Scleromyxedema.

Scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. Electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement.

[Porphyria cutanea tarda in a long-term hemodialysis patient]. / Porphyria cutanea tarda bei einem Langzeithämodialysepatienten.

A 61 year old man developed bullous skin lesions typical for porphyria cutanea tarda (PCT) after being on maintenance hemodialysis for 3 years. The porphyrin level in the urine, plasma, erythrocytes and stool supported the diagnosis of porphyria cutanea tarda, a very rare disease in patients with chronic renal failure on hemodialysis. Therapeutic possibilities are reviewed.

Postoperative lower extremity bypass surveillance: beyond ankle arm blood pressures.

Surveillance is essential to the postoperative follow-up of lower extremity bypass grafts. Early, intermediate, and late thrombosis place the patient's limb at risk, so detection of problems before the graft fails is critical. Because contrast angiography is not routinely performed for surveillance, most vascular surgeons rely on history, physical examination, and noninvasive vascular studies (NVS) to assess perfusion to the lower extremity after bypass grafting. These NVS include ankle/brachial waveforms, blood pressures, and indexes before and after exercise. The purpose of this study is to report our findings with duplex color-flow ultrasonography (DCU) to examine lower extremity bypass grafts. According to our protocol, we monitor lower extremity grafts with ankle/brachial Doppler pressures, analogue waveforms, and lower extremity exercise when possible. These NVS are performed by nurses in the vascular laboratory before the patient is discharged from the hospital, at least twice during the first year, and then annually. DCU is also performed at least two times during the first year and then annually. If the study results are abnormal or if the patient has symptoms, testing is usually repeated. When abnormalities persist contrast angiography may be warranted. We have detected anatomic and hemodynamic changes in lower extremity bypasses by use of our protocol. By adding DCU to ankle/brachial blood pressures, we have identified aneurysmal dilation, diffuse atherosclerosis, focal narrowing, arteriovenous fistulas caused by unligated venous branches, retained venous valves, and disease progression proximal or distal to the graft.(ABSTRACT TRUNCATED AT 250 WORDS)

[Chronic hemodialysis-related porphyria/pseudoporphyria]. / Krónikus hemodialízissel kapcsolatos porphyria/pseudoporphyria.

In a considerable proportion of the patients with chronic renal failure, skin changes resembling porphyria cutanea tarda (PCT) develop some months to years after the onset of maintenance hemodialysis. This can be either real PCT, or secondary PCT, or PCT-like bullous dermatosis. In a minor proportion, real PCT can be diagnosed. In such cases, elevated total porphyrin levels with a predominance of uro- (I > III) and heptacarboxyl porphyrins (III > I) can be measured in the plasma (also in the urine, if not anuric), and fecal (perhaps urinary as well) isocoproporphyrin can be detected. The activity of the hepatic uroporphyrinogen decarboxylase (UD) is decreased in every type of PCT; in PCT-II, also that of the erythrocyte UD. In a higher proportion, secondary PCT (pseudo-PCT) develops. In this group, porphyrins are accumulated in the plasma due to the unsatisfactory renal function. Uro and hepta are the dominant fractions here as well, but alteration in the ratio of the uro isomers or the presence of isocoproporphyrin can not be expected. The UD activity is probably normal in every tissue. In 1% to 18% of the cases, PCT-like bullous dermatoses develop, but porphyrins are at normal levels in all compartments. The phototoxic agent here is other than porphyrin (e.g. nalidixic acid, furosemide, tetracycline, etc., or unknown). The authors review the knowledge on chronic hemodialysis-related PCT or the PCT-like bullous dermatoses: development of the above-mentioned conditions, clinical and morphological and biochemical features, difficulties in diagnosis, or the possibilities in therapy.

The role of duplex and color Doppler imaging in the operating room.

