Stiff person syndrome

Case report and brief review.

Rethinking sepsis resuscitation

Here’s a provocative review article.

Some interesting questions were asked. For example, is lactate an indicator of tissue oxygenation in sepsis? Evidence suggests it may be due to a metabolic effect instead, unrelated to tissue hypoxia, that is, type b lactic acidosis. Nevertheless it appears to be a useful marker to follow. Certainly its elevation portends a worse outcome but maybe not by the mechanism we think. See here. All this is not to say that type a lactic acidosis does not exist (that is, is not due to tissue ischemia in other forms of shock, in low cardiac output states or in high demand states).

How strong is the evidence in favor of fluid resuscitation in sepsis? Pretty weak. The author talks around the idea, but comes short, of saying we should stop doing it. He also talks around the idea, but comes just short, of saying there is equipoise for a controlled trial of fluid resuscitation versus no fluid resuscitation.

Short telomere syndromes

From a recent review:

Short telomere syndromes (STSs) are accelerated aging syndromes with multisystemic manifestations that present complex management challenges. In this article, we discuss a single-institution experience in diagnosing and managing patients with inherited STSs. In total, we identified 17 patients with short telomeres, defined by flow-fluorescence in-situ hybridization telomere lengths of less than first centile in granulocytes/lymphocytes OR the presence of a characteristic germline pathogenic variant in the context of a highly suggestive clinical phenotype. Genetic variations in the telomere complex were identified in 6 (35%) patients, with 4 being known pathogenic variants involving TERT (n=2), TERC (n=1), and DKC1 (n=1) genes, while 2 were variants of uncertain significance in TERT and RTEL1 genes. Idiopathic interstitial pneumonia (IIP) (n=12 [71%]), unexplained cytopenias (n=5 [29%]), and cirrhosis (n=2 [12%]) were most frequent clinical phenotypes at diagnosis. At median follow-up of 48 (range, 0-316) months, Kaplan-Meier estimate of overall survival, median (95% CI), was 182 (113, not reached) months. Treatment modalities included lung transplantation for IIP (n=5 [29%]), with 3 patients developing signs of acute cellular rejection (2, grade A2; 1, grade A1); danazol therapy for cytopenias (n=4 [24%]), with only 1 out of 4 patients showing a partial hematologic response; and allogeneic hematopoietic stem cell transplant for progressive bone marrow failure (n=2), with 1 patient dying from transplant-related complications. In summary, patients with STSs present with diverse clinical manifestations and require a multidisciplinary approach to management, with organ-specific transplantation capable of providing clinical benefit.

Antibiotic stewardship programs

Effective in reducing c diff as well as infection and colonization with resistant organisms in this systematic review.

Are specialists ruining medicine in the US?

Jamie Koufman seems to think so.

Her solution? Let primary care docs be the gatekeepers who control access to specialists. Didn’t we try that in the 90s with managed care? It didn’t work.

Sometimes more may be better: 30 day costs and MI outcomes

From AHA meeting abstracts:

Methods: Eligible patients were fee-for-service Medicare beneficiaries 65 years and older hospitalized at an acute-care hospital for AMI between 2011 and 2014. Spending was defined as hospital-level risk-standardized payments associated with a 30-day episode of AMI care, stratified into low ( less than 25 percentile), average (greater than or equal to 25 to less than or equal to 75 percentile), and high (greater than 75 percentile) spending groups. The primary outcome was mortality within 30 days of admission. To examine the association between hospital-level 30-day spending and mortality, we fitted mixed-effects logistic regression models with random hospital intercepts to model 30-day mortality as a function of patients’ admitting hospital’s expenditure.

Results: We included 642,105 index hospitalizations for AMI at 2,319 acute-care hospitals. Hospitals with higher 30-day spending were larger, tended to be teaching hospitals, were more often located in an urban area, were more likely to have cardiac catheterization laboratories and cardiac surgery capability, and also had higher rates of coronary revascularization. Across hospital groups, median 30-day spending per beneficiary was $20,207 (low), $22,018 (average), and $24,174 (high). Higher hospital-level 30-day spending was associated with lower 30-day mortality even after adjustment for patient and hospital characteristics (adjusted odds ratio for additional $1000 spending, 0.989; 95% confidence interval 0.982-0.996, p=0.002). This relationship was not attenuated following additional adjustment for hospital-level revascularization rates.

Sepsis treatment

Maybe early goal directed therapy wasn’t such a bad idea after all.

Silent atrial fibrillation in patients who have not had a stroke: what to do?

