1/18. Aneurysms and hypermobility in a 45-year-old woman.EDS type IV presents a diagnostic and therapeutic challenge to the primary care physician, surgeon, and rheumatologist. In patients for whom the diagnosis is known, avoidance of trauma, contact sports, or strenuous activities, joint bracing and protection, and counseling on contraception are helpful preventive strategies. In patients presenting with vascular, gastrointestinal, or obstetric complications, a history of hypermobility and skin fragility (easy bruising, abnormal scarring, poor wound healing) should lead to a suspicion of this diagnosis, and to caution in the use of certain invasive diagnostic and operative techniques. Efforts should be made to examine family members. Most importantly, when caring for such patients, the acute onset of headaches, chest pain, shortness of breath, and abdominal pain should arouse suspicion of a potentially catastrophic vascular or visceral event.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/18. microscopic polyangiitis that presented liver dysfunction prior to noted renal manifestations.In microscopic polyangiitis (MPA), renal manifestations are very common as first symptoms. Here, we report a case of MPA which presented liver dysfunction prior to noted renal manifestations. A 58-year-old woman was hospitalized because of a fever for 8 weeks. A laboratory examination revealed marked elevation of alkaline phosphatase and gamma-glutamyl transpeptidase, while blood urea nitrogen and creatinine levels remained normal. Although apparent renal dysfunction developed in this case soon after hospitalization, physicians should be aware of the variety of clinical manifestations in MPA. Moreover, antineutrophil cytoplasmic autoantibodies were found to be helpful for diagnosing MPA.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/18. Cases from the medical grand rounds of the Osler Medical Service at Johns Hopkins University.A 77-year-old man was in good health until he complained of fatigue 3 weeks before presentation. Two weeks before admission, he developed gradually worsening shortness of breath. One week before admission, he developed a cough that initially was nonproductive but later was associated with hemoptysis.His past medical history was remarkable for a history of colon cancer (Dukes' stage III), for which he underwent a hemicolectomy and treatment with adjuvant chemotherapy in 1993. He had a myocardial infarction in 1986 and underwent coronary artery bypass surgery in 1999. He also had a history of hypertension, type 2 diabetes, and gout. He smoked in the past but had stopped more than 30 years ago.He was initially evaluated by his primary care physician, who noted that he complained of diaphoresis but denied fevers, chills, or contact with others who were ill. His physical examination was remarkable for bilateral crackles that were more pronounced on the right. A chest radiograph demonstrated bilateral pulmonary infiltrates (Figure 1). He was treated with amoxicillin. The next day, however, his physician noted that his dyspnea had worsened and that his oxygen saturation on room air was poor. He was therefore admitted for further evaluation.The amoxicillin was discontinued, and he was treated with levofloxacin, followed by ceftriaxone and azithromycin as his pulmonary status continued to deteriorate. He received intravenous diuretic agents, which failed to improve his respiratory status.During the initial phase of hospitalization, he was anemic, with a hematocrit of 21.3%. His serum creatinine level, which had been 1.0 mg/dL in 1999, was now 2.5 mg/dL. urinalysis was remarkable for the presence of numerous red blood cells. His oxygen requirement increased, and he eventually required a 100% nonrebreather mask. A computed tomographic scan of the chest demonstrated prominent alveolar opacities throughout the right upper, middle, and lower lobes, with similar opacities in the left upper and left lower lobes (Figure 2). An echocardiogram showed an ejection fraction of 50%, as well as mild mitral regurgitation. Serologies were remarkable for an antinuclear antibody titer of 1:320 and a P-antineutrophil cytoplasmic antibody (P-ANCA) titer of greater than 1:320. C-ANCA was negative. Anti-glomerular basement membrane and anti-human immunodeficiency virus antibodies were undetectable.- - - - - - - - - - ranking = 2keywords = physician (Clic here for more details about this article) |
