AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA ...AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.展开更多
Peutz-Jeghers syndrome （PJS）, a rare autosomal dominant inherited disorder, is characterized by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome have a predisposition...Peutz-Jeghers syndrome （PJS）, a rare autosomal dominant inherited disorder, is characterized by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome have a predisposition to a variety of cancers in multiple organs. Mutations in the serine/threonine kinase 11 （STK11） gene have been identified as a major cause of PJS. Here we present the clinical and molecular findings of two unrelated Thai individuals with PJS. Mutation analysis by Polymerase Chain Reaction-sequencing of the entire coding region of STK11 revealed two potentially pathogenic mutations. One harbored a single nucleotide deletion （c.182delG） in exon 1 resulting in a frameshift leading to premature termination at codon 63 （p.Gly61AlafsX63）. The other carried an in-frame 9-base-pair （bp） deletion in exon 7, c.907_915del9 （p.Ile303_GIn305del）. Both deletions were de novo and have never been previously described. This study has expanded the genotypic spectrum of the STK11 gene.展开更多
AIM To assess the seroprevalence of hepatitis B virus(HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantat...AIM To assess the seroprevalence of hepatitis B virus(HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation.METHODS This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss. RESULTS In total, 50 children were recruited. The mean time from liver transplantation to anti-HBs testing was 2.53 ± 2.11 years. The mean anti-HBs levels before and after liver transplantation were 584.41 ± 415.45 and 58.56 ± 6.40 IU/L, respectively. The rate of nonimmunity(anti-HBs < 10 IU/L) in the participants was 46%(n = 26) at one year, 57%(n = 7) at two years and 82%(n = 17) at > three years following liver transplantation. The potential factors relating to HBV immunity loss after liver transplantation were identified as anti-HBs(P = 0.002), serum albumin(P = 0.04), total bilirubin(P = 0.001) and direct bilirubin(P = 0.003) before liver transplantation. A five-year-old boy with biliary cirrhosis received 4 doses of HBV vaccine with an anti-HBs titer of > 1000 IU/L and underwent liver transplantation; his anti-HBc-negative father was the donor. After liver transplantation, the boy had stenosis of the hepatic artery up to the inferior vena cava anastomosis and underwent venoplasty three times. He also received subcutaneous injections of enoxaparin for 5 mo and 20 transfusions of blood components. Three years and ten months after the liver transplantation, transaminitis was detected with positive tests for HBs Ag, HBe Ag, and anti-HBc(2169.61, 1706 and 8.45, respectively; cutoff value: < 1.00) and an HBV viral load of 33212320 IU/mL.CONCLUSION The present study showed that loss of hepatitis B immunity after liver transplantation is unexpectedly common. In our case report, despite high levels of antiHBs prior to transplantation, infection occurred at a time when, unfortunately, the child had lost immunity to hepatitis B after liver transplantation.展开更多
AIM:Inflammatory fibroid polyp (IFP) is a rare benign lesion that may occur throughout the digestive tract.IFP is more commonly found in the antrum of the stomach in particular. It mostly affects adults at the average...AIM:Inflammatory fibroid polyp (IFP) is a rare benign lesion that may occur throughout the digestive tract.IFP is more commonly found in the antrum of the stomach in particular. It mostly affects adults at the average age of 60 years.These polyps are able to cause abdominal pain,gastrointestinal bleeding,intestinal obstruction or intussusception.In this paper we report a case of gastric TIP with unusual presenting features. METHODS:A child with gastric IFP was described and the literature was reviewed, RESULTS:A 4-year-old girl presented with fever for 2 months,arthralgia of knees and ankles,iron defidency anemia, and hypoalbuminemia.Her stool examination was positive for occult blood.The upper gastrointestinal study demonstrated a large Iobulated mass at the upper part of gastric body.Partial gastrectomy en blocwith this 5 cm×8 cm mass was subsequently performed.Pathological examination was consistent with IFP.Following the mass excision,her fever abruptly declined and disappeared together with anemia and arthralgia.She remained asymptomatic and the abdominal ultrasonography performed at the 24-month follow-up demonstrated no recurrence of the tumor. CONCLUSION:The etiopathogenesis of IFP still remains unclear,The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose,The inflammatory symptoms found in this patient support the hypothesis of inflammatory benign lesions of IFP.展开更多
AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 ...AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 （sICAM-1） and clinical outcome in BA patients after surgical treatment, METHODS： Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA rnebhod from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups： patients without jaundice and those with persistent jaundice. RESULTS： There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1 genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1 polymorphism and the status of jaundice in BA patients after Kasai operation. CONCLUSION： ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469E ICAM-1 polymorphism.展开更多
