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Biomedical subjects

I Krajnc

Publications and source records attributed to I Krajnc.

At least 19 recordsLinked to original sources

[Functional capacity and cartilage oligomeric protein (COMP) in serum of patients with maturity-onset polyarthritis].

OBJECTIVE: To compare late onset with adult onset rheumatoid arthritis. METHODS: Fifty-eight patients with late onset rheumatoid arthritis (LORA) were compared to 117 patients with adult onset rheumatoid arthritis (AORA) with respect to clinical and functional parameters. Furthermore, in 104 patients serum cartilage oligomeric matrix protein (COMP) was measured. Results were compared by means of ANOVA and possible influences of age, gender and clinical parameters were evaluated by Spearman rank correlation. RESULTS: Except a different distribution in gender (40% males in the LORA group) and a higher ESR, no differences could be found with respect to clinical parameters. However, a significantly higher HAQ score and significantly higher serum-COMP levels could be shown in the LORA group. HAQ scores correlated not only with disease activity parameters (C-reactive protein, disease activity score) but also with the age. Serum-COMP levels did show a correlation with the age as well, but not with disease activity. CONCLUSION: It is concluded that the higher serum-COMP levels in late onset rheumatoid arthritis could be due to concomitant osteoarthritic processes in larger joints, which are not symptomatic. The age dependence of the HAQ score is only weak, but may be the reason why patients with LORA show a worse functional capacity compared to patients with adult onset rheumatoid arthritis.

Activities of Daily Living↗

[Simple renal cysts and hypertension].

BACKGROUND: Simple renal cysts are frequent findings in mid-aged people, their frequency increases with age. They are often detected by abdominal ultrasound and/or computed tomography during diagnostic procedures. Hypertension is also a frequent disease and its prevalence increases with age as well. The aim of our study was to evaluate the association between simple renal cysts and hypertension in a group of patients with normal renal function. PATIENTS AND METHODS: Ninety-one patients (58 female and 33 male; mean age 50.5 +/- 16.9 years) were included in the study, all with normal renal function (serum creatinine <100 micromol/l). The patients were examined with a real-time ultrasound device with a 2-4 MHz convex transducer. Kidney size, presence, number and diameter of cysts were examined and the presence of hypertension was determined. The presence of hypertension was defined by the administration of antihypertensive agents, systolic blood pressure greater than 140 mmHg and/or diastolic blood pressure greater than 90 mmHg. RESULTS: Simple renal cysts were found in 19 (20.88%) patients. Only cortical cysts were found in 11 (12.09%) patients, only parapelvinic cysts were found in 4 (4.39%) patients. Four (4.39%) patients had cortical and parapelvinic cysts. Cortical cysts in both kidneys were found in five (5.49%) patients, parapelvinic cysts in both kidneys were found in three (3.29%) patients. Bilateral renal cysts were present in 10 (10.99%) patients, six (60%) of them had hypertension. We found no difference in the presence of simple renal cysts according to sex. Hypertension was detected in 30 (32.97%) patients. Hypertension was significantly more frequent in patients with simple renal cysts (P < 0.041) than in with patients without cysts. Simple renal cysts were also significantly associated with age (P < 0.01). With multiple regression analysis we found a significant association with age only (P < 0.0001), but not with hypertension (P = 0.394). CONCLUSIONS: Simple renal cysts are frequent findings and their incidence increases with age. We found an association between simple renal cysts and hypertension. However, with multiple regression analysis only an association between simple renal cysts and age could be detected.

Age Factors↗

Primary breast cancer of the vulva: a case report and review of the literature.

Since 1872, 40 cases of ectopic mammary gland tissue in the vulva have been reported in the literature. Out of these, 12 had a primary cancer in the ectopic breast tissue. Seven metastases of an orthotopic breast cancer have been found in this location. We are presenting the 20th case of cancerous breast tissue in the vulva whom we classified as the 13th case of primary cancer based on clinical and histopathological criteria of primary and metastatic malignant disease. Because of the advanced age of the patient, wide local excision followed by adjuvant hormonal therapy was opted for. Nineteen months after surgery, there is no evidence of recurrent disease. Due to the rarity of this entity, its management presents therapeutic dilemmas, and variable treatment strategies are being found in the literature. In our opinion, the same basic principles used for treatment of cancers of the orthotopic breast should be applied in ectopic breast carcinoma.

Aged↗

Wegener's granulomatosis--a diagnostic challenge.

Wegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomatous lesions in the upper and lower respiratory tracts, glomerulonephritis and vasculitis involving other organs. Limited forms have been described in which some features of the disease may be absent. Four patients with this disease are being reported with special emphasis on differences in presentation, the ensuing diagnostic problems, and individual outcome. In three, the disease began as a limited form with upper respiratory tract and eye involvement, while in one patient, onset was systemic including affection of the lower respiratory tract. The mean delay from first symptoms to diagnosis was 20 months--much longer for the three limited forms than for the one with systemic onset, in whom the condition was recognized after 2 months following initial misdiagnosis of respiratory and urinary tract infections. One patient developed endocarditis and required aortic valve replacement. Immunofluorescence revealed antineutrophil cytoplasmic antibodies in all, three showing a cytoplasmic pattern and antibodies to proteinase 3, and the fourth a perinuclear pattern and antibodies to myeloperoxidase. Upper respiratory tract biopsies were not specific. Kidney biopsies were performed in all the patients and were crucial for definitive diagnosis and treatment of the disease, which was successful in three patients.

Adult↗

Pelvic actinomycosis.

Pelvic actinomycosis is a rare chronic infection caused by bacteria of the family Actinomycetaceae. Prolonged use of an intrauterine contraceptive device (IUD) is a well known risk factor. We report six patients with pelvic actinomycosis, all of whom had an IUD inserted for over six years. Diagnostic problems necessitated a laparotomy in all patients. The pathohistological diagnosis was based on the characteristic microscopic image and specific staining. The patients were treated with penicillin and amoxycillin for several months.

Actinomycosis↗

[Diagnosis and therapy of common bile duct stones in a patient with a history of hypersensitivity reaction to radiologic contrast media --a case report].

Common bile duct (CBD) stones may occur in patients who had been previously cholecystectomized because of gallbladder stones. CBD stones occur in 15%-20% of patients with symptoms secondary to cholecystolithiasis. After cholecystectomy they occur in 1%-5% of patients, either in the form of retained or recurrent choledocholithiasis. Endoscopic ultrasonography (EUS) is a novel and excellent diagnostic screening procedure for common bile duct stones. Numerous reports confirm an equal success rate for endoscopic ultrasound and endoscopic retrograde cholangiopancreatography (ERCP). However, unanimity of opinion with respect to the best diagnostic procedure for the detection of CBD stones does not exist to date. We report a 74-year-old female patient with CBD stones, who was shown to have a severe allergy to X-ray contrast medium at a previous examination. In view of the pre-existing contraindication for ERCP, we diagnosed the stones by EUS. The majority of patients would require ERCP for endoscopic treatment, but our patient rejected the administration of contrast medium as well as conventional surgical treatment. After introducing a guide wire into the bile duct to assure its position, endoscopic sphincterotomy (EST) without contrast imaging was performed successfully. The CBD stones were extracted with a basket. Based on our results we believe that EUS ranks as an important procedure in the diagnosis of CBD stones, and that successful endoscopic treatment can be performed without previous ERCP. We do realize that EST without ERCP in unsuitable as a routine procedure, but may be used successfully in specific cases such as that of the patient reported here.

Aged↗

Dermatomyositis. Diagnosis and evaluation of dermatomyositis, polymyositis, and inclusion-body myositis.

In diseases of an unknown etiology, such as the idiopathic inflammatory myopathies, we must tackle first of all the question of classification and the degree of disease activity before we can institute treatment. The majority of idiopathic inflammatory myopathies are diagnosed clinically and confirmed by biopsy. The presently applicable methods of diagnosis and evaluation of idiopathic inflammatory myopathy have certain limitations, and hence it is necessary to apply new methods of rating the disease activities. Magnetic resonance imaging (MRI) and 99m-technetium muscle-scanning are the latest noninvasive methods for evaluation of disease activities with myositis. Future laboratory methods to determine the numerous myositis-specific autoantibodies will probably enable identification of subsets of these diseases.

Autoantibodies↗

The significance of histologic analysis of skin lesions in porphyria cutanea tarda. Light microscopy, electron microscopy, immunohistochemical and immunofluorescence analysis.

Porphyria cutanea tarda (PCT) is one of several entities in the differential diagnosis of scleroderma. We report a 62-year-old man with PCT diagnosed since two decades. Clinical data and the results of light microscopy, immunohistochemistry, immunofluorescence and electron microscopy of skin biopsies are presented. The biopsy revealed sclerosis of the dermis mainly due to increased collagens I and III, and accumulation of collagen IV, which had caused the vessel wall to thicken. Immunofluorescence for detection of immune reactants was negative. It was concluded that the histomorphology of PCT of long duration may be similar to that of scleroderma. Nevertheless, in addition to clinical findings, detailed skin biopsy studies including contemporary techniques can contribute to the differentiation of these diseases.

