RESUMO
A 46-year-old woman was type 1 diabetes diagnosed at the age of 9 who had previously been on an insulin pump. Other co-morbidities included CKD IV, HTN, and hypothyroidism. She presented with hyperglycemia of 400 mg/dl and fluid retention. Her GFR had decreased to 13. Her physical exam was notable for respiratory distress and anasarca. She failed to respond to aggressive IV diuresis and urgent hemodialysis was initiated. The patient had been lost to outpatient follow-up for a year. She had been co-managed by an endocrinologist and a primary care physician but had stopped going to her endocrinologist over a year ago due to inability to afford the co-pays. She subsequently lost her insurance and had to pay out of pocket for her insulin; at this point, she decided to stop seeing her PCP and began to ration her insulin. Due to social stigma, she did not mention her financial issues to her healthcare providers. After identifying these challenges, we decided to start her on a more affordable regimen of NPH insulin. Through social work assistance, we were able to obtain a charity hemodialysis chair and discharge her home. She applied to Medicaid. Healthcare expenditure with regard to diabetes rose to $327 billion from $245 billion in 2012. The price of insulin has continued to increase even after the drug's patent has expired due to the combination of FDA requirements, a monopoly in the insulin market, and the lack of federal price controls and Pharmacy Benefits Managers. The high out of pocket costs for insulin has led to many instances of insulin rationing among both uninsured and insured. This led to death in some cases as well as poorly controlled diabetes with increased complications and mortality as in our case. We present a case report and narrative review on insulin affordability.
RESUMO
Neurofibromatosis-1 or von Recklinghausen's disease is an autosomal dominant disorder. Cafe au lait macules are generally the initial presenting feature of the disease, and there can be varying degrees of involvement of the skeletal, neurological, and pulmonary organ systems as the disease progresses. The existence of neurofibromatosis-associated diffuse lung disease (NF-DLD) as a separate entity has always been questioned and, is often attributed to cigarette smoking, rather than a manifestation of NF-1. A 59-year-old male with a history of neurofibromatosis presented with shortness of breath and ataxia for 10 days. Exam findings were pertinent for tachycardia, tachypnea, and diffuse cutaneous neurofibromas. Workup showed white blood count (WBC) of 15.9 k/ul, electrocardiogram with biatrial enlargement and right axis deviation, and a chest X-ray showed left lower lobe infiltrate concerning for pneumonia. Computed tomography (CT) scan of the chest revealed left basilar consolidation with surrounding ground-glass opacities and innumerable bilateral thin-walled cysts. The latter finding raised suspicion for NF-DLD. The patient was evaluated by pulmonology with recommendations to continue treatment for pneumonia and follow-up with high-resolution CT of the chest and complete pulmonary function testing in 12 weeks. He was discharged in a stable condition after five days of hospitalization. NF-DLD is a pulmonary manifestation of NF-1 with non-specific respiratory symptoms and a characteristic pattern of upper lobe cystic and basilar interstitial lung disease. It usually presents in the 4th or 5th decade, earlier in tobacco users, but a few pediatric cases have also been reported. The presentation of NF-DLD can be variable, ranging from dyspnea, chest pain, chronic cough, hemoptysis, or an incidental finding on CT. Multiple complications, including spontaneous pneumothorax due to the rupture of subpleural blebs, pulmonary hypertension, and chronic respiratory failure, are associated with NF-DLD. NF-DLD can be prevented by smoking cessation but, there are no known modalities for treatment; however, complications can be managed symptomatically. This case illustrates the diagnostic challenge that NF-DLD represents to clinicians. The patient's CT from two years ago showed emphysematous changes along with scattered fibrosis and scarring, and no cystic changes were mentioned, unlike his latest CT, which showed innumerable cysts. This patient had a history of smoking, which likely put him at a higher risk for the development of cysts. However, he quit smoking 10 years prior, which suggests that his lung changes are not secondary to cigarette smoke, further confirming our suspicion for NF-DLD. Although routine screening is not implemented due to the rarity of the disease, NF-DLD should not be ruled out in patients with NF-1 presenting with pulmonary symptoms until a high-resolution computed tomography (HRCT) is obtained.
RESUMO
Historically, elevations in procalcitonin (PCT) have been implicated in medullary thyroid cancer and neuroendocrine tumors. More recently, the trending of PCT has been suggested as a monitor of infection to assess the presence, clearance and eradication of infection, especially in cancer patients. Its increase serves as a marker of bacterial infections. During homeostasis it is produced by most tissues in the body at an extremely low level (<.01 ng/mL) and is often induced by bacterial endotoxins. In cancer patients additional factors influence these levels. Metastasis in particular is linked with relatively higher PCT levels. We present a case of an afebrile patient with undifferentiated pleomorphic sarcoma who underwent 25 cycles of radiation therapy and presented one month later with elevated procalcitonin, lactic acid, and leukocytosis. All infectious work up was negative. Findings were incidental after a hospital visit for dehydration. Leukocytosis and lactic acidosis resolved after four days into the hospitalization. Procalcitonin, however, remained elevated over four months in the range of 2-5 ng/mL. The patient has no findings of metastatic disease. To our knowledge, there has never been a report in the literature describing a prolonged elevation of procalcitonin in a patient with a non-metastatic sarcoma without any signs of infection or any other underlying cause. The elevation of PCT has been noted in patients who suffered burns, trauma, minor and major surgery, and cardiogenic shock in addition to infection. Increases have served as signs of worsening patient outcomes and elevated rate of complications. Trending PCT can help in appropriated antibiotic use as it has been shown to decrease antibiotic use by 2.4 days. PCT trends have been increasing in value making idiopathic elevations found in combination undifferentiated pleomorphic sarcoma an important addition to the literature.
