Abnormalities in synovial fluid of patients with septic arthritis detected by gas-liquid chromatography.

Gas liquid chromatography was performed on synovial fluid of 94 patients with exudative arthritis. A relatively constant pattern of peaks was obtained in all synovial fluid samples. Lactic acid was increased in synovial fluid of patients with septic arthritis excluding gonococcal arthritis. In addition 2 constant peaks with retention time of 546 and 848 seconds in the chromatogram usually showed a significant increase in the synovial fluids of patients with septic arthritis (including gonococcal arthritis) but not in those from patients with sterile inflammatory or degenerative arthritis. In 1 patient with pseudomonas arthritis an increase in the peaks was noted with clinical and bacteriological relapse and subsequently there was a gradual return to normal levels during clinical improvement. Increase in synovial fluid lactic acid is useful in the diagnosis of septic arthritis. Identification of the compounds represented by the 2 peaks, which presumptively correspond to n-valeric and n-hexanoic acid, may provide further information on diagnosis, prognosis and pathogenesis of arthritis and may help in the differentiation between gonococcal and nongonococcal arthritis.

Sjogren syndrome presenting as pulmonary pseudolymphoma: report of a case.

A case of pulmonary pseudolymphoma and Sjogren syndrome is presented. Unusually, the lung involvement preceded the salivary disease by more than two years. The initial gammopathy and abnormal serologies are more consistent with uncomplicated Sjogren syndrome, but were present when the pseudolymphoma was solely apparent.

Constrictive pericarditis in systemic lupus erythematosus. Demonstration of immunoglobulins in the pericardium.

A 17-year-old male with systemic lupus erythematosus presented with clinical signs of severe constrictive pericarditis that was confirmed by cardiac catheterization. At surgery, a fibrous pericardium was removed, followed by complete relief of symptoms. Immunopathology of the pericardial tissue revealed deposition of IgG, IgM, and C3. A review of the literature produced only three similar cases. Although rare, constrictive pericarditis, a potentially life threatening complication, may occur in systemic lupus erythematosus presumably with an immunological basis.

Synovial fluid lactic acid. A diagnostic aid in septic arthritis.

Lactic acid concentrations in the synovial fluid of 84 patients with acute monoarticular arthritis were determined by gas liquid chromatography. Lactic acid values in 27 cases of nongonococcal septic arthritis were strikingly higher (mean 1170 mg/100ml) than in 45 cases of inflammatory or degenerative arthritis (mean 34 mg/100 ml), as well as in 12 cases of gonococcal arthritis (mean 27 mg/100 ml). With the proper equipment, determination of lactic acid can be a relatively rapid, reliable procedure. Synovial fluid lactic acid concentrations therefore can be used as a rapid, supplemental diagnostic aid in differentiating nongonococcal septic arthritis from both gonococcal and nonseptic acute arthritis.

Recurrent myoglobinuria due to muscle carnitine palmityl transferase deficiency.

Three new cases of carnitine palmityl transferase deficiency are described. The syndrome consists of recurrent attacks of muscle cramps, weakness, malaise, and myoglobinuria. These attacks are especially likely to occur during prolonged exercise after fasting, eating a high-fat diet, or during cold weather. Occasionally after fasting alone, spontaneous muscle breakdown may occur. One patient studied in detail was excessively slow in producing ketones when he fasted. His mylagias and weakness appeared to be alleviated by beta-hydroxybutyrate. Of eight other patients thought to have idiopathic recurrent myoglobinuria, three were found to have myophosphorylase deficiency, whereas five did not have deficiency of either enzyme. Carnitine palmityl transferase deficiency may be more common than previously supposed, may be in part amenable to dietary therapy, can be easily distinguished from myophosphorylase deficiency, and may provide insight into the metabolism of fatty acids and ketone bodies as well as energy requirements of skeletal muscle.

Behçet's disease: a case with hemoptysis, pseudotumor cerbri, and arteritis.

