Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities

We report six cases that were positive for anti-centromere antibodies, with ulcer and gangrene in the extremities but mild or no skin thickening. The patients were five women and one man, and the mean age at onset of gangrene was 56 yr. Raynaud's phenomenon was found in five patients and calcinosis cutis in two patients. Three patients did not satisfy the criteria for systemic sclerosis and CREST syndrome in this study. Ulcer and gangrene occurred in the fingers in three patients, and in the fingers and toes in two patients. The gangrene was refractory to treatment, and amputation of fingers or toes was inevitable in five patients. Regardless of cutaneous lesion, the presence of anti-centromere antibodies may cause the same pathological presentation of vascular damage as seen in systemic sclerosis.     

CT angiogram sign: incidence and significance in lobar consolidations evaluated by contrast-enhanced CT

BACKGROUND: Neuropathological data are very scarce in systemic sclerosis and fail to demonstrate primary changes in the brains of such patients. CASE DESCRIPTIONS: A 41-year-old woman with CREST syndrome developed signs of dementia after an episode of severe dehydration and died two months later of septic shock. A 63-year-old woman with CREST syndrome and a history of two unexplained transient ischemic attacks had had balance disorders since age 62. She died of severe pulmonary hypertension. In both cases, the autopsy showed extensive wall calcification of small arteries and arterioles in the brain, primarily in the basal ganglia, and also in the frontal lobes and the cerebellar area in the second case. No known cause of cerebrovascular calcification was found in either patient. CONCLUSION: The neuropathological findings in these two patients suggest that systemic sclerosis may induce primary vascular changes in the brain, of which calcification may be a marker.     

Resurfacing of atrophic facial acne scars with a high-energy, pulsed carbon dioxide laser

BACKGROUND: Treatment of atrophic acne scars has been limited to the use of such traditional treatments as dermabrasion and chemical peels for many years. Recently, the addition of high-energy, pulsed carbon dioxide (CO2) lasers to the treatment armentarium has created renewed enthusiasm for cutaneous resurfacing due to their ability to create specific thermal injury with limited side effects. OBJECTIVE: To determine the effectiveness of a high-energy, pulsed CO2 laser in eliminating atrophic facial scars and to observe for side effects. METHODS: Fifty patients with skin phototypes I-V and moderate to severe atrophic facial acne scars were included in the study. Each patient received one high-energy, pulsed CO2 laser treatment using identical laser parameters by the same experienced laser surgeon. Baseline and 1-, 4-, 8-, 12-, and 24-week postoperative photographs and clinical assessments were obtained in all patients. Textural analysis of skin before and after laser irradiation were obtained in 10 patients to confirm clinical impressions. Clinical evaluations were conducted independently by two blinded assessors. RESULTS: There was an 81.4% average clinical improvement observed in acne scars following laser treatment. Skin texture measurements of laser-irradiated scars were comparable to those obtained in normal adjacent skin. Side effects were limited to transient hyperpigmentation lasting an average of 3 months in 36% of patients. Prolonged erythema (2 months average) was usual and considered to be a normal healing response. No hypertrophic scarring was observed following laser treatment. CONCLUSION: High-energy, pulsed CO2 laser treatment can safely and effectively improve or even eliminate atrophic facial scars and provides many benefits over traditional treatment methods.     

Echocardiographic diagnosis of single coronary artery in double-outlet right ventricle

A 90-year-old woman was admitted to our hospital in December 1993 because of dyspnea on exertion and malaise. She had been well until October 1993, when she first noticed Raynaud's phenomenon, skin tightening, digital ulceration and scarring of her hands. On physical examination, generalized edema was found, along with acrosclerosis with contracture, especially in the fingers, wrists, and elbows. Inspiratory crackles were noted. A roentgenogram of the chest and an echocardiogram revealed pulmonary fibrosis, pulmonary congestion, and massive pleural and pericardial effusions. The pleural effusion was a transudate. Progressive systemic sclerosis was diagnosed, and furosemide and isosorbide were given. The edema and pulmonary congestion resolved, but the pleural and pericardial effusions did not. Prednisolone was given, which reduced the pleural effusion but not the pericardial effusin. The pleura and the pericardium are not usually involved in progressive systemic sclerosis, and this disease rarely occurs in patients over 70 years old. To the best of our knowledge, this was one of the oldest patients with progressive systemic sclerosis. The combination of massive pleural and pericardial effusions, and the advanced age of onset make the present case unusual.     

