Naruhiko Murase Hiroyuki Koga Joel Cazares Geoffrey J. Lane Hiroo Uchida Atsuyuki Yamataka 《Pediatric surgery international》2016,32(12):1209-1212
Purpose
According to Kasai’s classification of biliary atresia (BA), type III is diagnosed when micro bile ducts (MBD) cannot be identified macroscopically on the surface of the biliary remnant transected at the porta hepatis. However, during laparoscopic Kasai (lapKasai), magnification produced by a 30° 10 mm scope at a focal length of 5 cm is ×38 and ×100 when zooming, enabling more MBD to be identified than with the naked eye rendering Kasai’s original classification questionable in the laparoscopic era.Methods
Intraoperative video recordings of 36 consecutive lapKasai cases (2009–2015) were reviewed to confirm MBD visibility. 85 consecutive open Kasai cases examined macroscopically served as controls.Results
MBD were not visible under regular laparoscopic magnification during lapKasai in 6/36 (lapMBD?; 16.7 %) cases and visible in 30/36 (83.3 %). However, in open cases, MBD could not be identified macroscopically in 77/85 (macroMBD?; 90.6 %), a typical result reported internationally. For our lapKasai cases, jaundice clearance was lower in lapMBD-cases (4/6 = 66.7 % versus 26/30 = 86.7 %), which was not statistically significant (p = 0.26). Conversely, survival with the native liver was significantly lower in lapMBD-cases (4/6 = 66.7 % versus 23/30 = 76.7 %) (p < 0.05).Conclusions
BA classification may benefit from revision to include laparoscopic findings to categorize BA more comprehensively.2.
Purpose
Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis.Methods
A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason.Results
We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23–12.89, p?<?0.00001; odds ratio: 25.88, 95% CI 2.83–236.84, p?<?0.004; odds ratio: 15.05, 95% CI 2.70–83.93, p?=?0.002, respectively).Conclusion
The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.3.
Hideyuki Sasaki Hiromu Tanaka Motoshi Wada Takuro Kazama Megumi Nakamura Hironori Kudo Ryoji Okubo Tsuyoshi Sakurai Masaki Nio 《Pediatric surgery international》2016,32(9):839-843
Purpose
Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure.Methods
This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis.Results
The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors.Conclusions
The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.4.
Introduction
Owing to several therapeutic advancements, more patients with biliary atresia now survive into adulthood while retaining their native liver. However, the optimal strategy for long-term management of such patients remains unclear.Methods
Aiming to establish the current management strategies, we reviewed previous reports of long-term outcome of BA who underwent surgery at our institution as well as the relevant literature, focusing particularly on the treatment of late complications.Results
Approximately 30–40% of long-term survivors of biliary atresia who retain their native liver exhibit late sequelae such as cholangitis and portal hypertension. Early and appropriate intervention with Kasai portoenterostomy is essential for ensuring long-term survival with good quality of life. In our hospital, the current standard for Kasai portoenterostomy involves dissecting the fibrous remnants along the porta hepatis, just on the level of the liver capsule. Cholangitis is an important late complication in biliary atresia, and the possibility of mechanical obstruction of the biliary drainage route or deformity of the intrahepatic bile ducts with or without gallstones should be thoroughly evaluated in patients with intractable cholangitis. Regarding portal hypertension, appropriate interventions such as endoscopic variceal treatment and partial splenic embolization are considered to provide good quality of life when hepatic function is preserved.Conclusion
Appropriate therapeutic management is strongly recommended in selected patients with late complications.5.
Pan-Liang Wang Jun Wang Ying Zhou Xiao-Song Chen Ke-Jun Zhou Jie Wen Jian-Jun Zhang Wei Cai 《World journal of pediatrics : WJP》2017,13(6):604-610
Background
An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA). Programmed death-1 (PD-1) and its two ligands, programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2, respectively) play an important inhibitory role in immune reactions. We aimed to illustrate the expression of these molecules in BA.Methods
Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and liver transplantation (late BA). Intrahepatic expression of PD- 1, PD-L1, and PD-L2 were examined by immunostaining and compared with that in patients with neonatal hepatitis syndrome and normal controls. The correlation between the expression levels of these molecules in the liver and clinicopathological parameters was analyzed for each group.Results
Enhanced expression of PD-1 and its ligands occurred in the livers with early BA. In the BA-affected livers, PD-1 was correlated with the degree of peri-biliary inflammation, while PD-L2 was linked more directly with portal fibrosis. None of the three molecules was correlated with the prognosis of the Kasai procedure in patients with early BA.Conclusions
Only PD-1 and PD-L1 are involved in the immune reactions of early BA. Elucidation of the detailed role of PD-L2 in BA requires further research.6.