AIMTo investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in posto...AIMTo investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in postoperative BA children. METHODSEighty-five postKasai BA children and 24 control subjects were registered. Circulating uPAR was measured using enzyme-linked immunosorbent essay. Liver stiffness was analyzed using transient elastography. RESULTSBA children had significantly greater circulating uPAR and liver stiffness scores than control subjects (P P r = 0.507, P r = 0.364, P r = 0.559, P r = 0.325, P r = 0.508, P CONCLUSIONCirculating uPAR and liver stiffness values were greater in BA children than healthy controls. The increased circulating uPAR was associated with liver dysfunction in BA. As a consequence, serum uPAR and liver stiffness may be used as noninvasive biomarkers indicating the progression of liver fibrosis in postKasai BA.展开更多
BACKGROUND Liver transplantation(LT)has become an acceptable curative method for children with several liver diseases,especially irreversible acute liver failure and chronic liver diseases.King Chulalongkorn Memorial ...BACKGROUND Liver transplantation(LT)has become an acceptable curative method for children with several liver diseases,especially irreversible acute liver failure and chronic liver diseases.King Chulalongkorn Memorial Hospital is one of Thailand’s largest liver transplant centers and is responsible for many pediatric cases.AIM To report the experience with pediatric LT and evaluate outcomes of livingrelated vs deceased-donor grafts.METHODS This evaluation included children who underwent LT between August 2004 and November 2019.Data were retrospectively reviewed,including demographics,diagnoses,laboratory values of donors and recipients,the pediatric end-stage liver disease(PELD)or model for end-stage liver disease(MELD)score,graft source,wait time,perioperative course,postoperative complications,and survival rates.Continuous data were reported using the median and interquartile range.The Mann–Whitney U-test was used to compare the wait time between the living-related and deceased-donor groups.The chi-square or Fisher's exact test were used to compare the frequencies of between-group complications.Survival rates were calculated using the Kaplan–Meier method.RESULTS Ninety-four operated pediatric liver transplant patients were identified(54%were females).The median age at transplantation was 1.2(0.8-3.8)years.The median PELD and MELD scores were 20(13-26.8)and 19.5(15.8-26.3),respectively.Most grafts(81.9%)were obtained from living-related donors.The median wait time for the living donors was significantly shorter compared with the deceased donors at 1.6(0.3-3.1)mo vs 11.2(2.1-33.3)mo(P=0.01).Most patients were diagnosed with biliary atresia(74.5%),and infection was the most common complication within 30 d posttransplantation(14.9%).Without a desensitization protocol,9%of transplants were ABOincompatible.Eight hepatitis B core antibodies(anti-HBc)-negative recipients received positive anti-HBc grafts without different observed complications.The overall survival rate was 93.6%and 90.3%at 1 and 5 years,respectively.No graft loss during follow-up was noted among survivors.CONCLUSION A significant number of pediatric LT cases were reported in Thailand.Based on relatively comparable outcomes,ABO-incompatible and HBc antibody-positive grafts may be considered in an organ shortage situation.展开更多
AIM To investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia(BA) children and the association of bone mineral density(BMD) and biochemical parameters in post Kasai BA subjects. MET...AIM To investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia(BA) children and the association of bone mineral density(BMD) and biochemical parameters in post Kasai BA subjects. METHODS A total of 70 patients with post Kasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energyX-ray absorptiometry.RESULTS The prevalence of low bone mass(osteopenia and osteoporosis) in BA patients were 51.4%(36 out of 70). Ten patients(35.7%) in the jaundice group and 8 patients(19.0%) in the non-jaundice group had osteopenia. Sixteen patients(57.1%) in the jaundice group and 2 patients(4.8%) in the no jaundice group had osteoporosis. In addition, lumbar spine BMD Z-score was substantially lower in the jaundice BA patients compared with non-jaundice patients. BA subjects with persistent jaundice had significantly lower serum 25-hydroxyvitamin D than those without jaundice. Further analysis revealed that lumbar spine BMD was correlated with age(r = 0.774, P < 0.001), serum albumin(r = 0.333, P = 0.005), total bilirubin(r =-0.476, P < 0.001), aspartate aminotransferase(r =-0.583, P < 0.001), alanine aminotransferase(r =-0.428, P < 0.001), and alkaline phosphatase(r =-0.456, P < 0.001).CONCLUSION Low BMD was associated with biochemical parameters reflecting the severity of cholestasis in post Kasai BA patients.展开更多