Factors predictive of stroke outcome in a rehabilitation setting

We describe the first case of hypothenar hammer syndrome followed by accelerated systemic sclerosis (SSc). A sawmill worker with repeated wrist trauma presented with bilateral digital ischemia and fingertip ulcerations. Hypothenar hammer syndrome involving both hands was diagnosed based on classic angiographic studies showing occlusion of the ulnar arteries at the level of the hamate and occlusions of multiple digital arteries. He also had minimal digital swelling, telangiectatic lesions on the fingers, and a positive FANA test. Resected ulnar arteries showed clearcut changes compatible with both microtrauma and SSc. After surgical repair of both ulnar arteries, he rapidly developed severe SSc with significant cardiopulmonary involvement.     

Symptomatic spinal calcinosis in systemic sclerosis (scleroderma)

Two patients with diffuse cutaneous systemic sclerosis and spinal calcification, involving the lumbar spine in one and the cervical spine in the other, are described. Computed tomography-guided aspiration of the calcific masses was performed, and material aspirated from one patient was shown to be apatite, Ca5(PO4)3OH. One patient showed improvement following lumbar laminotomy, hemilaminectomy, and diskectomy.     

Diode laser cyclophotocoagulation of secondary glaucoma caused by anterior, necrotizing scleritis

PATIENT: A 60-year-old female patient presented with recurrent anterior, necrotizing scleritis with inflammation and a newly developed secondary glaucoma in the right eye. Anterior uveitis occurred some years before. Severe scleral thinning was circumferentially present and focal scleral ectasia was found. Physical examination revealed no systemic association of scleritis. Immunosuppressive therapy with metotrexate was initiated and control of scleritis achieved. Intraocular pressure elevation persisted and was refractory to glaucoma medication. Diurnal pressure curve showed IOP-values of 40 mm Hg despite the use of systemic carbonic anhydrase inhibitors. Visual acuity was 20/50 in the right and 20/25 in the left eye. METHOD: Diode laser cyclophotocoagulation (Oculight SLx 810 nm, Iris Medical Instruments Inc. California, USA) was performed under general anaesthesia using reduced parameters for application (12 laser spots, 1 second, 1.25 W). No complications occurred during and after laser application. Postoperatively, intraocular pressure was within normal range between 14 and 18 mm Hg. No reactivation of scleritis or uveitis was seen. CONCLUSION: In our experience, diode laser cyclophotocoagulation is effective and safe in treating secondary glaucoma associated with anterior, necrotizing scleritis with inflammation and uveitis using reduced parameters for application.     

Oligoclonal banding of IgG in CSF, blood-brain barrier function, and MRI findings in patients with sarcoidosis, systemic lupus erythematosus, and Beh?et's disease involving the nervous system

A retrospective study of CSF and serum analysis from a total of 43 patients with sarcoidosis, 20 with systemic lupus erythematosus, and 12 with Beh?et's disease with neurological involvement found local synthesis of oligoclonal IgG using isoelectric focusing and immunoblotting in 51%, 25%, and 8% respectively at some stage in their disease. Blood-brain barrier breakdown, when assessed with an albumin ratio found 47% of patients with sarcoidosis, 30% of those with systemic lupus erythematosus, and 42% of patients with Beh?et's disease exhibiting abnormal barrier function at some time. Serial CSF analysis showed that clinical relapses were associated with worsening barrier function and in some patients the development of local oligoclonal IgG synthesis; conversely steroid treatment led to a statistically significant improvement in barrier function, and in two patients a loss of oligoclonal IgG bands. A higher proportion of patients had MRI abnormalities than oligoclonal IgG or blood-brain barrier breakdown, MRI being abnormal in 16 of 19 patients with sarcoidosis, three of four patients with systemic lupus erythematosus, and seven of nine patients with Beh?et's disease, although this may have been due to temporal factors. In the differential diagnosis of chronic neurological disorders, locally synthesised oligoclonal IgG cannot distinguish between diseases, but the loss of bands seen in two patients contrasts with what is seen in multiple sclerosis, and thus may be a useful diagnostic clue.     