4/18. Localized versus systemic vasculitis: diagnosis and management.Localized vasculitis restricted to a specific anatomic site or organ is often histologically indistinguishable from more severe, systemic forms of vasculitis. By definition, localized vasculitis involves blood vessels within a confined vascular distribution or single organ without clinical evidence of generalized inflammation. Important factors that determine treatment and prognosis, in what appears initially to be a localized process, include histopathologic type, organ site, and the presence of systemic inflammatory markers and symptoms. The major issue is whether single organ vasculitis is actually an isolated form of the disease in which case surgical excision is curative, or whether the single organ involvement is simply a precursor of more threatening systemic vasculitis. The Birmingham Vasculitis Activity Score is a valuable tool to identify those patients with concurrent systemic involvement. The physician's recognition of a localized versus systemic vasculitic process is important in terms of making the correct diagnosis, prescribing treatment, and arranging appropriate clinical follow-up.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/18. The pulmonary physician in critical care * illustrative case 3: pulmonary vasculitis.The case history of a patient admitted to the ICU with severe hypoxic respiratory failure later diagnosed as Wegener's granulomatosis is presented. The diagnosis and management of patients with suspected pulmonary vasculitis is discussed.- - - - - - - - - - ranking = 4keywords = physician (Clic here for more details about this article) |
6/18. Asymptomatic vasculitis of the uterine cervix in presence of cervical intraepithelial neoplasia grade III.CASE REPORT: A 34-year-old woman was diagnosed to have a high-grade cervical intraepithelial neoplasia and was treated by large loop excision of the transformation zone. histology of the excised cone confirmed the diagnosis but also showed evidence of vasculitis of medium-sized vessels of the cervix. The woman was referred to a physician to rule out underlying systemic disease. Extensive laboratory and clinical screening was negative. DISCUSSION: The clinical significance and management of asymptomatic isolated vasculitis of the uterine cervix are discussed.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/18. Laryngeal involvement in systemic lupus erythematosus.Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
8/18. Treatable complications of cancer patients referred to an in-patient hospice.This paper illustrates the importance of accurate diagnoses and treatments of complications in terminally ill cancer patients. The paper reports on five hospice in-patients who completely recovered from life-threatening complications; three of them had been incorrectly labeled as "imminently dying" by the referring physicians. The paper concludes that it would be beneficial for patients to receive examinations and a trial of medical treatment in their continuing treatment settings.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
9/18. Echogenic vasculature in the basal ganglia of neonates: a sonographic sign of vasculopathy.The vessels that supply the basal ganglia and thalami are not normally conspicuous on the cranial sonograms of neonates. Twelve neonates with abnormally echogenic or "bright" vessels on cranial sonograms were studied. Records of these 12 patients were reviewed and were correlated with the neuropathologic findings available in four. The clinical diagnoses were cytomegalovirus infection (five patients), rubella (two patients), congenital syphilis (one patient), and trisomy 13 syndrome (three patients). No diagnosis was made in one infant. At neuropathologic examination, perforating medium-sized arteries to the basal ganglia and thalami had thickened hypercellular walls, with deposits of amorphous basophilic material in three infants. Results of computed tomography and radiography of brain sections were normal in these areas. Sonography is helpful in detecting early noncalcific inflammation and mineralization in vasculitis. Although nonspecific, these findings should alert the physician to the possibility of congenital infection or chromosomal abnormality.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
10/18. plasma concentrations of complement-activation complexes correlate with disease activity in patients diagnosed with isolated central nervous system vasculitis.Isolated central nervous system (CNS) vasculitis is rare medium- sized vessel disease limited to intracerebral vessels. The two most common symptoms of this inflammatory disorder observed at entry to a hospital are headaches and mild memory deficits. Further progression of this disease may result in focal neurologic alterations and seizures. Currently, the most common laboratory abnormality noted is an elevated erythrocyte sedimentation rate. The complement (C) system is known to play a role in many inflammatory processes; it may also be involved in CNS vasculitis. In this longitudinal study of patients with CNS vasculitis, we detected C activation by highly sensitive and specific assays that are capable of identifying breakdown products formed after C activation: C3a des arg, C4a des arg, C5a des arg, C1rC1s-C1-inhibitor complex, and terminal C complex (C5b-9). We present two cases of documented CNS vasculitis in which serial measurements of these C-activation products correlate with disease activity. Our results indicate that a temporal association exists between C activation and the clinical presentation of CNS vasculitis. We conclude that physicians should monitor C-activation by-products in plasma when they attempt to follow the clinical course of CNS vasculitis.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
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