Faculty Bibliography

LEARNING OBJECTIVE #1: Recognize the clinical presentation and physical exam findings of chronic tophaceous gout LEARNING OBJECTIVE #2: Discuss the management and complications of tophaceous gout CASE: A 40 year-old man with no past medical history presented with 8 months of worsening pain and deforming growths in multiple joints. He first noticed intermittent pain and swelling in the heels of both feet 2 years prior to admission, which progressed to constant pain in his feet, knees, hands, and elbows. Starting 8 months ago, distinct nodules formed on the affected joints; these occasionally burst and released a "toothpaste-like" discharge. He increasingly had difficulty walking and using his hands. The patient had not sought any medical care since emigrating from Mexico 8 years ago, due to lack of insurance. On exam, he had diffuse subcutaneous nodules on the feet, ankles, knees, elbows, earlobes, and hands, most concentrated in the joint spaces. They were firm, non-tender, and yellow to white in color. One nodule was draining a chalky white exudate. He had numerous cyst-like lesions on the shaft of the penis, restricting movement of the foreskin. Range of motion was severely impaired in all extremities. He had a uric acid level of 10.4, alkaline phosphatase of 164, and C-reactive protein of 16.5. Rheumatologic serologies were negative. X-rays of the affected joints showed osteopenic bone, with areas of periarticular erosions, joint space narrowing, and subchondral sclerosis with overlying tophi, all consistent with advanced gout. Fluid from the draining tophus revealed large collections of monosodium urate crystals. IMPACT: This case is a reminder that physicians should be prepared to recognize and manage not only acute gout flares, but also advanced presentations of chronic gout. Particularly when working in populations with limited healthcare access, the diagnosis and treatment of gout may be considerably delayed. Perhaps more importantly from an internist's perspective, recognizing and treating the sequelae and comorbidities of gout may reduce morbidity and mortality in these patients. DISCUSSION: In gout patients, the presence of one or more tophi is an indication to begin urate-lowering therapy (ULT), using xanthase-oxidase inhibitors, which decrease production of urate, and uricosuric agents, which increase renal excretion of uric acid. ULT targets a serum urate of <6 mg/dL; in severe cases, a target of <5 mg/dL is often used. If serum urate remains elevated on both agents, the next step is to add pegloticase, a recombinant uricase that converts urate into the renally cleared allantoin. Even with optimal control of hyperuricemia, tophi may persist for years before disappearing. Notably, all gout patients are at increased risk for cardiovascular disease, metabolic syndrome, and renal disease. However, patients with tophi have additional complications of joint and bone destruction, compressive neuropathies, and ulceration and infection of the tophi. The presence of tophi is also an independent predictor of mortality

LEARNING OBJECTIVE #1: Recognize the inciting factors of orthodeoxia with a patent foramen ovale. CASE: An 82 year-old presented with acute onset hypoxia upon extubation after a laparoscopic gastrectomy. She had positional episodes of desaturation to 79% when sitting up, with other vitals signs normal and no dyspnea. Her oxygen saturation was 91% on 2L nasal cannula when supine. Her lungs were clear to auscultation bilaterally. PaO2 was 45mmHg on roomair and increased to only 74 mmHg with 100% O2. Initial transthoracic echocardiogram with bubble study demonstrated a patent foramen ovale (PFO) with normal right atrial and pulmonary artery pressures and function. She had a stable 4.1 cm ascending thoracic aortic aneurysm and multiple, stable hepatic cysts. She was diagnosed with Platypnea-Orthodeoxia Syndrome (POS) due to PFO and underwent successful percutaneous transfemoral closure of the defect. Upon discharge, she was saturating 97% on room air, regardless of position. IMPACT: While PFOs can be seen in up to 29% of the general population, orthodeoxia is much more rare, with less than 200 cases described in the literature. With the elderly population increasing, the incidence of aortic aneurysms and other cardiovascular conditions distorting previously silent PFOs may increase as well, so it is important to maintain a high index of suspicion and perform the gold standard diagnostic test early: echocardiography with bubble study. DISCUSSION: Orthodeoxia, or the arterial deoxygenation that accompanies the positional change from supine to erect, requires two conditions to coexist: an anatomical component (interatrial communication such as a patent foramen ovale [PFO] or atrial septal defect [ASD]) and a functional component that redirects shunted blood flow through the atrial septum. These functional defects can either preferentially direct blood flow through the anatomical defect or cause a transient increase in right atrial pressure, reversing the leftright gradient. The former can be caused by conditions that direct the jet of deoxygenated blood through the interatrial communication by repositioning the atrial septum, such as an ascending aortic aneurysm, intracardiac lipoma, hepatic cyst distorting the right atrium, or aortic valve replacement. Conditions which transiently increase right sided pressures include pulmonary embolism, pulmonary hypertension, pericardial effusion, pneumonectomy, chronic obstructive pulmonary disease and constrictive pericarditis. Measured right sided pressures are typically normal in POS. The pathophysiology in our patient is likely due to her thoracic aortic aneurysm, elongating in the erect position and stretching the interatrial septum and PFO. While a possibility, it is unlikely her hepatic cysts contributed to her presentation as they were small and there has only been one case report of a large liver cyst causing this presentation

BACKGROUND: The laparoscopic adjustable gastric band (LAGB) has been widely used to treat morbid obesity. There is conflicting data on its long-term effect on esophageal function. Our aim was to assess the long-term impact of the LAGB on esophageal motility and pH-metry in patients who had LAGB who had normal and abnormal esophageal function at baseline. METHODS: Consecutive patients referred for bariatric surgery were prospectively enrolled. A detailed medical history was obtained, and esophageal manometric and 24-h pH evaluations were performed in standard fashion preoperatively and 6 and 12 months postoperatively; patients served as their own controls. RESULTS: Twenty-two patients completed manometric evaluation. Ten patients had normal manometric parameters at baseline; at 6 months, mean lower esophageal sphincter (LES) residual pressure increased significantly from baseline (3.9 +/- 2 vs. 8.9 +/- 4 mmHg, p = 0.014). At 12 months, the mean peristaltic wave duration increased from 3.6 +/- 1 at baseline to 6.8 +/- 2 s, p = 0.025 and wave amplitude decreased during the same period (98.7 +/- 22 vs. 52.3 +/- 24, p = 0.013). LES pressure and percent peristalsis did not differ significantly pre- and post-LAGB. Twelve patients had one or more abnormal manometric findings at baseline; at 12 months, LES pressure in these 12 patients decreased significantly (31.1 +/- 10 vs 23.6 +/- 7, p = 0.011) and wave amplitude was significantly reduced (125.9 +/- 117 vs 103 +/- 107, p = 0.039). LES residual pressure did not change significantly pre- and post-LAGB. Twenty-two individuals were evaluated for impact of Lap-Band on esophageal acid exposure. Sixteen of these patients had normal esophageal pH-metry values at baseline and had no significant changes in 12 months in any pH-metry measurement. Six patients had abnormal pH-metry values at baseline. Among these patients, time with pH < 4.0 and Johnson/DeMeester score did not change significantly during follow-up. There was a significant decrease in the number of reflux episodes from baseline to 6 months (159 +/- 48 vs. 81 +/- 61, p = 0.016). CONCLUSIONS: Abnormal manometric findings are frequently encountered post-LAGB. Increases in LES residual pressure and peristaltic wave duration were the most significant changes. LAGB is not associated with an increase in total esophageal acidification time. Further evaluation of the clinical significance of manometric abnormalities is warranted