Deconjugation ability of bacteria isolated from the jejunal fluid of patients with progressive systemic sclerosis and its gastric pH

BACKGROUND/AIMS: Our goal was to demonstrate the role of bacteria in altered bile acid metabolism, which overgrow in the upper small intestine of patients with progressive systemic sclerosis. We identified the bacterial species, isolated from the jejunal fluid obtained from patients with progressive systemic sclerosis, who had previously shown an increase in 14CO2, specific activity on breath test, and normal controls. After which, we investigated the deconjugation ability of the isolated bacteria and the relationship between 14CO2, specific activity and gastric pH. METHODOLOGY: Bile acid breath tests were performed on 12 patients, and 19 normal controls using 5 microCi of oral glycine-1-(14)C-labeled glycocholate. Jejunal fluid was aspirated through a double lumen-tube with a rubber cover on the tip. Deconjugation ability was examined by thin-layer chromatography using conjugated bile acids in ox gall. RESULTS: The following species were identified in jejunal fluid samples obtained from patients: Bacteroides vulgatus, Eubacterium lentum, enterococcus, Lactobacillus bifidus, Escherichia (E) coli, Aerobacter (A) aerogenes. Except for E. coli and A. aerogenes, these species were capable of hydrolyzing conjugated bile acids in ox gall. The administration of chloramphenicol (1 g orally per day for 14 days in divided doses) significantly reduced the 14CO2, specific activity (p<0.05) in the patients with progressive systemic sclerosis. On the other hand, nineteen healthy control subjects demonstrated no increase in CO2 excretion, and 16 of the 19 had no bacteria isolated from jejunal fluid. The remaining healthy man showed an overgrowth of E. coli and Pseudomonas (P) aeruginosa, but the E. coli and P. aeruginosa did not have the ability of deconjugation. CO2 specific activity of expired breath samples in the patients with progressive systemic sclerosis was correlated with gastric pH (n=12, r=0.588, p<0.05). CONCLUSIONS: Our results demonstrated that some of the bacterial species that overgrow in the upper small intestine of patients with progressive systemic sclerosis can deconjugate bile acids, and that a shift to neutral pH in gastric juice, may promote the bacterial overgrowth related to their impaired peristaltic activity.     

The effects of warfarin on calcinosis in a patient with systemic sclerosis

We describe the use of low doses of warfarin to treat calcinosis in a patient with systemic sclerosis or CREST syndrome. Our patient had Raynaud's phenomenon, skin sclerosis of the neck and the distal surface of the elbows, and pitting ulcers and scarification of the fingers as well as cutaneous calcinosis. After beginning warfarin, no calcium containing substance was discharged from the fingertip ulcers. There was no tendency to bleed and activated partial thromboplastin time and prothrombin time were normal. Sequential radiographs of the hands showed that calcinosis had improved. Since there seem to be few adverse effects, the use of warfarin in patients with calcinosis warrants further study.     

