Successful use of adalimumab for treating fistulizing Crohn's disease with pyoderma gangrenosum: Two birds with one stone.

Crohn's disease (CD) is a chronic relapsing and remitting autoinflammatory disorder of the gastrointestinal tract that has many intestinal and extraintestinal complications. The purpose of treatment is long-term remission, reduction of complications, and improvement of patients' quality of life. In many cases, this can be quite challenging and it is necessary to have a well thought out management strategy. We present the case of a 38-year-old woman with fistulizing CD that manifested as diffuse abdominal pain and bloody diarrhea accompanied by arthralgia. In addition, there were ulcerative lesions surrounded by cutaneous inflammation and erythema on her extremities, indicative of pyoderma gangrenosum. The patient was treated with high doses of parenteral methylprednisolone without any improvement and was started on adalimumab. A positive response to adalimumab therapy was observed: after 2 mo of therapy, the ulcerative skin lesion healed completely and the enterogastric fistula was closed after 5 mo adalimumab treatment. Adalimumab might be a suitable initial as well as maintenance therapy in patients with complicated CD.

[Successful renal transplantation following lung transplantation: a survey of the first Hungarian case]. / Sikeres vesetranszplantáció tüdoátültetés után: az elso magyarországi eset ismertetése.

The authors review the case of their young patient, who underwent a lung transplantation in Vienna because of an end stage idiopathic pulmonary fibrosis. During the prolonged postoperative phase renal failure full of complications developed, which necessitated haemodialysis. As the pulmonological rehabilitation was complete, but the regular dialyses considerably raised the risk of opportunistic infections, and also significantly reduced the quality of life of the patient, renal transplantation was performed in Debrecen four years after the lung transplantation. This is the first lung transplanted patient in Hungary who also underwent renal transplantation. Now, more than two years after the renal transplantation the patient lives a compensated, rehabilitated life, the respiratory function values have improved and the renal function values are also acceptable. The number of transplanted patients has significantly increased in recent years worldwide, and also in Hungary. However, due to immunosuppressive medications, calcineurin inhibitors mainly, numerous complications must be reckoned with. An outstanding one among them is the secondary renal failure which may occur both in acute and chronic form and may even necessitate renal transplantation. Renal transplantation is an excellent treatment option for end stage renal failure patients, who had received another organ transplant earlier. Kidney transplantation improves quality of life and increases expected survival, too.

Pulmonary arterial hypertension in mixed connective tissue disease: successful treatment with Iloprost.

This paper describes a 61-year-old woman who presented with mixed connective tissue disease, which was complicated by the development of pulmonary arterial hypertension (PAH). Her condition worsened rapidly, with development of haemopthysis, tachypnoe and cardiac arrest. Doppler echocardiography showed a high systolic pulmonary arterial pressure (98 mmHg), confirmed by the right heart catheterization. Vasculopathy of the pulmonary artery vessels was detected following open lung biopsy. No pulmonary embolism was found. Because of suspicion of flare of her underlying disease, which leads to PAH, immunosuppressive treatment was started with high doses of corticosteroid and cyclophosphamide, in combination with the prostacyclin analogue, Iloprost, and low molecular weight heparin. The therapy resulted in slow recovery over 6 weeks, with control echocardiography showing normalization of the high pulmonary pressure, and the patient being capable of returning to everyday activities.

[The role of volume reduction surgery in the therapy of early stage chronic obstructive pulmonary disease]. / A volumenredukciós mutét, mint kezelési lehetoség a korai stádiumú krónikus obstruktív tüdobetegség terápiájában.

The role of volume reduction surgery in the therapy of early stage chronic obstructive pulmonary disease. COPD is a leading cause of morbidity and mortality worldwide. Assessment of COPD severity is based on the level of patient's symptoms, the severity of the spirometric abnormality and the presence of complications, such as respiratory failure, and right heart failure. Management of mild to moderate COPD is complex, involves the avoidance of risk factors to prevent disease progression, pharmacotherapy as needed to control symptoms, physiotherapy, and rehabilitation. The bases of pharmacotherapy are bronchodilators, which represent the first line symptom based pharmacologic intervention in COPD. Surgical treatment of emphysema was basically designed to improve subjective dyspnoea, respiratory function and quality of life in severely disabled patients. Three surgical options are currently employed, namely bullectomy, lung volume surgery and lung transplantation. During volume reduction surgery the hyperinflated areas are resected, so the functional residual volume is getting reduced, the respiratory muscle function is increased; the quality of life is improved. The authors are report a young early stage COPD patient who underwent a volume reduction surgery because of decline in physical condition, improving her disability. In conclusion in properly selected patients, lung volume surgery can offer prolonged functional benefit and satisfactory long term survival, even in early stage. The invasive options has a risk of both mortality and morbidity, they are directed only at patients who remain symptomatic despite optimal medical treatment.

[Interstitial lung disease in mixed connective tissue disease]. / Interstitialis légzoszervi betegség kevert kötooszöveti betegségben.

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.

Mediastinal bulky tumour in Hodgkin's disease and prognostic value of positron emission tomography in the evaluation of post-treatment residual masses.

Among the 193 patients (82 female, 111 male) treated primarily for Hodgkin's disease at our clinic between 1990 and 2001 and followed up until 2003, 42 (22%) had mediastinal bulky tumours (MBTs) by the Cotswolds criteria. The rate of MBT diagnosis was significantly greater in the early stage of the disease, these patients were younger and--in contrast to the other group--they all received combined therapy. No significant differences were found in the overall and relapse-free survival rate in the two groups, but relapse and death rates were lower in the patients with bulky tumours. Of the total number of patients, 27 underwent a total of 31 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) examinations, mainly for the evaluation of post-treatment residual mass viability. In the 12 positive cases, the majority of patients received further therapy. During the mean follow-up time of 58 months (range 5-98 months) after obtaining negative results, progression of the disease was found in 2 cases 14 and 23 months later, respectively. Based on our results, we conclude that FDG-PET examinations show a good correlation with clinical follow-up results.

[Liver resection for a rare parasitic infection--visceral larva migrans syndrome]. / Májresectio ritka parazitás fertozés miatt. Visceralis típusú larva migrans szindróma.

The authors describe a case of an 38-year-old woman suffering from a parasitical infection which is rare in Hungary. It was diagnosed in connection with a surgical liver segment resection. Visceral larval migrans is an infection caused by migration of the roundworm Toxocara larvae to organs and tissues. The authors describe the pathophysiology of the disease, clinical symptoms, diagnostic and therapeutic possibilities.

[New indications in the treatment of advanced esophageal cancer using self-expanding stent]. / A malignus nyelócsó szúkületek kezelésének új indikációi polyflex sztent alkalmazásával.

In the past four years we performed palliative reconstruction in 69 patients with Tygon or Polyflex self-expanding stents. The procedure was performed in 35 patients because of mid-third oesophageal cancer, in 18 patients because of cancer of the cardia, in 7 patients because of mediastinal tumour and in 8 patients because of oesophago-respiratory fistula. The procedures improved the swallowing greatly, the oesophago-respiratory fistulae closed. During endoscopic intubation of cardia tumours the risk of perforation is high, while it is lower in case of open operative pull-through method. Insertion of Polyflex self-expanding stent under X-ray is safe. Improvement of the stents helps not only safer use, but it encourages its use in new indications, such as iatrogen benign perforation, corrosive oesophageal stricture, long preoperative period, when the swallowing for better nutrition is essential. The procedures improve the quality of life and improve survival time because of the restored swallowing, closed oesophago-respiratory fistula with the prevention of aspiration.