Category Archives: Educational resources

Should new HCV therapies be rationed?

The Annals of Internal Medicine published an interesting article last March investigating the cost effectiveness of new HCV antiviral therapies. The authors concluded that, assuming a cutoff cost of $100,000 per quality-adjusted life-year, sofosbuvir containing regimens are cost effective for most genotypes. With that said,  treatment of all eligible HCV sufferers would result in overwhelming financial burden to the American healthcare system – estimated at $27 billion per year or 10% of prescription drug spending. Therefore, without a massive increase in funding to government and private insurers, rationing of these therapies is currently necessary. This article poses interesting questions regarding cost conscious care, drug expense and the allocation of scarce resources in a time of exponentially growing healthcare expenditure. It’s certainly worth a look.

Cyclosporiasis again?

Our own Dr. Christian Mayorga was interviewed on KERA yesterday about the recent spike in cases of cyclosporiasis in Texas. Following outbreaks in 2013 (133 cases) and 2014 (270 cases), the Texas Department of Health State Services has already received reports of 113 cases as of July 3 with the majority of these occurring in the past two weeks.  Remember to ask your patients about fresh produce consumption and sick contacts!

Cyclospora 101

  • single-celled organism (Cyclospora cayetanensis)
  • typically found in imported fresh produce
  • dominant symptom is watery diarrhea
  • Diagnosis: acid-fast stool stain
  • Treatment: typically supportive but persistent diarrhea treated with bactrim

cyclospora_lifecycle

Figure courtesy of DPDx

Journal Watch June Edition

A few days late, but here is the June Edition of our resident-led publication with micro-reviews of recent medical articles. Stay up to date on recent advances with brief but comprehensive analyses by our own residents! Articles highlighted this month:

  • Idarucizumab for Dabigatran Reversal (NEJM) reviewed by Dr. Haomin Ye
  • Ezetimibe Added to Statin Therapy after Acute Coronary Syndromes (NEJM) reviewed by  Dr.Haomin Ye and Dr. Tri Le
  • EKG Challenge (Wide-complex Tachycardia) by Dr. Steve Dickson
  • Early versus on-demand nasoenteric tube feeding in acute pancreatitis (NEJM) reviewed by Dr. Udayan Shah
  • Antibiotic Therapy vs Appendectomy for Treatment of Uncomplicated Acute Appendicitis: The APPAC Randomized Clinical Trial (JAMA) reviewed by Dr. Udayan Shah
  • Nonalcoholic Fatty Liver Disease: A Systematic Review (JAMA) reviewed by Dr. Brian Davis
  • Prednisolone or Pentoxifylline for Alcoholic Hepatitis (the STOPAH trial) (NEJM) reviewed by Dr. Brian Davis
  • Cannabinoids for Medical Use: A Systematic Review and Meta-analysis (JAMA) reviewed by Dr. Matt Hancock
  • Nivolumab versus Docetaxel in Advanced Squamous-Cell Non–Small-Cell Lung Cancer (NEJM) and Combined Nivolumab and Ipilimumab or Monotherapy in Untreated Melanoma (NEJM) reviewed by Dr. Arjun Gupta
  • Continuous proton pump inhibitor therapy and the associated risk of recurrent Clostridium difficile infection (JAMA Int Med) reviewed by Dr. Fernando Woll
  • Latent Mycobacterium tuberculosis infection (NEJM) reviewed by Dr. Fernando Woll
  • Effect of Remote Ischemic Preconditioning on Kidney Injury Among High-Risk Patients Undergoing Cardiac Surgery. A Randomized Clinical Trial (JAMA) reviewed by Dr. Natalia Rocha
  • Glycated Hemoglobin Level and Mortality in a Nondiabetic Population with CKD (Clin J Am Soc Neph) reviewed by Dr. Natalia Rocha
  • High-Flow Oxygen through Nasal Cannula in Acute Hypoxemic Respiratory Failure (NEJM) reviewed by Dr. Mark Weinreich
  • Adjunct Prednisone Therapy for Patients with Community-Acquired Pneumonia: A multicenter, double-blind, randomized, placebo controlled trial (Lancet) reviewed by Dr. Mark Weinreich
  • Comparative effectiveness of pharmacologic interventions for knee osteoarthritis: a systematic review and network meta-analysis. (Ann Int Med)  reviewed by Dr. James Galloway
  • Evaluating relationships between symptom duration and persistence of rheumatoid arthritis: does a window of opportunity exist? Results of the Leiden Early Arthritis Clinic and ESPOIR cohorts. (Ann Rheum Dis)  reviewed by Dr. James Galloway

Interesting Journal Articles to Read!

Today at morning report we discussed high value care and professionalism/managerial skills. We specifically noted a recent article published in JAMA by oncologist Dr. Ezekiel Emanuel from the University of Pennsylvania and an article in NEJM by Dr. Michael Porter of the Harvard Business School published in 2010. Click on the links below to read the articles!