Background
Synovial arteriovenous malformation is rare.Case characteristics
We present three children with recurrent monoarthritis secondary to synovial arteriovenous malformation.Outcome
Two children underwent excision of arteriovenous malformation. Another child had diffuse arteriovenous malformation, which was inoperable.Message
Synovial arteriovenous malformations should be considered in the differential diagnosis of monoarthritis, especially of the knee.7.
Purpose
Biliary atresia (BA) is characterized by progressive inflammation of the biliary system. This inflammation persists and causes liver fibrosis, although jaundice disappears after Kasai portoenterostomy (KP). We aimed to confirm whether the oral administration of eicosapentaenoic acid (EPA) suppresses liver fibrosis in postoperative patients with BA.Methods
We reviewed patients who underwent laparoscopic KP (lapKP) between January 2014 and September 2017. From December 2016, 30 mg/kg/day of EPA was orally administered to patients who opted to take medicine (EPA group). Patients who did not receive EPA were assigned to the non-EPA group. Mac-2 binding protein sugar chain modified isomer (M2BPGi) and hyaluronic acid (HA) levels were compared between the two groups in patients showing disappearance of jaundice at 6 months after the first lapKP.Results
Seventeen patients in the non-EPA group and 11 in the EPA group were enrolled. At 6 months after the first lapKP, 10 patients in the non-EPA group and six in the EPA group were without jaundice. M2BPGi and HA levels were significantly lower in the EPA group.Conclusions
Liver fibrosis was suppressed in patients without jaundice 6 months after lapKP, who were administered EPA. We believe that periductular inflammation was alleviated by EPA supplementation.8.
Manoja Kumar Das Vidyut Bhatia Anupam Sibal Abha Gupta Sarath Gopalan Raman Sardana Reeti Sahni Ankur Roy Narendra K Arora 《Indian pediatrics》2017,54(12):1012-1016
Objective
To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normalweight and overweight schoolchildren.Design
Cross-sectional study.Setting
Thirteen private schools in urban Faridabad, Haryana.Participants
961 school children aged 5-10 years.Methods
Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing.Outcome measures
Prevalence of fatty liver on ultrasound, and NAFLD and its association with biochemical abnormalities and demographic risk factors.Results
On ultrasound, 215 (22.4%) children had fatty liver; 18.9% in normal-weight and 45.6% in overweight category. Presence and severity of fatty liver disease increased with body mass index (BMI) and age. Among the children with NAFLD, elevated SGOT and SGPT was observed in 21.5% and 10.4% children, respectively. Liver enzyme derangement was significantly higher in overweight children (27% vs 19.4% in normal-weight) and severity of fatty liver (28% vs 20% in mild fatty liver cases). Eleven (8.1%) children with NAFLD had metabolic syndrome. Higher BMI (OR 35.9), severe fatty liver disease (OR 1.7) and female sex (OR 1.9) had strong association with metabolic syndrome.Conclusions
22.4% of normal-weight and overweight children aged 5-10 years had fatty liver. A high proportion (18.9%) of normal-weight children with fatty liver on ultrasound indicates the silent burden in the population.9.
Mullai Baalaaji Muralidharan Jayashree Karthi Nallasamy Sunit Singhi Arun Bansal 《Indian pediatrics》2018,55(4):311-314
Objective
To identify predictors and outcome of acute kidney injury (AKI) in children with diabetic ketoacidosis (DKA) admitted to a Pediatric Intensive Care Unit (PICU).Methods
Retrospective case review of 79 children with DKA admitted between 2011-2014.Results
28 children developed AKI during the hospital stay; 20 (71.4%) recovered with hydration alone. Serum chloride at 24 hours was independently associated with AKI. Children with AKI had prolonged acidosis, longer PICU stay, and higher mortality.Conclusions
Majority of children with AKI and DKA recover with hydration. Hyperchloremia at 24 hours had independent association with AKI, although cause-effect relation could not be ascertained.10.