IgA class serum antibodies against three different Klebsiella serotypes in ankylosing spondylitis

BACKGROUND: Over the past decade, the 585-nm pulsed dye laser (PDL) has been used successfully to treat a variety of cutaneous vascular lesions as well as hypertrophic scars. Laser scar revision has been revolutionized by the recent development of high-energy, pulsed carbon dioxide (CO2) laser systems. These new CO2 lasers allow controlled vaporization of thin layers of skin while minimizing damage to surrounding dermal structures. OBJECTIVE: To determine the effect of a high-energy, pulsed CO2 laser alone and in combination with a 585-nm PDL on nonerythematous hypertrophic scars. METHODS: Twenty patients with nonerythematous hypertrophic scars were treated with a high-energy, pulsed CO2 laser. One-half of each scar was additionally treated with the 585-nm PDL laser. Sequential clinical and photographic analyses were performed independently by two blinded assessors. In addition, erythema reflectance spectrometry measurements were obtained from the scars before and at regular postoperative intervals. RESULTS: Global assessment scores and erythema spectrometry measurements were significantly improved after laser treatment. Combination CO2 and PDL laser treatment resulted in more significant improvement than CO2 laser irradiation alone. CONCLUSION: Concomitant use of the high-energy, pulsed CO2 and PDL laser systems was superior to CO2 laser vaporization alone for revision of nonerythematous hypertrophic scars. Once again, the vascular specificity of the 585-nm PDL has been linked to improvement in hypertrophic scar tissue.     

Sj?gren's syndrome

Sj?gren's syndrome is a multisystemic connective tissue disease. The syndrome is associated with other major connective tissue disorders, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others, Reticulo-endothelial malignances have repeatedly been reported in patients with Sj?gren's syndrome. A clinical study of 20 patients with Sj?gren's syndrome was performed in the Department of otolaryngology, Rikshospitalet, Oslo, Norway. A considerable complexity of symptoms was seen. One patient with malignant lymphoma and one with a thyroid carcinoma were encountered.     

Low energy fluence CO2 laser treatment of lymphangiectasia

Lymphangiectasia is an uncommonly reported complication of lymphatic insufficiency. These dilatations of lymphatic vessels may be symptomatic, necessitating treatment. While CO2 laser ablation has been used with success in the treatment of lymphangiectasia, it is infrequently reported and previous laser protocols have relied on high irradiances. The successful use of low fluence CO2 laser in the treatment of multiple lymphangiectases on the lower limb of a middle-aged Caucasian woman with unilateral chronic lymphoedema is described.     

Widespread expression of MRP8 and MRP14 in human cerebral malaria by microglial cells

Recently, laser treatment of the prostate has been added to the urologist's armamentarium for the treatment of bladder outlet obstruction secondary to benign prostatic hyperplasia (BPH). Until now, limited data on long-term outcome are available notwithstanding the fact that such information is crucial in determining the ultimate role of laser prostatectomy in the treatment of BPH. We now have 3-year data of a comparative study using the Urolase and Ultraline fiber in Nd:YAG sidefiring laser prostatectomy. The study was performed to compare laser prostatectomy using a pure coagulation (Urolase fiber) and a combination of a coagulation and vaporization (Ultraline fiber). In a period of 15 months, 93 men were randomized for laser treatment with the Ultraline fiber (N = 44) or the Urolase fiber (N = 49). Symptom scores, maximal uroflow, postvoiding residual volume, and sexual history were noted over a 3-year period. Adverse events and retreatments were also recorded. The mean postoperative catheterization time was 18 days, without significant difference between the two groups. After 3 years, we demonstrated a durable improvement in maximal flow rate, from 7.8 to 13.9 mL/sec in the Urolase group and from 7.9 to 13.6 mL/sec in the Ultraline group. In both groups, however, a considerable decrease in the maximal flow rate was noted after 3 years compared with 3 months after treatment, from 18.7 to 13.9 mL/sec in the Urolase group and from 20.0 to 13.6 mL/sec in the Ultraline group. The symptom scores showed marked and lasting improvement. The postvoiding residual urine volume became very low in the early postoperative period but did significantly increase after 3 years; nevertheless, it was still only 50% of the preoperative value. Although after 3 years, the maximal uroflow rate was still significantly improved compared with baseline, a considerable decrease was noted when compared with the early postoperative value. The same considerable and lasting improvement in subjective outcome (symptom scores) was seen in both groups. Although the Ultraline fiber also causes vaporization of prostatic tissue, no differences could be noted in the clinical outcome obtained with the two fibers.     

