Patient with antibody-negative relapse of Goodpasture syndrome.

Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of hemodialysis treatment. It is important to consider that in patients with anti-GBM disease antibody-negative relapses are possible.

[The multilocular occlusive form of fibromuscular hyperplasia]. / Die multilokulär okkludierende Form der fibromuskulären Hyperplasie.

The rare case of a 19-year-old man with fibromuscular hyperplasia (FMH) affecting several arteries with tubular stenosis and occlusions is presented. Involved were both internal carotid arteries, the right renal artery, celiac axis and the superior mesenteric artery. The disease pattern of FMH is discussed regarding frequency, vascular morphology and differential diagnosis.

Innovations in breast cancer imaging: PET for diagnosis and follow-up.

Could positron emission tomography (PET), which identifies the higher metabolism of malignant tumors, become the noninvasive test needed to assess the thousands of falsely positive mammograms? Could serial scans be used to evaluate response to chemotherapy? This team examines the data on current and potential uses of PET.

Axillary lymph node metastases: screening with [F-18]2-deoxy-2-fluoro-D-glucose (FDG) PET.

PURPOSE: To evaluate [fluorine-18]2-deoxy-2-fluoro-D-glucose (FDG) positron emission tomography (PET) of the axilla as a screening test for detecting regional spread of breast cancer. MATERIALS AND METHODS: High-dose FDG PET of the axilla was successfully performed in 50 patients (age range, 36-79 years) with breast cancer before 52 axillary lymph node dissections. Two additional patients had scans that were uninterpretable because of intense myocardial activity that obscured the axilla. RESULTS: The sensitivity and negative predictive value were both 95%, the specificity was 66%, and the overall accuracy was 77%. The only false-negative PET scan was obtained in the largest patient, who had a low-quality scan. CONCLUSION: Patients with negative PET scans had such a low risk for axillary lymph node metastases that axillary dissection was not warranted. Patients with positive PET scans required dissection to confirm the presence and determine the number of positive lymph nodes. Had this algorithm been used to select patients for dissection, approximately $120,000 in charges ($2,300 per patient) would have been saved and 22 patients would have been spared the morbidity of axillary lymph node dissection. Within this study population, PET scans of the axilla were interpreted with sufficient sensitivity for PET to serve as a cost-effective screening test for axillary lymph node metastases.