Sunday, January 21, 2018

Calcium pyrophosphate crystal deposition disease

Below are some key points from a couple of free full text reviews [1] [2].

Terminology has changed and can be confusing

The current official term is calcium pyrophosphate crystal deposition (CPPD). Pseudogout, a term which historically referred to the acute attacks of CPPD, has been replaced by “acute calcium pyrophosphate crystal arthritis.” The plain radiographic finding known as chondrocalcinosis is frequently replaced by the term “cartridge calcification.” CC is not universal in patients with attacks nor is it entirely specific for CPPD.

Crystal identification is important in diagnosis

As opposed to the needle like negatively birefringent crystals of gout, the crystals of CPPD are variable in shape, often rectangular, and if birefringent at all, only weakly positively birefringent.

There are some disease associations

Metabolic disease associations of CPPD include haemochromatosis (18), hyperparathyroidism (19, 20), hypomagnesemia (21) and hypophosphatasia (22). Other diseases such as diabetes mellitus and hypothyroidism do not associate with CPPD once adjusted for age (22, 23). Haemochromatosis is the only metabolic disease associated with CPPD that results in structural arthropathy, and this commonly affects the knees, wrists, hips, MCPJs, and ankles (18, 24).

There are some genetic determinants as well.

Saturday, January 20, 2018

The importance of nutrition in cystic fibrosis

From a recent review:

Recent findings

Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status. For infants and young children, the aim is to achieve the 50th percentile of weight and length for a healthy same-age population up to age 2 years. For older children and adolescents 2–18 years, the target is a BMI of at or above the 50th percentile for healthy children. For CF adults of at least 18 years, the target is a BMI of at or above 22 kg/m 2 for women and at or above 23 kg/m 2 for men. Recently, new drugs are developed with the aim to treat the malfunction of the cystic fibrosis transmembrane conductance regulator gene. This potentiator/corrector therapy improves lung function and nutritional status and decreases the number of infective exacerbations. As survival is improving and the CF population is aging, it is important to focus on micronutrient and macronutrient intake of CF patients in different age and disease stages.


Recent evidence-based nutritional guidelines and improved medical treatment support the nutritional monitoring and interventions in CF patients. Nutritional care should be personalized and provided by a specialized CF dietitian because patients’ care needs may change dramatically during their disease progress.

Friday, January 19, 2018

Insomnia as a cardiovascular disease risk factor

Insomnia is the most prevalent sleep disorder in the United States and has high comorbidity with a number of cardiovascular diseases (CVDs). In the past decade, a number of observational studies have demonstrated an association between insomnia and incident cardiovascular disease (CVD) morbidity and mortality, including hypertension (HTN), coronary heart disease (CHD), and heart failure (HF). Despite some inconsistencies in the literature, likely due to variations in how insomnia is defined and measured, the existing data suggest that insomnia, especially when accompanied by short sleep duration, is associated with increased risk for HTN, CHD and recurrent acute coronary syndrome, and HF. Purported mechanisms likely relate to dysregulation of the hypothalamic-pituitary axis, increased sympathetic nervous system activity, and increased inflammation. This paper reviews the most recent studies of insomnia and CVD and the potential pathophysiological mechanisms underlying this relationship and highlights the need for randomized trials to further elucidate the nature of the relationship between insomnia and CVD.

Thursday, January 18, 2018

Insulin in type 2 diabetes: are we pushing too hard?


There is no significant evidence of long term efficacy of insulin on any clinical outcome in T2D. However, there is a trend to clinically harmful adverse effects such as hypoglycaemia and weight gain. The only benefit could be limited to reducing short term hyperglycemia. This needs to be confirmed with further studies.

Wednesday, January 17, 2018

Inhaled steroid/beta agonist treatments improved oxygenation in patients at risk for ARDS

Objectives: Effective pharmacologic treatments directly targeting lung injury in patients with the acute respiratory distress syndrome are lacking. Early treatment with inhaled corticosteroids and beta agonists may reduce progression to acute respiratory distress syndrome by reducing lung inflammation and enhancing alveolar fluid clearance.

Design: Double-blind, randomized clinical trial ( NCT01783821). The primary outcome was longitudinal change in oxygen saturation divided by the FIO2 (S/F) through day 5. We also analyzed categorical change in S/F by greater than 20%. Other outcomes included need for mechanical ventilation and development of acute respiratory distress syndrome.

Setting: Five academic centers in the United States.

Patients: Adult patients admitted through the emergency department at risk for acute respiratory distress syndrome.

Interventions: Aerosolized budesonide/formoterol versus placebo bid for up to 5 days.

Measurements and Main Results: Sixty-one patients were enrolled from September 3, 2013, to June 9, 2015. Median time from presentation to first study drug was less than 9 hours. More patients in the control group had shock at enrollment (14 vs 3 patients). The longitudinal increase in S/F was greater in the treatment group (p = 0.02) and independent of shock (p = 0.04). Categorical change in S/F improved (p = 0.01) but not after adjustment for shock (p = 0.15). More patients in the placebo group developed acute respiratory distress syndrome (7 vs 0) and required mechanical ventilation (53% vs 21%).

Conclusions: Early treatment with inhaled budesonide/formoterol in patients at risk for acute respiratory distress syndrome is feasible and improved oxygenation as assessed by S/F. These results support further study to test the efficacy of inhaled corticosteroids and beta agonists for prevention of acute respiratory distress syndrome.