“Higher income, higher educational attainment, and a cleaner environment are actually more important in determining patients’ well-being than health care services.” Dr. Ezekiel Emanuel

What is Value in Health Care

Enhancing Professionalism Through Management

Common Variable Immunodeficiency

CVID is a rare form of severe antibody deficiency with an incidence of 1 in 25,000 to 1 in 100,000. The mean age of diagnosis is 30 years, although there can be a delay in diagnosis by many years as demonstrated in our patient. The disorder results from failed B-cell differentiation. Thus, plasma cells do not develop and immunoglobulin secretion is impaired. A number of defects of T-cell function and deficits in the memory B-cell pool have been identified, but the underlying cause of this defect remains unknown.

CVID has variable clinical manifestations, the most common being recurrent bacterial infections caused by encapsulated bacteria [1]. Bacterial infections commonly involve the sinuses and respiratory tract leading to sinusitis, otitis media, bronchitis and pneumonia. Chronic sinusitis and bronchiectasis are frequent complications in untreated patients leading to significant morbidity and mortality. Giardiasis is a frequent infection in patients leading to chronic diarrhea. They can also have diarrhea secondary to dysgonic fermenter 3, which is unusual in immunocompetent patients. They are prone to severe herpes simplex, cytomegalovirus infections of the gastrointestinal tract and meningoencephalitis from enteroviral infection. Patients who are not receiving IVIG owing to a delay in diagnosis may develop sepsis or meningitis, which can be fatal. These patients are also at higher risk of developing other autoimmune diseases such as thrombocytopenic purpura, hemolytic anemia and/or neutropenia. In the largest published case series of 248 patients, a 7.7% incidence of non-Hodgkin’s lymphoma (NHL) was reported. Mucosal associated lymphoid tissue lymphomas, an uncommon form of NHL, can occur in these patients in the stomach or bronchial tissue. Other uncommon manifestations include granulomatous lung disease, follicular bronchiolitis, inflammatory bowel disease, sprue-like illness, nodular lymphoid hyperplasia and lymphoid interstitial pneumonia.

CVID should be suspected in any patient with recurrent infections, especially of the upper or lower respiratory tract. IgG, IgA or IgM levels should be less than two standard deviations below the mean for age-adjusted standardized reference. They should also have inadequate antibody response to pneumococcal vaccine and tetanus toxoid or absent isohemagglutinins to confirm the diagnosis.

The mainstay of treatment is IVIG. The target trough level should be 400–500 mg/dl, which is achieved by infusing a dose of 200–400 mg/kg every three or four weeks. The dosage varies from patient to patient, and IgG levels should be checked periodically to attain a target trough level. Autoimmune and granulomatous components of this disease do not respond to treatment with IVIG. There has been recent interest in the use of tumor necrosis factor (TNF) antagonists and anti-CD20 immunomodulators in treating autoimmune and granulomatous diseases based on the dramatic improvement of some clinical manifestations documented in some case reports. However, long-term immunomodulators should be used with extreme caution as these patients are at high risk of developing malignancies.

Journal of Medical Case Reports 2008, 2:117

Wilson’s Disease

General Information:

  • Typically, we ingest more copper in the diet than necessary – most is excreted through the biliary system (some through the urine)
  • Normally, ingested copper is stored in plasma as ceruloplasmin.
  • Patients with Wilson’s disease, have a decreased rate of copper incorporation into ceruloplasmin and a decreased rate of biliary excretion

Clinical Manifestations:

  • Typically considered a disease that presents in young people, but can present at any age
  • The most common presentations are with liver disease or neuropsychiatric disturbances
  • Wilson’s disease can manifest with an impressive spectrum of neurological, behavioral or psychiatric disorders, which may be its first clinical manifestation, appearing simultaneously with hepatic signs, or some years later.
  • System-specific
    • Liver: cirrhosis; increased bilirubin out of proportion to alkaline phosphatase, which may be abnormally low; AST>>ALT
    • Heme: hemolytic anemia, caused by release of copper into the circulation
    • Neurologic: movement disorder (tremor, poor coordination) or rigid dystonia, including bulbar sx
    • Psych: highly variable—from OCD to psychosis to depression
    • Ocular: Kayser-Fleischer rings caused by copper deposition (not required for dx, nor are they specific for Wilson’s—may be found in other types of chronic liver disease)
    • Renal: Fanconi’s syndrome (proximal tubular dysfunction resulting in Type 2 RTA, glucosuria with normal serum glucose, hypouricemia and hypophosphatemia)
    • Also: arthritis, cardiomyopathy, rhabdo, pancreatitis

Diagnostic Tests: 

  • Serum ceruloplasmin: classically, this is low. May be normal in up to 5% of patients; can also be low in other forms of chronic liver disease
  • 24 hour urinary copper: elevated; good confirmatory test
  • Slit-lamp exam for Kayser-Fleischer ring
  • Liver biopsy

Management:

  • Copper chelation: penicillamine, trientene, or zinc
  • Fulminant hepatic failure generally requires transplant
  • Family screening is key for siblings of those affected, as early treatment can greatly improve prognosis