[100 years Peutz-Jeghers syndrome]. / 100 jaar Peutz-Jeghers-syndroom.

In 2021 it was 100 years since drPeutz published his case report titled: 'a very remarkable case of familial polyposis of mucous membranes of intestinal tract and nasopharynx accompanied by peculiar pigmentations of skin and mucous membrane'. This is the first description of the Peutz-Jeghers syndrome, which is named after him. Like Peutz already suggested a century ago, we know now that this is a genetic disorder (autosomal dominant) caused by mutations in the STK11 gene. The clinical symptoms are typical pigmentations of the mucous membranes and hamartomatous polyps which already at a young age can result in polyp related complications like intussusception. Thereby patients with the Peutz-Jeghers syndrome have a high risk of developing an intestinal or extra-intestinal malignancy. For this reason there are strict surveillance guidelines for these patients. Even after a hundred years there is still a high mortality risk for patients with this syndrome.

Variability in the Use of Protective Mechanical Ventilation During General Anesthesia.

BACKGROUND: The purpose of this study was to determine whether significant variation exists in the use of protective ventilation across individual anesthesia providers and whether this difference can be explained by patient, procedure, and provider-related characteristics. METHODS: The cohort consisted of 262 anesthesia providers treating 57,372 patients at a tertiary care hospital between 2007 and 2014. Protective ventilation was defined as a median positive end-expiratory pressure of 5 cm H2O or more, tidal volume of <10 mL/kg of predicted body weight and plateau pressure of <30 cm H2O. Analysis was performed using mixed-effects logistic regression models with propensity scores to adjust for covariates. The definition of protective ventilation was modified in sensitivity analyses. RESULTS: In unadjusted analysis, the mean probability of administering protective ventilation was 53.8% (2.5th percentile of provider 19.9%, 97.5th percentile 80.8%). After adjustment for a large number of covariates, there was little change in the results with a mean probability of 51.1% (2.5th percentile 24.7%, 97.5th percentile 77.2%). The variations persisted when the thresholds for protective ventilation were changed. CONCLUSIONS: There was significant variability across individual anesthesia providers in the use of intraoperative protective mechanical ventilation. Our data suggest that this variability is highly driven by individual preference, rather than patient, procedure, or provider-related characteristics.

Liver test abnormalities predict complicated disease behaviour in patients with newly diagnosed Crohn's disease.

BACKGROUNDS: In coeliac disease, the prevalence of liver test abnormalities (LTAs) is higher in patients with more severe mucosal inflammation. In Crohn's disease, prognosis is related to the severity of mucosal inflammation. AIM: The aim of this study was to investigate whether the presence of LTA predicts the occurrence of complicated disease behaviour in newly diagnosed Crohn's disease. METHODS: A retrospective cohort study was performed in patients newly diagnosed with Crohn's disease between 2002 and 2011. The complicated disease was defined as the occurrence of stricturing and/or perforating disease. LTAs were defined as a value of any of alkaline phosphatase (AP), gamma-glutamyl transpeptidase (GGT), aspartate aminotransferase (AST), or alanine aminotransferase (ALT) over the upper limit of normal. RESULTS: Three hundred eighty-three patients were included, of whom 34.1% had LTA. LTAs were mostly mild (less than two times the upper limit of normal). During the 5-year follow-up, 33.1% of patients in the group with LTA developed complicated disease behaviour compared to 14.6% in patients without LTA (p < 0.001). The presence of LTA was identified as a risk factor for complicated disease behaviour (HR 2.6, 95% confidence interval (CI) 1.5-4.2, p < 0.0001). CONCLUSIONS: In newly diagnosed Crohn's disease, the presence of LTA was an independent risk factor for the development of complicated disease behaviour.

Differential Effects of Intraoperative Positive End-expiratory Pressure (PEEP) on Respiratory Outcome in Major Abdominal Surgery Versus Craniotomy.

OBJECTIVES: In this study, we examined whether (1) positive end-expiratory pressure (PEEP) has a protective effect on the risk of major postoperative respiratory complications in a cohort of patients undergoing major abdominal surgeries and craniotomies, and (2) the effect of PEEP is differed by surgery type. BACKGROUND: Protective mechanical ventilation with lower tidal volumes and PEEP reduces compounded postoperative complications after abdominal surgery. However, data regarding the use of intraoperative PEEP is conflicting. METHODS: In this observational study, we included 5915 major abdominal surgery patients and 5063 craniotomy patients. Analysis was performed using multivariable logistic regression. The primary outcome was a composite of major postoperative respiratory complications (respiratory failure, reintubation, pulmonary edema, and pneumonia) within 3 days of surgery. RESULTS: Within the entire study population (major abdominal surgeries and craniotomies), we found an association between application of PEEP ≥5 cmH2O and a decreased risk of postoperative respiratory complications compared with PEEP <5 cmH2O. Application of PEEP >5 cmH2O was associated with a significant lower odds of respiratory complications in patients undergoing major abdominal surgery (odds ratio 0.53, 95% confidence interval 0.39 - 0.72), effects that translated to deceased hospital length of stay [median hospital length of stay : 6 days (4-9 days), incidence rate ratios for each additional day: 0.91 (0.84 - 0.98)], whereas PEEP >5 cmH2O was not significantly associated with reduced odds of respiratory complications or hospital length of stay in patients undergoing craniotomy. CONCLUSIONS: The protective effects of PEEP are procedure specific with meaningful effects observed in patients undergoing major abdominal surgery. Our data suggest that default mechanical ventilator settings should include PEEP of 5-10 cmH2O during major abdominal surgery.