BACKGROUND Children with esophageal atresia(EA)have risk of gastroesophageal reflux disease(GERD),suggesting reflux monitoring for prompt management.AIM To evaluate GERD in children with EA and specific symptom associ...BACKGROUND Children with esophageal atresia(EA)have risk of gastroesophageal reflux disease(GERD),suggesting reflux monitoring for prompt management.AIM To evaluate GERD in children with EA and specific symptom association from combined Video with Multichannel Intraluminal Impedance and pH(MII-pH)study.METHODS Children diagnosed with EA with suspected GERD and followed up at King Chulalongkorn Memorial Hospital between January 2000 and December 2018 were prospectively studied.All underwent esophagogastroduodenoscopy with esophageal biopsy and Video MII-pH study on the same day.Symptoms of GERD which included both esophageal and extra-esophageal symptom were recorded from video monitoring and abnormal reflux from MII-pH study based on the statement from the European Paediatric Impedance Group.Prevalence of GERD was also reported by using histopathology as a gold standard.Endoscopic appearance was recorded using Los Angeles Classification and esophagitis severity was graded using Esohisto criteria.RESULTS Fifteen children were recruited with age of 3.1(2.2,9.8)years(40%,male)and the common type was C(93.3%).The symptoms recorded were cough(75.2%),vomiting(15.2%),irritability or unexplained crying(7.6%)and dysphagia(1.9%)with the symptom-reflux association of 45.7%,89%,71%and 0%,respectively.There were abnormal endoscopic appearance in 52.9%,esophagitis in 64.7%and high reflux score in 47.1%.Video MII-pH study has high diagnostic value with the sensitivity,specificity and accuracy of 72.7%,100%and 82.4%,respectively.CONCLUSION Prevalence of GERD in children with EA was high.Video MII-pH study to detect GERD in children with EA had high diagnostic value with the trend of specific symptom association.展开更多
基金Supported by Ratchadapiseksompotch Fund, Faculty of Medicine, Chulalongkorn University, Thailand Research Fund, and the Commission on Higher Education
文摘AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.
基金Supported by Chulalongkorn University,National Science and Technology Development Agency,and the Thailand Research Fund
文摘Peutz-Jeghers syndrome （PJS）, a rare autosomal dominant inherited disorder, is characterized by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome have a predisposition to a variety of cancers in multiple organs. Mutations in the serine/threonine kinase 11 （STK11） gene have been identified as a major cause of PJS. Here we present the clinical and molecular findings of two unrelated Thai individuals with PJS. Mutation analysis by Polymerase Chain Reaction-sequencing of the entire coding region of STK11 revealed two potentially pathogenic mutations. One harbored a single nucleotide deletion （c.182delG） in exon 1 resulting in a frameshift leading to premature termination at codon 63 （p.Gly61AlafsX63）. The other carried an in-frame 9-base-pair （bp） deletion in exon 7, c.907_915del9 （p.Ile303_GIn305del）. Both deletions were de novo and have never been previously described. This study has expanded the genotypic spectrum of the STK11 gene.
基金Supported by the Development of New Faculty Staff,Ratchadaphiseksomphot Endowment Fund to Sintusek PThe Special Task Force for Activating Research in Immune Response in Children with Chronic Liver Diseases and Children after Liver Transplantation,Chulalongkorn University and King Chulalongkorn Memorial Hospital,Bangkok,Thailand to Sintusek P+1 种基金the Research Chair Grant from the National Science and Technology Development Agency,No.P-15-50004 to Poovorawan YThe Center of Excellence in Clinical Virology,Chulalongkorn Unversity and King Chulalongkorn Memorial Hospital,No.GCE 5900930-005 to Poovorawan Y
文摘AIM To assess the seroprevalence of hepatitis B virus(HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation.METHODS This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss. RESULTS In total, 50 children were recruited. The mean time from liver transplantation to anti-HBs testing was 2.53 ± 2.11 years. The mean anti-HBs levels before and after liver transplantation were 584.41 ± 415.45 and 58.56 ± 6.40 IU/L, respectively. The rate of nonimmunity(anti-HBs < 10 IU/L) in the participants was 46%(n = 26) at one year, 57%(n = 7) at two years and 82%(n = 17) at > three years following liver transplantation. The potential factors relating to HBV immunity loss after liver transplantation were identified as anti-HBs(P = 0.002), serum albumin(P = 0.04), total bilirubin(P = 0.001) and direct bilirubin(P = 0.003) before liver transplantation. A five-year-old boy with biliary cirrhosis received 4 doses of HBV vaccine with an anti-HBs titer of > 1000 IU/L and underwent liver transplantation; his anti-HBc-negative father was the donor. After liver transplantation, the boy had stenosis of the hepatic artery up to the inferior vena cava anastomosis and underwent venoplasty three times. He also received subcutaneous injections of enoxaparin for 5 mo and 20 transfusions of blood components. Three years and ten months after the liver transplantation, transaminitis was detected with positive tests for HBs Ag, HBe Ag, and anti-HBc(2169.61, 1706 and 8.45, respectively; cutoff value: < 1.00) and an HBV viral load of 33212320 IU/mL.CONCLUSION The present study showed that loss of hepatitis B immunity after liver transplantation is unexpectedly common. In our case report, despite high levels of antiHBs prior to transplantation, infection occurred at a time when, unfortunately, the child had lost immunity to hepatitis B after liver transplantation.