From the green journal:

A new suspected cause of cryptic strokes is “silent atrial fibrillation.” Pacemakers and other implanted devices allow continuous recording of cardiac rhythm for months or years. They have discovered that short periods of atrial fibrillation lasting minutes or hours are frequent and usually are asymptomatic. A meta-analysis of 50 studies involving more than 10,000 patients with a recent stroke found that 7.7% had new atrial fibrillation on their admitting electrocardiogram. In 3 weeks during and after hospitalization, another 16.9% were diagnosed. A total of 23.7% of these stroke patients had silent atrial fibrillation; that is, atrial fibrillation diagnosed after hospital admission. Silent atrial fibrillation is also frequent in patients with pacemakers who do not have a recent stroke. In a pooled analysis of 3 studies involving more than 10,000 patients monitored for 24 months, 43% had at least 1 day with atrial fibrillation lasting more than 5 minutes. Ten percent had atrial fibrillation lasting at least 12 hours. Despite the frequency of silent atrial fibrillation in these patients with multiple risk factors for stroke, the annual incidence of stroke was only 0.23%. When silent atrial fibrillation is detected in patients with recent cryptogenic stroke, anticoagulation is indicated. In patients without stroke, silent atrial fibrillation should lead to further monitoring for clinical atrial fibrillation rather than immediate anticoagulation, as some have advocated.

Pulmonary manifestations of inflammatory bowel disease

Here is a free full text review.

These can take the form of extraintestinal manifestations of the IBD itself or disease associations due to shared risk factors.

They can occur in both ulcerative colitis and Crohn disease.

Direct extraintestinal manifestations include bronchiolitis, obliterative bronchiolitis and bronchiectasis.

Associated diseases include asthma and sarcoidosis.

Readmission penalties five years later

There’s modest evidence that they’ve had an impact.

Review of presentation platforms

Slideshare and much more reviewed here.

RAI vs antithyroid drugs

This is from an evidence summary from AFP:

Radioactive iodine therapy and antithyroid medications produce similar health-related quality-of-life outcomes in patients with Graves disease. Radioactive iodine therapy is an appropriate choice for patients who prefer definitive treatment. Antithyroid medications are appropriate in patients attempting to avoid long-term thyroid hormone therapy and should be considered in those with increased risk of Graves ophthalmopathy, such as smokers.1 (Strength of Recommendation: B, based on inconsistent or limited-quality patient-oriented evidence.)

Stress cardiomyopathy in cancer patients

From a recent study:

Takotsubo syndrome, also known as stress-induced cardiomyopathy (SC), is underrecognized in cancer patients. This study aims to investigate the incidence, natural history, and triggers of SC in cancer patients and its impact on cancer therapy and overall survival. A total of 30 subjects fulfilled the diagnostic criteria for SC at MD Anderson Cancer Center over a 6-year period. Clinical presentation, electrocardiogram, laboratory data, and transthoracic echocardiogram results registered during the acute phase and follow-up were collected. All patients underwent coronary angiography. The most frequent presenting symptoms were chest pain in 63.3% of the patients and shortness of breath/dyspnea on exertion in 27% of the patients. T-wave inversion was a more common electrocardiographic presentation (60%) than ST elevation (13.3%). The median and interquartile range of peak creatine kinase MB fraction, troponin I, and brain natriuretic peptide were creatine kinase MB fraction 8.9, 4.6 to 21.1; troponin I 1.31, 0.7 to 3.3; and brain natriuretic peptide 1,124, 453.5 to 2,369.5. The most common complication of SC was cardiogenic shock requiring inotropic agents (20%). Of the 21 patients who required ongoing cancer treatment, 16 were able to resume chemotherapy, 5 underwent surgery, and 4 received radiation treatment. Median time to resume cancer treatment was 20 days after SC. None of the patients experienced recurrence of SC and other cardiac events. In conclusion, SC should be considered in the differential diagnosis of cancer patients who present with chest pain and ECG findings characteristic of acute coronary syndrome. Most of these patients normalize ejection fraction and may resume cancer therapy early.

Treating portal vein thrombosis in patients with cirrhosis

From a recently published study:

Highlights

•LMWH is effective in about 60% of LC with PVT patients.

•The resolution of thrombus associated with LMWH use is accompanied by improvement in liver function.

•It is more effective for PVT treatment to start LMWH early and to maintain it for a long time.

•LMWH treatment of usual dose and schedule is tolerable for LC patients.

Abstract

Introduction

Portal vein thrombosis (PVT) is a well-known complication in patients with liver cirrhosis (LC). The aim of this study is to investigate the outcomes of cirrhotic patients with PVT treated with low-molecular-weight heparin (LMWH).

Method

Ninety-one LC patients with PVT were treated with dalteparin or enoxaparin for six months. Patients with major bleeding during the last three months, severe thrombocytopenia, or impaired renal function were excluded.

Results

The median age was 62.9 years, and 59 patients had hepatocellular carcinoma. The overall recanalization rate was 61.5%. Patients with a favorable Child-Pugh class and those recently diagnosed as having a thrombus showed significantly better responses. In those who responded to the anticoagulation therapy, the post-treatment bilirubin and platelet levels were improved compared to those in the pre-treatment state. The relapse rate for PVT was 56.6%, and the median time to relapse was 4.0 months. Bleeding was reported in 13 patients (14.4%), and two patients died due to fatal bleeding. A history of variceal bleeding and low serum albumin were risk factors for bleeding.

Conclusion

LMWH therapy for PVT in LC is effective. Advanced LC and a delayed start of anticoagulation treatment decrease the effect of LMWH. Despite its effectiveness, there is a risk of hemorrhage, hence anticoagulation should be carefully considered, especially in patients with advanced LC and a history of variceal bleeding.