For years vascular surgeons have used objective methods in the operating room to assess the technical quality of vascular procedures. Black and white (duplex) and color Doppler imaging are critical to the preoperative work-up and postoperative follow-up of many patients. With advancing technology it is possible to adapt these imaging techniques for use in the operating room as well. Anatomic and hemodynamic results can therefore be ascertained before the patient leaves the operating room. Over the past 31 months, vascular surgeons at St. John's Mercy Medical Center (SJMMC) have regularly used duplex and color Doppler imaging in the operating room after carotid thromboendarterectomy and renal artery bypass. A peripheral vascular laboratory nurse is called to the operating room and brings the imaging equipment and videotape recorder. The imaging transducer is draped with a sterile sheath and positioned directly on the vessel. Longitudinal and transverse views are obtained; the presence of intimal flap or turbulent color flow is noted. Sound spectral analysis and peak systolic velocity are obtained in centimeters per second (cm/sec) at various locations proximal and distal to the arteriotomy and within the graft or endarterectomized segment. Results are classified as normal or abnormal. Abnormal findings are further classified as accepted or warranting reexploration. Of 81 carotid scans, 55 (67%) were normal and 26 (33%) abnormal by imaging. Of 28 abnormal sites, 14 (17%) were reexplored and the abnormality repaired. The other 14 abnormalities were accepted. Duplex and color Doppler imaging have proved helpful to surgeons in assessing technical results before the patient leaves the operating room.(ABSTRACT TRUNCATED AT 250 WORDS)

Teicoplanin in the treatment of skin and soft tissue infections: results of a multicentre study.

The clinical efficacy and safety of teicoplanin was studied in hospitalized patients with skin and soft tissue infections. In an open multicentre study 64 patients were treated with teicoplanin i.v. and/or i.m. Predisposing or complicating factors for infection were present in almost 80% of the patients. Teicoplanin was usually given as an initial loading dose of 400 mg (87.1%), or 800 mg (6.5%) or various doses (6.5%). During the course of the study, the mean daily dose of teicoplanin was 261.3 mg. Sixty of the 62 evaluable patients responded to treatment. 58 gram-positive pathogens were isolated, consisting of Staphylococcus aureus (n = 41), coagulase-negative staphylococci (n = 6) and streptococci (n = 11). Elimination of pathogens was seen in 37/47 of all microbiologically evaluable cases. Persistence, recurrence or reinfection occurred in 7/47, 2/47 and 1/47, respectively. Adverse reactions were reported in only three patients with allergic reaction, local reaction and rise in transaminases in one case each. Therapy failed only in two patients.

An evaluation of a rapid latex test for the diagnosis of Clostridium difficile-associated diarrhea.

We present here the results of an evaluation of a rapid latex test for detection of Cl. stridium difficile-associated in comparison with our standard cytotoxin assay and culture for C. difficile. Some 515 diarrheal stools were examined. C. difficile was cultured from 70 specimens (13.5%); 53 specimens (10.2%) were positive with the latex test, and 50 (9.6%) by cytotoxin assay. The latex test did not differ significantly from the cytotoxin assay in sensitivity or specificity compared to culture results. There was also no significant difference in the specificity of the latex test compared to cytotoxin assay in patients in whom the diagnosis of C. difficile-associated diarrhea was negative. Positive and negative predictive values of the latex test for C. difficile-associated diarrhea were similar to those of cytotoxin assay. The latex test thus appears to be a rapid and practical test for the laboratory diagnosis of C. difficile-associated diarrhea. To optimize specificity and sensitivity its use should be restricted to patients where the diagnosis is strongly suspected and a rapid answer is required. As it does not distinguish between toxigenic virulent C. difficile strains and non-toxigenic avirulent strains, it would seem prudent to confirm positive results subsequently by demonstrating in-vivo or in-vitro cytotoxin production.

Reproducibility of transcutaneous continuous-wave Doppler testing of the carotid arteries.

Hand-held, continuous-wave Doppler probes, coupled with sound spectral analysis, can successfully predict carotid artery stenosis. Changing either the emitting frequency of the probe, the beam/artery angle of the carotid flow velocity (e.g. cardiac output) may alter the recorded frequency shifts. These effects raise questions as to the efficacy of this technique to serially follow carotid atheroma for progressive stenosis. To test the inherent problems with this methodology, a study of reproducibility was conducted. Two Doppler probes (5 MHz and 8 MHz) were compared at the same sitting in 24 patients; 12 were restudied on two subsequent occasions. Peak systolic frequency was 135% higher with the 8 MHz probe; this was lower than the 160% calculated by substitution for emitting frequency in the Doppler formula. The linear correlation coefficient of the two probes was 0.88. In relationship to established laboratory criteria of a greater than 75% area stenosis, no errors were noted with the 5 MHz probe while four errors were noted with the 8 MHz probe. A serial study variation of peak systolic frequency was noted for both probes; these variations did not cross established criteria levels of a severe stenosis when the 5 MHz probe was used, but did with the 8 MHz probe for two carotids. A standard examining probe is recommended. Angle and cardiac output changes do result in peak systolic frequency variation from test to test, but these were not clinically significant with the 5 MHz probe. Thus, significant changes during follow-up testing should provide an index of evolving carotid stenosis.