基金Supported by the Thailand Research Fund and Center of Excellence,Viral Hepatitis Research Unit,Chulalongkorn University
文摘AIM:Inflammatory fibroid polyp (IFP) is a rare benign lesion that may occur throughout the digestive tract.IFP is more commonly found in the antrum of the stomach in particular. It mostly affects adults at the average age of 60 years.These polyps are able to cause abdominal pain,gastrointestinal bleeding,intestinal obstruction or intussusception.In this paper we report a case of gastric TIP with unusual presenting features. METHODS:A child with gastric IFP was described and the literature was reviewed, RESULTS:A 4-year-old girl presented with fever for 2 months,arthralgia of knees and ankles,iron defidency anemia, and hypoalbuminemia.Her stool examination was positive for occult blood.The upper gastrointestinal study demonstrated a large Iobulated mass at the upper part of gastric body.Partial gastrectomy en blocwith this 5 cm×8 cm mass was subsequently performed.Pathological examination was consistent with IFP.Following the mass excision,her fever abruptly declined and disappeared together with anemia and arthralgia.She remained asymptomatic and the abdominal ultrasonography performed at the 24-month follow-up demonstrated no recurrence of the tumor. CONCLUSION:The etiopathogenesis of IFP still remains unclear,The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose,The inflammatory symptoms found in this patient support the hypothesis of inflammatory benign lesions of IFP.
文摘AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 （sICAM-1） and clinical outcome in BA patients after surgical treatment, METHODS： Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA rnebhod from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups： patients without jaundice and those with persistent jaundice. RESULTS： There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1 genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1 polymorphism and the status of jaundice in BA patients after Kasai operation. CONCLUSION： ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469E ICAM-1 polymorphism.
基金the Thailand Research Fund (RSA5880019)the Research Chair Grant from the National Science and Technology Development Agency+2 种基金the 100th Anniversary Chulalongkorn University Fund for Doctoral Scholarship to WUNational Research University Project, through the Ageing Cluster (NRU59056-AS)Chulalongkorn University
文摘AIMTo investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in postoperative BA children. METHODSEighty-five postKasai BA children and 24 control subjects were registered. Circulating uPAR was measured using enzyme-linked immunosorbent essay. Liver stiffness was analyzed using transient elastography. RESULTSBA children had significantly greater circulating uPAR and liver stiffness scores than control subjects (P P r = 0.507, P r = 0.364, P r = 0.559, P r = 0.325, P r = 0.508, P CONCLUSIONCirculating uPAR and liver stiffness values were greater in BA children than healthy controls. The increased circulating uPAR was associated with liver dysfunction in BA. As a consequence, serum uPAR and liver stiffness may be used as noninvasive biomarkers indicating the progression of liver fibrosis in postKasai BA.