Subclinical atrial fibrillation

It was common in the elderly who had risk factors.

The problem with public reporting in health care

Peter Pronovost, a champion of this sort of thing, along with a couple of other authors, cites weaknesses of public reporting, correctly pointing out that when hospitals do it themselves it’s largely just self promotional. Their solution? Beef up the standards by getting outside organizations like CMS and Leapfrog involved. Seriously? They’ve been at this for years with little evidence that patients are really interested, let alone helped.

The CMS sepsis measure

The definition of sepsis is a mess. Sepsis is difficult to reduce to a set of criteria. If you are an experienced clinician you know it when you see it. This is just one reason why the CMS measure is a disaster.

From the linked article:

In changing the clinically significant value of lactate, CMS mandated that clinical practice, hospital protocols, and medical education had to adopt the lower threshold of 2 mmol/L to define severe sepsis and an initial lactate of greater than 4 mmol/L to define septic shock in the absence of robust supportive literature. Physicians are being forced to use government-issued standards of practice and patient care that have not been fully investigated as appropriate and safe. Doctors are no longer permitted to doctor but rather forced to practice cookie cutter one-size-fits-all algorithms with regard to sepsis care. These constraints leave the clinician in the predicament of using best practices versus following mandated guidelines.

We have demonstrated that there are various proposed definitions for sepsis, severe sepsis and septic shock. This is likely due to the fact that unlike myocardial infarction, which has a very precise pathophysiology and organic effect, sepsis is a spectrum of any number of factors. It is not due to one distinct insult but can be caused by a large variety of infectious agents that can infect a variety of anatomic locations. It is not due to one region of the body suffering hypoxia; rather it is due to a dysregulated host response to infection. And that host response is dependent on a variety of uncontrolled factors such as age, sex and comorbidities. It may be impossible to develop definitions that appropriately identify a disease state that is so dependent on multiple variables. Each patient is different and cannot be defined and treated exactly the same way. The CMS definitions are premature and, unlike the various other definitions presented, are mandatory and must be followed by clinicians practicing in the United States.

Why not let doctors be doctors? Because there is so much variation, of course. And as we all know variation is the enemy, right?

SEP-1: another example of performance as a poor surrogate for quality

Here is a report on hospitals’ perceptions of SEP-1:

BACKGROUND: In October 2015, the Centers for Medicare and Medicaid Services (CMS) implemented the Sepsis CMS Core Measure (SEP-1) program, requiring hospitals to report data on the quality of care for their patients with sepsis.

OBJECTIVE: We sought to understand hospital perceptions of and responses to the SEP-1 program.

DESIGN: A thematic content analysis of semistructured interviews with hospital quality officials.

SETTING: A stratified random sample of short-stay, nonfederal, general acute care hospitals in the United States.

SUBJECTS: Hospital quality officers, including nurses and physicians.

INTERVENTION: None.

MEASUREMENTS: We completed 29 interviews before reaching content saturation.

RESULTS: Hospitals reported a variety of actions in response to SEP-1, including new efforts to collect data, improve sepsis diagnosis and treatment, and manage clinicians’ attitudes toward SEP-1. These efforts frequently required dedicated resources to meet the program’s requirements for treatment and documentation, which were thought to be complex and not consistently linked to patient-centered outcomes. Most respondents felt that SEP-1 was likely to improve sepsis outcomes. At the same time, they described specific changes that could improve its effectiveness, including allowing hospitals to focus on the treatment processes most directly associated with improved patient outcomes and better aligning the measure’s sepsis definitions with current clinical definitions.

CONCLUSIONS: Hospitals are responding to the SEP-1 program across a number of domains and in ways that consistently require dedicated resources. Hospitals are interested in further revisions to the program to alleviate the burden of the reporting requirements and help them optimize the effectiveness of their investments in quality-improvement efforts.

Science is the way out of the opioid epidemic

Well, it was pseudoscience that got us into it.

Report of a large experience with severe systemic capillary leak syndrome

From the paper:

Objective: Systemic capillary-leak syndrome is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission. This study was undertaken to describe the clinical pictures and ICU management of severe systemic capillary-leak syndrome episodes.

Design, Setting, Patients: This multicenter retrospective analysis concerned patients entered in the European Clarkson’s disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016.

Measurements and Main Results: Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± SD age, 51 ± 11.4 yr) were included. Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains. ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9–22 g/dL) and 50 g/L (36.5–58.5 g/L). IV immunoglobulins were infused (IV immunoglobulin) during 15 episodes (25.4%). A compartment syndrome developed during 12 episodes (20.3%). Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients’ last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2–140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6–180]; p = 0.02) as independent predictors of hospital mortality.

Conclusions: We described the largest cohort of severe systemic capillary-leak syndrome flares requiring ICU admission. High-volume fluid therapy was independently associated with poorer outcomes. IV immunoglobulin use was not associated with improved survival; hence, their use should be considered prudently and needs further evaluation in future studies.