Pneumatic compression devices for prophylaxis of deep venous thrombosis (DVT).

A pneumatic compression device was applied to 155 patients with a normal Doppler venous examination who underwent a general surgical procedure of at least 1 hr in duration. One hundred fifty-three patients had neither PE nor DVT clinically or by Doppler studies, one patient had a venographically proven DVT, and one patient had a clinical pulmonary embolism verified by lung scanning. Using clinical and Doppler criteria, the device was effective in the prophylaxis of thromboembolic complications.

[Transient acantholytic dermatosis (Grover)]. / Transitorische akantholytische Dermatose (Grover).

Case report about a 79 year old man with transient acantholytic dermatosis (TAD). Review of the literature of Grover's disease.

In-vitro effects of vancomycin, rifampicin, and fusidic acid, alone and in combination, against methicillin-resistant Staphylococcus aureus.

Minimal inhibitory and minimal bactericidal concentrations were determined for eighteen methicillin-resistant Staphylococcus aureus isolates, the majority also resistant to gentamicin, obtained from the blood of bacteraemic patients. Fifty per cent of organisms had a greater than four-fold difference in M.I.C. and M.B.C. for vancomycin, 83% for rifampicin, and 89% for fusidic acid. In-vitro effects of two-drug combinations of vancomycin, rifampicin, and fusidic acid demonstrated neither synergy nor antagonism when measured by a checkerboard dilution technique. The relevance of these findings to choice of therapy of serious infection due to methicillin-gentamicin resistant Staph. aureus is yet to be determined.

Vancomycin therapy for methicillin-resistant Staphylococcus aureus.

Ten patients with bacteremia due to methicillin-resistant Staphylococcus aureus were treated with vancomycin. These patients were compared with matched controls, nine bacteremic patients with methicillin-sensitive S. aureus, and one patient with penicillin-sensitive S. aureus. Controls were treated with a penicillin. There were no significant differences in time to defervescence, metastatic infections, relapse, mortality, need for surgical drainage, or duration of therapy. Fifteen of 19 episodes of serious methicillin-resistant S. aureus infection responded to vancomycin. Severe toxic effects included tinnitus, neutropenia, rash, and possible nephrotoxicity. Tolerance (a minimal bactericidal concentration to minimal inhibitory concentration ratio of at least 32), but not a minimal bactericidal concentration of at least 32 mg/L, correlated with therapeutic failure (respectively, p = 0.04 and p = 0.11, Fisher's exact test). Bacteremic infections due to methicillin-resistant and methicillin-sensitive S. aureus cause similar morbidity and mortality. Vancomycin is effective but potentially toxic therapy for most serious infections due to methicillin-resistant S. aureus. In-vitro tests may not predict therapeutic efficacy.

Cholesterol esterification of mechanically-induced aortic lesions in normolipemic pigs.

Intimal thickenings were produced in the abdominal aortae of normolipemic pigs by longitudinal mechanical injury. In two-week lesions there was increased I-14C-oleic acid incorporation into esterified cholesterol (CE), accompanied by lipid droplet formation. In twelve-week lesions, lipid droplets had largely disappeared, and the incorporation of 1-14C-oleic acid into CE was similar to that of normal aorta. Increased esterification of cholesterol with oleic acid may be a protective mechanism during the early phases of the arterial repair reaction. Regression of the lesions may be associated with the return of cholesterol esterification activity to normal.

Serum immune complexes and disease.

The solid phase Clq radioimmunoassay was used to detect immune complexes in sera from patients with systemic lupus erythematosus (14/25), rheumatoid arthritis (4/5), vasculitis (5/15), infective endocarditis (2/2), acute rheumatic fever (2/3), pre-eclamptic toxaemia (0/14), lung cancer (3/7), glomerulonephritis (26/98) and renal transplant patients (0/5). The best correlation with disease activity was seen in systemic lupus erythematosus and infective endocarditis where serial immune complex determinations were clearly of value in monitoring therapy. The findings in primary glomerulonephritis indicate only a limited usefulness of the assay in that serum immune complexes were detected in a minority (22/73) of patients with glomerular immune deposits. In particular the data do not support a role for Clq fixing immune complexes in the pathogenesis of membranous glomerulonephritis or in pre-eclamptic toxaemia.