Background
Immunodeficient children are more prone for invasive cryptococcal infections.Case characteristics
A 2-year-old boy with disseminated cryptococcosis was evaluated for underlying immunodeficiency without success.Intervention/outcome
Child was managed successfully.Message
Immunocompetent children with disseminated cryptococcosis can present diagnostic or therapeutic challenge in resource-limited settings.11.
Objective
To compare the efficacy and safety of laparoscopic appendectomy and open appendectomy for acute appendicitis in children.Methods
This study was conducted as a retrospective comparison of hospital records for postoperative complications, duration of operation, and postoperative length of stay between children (aged <18y) who underwent laparoscopic (n=190) or open (n=199) appendectomy over a six-year period. Quality of life was evaluated immediately and 1 month postoperatively.Results
The major complication rate after surgery in laparoscopic group was significantly lower than that of open appendectomy group (13% vs 27%, P<0.05). The mean (SD) postoperative hospital stay was also shorter in laparoscopic group (2.4 (0.6) days vs 3.7 (1.1) days, P<0.05). The postoperative minor complication rate and hospital expenses were not significantly different between the two groups. The duration of surgery was longer in laparoscopic group (P<0.05). Children in laparoscopic group had less postoperative pain and higher quality of life after one month than those in open appendectomy group.Conclusions
Laparoscopic technique seems to be safer than open appendectomy for acute appendicitis in children.12.
Background
The aim of this study was to retrospectively investigate whether Kasai Procedure (KP) improves nutritional status and whether KP decreases liver transplantation (LT)-associated complications in children with biliary atresia (BA).Methods
From March 1st 2014 to February 28th 2015, 103 patients underwent LT and the other 17 without prior KP died during the waiting period. In 103 patients undergoing LT, 58 patients received KP previously (Group A), and 45 only underwent primary LT (Group B). The nutritional status, liver function and LT-related short-term complications were analyzed.Results
Compared to Group B, patients in Group A had significantly increased time interval when undergoing LT (p?<?0.05). Persistently, the bodyweight, albumin and hemoglobin in Group A were significantly increased compared to Group B (all, p?<?0.0001). The bilirubin, ALT/AST and PELD score in Group A were all decreased compared to Group B (p?<?0.05) prior to LT. The incidences of post-LT severe infection and hypoalbuminemia in Group A were significantly lower than those of Group B (p?<?0.05).Conclusions
KP improves the nutritional status, preserves liver function for patients who finally undergo LT, and decreases the incidence post-LT severe infection and hypoalbuminemia. Mental health can be improved in parents whose children underwent previous KP.13.
Background
Congenital tracheobiliary fistula is a rare developmental anomaly with a persistent communication between the biliary system and the trachea.Characteristics
A 7-day-old baby with severe respiratory distress and aspiration pneumonia.Outcome
Tracheobilliary fistula identified on bronchoscopy. Open surgical excision of fistula was followed by improvement.Message
This condition should be considered in the differential diagnosis of intractable aspiration pneumonia.14.
Abhijeet Botre Vrajesh Udani Neelu Desai Spoorthy Jagadish Milind Sankhe 《Indian pediatrics》2017,54(8):678-680
Background
Management of refractory status epilepticus in children is extremely challenging.Case characteristics
Two children with medically refractory status epilepticus, both of whom had lesional pathology on MRI and concordant data on EEG and PET scan.Intervention
Emergency hemispherotomy performed in both patients. A complete, sustained seizure freedom obtained postoperatively.Message
Emergency surgery is a treatment option in selected cases of drug refractory status epilepticus with lesional pathology and concordant data.15.
Background
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP.Case characteristics
Three children with features of HSP presented with seizures and CNS dysfunction.Observation
All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage.Message
HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment.16.