Presence of genomes of phage PBV-1 in the mycelia of the fungus Penicillium brevi-compactum

Progressive systemic sclerosis may be associated with focal myocardial fibrosis. Electrocardiographic abnormalities including conduction block are common in progressive systemic sclerosis but whether they are due to direct destruction of the specialized conduction tissue of the heart is uncertain. The conduction systems of 35 patients with progressive systemic sclerosis were studied. Of these 35 patients, 17 (50 per cent) had myocardial fibrosis of the type seen in progressive systemic sclerosis. In 10 of the 17, it was severe. Sinus node fibrosis was present in 13 patients and was nearly as frequent in those with as in those without the progressive systemic sclerosis myocardial lesion. Overlying pericarditis may have contributed to the fibrotic changes within the sinoatrial nodes in seven of the 13 patients. The atrioventricular node and main His bundles were normal. However, fibrotic changes were found in the proximal bundle systems in six patients. In three of the six, severe myocardial progressive systemic sclerosis was present, two had focal fibrous atrophy of the left bundle, and one had complete interruption of the right bundle. In only the latter patient was this reflected in the electrocardiogram which showed a right bundle branch block. Three patients without progressive systemic sclerosis myocardial lesions also had fibrous atrophy of a portion of the proximal left bundle branch, and in one the electrocardiogram showed an isolated left anterior hemiblock. Thus, morphologic abnormalities within the conduction system in our patients are difficult to attribute to progressive systemic sclerosis per se. Furthermore, although conduction abnormalities were more frequent in patients with myocardial disease, specific conduction system disease was not the cause in most patients. As has been noted in ischemic heart disease, the conduction system appears to be relatively spared from the myocardial changes of progressive systemic sclerosis, and the high incidence of conduction disturbances in this condition may be a consequence, rather, of damage to working myocardium.     

Dependency scoring in a critical care area: a direct nursing assessment method

The urinary symptoms characteristic of benign prostatic hyperplasia (BPH) can have a considerable impact on patients' quality of life. Symptom score assessment is now used in BPH, although a number of different instruments are available. Controlled clinical trials with selective alpha 1 adrenoceptor antagonists such as doxazosin, prazosin and terazosin have shown these agents to be effective in the treatment of BPH. The effects of doxazosin on the severity and bothersomeness of BPH symptoms were determined in three multicentre, double-blind, placebo-controlled clinical studies, involving a total of 609 normotensive and hypertensive patients. Doxazosin was initiated at a dosage of 0.5 or 1 mg once daily, with a final dose range of up to 12 mg once daily. The duration of active treatment was 12 to 14 weeks. Significant improvements were seen in symptom severity and bothersomeness with doxazosin compared with placebo, in both patient populations. The onset of symptomatic improvement was rapid, occurring within two weeks of treatment initiation, and efficacy was sustained throughout the treatment period. A long-term, open label extension of these studies has demonstrated sustained efficacy during 48 months of follow-up. Since symptom relief is the primary goal of therapy in BPH, and since doxazosin's effects are rapid in onset and sustained in duration, it appears that doxazosin is an effective agent for the treatment of symptomatic BPH in both normotensive and hypertensive men.     

Morphological equivalents of lymphocytic-epithelial interactions in kidney and liver regeneration