Tuesday, January 16, 2018

Reperfused STEMI and intramyocardial hemorrhage


Background Findings from recent studies show that microvascular injury consists of microvascular destruction and intramyocardial hemorrhage (IMH). Patients with ST‐segment elevation myocardial infarction (STEMI) with IMH show poorer prognoses than patients without IMH. Knowledge on predictors for the occurrence of IMH after STEMI is lacking. The current study aimed to investigate the prevalence and extent of IMH in patients with STEMI and its relation with periprocedural and clinical variables.

Methods and Results A multicenter observational cohort study was performed in patients with successfully reperfused STEMI with cardiovascular magnetic resonance examination 5.5±1.8 days after percutaneous coronary intervention. Microvascular injury was visualized using late gadolinium enhancement and T2‐weighted cardiovascular magnetic resonance imaging for microvascular obstruction and IMH, respectively. The median was used as the cutoff value to divide the study population with presence of IMH into mild or extensive IMH. Clinical and periprocedural parameters were studied in relation to occurrence of IMH and extensive IMH, respectively. Of the 410 patients, 54% had IMH. The presence of IMH was independently associated with anterior infarction (odds ratio, 2.96; 95% CI, 1.73–5.06 [P less than 0.001]) and periprocedural glycoprotein IIb/IIIa inhibitor treatment (odds ratio, 2.67; 95% CI, 1.49–4.80 [P less than 0.001]). Extensive IMH was independently associated with anterior infarction (odds ratio, 3.76; 95% CI, 1.91–7.43 [P less than 0.001]). Presence and extent of IMH was associated with larger infarct size, greater extent of microvascular obstruction, larger left ventricular dimensions, and lower left ventricular ejection fraction (all P less than 0.001).

Conclusions Occurrence of IMH was associated with anterior infarction and glycoprotein IIb/IIIa inhibitor treatment. Extensive IMH was associated with anterior infarction. IMH was associated with more severe infarction and worse short‐term left ventricular function in patients with STEMI.

Clinical Perspective
What is New?

This is the first study to link periprocedural additional glycoprotein IIb/IIIa inhibitor treatment to higher occurrence of intramyocardial hemorrhage in patients with reperfused ST‐segment elevation myocardial infarction.

What are the Clinical Implications?

The optimal application of aggressive antithrombotic therapies in patients with ST‐segment elevation myocardial infarction undergoing percutaneous coronary intervention remains to be studied, especially in the era of adequate double antiplatelet preloading.

Anterior infarct location predicted presence and severity of intramyocardial hemorrhage and may prove useful in direct risk stratification.

Monday, January 15, 2018

What the hospitalist needs to know about interstitial lung disease

Here are some key points from a review in the Journal of Hospital Medicine:

What is the classification?

Categories are exposure related (eg hypersensitivity pneumonitis, drugs, occupational), connective tissue disease related, idiopathic and miscellaneous (including sarcoid, Langerhans cell histiocytosis, eosiniphilic pneumonia and vasculitis (including diffuse alveolar hemorrhage).

What labs should be ordered initially on a patient suspected for the first time to have ILD?

According to the review, ANA, RA, anti cyclic citrullinated peptide, CK and aldolase. (Aldolase may be elevated while the CK is normal in some cases of inflammatory myopathy). (I would wonder if synthetase antibody testing should be added to this list). After this round of testing, subsequent tests can be added based on initial results and the ensuing clinical course.

What imaging studies should initially be done?

CXR and HRCT according to the article, which also mentioned that if clinical conditions warrant, instead of just doing a HRCT, get a CT PA gram with simultaneous high resolution images of the lung parenchyma.

What about bronchoscopy?

This is not considered routine and what I get from the review is that the diagnostic confidence derived from HRCT and the anticipation of how the results might change treatment will influence this decision. Circumstances that might favor doing bronchoscopy include suspected acute eosinophilic pneumonia (AEP), suspected acute hypersensitivity pneumonitis, suspected sarcoid and suspected unusual infection.

When should antibiotics be given?

According to the review, onset or exacerbation of ILD can be difficult to distinguish from infection, so always at least consider them. (Given the all too often undifferentiated nature of the presentation in critically ill patients, I suspect the threshold would be low). The review did not comment on the specific type of coverage. However, many such patients are immunosuppressed, have had frequent hospitalizations, or are critically ill and those factors would guide antibiotic choices.

Should corticosteroids be used?

In cases of clinical deterioration or respiratory failure, which is the case with most patients who require hospitalization, yes. The etiology is often unknown, and it must be kept in mind that some etiologies are known to be highly steroid responsive, particularly AEP and COP. Recommendations also support the use of steroids for CTD related ILD, acute HP and drug induced ILD. IPF by comparison is poorly steroid responsive but current guidelines conditionally recommend their use.

Cautions that would apply include the ever present risk of unusual infection and the potential for steroids to increase the risk of renal crisis in patients with systemic sclerosis.

Lung transplantation is a consideration for certain non responding patients

Form the article:

In these cases, lung transplantation may be the only remaining treatment option. This is particularly true for patients presenting with IPF, and it is 1 of the most common indications for lung transplantation. Patients with respiratory failure and ILD should be evaluated early in the hospital course for transplantation or considered for transfer to a transplant center. General contraindications to transplant are age older than 70 years, underweight or elevated BMI (generally higher than 30), malignancy within the last 2 years (with the exception of cutaneous squamous and basal cell tumors), untreatable major organ dysfunction other than the lung, noncurable chronic extrapulmonary infection (chronic active viral hepatitis B, hepatitis C, human immunodeficiency virus), significant chest wall deformity, untreatable psychiatric or psychologic disease, substance addiction within the last 6 months, or lack of dependable social support.4

Another excellent review, free full text, is here. Though a couple of years old it is still relevant and has lots of pearls.