Gastric Peroral Endoscopic Myotomy: A Specific Learning Curve.

INTRODUCTION: Gastric peroral endoscopic pyloromyotomy (G-POEM) is a novel option for patients with gastroparesis. It involves submucosal tunneling across the pylorus, followed by pyloromyotomy, and subsequent closure of the endoscopic tunnel. The aim of this study was to determine the learning curve for G-POEM. METHODS: Consecutive patients undergoing G-POEM by a single operator were included from a prospective registry over 2 years. Demographics, procedure info, postprocedure follow-up data, and adverse events were collected. Nonlinear regression and cumulative sum control chart analyses were conducted for the learning curve. Clinical outcomes were improvement in Gastroparesis Cardinal Symptom Index score and gastric emptying scintigraphy. RESULTS: Thirty-six patients were included (16.7% M, mean age 46 y). The majority had idiopathic gastroparesis (n=16, 44%), with the remaining having diabetes (n=5, 17%), postsurgical (n=10, 28%), or other (n=4, 11%). Technical success was achieved in 35 of 36 (97%). There was a significant reduction in the total Gastroparesis Cardinal Symptom Index score (2.09 units, P<0.00001) and a significant reduction in postoperative gastric emptying scintigraphy (82.44 mins, P<0.00001). Mean follow-up was 15 months (SD, 1.05). Median procedure time was 60.5 minutes (range, 35 to 136). Cumulative sum control chart shows 60-minute procedure was achieved at the 18th procedure. Procedure durations further reduced with consequent procedures with the last 3 being 45 minutes, thus demonstrating continued improvement with ongoing experience (nonlinear regression P<0.0001). CONCLUSION: Endoscopists experienced in G-POEM are expected to achieve a reduction in procedure time over successive cases, with efficiency reached at 60.5 minutes and a learning rate of 18 cases with continuing improvement.

Learning curve for EUS-guided biliary drainage: What have we learned?

BACKGROUND AND OBJECTIVE: EUS-guided-biliary drainage (EUS-BD) is an efficacious and safe option for patients who fail ERCP. EUS-BD is a technically challenging procedure. The aim of this study was to define the learning curve for EUS-BD. METHODS: Consecutive patients undergoing EUS-BD by a single operator were included for a prospective registry over 6 years. Demographics, procedural information, adverse events, and follow-up data were collected. Nonlinear regression and CUSUM analyses were conducted for the learning curve. Technical success was defined as successful stent placement. Clinical success was defined as resolution of jaundice and/or at least a 30% reduction in the pretreatment bilirubin level within a week after placement or normalization of bilirubin within 30 days. RESULTS: Seventy-two patients were included in the study (53% male, mean age 67 years). Technical success was achieved in 69 patients (96%). Clinical success was achieved in 59/69 patients (86%). Seven patients (10%) had adverse events including bleeding (n = 6) and liver abscess (n = 1). The median procedural time was 59 min (range 36-138 min). This was achieved at the 32nd procedure. Procedural durations were further reduced to 50 min and below after the 50th procedure in a nonlinear pattern. This suggests that procedural durations approach a potential plateau after 100 cases. CONCLUSION: Endoscopists experienced in EUS-BD are expected to achieve a reduction in procedural time over successive cases, with efficiency reached at 59 min and a learning rate of 32 cases. Continued improvement is demonstrated with additional experience, with mastery suggested after approximately 100 cases.

Endoscopic ultrasound guided biliary drainage: a comprehensive review.

Endoscopic ultrasound-guided biliary drainage (EUS-BD) has become widely accepted over the past several years for complicated biliary drainage. When conventional endoscopic retrograde cholangiopancreatography (ERCP) is not successful or feasible due to a variety of constraints, an ultrasound-guided technique through intrahepatic and extrahepatic approaches allows adequate management of hepatobiliary obstruction. Other management alternatives include percutaneous transhepatic biliary drainages (PTBD) and complicated surgical approaches, both of which can be associated with increased morbidity and mortality. There are various technical approaches to allow successful biliary drainage which will be reviewed in this article.

Posterior Reversible Encephalopathy Syndrome following Ustekinumab Induction for Crohn's Disease.

Biological agents are frequently used in the management of inflammatory bowel disease, and it is important to understand the potential adverse effects of these therapies. Ustekinumab is a human monoclonal antibody that interferes with interleukin-12 and -23 cytokine signaling and is approved for the treatment of moderate to severe Crohn's disease. We report 2 cases of neurological adverse events, one of which is consistent with posterior reversible encephalopathy syndrome (PRES), in the setting of ustekinumab therapy for Crohn's disease. The first patient had a seizure and classic neuroimaging features of PRES following induction with ustekinumab. The second patient presented with acute encephalopathy and atypical imaging findings concerning for PRES after ustekinumab induction. Both patients recovered fully following cessation of ustekinumab therapy. PRES associated with ustekinumab is uncommon, but must be a consideration in Crohn's disease patients receiving this therapy who present with focal neurological symptoms or change in mentation.

Prognostic Models in Acute Liver Failure.