AIM: The trial of efficacy of 6-month therapy with madecassol (tablets, ointment, powder) of patients with systemic and focal scleroderma (SS and FS). MATERIALS AND METHODS: 54 patients (49 females and 5 males) aged 15 to 70 years with scleroderma running from 3 months to 15 years entered the study. 30 patients had typical SS, 24 patients had FS. Tablets were given to 18 patients, ointment was applied in 42 patients, powder in 3 and tablets + ointment in 9 patients. Madecassol 10 mg tablets were taken 3 times a day by patients with SS and advanced FS. The ointment was preferred in ulcers and scars on fingers and toes in SS and vascular trophic lesions in FS. In active focal scleroderma the ointment was applied to the skin lesions. The ointment was used 2 times a day (in the morning and evening) for 1-6 months. Madecassol powder was employed rarely, primarily of anal and vulval lesions. RESULTS: 6-month oral course (30 mg/day) in 12 SS patients brought about a decrease of indurative lesions, hyperpigmentation (8), vascular trophic disorders (6) and improvement of general condition (5). Subjective response was good in 10 patients and corresponded to absence of progression. In progressive disease and diffuse skin lesions the drug was ineffective. The best response was obtained in local application of madecassol ointment on digital ulcers in SS. CONCLUSION: Madecassol is effective and well tolerated and therefore recommended for oral and local use in combined treatment of SS adn FS. Indications for per os utelization are: chronic or subchronic SS with limited skin involvement, advanced and/or prone to progression FS in which combined administration of the tablets and ointment is proposed.     

Increased restenosis in diabetes mellitus after coronary interventions is due to exaggerated intimal hyperplasia. A serial intravascular ultrasound study

Hepatic hydrothorax is a rare complication of portal hypertension. Conservative therapy may be successful but refractory hepatic hydrothorax is not uncommon. Management of refractory hydrothorax is usually ineffective and can result in a worsened clinical status. Transjugular intrahepatic portosystemic shunts (TIPS) lower portal pressure and have been used in the treatment of refractory ascites. The aim of this study was to determine the efficacy of TIPS in the treatment of symptomatic refractory hepatic hydrothorax. A TIPS was placed in 24 consecutive cirrhotic patients with symptomatic refractory hepatic hydrothorax. Five patients (20.8%) were Child's/Pugh class B and 19 (79.2%) were class C. All had undergone multiple thoracenteses and were hypoalbuminemic. Mean follow-up was 7.2 months (range, 0.25-49 months). Fourteen (58.3%) of 24 patients had complete relief of symptoms after shunt placement and did not require further thoracentesis. Five (20.8%) additional patients required fewer thoracenteses. Five (20.8%) patients developed worsening liver function and died within 45 days. In eight (66.7%) of 12 patients with > or = 60 days of follow-up, the serum albumin increased by a mean of 1.2 g/dL (range, 0.1-2.2 g/dL). The Child's-Pugh score improved in 7 (58.3%) of these 12 patients and two patients improved from class C to class A. These two patients no longer require liver transplantation. This study shows that TIPS can be effective in the management of symptomatic, refractory hepatic hydrothorax. Clinical and laboratory improvement may be seen and liver transplantation may become unnecessary.     

Assessment of the psychotropic activity of analogs of cholecystokinin tetrapeptide fragment]

BACKGROUND: Both dermabrasion and high-energy pulsed carbon dioxide (CO2) laser resurfacing can improve the appearance of surgical scars. Although the results of these two procedures have been compared using historical data, a prospective evaluation has never been performed in humans. OBJECTIVE: To prospectively compare the clinical effects of dermabrasion and high-energy pulsed CO2 laser resurfacing in the revision of surgical scars. METHODS: Facial surgical scars in four patients were prospectively revised using a split scar model. One half of the scar was dermabraded and the other half was resurfaced with the high-energy pulsed CO2 laser. Comparisons of the two treatment modalities were performed through clinical assessment, photographic evaluation, and textural analysis of the scars. RESULTS: The high-energy pulsed CO2 laser-resurfaced halves of the scar were bloodless with less postoperative crusting in comparison with the dermabraded halves. Reepithelialization time and degree and duration of postoperative erythema were similar for both treatment halves. Photographic evaluation and textural analysis showed comparable improvement in the clinical appearance and surface texture of the scars with both treatment modalities. CONCLUSIONS: Both the high-energy pulsed CO2 laser and dermabrasion can achieve comparable clinical improvement in the revision of surgical scars. The high-energy pulsed CO2 laser offers the advantage of a bloodless field and a more precise method of tissue ablation. Postoperative erythema, however, is an expected finding with both treatment modalities.