Mini Sreedharan Kalpana Devadathan P. A. Mohammed Kunju Bindusha Sasidharan Jayakumar Parameswaran Pillai Minikumari Amma Vasumathy Amma Saboorabeegum MuthuBeevi 《Indian pediatrics》2018,55(4):307-310
Objective
To assess the effect of monotherapy with Carbamazepine (CBZ) and Sodium valproate (VPA) on serum 25-OH vitamin D levels in children with epilepsy compared to controls.Design
Cross-sectional study.Setting
Outpatient department of a tertiary-care Pediatric Neurology centre, and a nearby day-care centre and school.Study period
June 2012 to May 2013Participants
Children with epilepsy aged 2 to 13 years on monotherapy with CBZ (n=28) or VPA (n=28) for at least 6 months; 109 age-matched controls from a nearby day-care centre and school.Results
The median (IQR) values of 25 (OH) vitamin D was 18.0 ng/mL (13.7-27.3), 21.35 ng/mL (16.4 -25.2) and 30.5 ng/mL (19.1-43.7) in CBZ, VPA and control group, respectively (P=0.008). 60.7% of patients in CBZ group and 35.7 % in VPA group had low 25 (OH) D levels (<20 ng/mL) compared to 27.8% in controls (P=0.001).The serum alkaline phosphatase level was higher in children on carbamazepine therapy (P=0.001) than controls.Conclusion
This study identifies significant risk of vitamin D deficiency in ambulant children with epilepsy on monotherapy with CBZ or VPA.17.
Objective
To evaluate the perioperative risk factors for 30-day complications of the Kasai procedure in a large, cross-institutional, modern dataset.Study design
The 2012–2015 National Surgical Quality Improvement Program Pediatric database was used to identify patients undergoing the Kasai procedure. Patients’ characteristics were compared by perioperative blood transfusions and 30-day outcomes, including complications, reoperations, and readmissions. Multivariable logistic regression was used to identify risk factors predictive of outcomes. Propensity matching was performed for perioperative blood transfusions to evaluate its effect on outcomes.Results
190 children were included with average age of 62 days. Major cardiac risk factors were seen in 6.3%. Perioperative blood transfusions occurred in 32.1%. The 30-day post-operative complication rate was 15.8%, reoperation 6.8%, and readmission 15.3%. After multivariate analysis, perioperative blood transfusions (OR?3.94; p?<?0.01) and major cardiac risk factors (OR?7.82; p?<?0.01) were found to increase the risk of a complication. Perioperative blood transfusion (OR?4.71; p?=?0.01) was associated with an increased risk of reoperation. Readmission risk was increased by prematurity (OR?3.88; p?=?0.04) and 30-day complication event (OR?4.09; p?=?0.01). After propensity matching, perioperative blood transfusion was associated with an increase in complications (p?<?0.01) and length of stay (p?<?0.01).Conclusion
Major cardiac risk factors and perioperative blood transfusions increase the risk of post-operative complications in children undergoing the Kasai procedure. Further research is warranted in the perioperative use of blood transfusions in this population.Level of evidence
IV.18.
Objective
To study the prevalence of covert or early renal involvement among human immunodeficiency virus (HIV) positive children.Methods
A cross-sectional observational study was carried out on 250 HIV positive children (2–18 y) on follow-up in a tertiary care hospital from January 2014 through June 2015. Those who met the study criteria were evaluated for microalbuminuria and glomerular and tubular dysfunctions.Results
Fifty-one out of two hundred fifty (20%) children were found to have microalbuminuria though none had any other evidence of renal dysfunction. The incidence of microalbuminuria in children on Anti Retroviral Therapy (ART) and those not on ART was 20% and 21% respectively. Neither was there any difference in those with a CD4 count ≤500/cu mm compared with those with counts >500/cu mm. However, the overall CD4 counts were significantly lower in children with microalbuminuria.Conclusions
Microalbuminuria was detected in 20% of asymptomatic HIV positive children suggesting early glomerular dysfunction and need for regular screening and follow-up.19.
Background
Plastic bronchitis is characterized by formation of extensive obstructive endobronchial casts and high recurrence rates.Case characteristics
Two children (1-year-old girl, 7-year-old boy) who had recurrent episodes of respiratory distress with acute worsening. Bronchoscopy revealed membrane-like casts. Both children were managed with nebulized N-acetylcysteine in addition to management for asthma.Outcome
Symptom-free without recurrence for more than 9 months of follow-up.Message
Nebulized N-acetylcysteine may be helpful in prevention of recurrence of plastic bronchitis due to asthma.20.
Rahul Jain Anshita Arora Rohit Anand Shahzadi Malhotra Medha Mittal Monica Juneja 《Indian pediatrics》2017,54(7):550-555