A case of Behçet's diease with massive hemoptyis is described. The hemoptysis occurred during disease exacerbation with extensive oropharyngeal and laryngeal ulcerations, and responded to high-dose corticosteroid therapy. Other unusual manifestations included a light sensitive dermatitis, pseudotumor cerebri, and right bundle branch block. Also, arteritis and subsequent occlusion of the femoral and popliteal arteries occurred. Although uncommon, pumonary involvement may be life-threatening and should be treated with corticosteroids. Both pulmonary and neurologic involvement tend to occur with active aphthosis and respond to corticosteroids. This case underscores the protean nature of the organ system involvement in Behçet's disease and supports the concept that vasculitis is a primary pathophysiologic event.

Mitral valve prolapse and conduction defects in Ehlers-Danlos syndrome.

A case of type 3 Ehlers-Danlos syndrome with associated mitral valve prolapse, right bundle-branch block, and left anterior fascicular block was studied, including His bundle electrocardiography and echocardiography. Mitral valve prolapse and HV prolongation were reported. The present study suggests that the basic underlying collagen defect in this syndrome is responsible for these findings, and that the association may be more widespread in the syndrome than currently acknowledged. The possibility also is considered that the connective tissue abnormalities of Ehlers-Danlos syndrome involving the heart may be the cause of some cases of idiopathic conduction system disease or valvular insufficiency.

Multiple endocrine adenomatosis type II (Sipple's syndrome) in twins.

Medullary carcinoma of the thyroid, pheochromocytoma and multiple mucosal neuromas (MEA-II), a familial disorder of neuroectodermal tissue, is believed to be inherited in an autosomal dominant pattern. The occurrence of this syndrome in twins has not previously been reported. We have documented the presence of MEA-II in a pair of twins. The high incidence of bilaterality of pheochromocytoma is emphasized, as well as the usefulness of preoperative catecholamine fractionation, and vena cava catheterization sampling.

Wegener's granulomatosis. Long-term followup of patients treated with cyclophosphamide.

Ten patients with Wegener's granulomatosis were treated with cyclophosphamide and followed for periods up to 7 years. In all cases cyclophosphamide induced complete remissions. The mean duration of remission (to date) was 38 months. Six patients have been in remission for a mean of 46 months after cyclophosphamide was discontinued; 2 have been disease-free for 7 years. Of the 10 patients treated, only 1 relapsed and she responded to a second course of cyclophosphamide. These results indicate that cyclophosphamide is the drug of choice in Wegener's granulomatosis. The long-term effectiveness of this drug suggests that it may induce permanent remissions in certain patients with Wegener's granulomatosis.

Pericardial effusion secondary to mucoepidermoid carcinoma of the parotid gland. A report of an unusual case.

This paper reports a unique case of malignant pericardial effusion secondary to metastatic mucoepidermoid carcinoma in a patient who previously had mucoepidermoid carcinoma of the parotid gland and subsequently was found to have a hypernephroma of the kidney. Its distinctive features warranted the diagnosis of mucoepidermoid carcinoma as the cause of the effusion, and excluded the more aggressive hypernephroma. Although pericardectomy was ultimately necessary, there was no evidence of viable malignancy in fluid specimens obtained after an initial course of radiation therapy or in the resected pericardium. This suggests that conservative treatment with local radiation therapy might be successful in future cases of pericardial effusion in this malignancy.

Hydralazine therapy in hypertensive patients with idiopathic systemic lupus erythematosus.

Seven hypertensive patients with idiopathic systemic lupus erythematosus were treated with hydralazine. They received a mean daily dose of 203 mg for a mean duration of 21 months. All were taking prednisone alone or in combination with azathioprine. During therapy with hydralazine, there were no new symptoms nor exacerbation of pre-existing symptoms attributable to systemic lupus. Laboratory parameters, including antinuclear antibody titers and complement levels, either improved or remained stable. The results indicate that hydralazine can be safely used in hypertensive patients with systemic lupus who are receiving concomitant immunosuppressive therapy.