There is a strong imperative to develop valid and accurate prognostic modeling for acute liver failure (ALF). Despite the numerous clinical models that have been proposed thus far and the use of some such models, that is, King's College Criteria and Model for End-Stage Liver Disease, in clinical practice to aid decision-making, there is a significant need for improvement for determining patients' clinical course, survival, and requirement for liver transplantation. Future prognostic models shall need a stronger statistical foundation and accountability for time and variability in the clinical course of ALF and be applied for pretransplant and posttransplant outcomes.

Esophageal Stricture Resulting from Systemic Chemotherapy for Solid Malignancy.

Although dysphagia in patients treated for malignancy is usually related to reflux esophagitis, infectious esophagitis, malignant infiltration, or as a complication of radiation therapy, acute esophageal stricture resulting from chemotherapy is very rare. Only 2 prior cases have been described in the treatment of an adult patient with malignancy. We present a unique case of isolated chemotherapy-induced esophageal stricture in a patient receiving treatment for metastatic testicular seminoma without prior history of gastroesophageal reflux disease, caustic ingestion, or other risk factors for esophageal stricture formation.

Blood loss during posterior spinal fusion for adolescent idiopathic scoliosis.

STUDY DESIGN: Retrospective uncontrolled case series. OBJECTIVE: The purpose of this study was to determine the association, if any, between intraoperative blood loss and need for transfusion with the use of periapical (Ponte) osteotomies, as well as other patient and surgical variables among patients with adolescent idiopathic scoliosis (AIS) undergoing posterior spinal instrumentation and fusion. SUMMARY OF BACKGROUND DATA: Blood loss during posterior spinal fusion for AIS can be substantial. Numerous techniques are used to minimize intraoperative blood loss and the need for allogeneic transfusion. However, it is unclear which patient and surgeon variables affect blood loss most significantly. METHODS: A review was conducted on consecutive patients with AIS who had undergone posterior spinal fusion from July 1997 to February 2013 by a single primary surgeon at 1 institution. The relationship of estimated blood loss, normalized blood loss (normalized blood loss = estimated blood loss/number of levels fused/patient's weight in kilograms), autologous blood retrieved, and allogeneic transfusion received with various patient- and procedure-related variables were analyzed. RESULTS: Estimated blood loss, normalized blood loss, and autologous blood retrieved were higher in patients who underwent periapical Ponte osteotomies (n = 38) (P < 0.0001, P < 0.001, P < 0.01, respectively). The mean major curve correction was 64% in patients without osteotomies, and 65% in patients with osteotomies (P = 0.81). All patients who underwent osteotomies (38/38) received allogeneic transfusion versus 26% (19/73) of those without osteotomies (P < 0.001). The likelihood of transfusion correlated with increasing number of osteotomies and a lower preoperative hemoglobin level (odds ratio, 3.34; P = 0.003; and odds ratio, 0.51; P = 0.02, respectively). CONCLUSION: In patients with AIS undergoing posterior spinal fusion with instrumentation, performing periapical osteotomies increased all measures of intraoperative blood loss and need for transfusion without substantially improving major curve correction. As expected, a lower preoperative hemoglobin level was observed in patients who received a blood transfusion after posterior instrumentation and fusion. LEVEL OF EVIDENCE: 4.

Can yoga be used to treat gastroesophageal reflux disease?

Yoga methods including Pranayama are the best ways to prevent many diseases and their progression. Even though, Yoga is widely practiced, its effects on certain medical conditions have not been studied or reported. Gastroesophageal reflux disease (GERD) is one of them. GERD is extremely common condition requiring frequent consumption of over-the-counter or prescribed proton pump inhibitors (PPI). In severe symptoms of GERD and in the presence of multiple etiologies, PPIs are insufficient to relieve the symptoms of gastric reflux. Regular and proper use of the Yoga along with PPI can control the severe symptoms of GERD and can avoid or delay the necessity of invasive procedures. This evidence-based case report focuses on the effects of Yoga on GERD. Our case report showed that regular practice of Kapalbhati and Agnisar kriya along with PPI, patients with hiatal hernia had improvement in severe symptoms of GERD, which were initially refractory to PPI alone.

An in vitro paradigm for diabetic cerebral oedema and its therapy: a critical role for taurine and water channels.

The pathophysiology of cerebral oedema (CE) in diabetic ketoacidosis (DKA) remains enigmatic. We investigated the role of the idiogenic osmol taurine and aquaporin channels in an in vitro model, the SH-SY5Y neuroblastoma cell line, by sequentially mimicking DKA-like hyperglycemia/hypertonicity and hypotonic fluid therapy. Exposure to DKA-like hyperosmolarity led to shrinkage, while hypotonic fluid exposure led to cell swelling and impaired viability. Low sodium compensated in part for elevated glucose, pointing to a critical role for overall osmolality. Taurine, was synthesized and retained intracellularly during DKA-like hypertonicity, and released during hypotonicity, in part mitigating neuronal swelling. Metabolic labeling showed that the rate of taurine release was inadequate to fully prevent neuronal swelling during hypotonic fluid therapy following DKA-like hypertonicity. Under these conditions, Aquaporin4 & 9 channels were respectively down and up-regulated. Our study provides further novel insights into molecular mechanisms contributing to CE in DKA and its therapy.

