Lege Artis Medicinae

[Idiopathic retroperitoneal fibrosis (Morbus Ormond) associated with severe cardiomyopathy in a young man: a case report]

KOVÁCS Erzsébet, ZEHER Margit, SZÁNTÓ János, PÁSZTOR Éva, TÓTH László, PFLIEGLER György

JULY 20, 2012

Lege Artis Medicinae - 2012;22(06-07)

[INTRODUCTION - The cause of idiopathic retroperitoneal fibrosis is unknown, but probably involves a chronic, nonspecific, autoimmune inflammation. This rare disease mostly affects middle-aged or elderly people, it hardly ever occurs in young people. It causes obstruction of the urinary tract, postrenal insufficiency as well as functional disorder of vital organs in the retroperitoneum and its surrounding area. The diagnosis is based on diagnostic imaging and histological examinations. Surgical treatment of the urinary obstruction is only efficient in the long-term if combined with immunomodulatory treatment. CASE REPORT - A young, 34-year-old man who presented with oedema in his legs was diagnosed with retroperitoneal fibrosis. Autoimmune serology was negative. He needed temporary nephrostomy on the right side. Treatment with steroid and colchicine was initially successful. His severe cardiomyopathy improved gradually. No regression occurred with tamoxifen, so we started to administer azathioprine with methylprednisolone. The control abdominal CT showed stagnation of the process. CONCLUSIONS - Because of the rarity of this disease, no randomised controlled trials are available regarding its treatment. The aim of treatment is to eradicate the symptoms, solve the urinary obstruction, maintain renal function and prevent progression of the disease. Improvement may be achieved by corticosteroids, cyclophosphamide, azathioprine, leflunomide, mycophenolate mofetil and tamoxifen. To our knowledge, this case is unique as no similar case with severe cardiomyopathy and idiopathic retroperitoneal fibrosis has been reported in the literature previously.]

COMMENTS

0 comments

Further articles in this publication

Lege Artis Medicinae

[Use of a drug-eluting stent for the treatment of in-stent restenosis of the superior mesenteric artery]

SZABÓ Réka, MÓZES Péter, VARGA István, VAJDA Gusztáv, HARANGI Mariann, MÁTYUS János, BALLA József

[INTRODUCTION - Diagnosis and treating intestinal ischaemia in time presents a great challenge for clinicians. CASE REPORT - In a 60-year-old woman on dialysis who presented with abdominal angina, angiography revealed stenosis of the superior mesenteric artery, which was treated by implantation of a 6×29 mm Genesis stent. After a year, her symptoms reoccurred and angiography revealed restenosis, which was treated with a 7×34 mm Wallstent, while her previous acetylsalicylic acid treatment was supplemented with clopidrogel. Nevertheless, her abdominal angina reoccurred again after a year. During the next intervention - because of the in-stent restenosis - a Taxus Liberte stent was implanted. During the dual antiplatelet therapy, her abdominal complaints did not reoccur, her body weight increased and angiography did not reveal restenosis in the affected artery even after 4 years. CONCLUSIONS - A drug-eluting stent can be a good choice in case of restenosis of the superior mesenteric artery. In a stented patient, it is crucial to use an appropriately controlled, long-term, dual antiplatelet therapy.]

Lege Artis Medicinae

[Suggestive Communication in Medicine ]

BUDA Béla

Lege Artis Medicinae

[Survival in multiple sclerosis: A randomized cohort study 21 years after the start of the pivotal IFN-β-1b trial]

RÓZSA Csilla

Lege Artis Medicinae

[Saint Agatha’s Cult in Northern Italy ]

HORVÁTH Zoltán György

Lege Artis Medicinae

[Network analysis in medicine]

BRYS Zoltán, BUDA Béla, PLUHÁR András

[Network analysis is an increasingly used research method in medicine. Network analysis is a research and interpretation model at the same time. Our article outlines the various applications of network research in medicine and at its interdisciplinary boundaries. ]

All articles in the issue

Related contents

Clinical Oncology

[Therapy of endometrial cancer - an update]

SIPOS Norbert

[Endometrial cancer is the most frequent gynecologic malignancy in developed countries. Recently, there is a signifi cant increase of incidence caused by epidemic obesity. While the etiology of endometrial cancer can be heterogeneous, the effective therapy should be rather personalized. The primary therapy of endometrial cancer is operative. The recommended surgery is total abdominal hysterectomy and bilateral salpingo-oophorectomy. Management of pelvic and paraaortic lymphadenectomy is supported by the latest international recommendations, except cases of low-risk tumors (stage I/A, grade 1 or 2, endometrioid type, diameter of tumor <2 cm). Method of adjuvant therapy, especially in developed stages, is still controversial. Efficacy of postoperative irradiation, chemotherapy and chemoirradiation is under investigation by several ongoing studies. Recurrent endometrial cancer has bad prognosis, the best solution in this case is chemotherapy. In recent years targeted therapy (especially antiangiogenetic drugs, mTORinhibitors and hormontherapy) gave us some promising results. Around 80% of endometrial cancers can be diagnosed at early stages and cured with efficacy. Unfortunately, there is a group of tumors with bad prognosis, low differentiation, or recurrency, which can be a real challenge for clinicians. In this review we discuss the latest and most promising studies and scientifi c results in connection with treatment of high-risk endometrial cancers.]