文摘BACKGROUND Liver transplantation(LT)has become an acceptable curative method for children with several liver diseases,especially irreversible acute liver failure and chronic liver diseases.King Chulalongkorn Memorial Hospital is one of Thailand’s largest liver transplant centers and is responsible for many pediatric cases.AIM To report the experience with pediatric LT and evaluate outcomes of livingrelated vs deceased-donor grafts.METHODS This evaluation included children who underwent LT between August 2004 and November 2019.Data were retrospectively reviewed,including demographics,diagnoses,laboratory values of donors and recipients,the pediatric end-stage liver disease(PELD)or model for end-stage liver disease(MELD)score,graft source,wait time,perioperative course,postoperative complications,and survival rates.Continuous data were reported using the median and interquartile range.The Mann–Whitney U-test was used to compare the wait time between the living-related and deceased-donor groups.The chi-square or Fisher's exact test were used to compare the frequencies of between-group complications.Survival rates were calculated using the Kaplan–Meier method.RESULTS Ninety-four operated pediatric liver transplant patients were identified(54%were females).The median age at transplantation was 1.2(0.8-3.8)years.The median PELD and MELD scores were 20(13-26.8)and 19.5(15.8-26.3),respectively.Most grafts(81.9%)were obtained from living-related donors.The median wait time for the living donors was significantly shorter compared with the deceased donors at 1.6(0.3-3.1)mo vs 11.2(2.1-33.3)mo(P=0.01).Most patients were diagnosed with biliary atresia(74.5%),and infection was the most common complication within 30 d posttransplantation(14.9%).Without a desensitization protocol,9%of transplants were ABOincompatible.Eight hepatitis B core antibodies(anti-HBc)-negative recipients received positive anti-HBc grafts without different observed complications.The overall survival rate was 93.6%and 90.3%at 1 and 5 years,respectively.No graft loss during follow-up was noted among survivors.CONCLUSION A significant number of pediatric LT cases were reported in Thailand.Based on relatively comparable outcomes,ABO-incompatible and HBc antibody-positive grafts may be considered in an organ shortage situation.
基金Supported by the Thailand Research Fund(RSA5880019)the Research Chair Grant from the National Science and Technology Development AgencyNational Research University Project,through the Ageing Cluster(NRU59-056-AS),Chulalongkorn University
文摘AIM To investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia(BA) children and the association of bone mineral density(BMD) and biochemical parameters in post Kasai BA subjects. METHODS A total of 70 patients with post Kasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energyX-ray absorptiometry.RESULTS The prevalence of low bone mass(osteopenia and osteoporosis) in BA patients were 51.4%(36 out of 70). Ten patients(35.7%) in the jaundice group and 8 patients(19.0%) in the non-jaundice group had osteopenia. Sixteen patients(57.1%) in the jaundice group and 2 patients(4.8%) in the no jaundice group had osteoporosis. In addition, lumbar spine BMD Z-score was substantially lower in the jaundice BA patients compared with non-jaundice patients. BA subjects with persistent jaundice had significantly lower serum 25-hydroxyvitamin D than those without jaundice. Further analysis revealed that lumbar spine BMD was correlated with age(r = 0.774, P < 0.001), serum albumin(r = 0.333, P = 0.005), total bilirubin(r =-0.476, P < 0.001), aspartate aminotransferase(r =-0.583, P < 0.001), alanine aminotransferase(r =-0.428, P < 0.001), and alkaline phosphatase(r =-0.456, P < 0.001).CONCLUSION Low BMD was associated with biochemical parameters reflecting the severity of cholestasis in post Kasai BA patients.
基金The author would like to thank Ratchadapiseksompotch Fund,Faculty of Medicine,Chulalongkorn University(Grant No RA62/001),the Pediatric Liver Diseases and Immunology STAR(Special Task Force for Activating Research),Department of Pediatrics,King Chulalongkorn Memorial Hospital and Faculty of Medicine,Chulalongkorn University for research funding.
文摘BACKGROUND Children with esophageal atresia(EA)have risk of gastroesophageal reflux disease(GERD),suggesting reflux monitoring for prompt management.AIM To evaluate GERD in children with EA and specific symptom association from combined Video with Multichannel Intraluminal Impedance and pH(MII-pH)study.METHODS Children diagnosed with EA with suspected GERD and followed up at King Chulalongkorn Memorial Hospital between January 2000 and December 2018 were prospectively studied.All underwent esophagogastroduodenoscopy with esophageal biopsy and Video MII-pH study on the same day.Symptoms of GERD which included both esophageal and extra-esophageal symptom were recorded from video monitoring and abnormal reflux from MII-pH study based on the statement from the European Paediatric Impedance Group.Prevalence of GERD was also reported by using histopathology as a gold standard.Endoscopic appearance was recorded using Los Angeles Classification and esophagitis severity was graded using Esohisto criteria.RESULTS Fifteen children were recruited with age of 3.1(2.2,9.8)years(40%,male)and the common type was C(93.3%).The symptoms recorded were cough(75.2%),vomiting(15.2%),irritability or unexplained crying(7.6%)and dysphagia(1.9%)with the symptom-reflux association of 45.7%,89%,71%and 0%,respectively.There were abnormal endoscopic appearance in 52.9%,esophagitis in 64.7%and high reflux score in 47.1%.Video MII-pH study has high diagnostic value with the sensitivity,specificity and accuracy of 72.7%,100%and 82.4%,respectively.CONCLUSION Prevalence of GERD in children with EA was high.Video MII-pH study to detect GERD in children with EA had high diagnostic value with the trend of specific symptom association.
