Clinical Effectiveness of an Anesthesiologist-Administered Intravenous Sedation Outside of the Main Operating Room for Pediatric Upper Gastrointestinal Endoscopy in Thailand

Objectives. To review our sedation practice and to evaluate the clinical effectiveness of an anesthesiologist-administered intravenous sedation outside of the main operating room for pediatric upper gastrointestinal endoscopy (UGIE) in Thailand. Subjects and Methods. We undertook a retrospective review of the sedation service records of pediatric patients who underwent UGIE. All endoscopies were performed by a pediatric gastroenterologist. All sedation was administered by staff anesthesiologist or anesthetic personnel. Results. A total of 168 patients (94 boys and 74 girls), with age from 4 months to 12 years, underwent 176 UGIE procedures. Of these, 142 UGIE procedures were performed with intravenous sedation (IVS). The mean sedation time was 23.2 ± 10.0 minutes. Propofol was the most common sedative drugs used. Mean dose of propofol, midazolam and fentanyl was 10.0 ± 7.5 mg/kg/hr, 0.2 ± 0.2 mg/kg/hr, and 2.5 ± 1.2 mcg/kg/hr, respectively. Complications relatively occurred frequently. All sedations were successful. However, two patients became more deeply than intended and required unplanned endotracheal intubation. Conclusion. The study shows the clinical effectiveness of an anesthesiologist-administered IVS outside of the main operating room for pediatric UGIE in Thailand. All complications are relatively high. We recommend the use of more sensitive equipments such as end tidal CO2 and carefully select more appropriate patients

Benign Recurrent Intrahepatic Cholestasis: A Case Report

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive cholestatic liver disease. Recurrent self-limited episodes of jaundice and severe pruritus are leading clinical manifestations. We report a 16-year-old Thai boy with three recurrent episodes of cholestasis. The first episode occurred at 30 months old. The subsequent recurrent episodes were at 13 and 16 years, respectively. Investigations including viral study, autoimmune hepatitis markers, abdominal ultrasound and magnetic resonance cholangiopancreatography (MRCP) were negative. Diagnosis of BRIC was made based on specific criteria. He was treated with cholestyramine and ursodeoxycholic acid when the first and second episodes occurred which resulted in good recovery. However, for the last recurrent episode, treatment with ursodeoxycholic acid failed to improve his condition, so his treatment was changed to rifampicin, which resulted in dramatic response. Although BRIC is a rare disease, it should be taken into account in all cases with recurrent cholestasis with normal or minimally elevated GGT level. Currently, genetic study for the mutation of ATP8B1 and ABCB11 genes are crucial for making a definite diagnosis

Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants

Abstract Background Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication. Case presentation A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of SPINK1. We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches. Conclusions PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy

Neonatal intrahepatic cholestasis caused by citrin deficiency: prevalence and <it>SLC25A13</it> mutations among thai infants

<p>Abstract</p> <p>Background</p> <p>The most common causes of cholestatic jaundice are biliary atresia and idiopathic neonatal hepatitis (INH). Specific disorders underlying INH, such as various infectious and metabolic causes, including neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) especially, in East Asian populations are increasingly being identified. Since most NICCD infants recovered from liver disease by 1 year of age, they often are misdiagnosed with INH, leading to difficulty in determining the true prevalence of NICCD. Mutation(s) of human <it>SLC25A13</it> gene encoding a mitochondrial aspartate/glutamate carrier isoform 2 (AGC2), can lead to AGC2 deficiency, resulting in NICCD and an adult-onset fatal disease namely citrullinemia type II (CTLN2). To study the prevalence of NICCD and <it>SLC25A13</it> mutations in Thai infants, and to compare manifestations of NICCD and non-NICCD, infants with idiopathic cholestatic jaundice or INH were enrolled. Clinical and biochemical data were reviewed. Urine organic acid and plasma amino acids profiles were analyzed. PCR-sequencing of all 18 exons of <it>SLC25A13</it> and gap PCR for the mutations IVS16ins3kb and Ex16+74_IVS17-32del516 were performed. mRNA were analyzed in selected cases with possible splicing error.</p> <p>Results</p> <p>Five out of 39 (12.8%) unrelated infants enrolled in the study were found to have NICCD, of which three had homozygous 851del4 (GTATdel) and two compound heterozygous 851del4/IVS16ins3kb and 851del4/1638ins23, respectively. Two missense mutations (p.M1? and p.R605Q) of unknown functional significance were identified. At the initial presentation, NICCD patients had higher levels of alkaline phosphatase (ALP) and alpha-fetoprotein (AFP) and lower level of alanine aminotransferase (ALT) than those in non-NICCD patients (<it>p</it>< 0.05). NICCD patients showed higher citrulline level and threonine/serine ratio than non-NICCD infants (<it>p</it>< 0.05). Fatty liver was found in 2 NICCD patients. Jaundice resolved in all NICCD and in 87.5% of non-NICCD infants at the median age of 9.5 and 4.0 months, respectively.</p> <p>Conclusion</p> <p>NICCD should be considered in infants with idiopathic cholestasis. The preliminary estimated prevalence of NICCD was calculated to be 1/48,228 with carrier rate of 1/110 among Thai infants. However, this number may be underestimated and required further analysis with mutation screening in larger control population to establish the true prevalence of NICCD and AGC2 deficiency.</p