Adult↗

The clinical significance of antinuclear antibodies in connective tissue disease.

Antinuclear antibodies (ANA) are often present in connective tissue diseases. In 279 non-selected patients with connective tissue disease, inflammatory and degenerative joint disease, in some patients with chronic infectious diseases and malignancies and in the presence of some unclear pathologic conditions in patients whose serum reacted positively to ANA, we analyzed the type of immunofluorescence and the presence of extractable antinuclear antibodies (ENA). In systemic lupus erythematosus, the prevailing immunofluorescence is type H (homogenous) (60.6%), anti-Ro/SS-A appears in 24.2%, anti-Sm and anti-RNP in 12.1%. In Sjögren's syndrome, type S prevails (47.6%), anti- Ro/SS-A and anti-La/SS-B are present in 52.4%, only anti-Ro/SS-A in 28.6%. In systemic sclerosis, the prevailing immunofluorescence is type S (37.5%), in 75% a positive anti-Scl-70 antibody is present. In mixed connective tissue disease, anti-RNP appears in 85.7%. In dermatopolymyositis, the anti-Jo-1 antibody is present in 33.3%. In undifferentiated connective tissue disease, type S immunofluorescence appears in 70%. In rheumatoid arthritis the prevailing immunofluorescence is type H (homogenous) (46.4%) and type S (speckled) (41.0%), while the presence of ENA is rare (anti-Ro/SS-A in 4.6%). In spondylarthritis, type S immunofluorescence appears most often (62.5%). Patients with chronic infectious disease, malignancies, undefined conditions and degenerative joint disease present with various types of immunofluorescence; the presence of ENA is extremely rare in these patients. The results of this study underline the significance of ANA and, particularly ENA, in the diagnosis of connective tissue disease. These antibodies however, can also be identified in various infectious and malignant diseases as well as in inflammatory and degenerative joint diseases.

Adult↗

[Porphyria cutanea tarda with severe clinical complications. A 20-year follow-up].

A patient with porphyria cutanea tarda developed distinctive sclerodermoid changes with extensive dystrophic calcification and ulcerations in the sclerotic areas. In addition to these skin complications, over the course of 20 years other internal problems also appeared, such as diabetes mellitus, fatty cirrhosis and ocular inflammation. These severe clinical complications were related to omission of treatment and the alcohol abuse.

Adult↗

[Significance of histopathologic analysis of skin lesions in scleromyxedema. Light microscopy, electron microscopy, immunohistochemistry and immunofluorescence microscopy].

Scleromyxedema (SM) may be considered as a possible disease entity in the differential diagnosis of scleroderma. Clinical data and the results of light, immunohistochemical, immunofluorescence and electron microscopic study of skin biopsies taken from a 53-year old patient with SM are reported. In the patient with SM in which abnormal serum paraprotein was not identified, the skin biopsy showed mucinous material in the dermis and proliferation of fibroblasts accompanied by mild dermal sclerosis. Immunofluorescence showed scanty granular IgG along the epidermal basement membrane and IgG and C1q focally along the connective tissue fibres in the dermis of clinically involved skin. In addition to clinical findings, detailed skin biopsy studies including contemporary techniques can contribute to the diagnosis of the disease.

Biopsy↗

[Arndt-Gottron scleromyxedema. Summary of 2 years treatment].

The case of a 53-year-old patient with a 2-year history of Arndt-Gottron scleromyxoedema is reported. Typical lichenoid papules were found clinically, along with infiltration of acid mucopolysaccharides into the skin, which induces extensive elephantine-like thickening and hardening of the skin. The presence of abnormal paraproteins is to be rated as the criterion for the diagnosis of scleromyxoedema. This type of paraproteinaemia is described as a monoclonal gammopathy of undetermined significance. We found no paraproteinaemia in our patient; bone marrow histology and urinalysis were normal. The oetiopathogenesis of the disease is unknown. Pathohisto-logical examination corroborates the presumption that fibro-blasts are producing acid mucopolysaccharides and that collagen fibres play an important role in inducing the infiltration of mucinous material into the skin. We treated our patient for 6 months with cyclosporin A and observed regression of the extensively thickened skin; it appeared smooth, especially over the region of the deltoid muscles and shoulders. The flexibility of the finger and mandible joints showed an improvement, but the neck was still stiff and painful. The patient complained of "burning" pain in the neck and back. Serum factors able to stimulate fibroblasts in scleromyxoedema are the cytokines IL-1 and TNF-alpha. Cyclosporin A inhibits both these substances and also inhibits the activation of helper T-cells, which express lymphokines.