Hypertension and nephrology

[Erythropoiesis-stimulating agents and oxidative stress in hemodialysis patients]

MONOSTORI Péter, VARGA Ilona, KISS Zoltán, KISS István, HASZON Ibolya, PAPP Ferenc, SÜMEGI Viktória, BERECZKI Csaba, NÉMETH Ilona, TÚRI Sándor

[Oxidative stress plays an important role in the elevation of the cardiovascular risk of patients with chronic kidney insufficiency. The oxidative stress becomes more severe together with the deterioration of the renal function, and the hemodialysis sessions may also induce repetitive oxidative insults. Erythropoesis-stimulating agents (ESAs) may alter the level of oxidative stress via their effects on hematopoiesis, resulting in indirect effects on changes of iron metabolism and the levels of antioxidants. We review the current knowledge about the administration of ESAs as concerns effects on oxidative parameters in hemodialysis patients. We discuss the relationship between the characteristics of the ESA therapy (type, administration frequency and dosage of ESA, length of the therapy, administration withdrawal) and the oxidative stress in view of earlier and recent research.]

Lege Artis Medicinae

[Diagnostic problems of ischemic colitis]

DEMETER Pál, SIKE Róbert, SZÉKELY György, KISS Sándor, SZILVÁS Ágnes

[Ischemic colitis is mainly caused by the impaired circulation of the inferior mesenteric artery. Most frequently it occurs as part of general arteriosclerotic disease or impaired left ventricular function, but it may also occur after abdominal aortic reconstruction. In connection with cocaine users, thrombophylia and vasculitis can also happen among young patients. Colonoscopy plays the most important role in making the diagnosis. In case of transitional ischemia conservative treatment is recommended, but surgery is needed in the progressive gangrenous form or when stricture in the colon developed. Authors describe the case of an 80-year-old man and discuss the etiology, clinical aspects, classical and up-to-date methods of diagnostics and the possibilities of conservative treatment.]

Hypertension and nephrology

[Causes of and therapeutic opportunities in resistant hypertension]

SIMONYI Gábor, GENCSI Kristína

[Hypertension is an independent cardiovascular risk factor and one of the most frequent diseases in Hungary. In the treatment of hypertensive patients usually more than two drugs are needed for the appropriate blood pressure control. Resistant hypertension (RH) is defined when blood pressure remains above target value despite full doses of antihypertensive medications, which consist of at least three different classes of drugs including a diuretic administered in maximal doses. The frequency of RH can reach 20-30% among hypertensive patients. RH increases the cardiovascular risk because of the lack of target blood pressure. RH is multifactorial and it is important to exclude pseudo-resistant hypertension (e.g. poor compliance, white coat effect). In the background of RH we can find lifestyle factors (e.g. obesity, excessive salt intake, alcoholism, etc.) and a variety of drugs (e.g. non-steroids, corticosteroids, sympathomimetics). In the pathogenesis of RH the increased activity of the sympathetic nervous system has an important role. In the treatment of RH we should manage lifestyle factors and it is important to assess the drugs and diseases (e.g. sleep apnea, chronic kidney disease, diabetes mellitus) which may cause increased blood pressure. It is no exact recommendations for the treatment of RH. Therapy often consists of 4-5 various drugs in combination. An important role has the device therapy of RH in recent years (e.g. stimulation of the carotid baroreceptors and renal denervation) as well.]

Lege Artis Medicinae

[NOVEL RESULTS ON SYSTEMIC LUPUS ERYTHEMATOSUS]

KISS Emese, GERGELY Péter, SZEGEDI Gyula

[Systemic lupus erythematosus is an autoimmune disorder. It stands at the focus of medical interest: basic scientists, clinicians and innovative biotechnologists all pay attention to lupus. Authors of this article present the novel scientific results on the etiopathogenesis, clinical and laboratory characteristics of SLE. Furthermore, authors discuss diagnostic problems and the possible therapeutic modalities. One of the most important results is the characterisation and mapping of the susceptibility genes as well as the analysis of their functional features. More and more is known about the relationship between natural and adaptive immunity, about the cooperation of T and B cells. The abnormalities of intracellular biochemical processes and signal transduction pathways have been cleared. The importance of cytokine network and infective agents in the pathogenesis of SLE has largely been investigated recently. With regards to the outcome of the disease, there is growing attention paid on chronic organ damages, such as end-stage renal disease, osteoporosis and atherosclerosis - in connection with the increased life expectancy. Evidence accumulates on the importance of immune-inflammatory processes in the initiation and perpetuation of osteoporosis and atherosclerosis. There is an urgent need for validated biomarkers which can predict the susceptibility, prognosis, clinical manifestations, activity and severity of SLE. To follow and measure the effectiveness of treatment is also required. Although the principals of lupus management have not changed, novel immune modulators, biological therapy and non-medical treatments (e.g. stem-cell transplantation) have become available. Further research and clinical observations may help to find the real place of such therapeutics.]