Sweat testing for cystic fibrosis: standards of performance in Australasia.

BACKGROUND: Accurate measurement of sweat chloride concentration is essential for the diagnosis of cystic fibrosis (CF). We surveyed all laboratories enrolled in the Royal College of Pathologists of Australasia Quality Assurance Program (QAP) for Sweat Electrolytes to determine how closely they comply with the Australian Guidelines for the performance of the sweat test for the diagnosis of CF. METHODS: A detailed questionnaire covering most aspects of sweat collection and analysis was sent to all participating laboratories in 2007. RESULTS: Twenty out of 38 laboratories completed the questionnaire. While adherence to accepted guidelines was noted in many areas, the following main variations were recorded: some laboratories were not doing enough sweat tests to maintain expertise; some were not collecting sweat for the recommended collection time; sweat conductivity was the only test available in some laboratories; there was a lack of agreement between the sweat chloride concentration used to indicate CF or define an equivocal result. CONCLUSIONS: There is room for improvement in the performance of the sweat test in some laboratories in Australasia. The Sweat Testing Working Party of the Australasian Association of Clinical Biochemists is the appropriate body to address the problems involved in sweat testing and to bring about change.

Diagnosis of cystic fibrosis by sweat testing: age-specific reference intervals.

OBJECTIVE: To develop reference intervals (RIs) for sweat chloride and sodium in healthy children, adolescents, and adults. STUDY DESIGN: Healthy, unrelated subjects aged from 5 to >50 years and subjects who were pancreatic insufficient with cystic fibrosis (CF) were recruited. Sweat collection was performed on all subjects with the Wescor Macroduct system. Sweat electrolytes were analyzed with direct ion selective electrodes. DeltaF508 mutation analysis was performed on the healthy subjects >/=15 years old. RESULTS: A total of 282 healthy and 40 subjects with CF were included for analysis. There was no overlap of sweat chloride between the group with CF and the group without CF, but there was some overlap of sweat sodium. Sweat chloride increased with age, with the rate of increase slowing progressively to zero after the age of 19 years. The estimated median (95% RI) for sweat chloride were: 5 to 9 years, 13 mmol/L (1-39 mmol/L); 10 to 14 years, 18mmol/L (3-47 mmol/L); 15 to 19 years, 20 mmol/L (3-51mmol/L); and 20+ years 23 mmol/L (5-56mmol/L). CONCLUSIONS: We have successfully developed the age-related RI for sweat electrolytes, which will be useful for clinicians interpreting sweat test results from children, adolescents, and adults.

The limitations of sweat electrolyte reference intervals for the diagnosis of cystic fibrosis: a systematic review.

The sweat test has been used for more than 50 years for the diagnosis of cystic fibrosis (CF) and remains an important diagnostic test in the genomic era. The currently used reference intervals for sweat electrolytes are applied to all patients regardless of age or sex. We performed a systematic review to summarise the studies with published reference values of sweat electrolyte concentrations for the diagnosis of CF. The MEDLINE (from 1950), EMBASE (from 1980) and PubMed (from 1950) databases were searched for English language studies. An abstract was also found by hand-searching. The search generated 1136 articles that matched the search key terms. Of these, 17 studies that contained data on sweat electrolyte concentrations were included in the analysis. Among these, seven studies did not perform the sweat test in accordance with current international and Australian guidelines. Of the ten remaining studies, four reported both the sweat sodium and chloride concentrations and six reported sweat chloride concentration only. A major limitation of these studies was the subject selection. Most recruited patients with various medical conditions including respiratory diseases or undefined recruitment criteria, whilst some did not report the subjects' age and some had small subject numbers. Only one study performed mutation analysis to determine carrier status. No study used appropriate statistical analysis to develop a sweat chloride reference interval. The literature review yielded no studies that reliably developed reference intervals for sweat electrolyte concentrations. The limitations of the studies highlight the need for reliable age-related reference intervals for sweat electrolyte concentrations in healthy subjects.

The relevance of sweat testing for the diagnosis of cystic fibrosis in the genomic era.

Cystic fibrosis (CF) is the most common inherited disorder of childhood. The diagnosis of CF has traditionally been based on clinical features with confirmatory evidence by sweat electrolyte analysis. Since 1989 it has been possible to also use gene mutation analysis to aid the diagnosis. Cloning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has advanced our understanding of CF, in particular the molecular basis of an expanded CF phenotype. However, because there are over 1000 mutations and 200 polymorphisms, many without recognised effects on CFTR, the molecular diagnosis can be troublesome. This has necessitated measurement of CFTR function with renewed interest in the sweat test. This review provides an overview of the clinical features of CF, the diagnosis and complex genetics. We provide a detailed discussion of the structure and function of CFTR and the classification of CFTR mutations. Sweat electrolyte analysis is discussed, from the physiology of sweating to the rigours of a properly performed sweat test and its interpretation. With this information it is possible to understand the relevance of the sweat test in the genomic era.