Cyclosporine↗

Malignant acanthosis nigricans and tripe palms in a patient with endometrial adenocarcinoma--a case report and review of literature.

BACKGROUND: In the world literature, uterine carcinoma associated with paraneoplastic malignant acanthosis nigricans and tripe palms has been mentioned in two review articles. Endometrial adenocarcinoma associated with malignant acanthosis nigricans without tripe palms has been cited in three case reports, and endometrial adenocarcinoma associated with tripe palms without malignant acanthosis nigricans has been cited in one case report. We present the case of a patient with endometrial adenocarcinoma associated with acanthosis nigricans and tripe palms which we have been following for the past 7 years. METHODS: Our 54-year-old patient had been operated for moderately differentiated endometrial adenocarcinoma. She had also received postoperative radiotherapy. RESULTS: Three years after surgery, tripe palms and acanthosis nigricans with generalized pruritus occurred. After treatment with etretinate, the skin symptoms were somewhat mitigated while the pruritus persisted. Six years after gynecologic treatment, a solitary inguinal metastatic lymph node of endometrial carcinoma was detected. Following lymphadenectomy there was additional but not complete mitigation of skin symptoms including pruritus. CONCLUSIONS: In clinical practice the association of malignant acanthosis nigricans and tripe palms with endometrial adenocarcinoma is found extremely rarely. Although the survival time of adenocarcinoma patients with malignant acanthosis nigricans is short, our patient has been treated and followed for more than 7 years.

Acanthosis Nigricans↗

[Autoantibodies and autoimmune rheumatologic diseases].

The paper describes the significance of various autoantibodies in autoimmune rheumatologic diseases. A greater number of laboratory tests also means a greater possibility of diagnosing these diseases. In some cases the antibodies correlate with disease activity, some antibodies serve as prognostic indicators, others allow the following of disease activity or success of treatment. In any case, the role of numerous autoantibodies in the pathogenesis of rheumatologic inflammatory diseases is still unclarified und further investigation will be necessary to obtain the answer to this important question.

Autoantibodies↗

[Acquired generalized cutis laxa with paraproteinemia (IgG lambda). Immunofluorescence study, clinical and histologic findings with review of the literature].

A 49-year-old patient developed acquired generalized cutis laxa, manifest as involvement of the elastic fibres of the dermis and the internal organs, leading to pulmonary emphysema, bilateral inguinal hernia and two oesophageal diverticula. The patient's serum levels of IgG lambda paraprotein were elevated. Direct immunofluorescence examination revealed intensive linear deposits of IgG lambda light chains, an early component of complement C1q and discrete C3 deposits. The deposits were found along the preserved elastic fibres in the dermis, especially in the papillary dermis, at the dermoepidermal junction around the sweat and sebaceous glands and in the walls of small vessels. Dermal alterations of this kind have not been described previously.

Basement Membrane↗

[Anti-Jo-1-positive polymyositis with interstitial alveolitis and pericardial effusion. Overview of 3 years treatment].

We describe the case of a 53-year-old woman who developed polymyositis with extramuscular complications: interstitional lung fibrosis, pericarditis and a pericardial effusion, polyarthritis, Raynaud's syndrome, carpal tunnel syndrome, sclerodactylia and positive anti-Jo-1-antibodies. We treated her for 3 years. Pericarditis and pericardial effusion with a fibrosing lung alveolitis were the first clinical manifestations of positive Jo-1-syndrome. We stress the patient's serious cardiac disease accompanied by pericardial effusion, which has seldom been described in specialist articles. Steroid treatment induced remission of the disease, but the Jo-1-antibodies did not disappear from the patient's serum.

Antibodies, Antinuclear↗

[Palindromic rheumatism--progression to erosive rheumatoid arthritis--presentation of 3 years treatment of a patient].

The 3-year course of treatment of a 35-year old male patient presenting with recurrent afebrile episodes of acute mono- or polyarthritis, periarthritis and at times paraarthritis of the left ankle and knee joint as well as the PIP and MCP joints of the hands is presented. The episodes lasted from a few hours up to several days, leaving no residual changes in the joints. After initial observation and treatment, palindromic rheumatism was diagnosed. During the second year of treatment, seroconversion from the rheumatoid factor negative to the rheumatoid factor positive arthritis form was established. In the third year of treatment, erosions of cartilage and bone in the left knee joint were confirmed radiologically. On the basis of the clinical picture, laboratory findings and radiologic changes in the joints, the diagnosis of erosive seropositive rheumatoid arthritis was made. The author stresses that the diagnosis "palindromic rheumatism" could be established sooner and more often, particularly if, regarding the typical clinical course and history, the possibility of this disease were taken into consideration at all.

Adult↗