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Lege Artis Medicinae

SEPTEMBER 20, 2018

[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]

BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna

[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion bet­ween experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]

Clinical Neuroscience

SEPTEMBER 30, 2018

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome

ETHEMOGLU Ozlem, KOCATÜRK Özcan, TARINI Zeynep Emine

Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

Hypertension and nephrology

SEPTEMBER 12, 2018

[Treatment of hypertension in kidney transplant patients]

KOVÁCS Tibor, WAGNER László

[Most of the renal transplant recipients suffer from hypertension. Hypertension substantially contributes to the high cardiovascular mortality in this population. The recommendation of the Hungarian Society of Hypertension and the international guidelines suggest to achieve less than 130/80 mmHg as target blood pressure in these patients. Several factors may be in the background of hypertension after kidney transplantation, which can be summarized as factors from the recipient-side, the donorside and factors provoked by transplantation itself. In most of the cases early after transplantation high doses of immunosuppressive drugs (especially calcineurin inhibitors and steroids) are responsible for the increased blood pressure. There are some further special methods apart from the general recommendations which are needed during the examination of hypertension of kidney transplant patients: e.g. measurement of blood trough-level of immunosuppressive drugs, investigation of bone-mineral disorder, screening for the level and causes of anaemia, check-up of the renal graft circulation. Kidney transplant patients suffering from hypertension usually need more than two antihypertensive drugs beyond the use of non-pharmaceutical antihypertensive methods. In the early posttransplantation period calcium channel blockers are preferred antihypertensive medications, because they counterbalance the vasoconstrictive effect of calcineurin inhibitors. The administration of renin-angiotensin-aldosterone inhibitors are rather suggested after the stabilization of renal function (from the 1-3 months posttransplantation). When designing antihypertensive strategy, comorbidities and special factors should be regarded as well, especially volume overload, proteinuria, allograft function (GFR), diabetes, other cardiovascular risk factors, previous cardiovascular events. The setup of an individual therapeutical strategy is advised in view of all these factors, which is different according to the timing after transplantation: the perioperative, the early postoperative phases and from 1-3 months after transplantation have special focuses.]

Lege Artis Medicinae

JUNE 20, 2018

[Diagnosis of interstitial lung diseases]

MÜLLER Veronika

[Recognition and diagnosis of interstitial lung diseases (ILD) is one of the most challenging tasks of respiratory medicine. In the wide range of different ILD-s idiopathic pulmonary fibrosis (IPF) is one of the most common. Improvements in the diagnosis made the recognition of IPF easier. Typical clinical features and usual interstitial pneumonia pattern on high resolution CT are now enough for the confident diagnosis of IPF in the multidisciplinary ILD-team discussions. Bronchoscopic cryobiopsy is a great achievement of histological sampling as this can give appropriate size tissue for histological analysis making surgical intervention rare. As IPF cannot be cured early diagnosis is crucial. Today available treatments can only slow down progression of the disease, so early stages and better clinical condition of the patients are essential for therapeutic success. Follow-up of IPF patients includes complex lung functional measurements in dedicated centers in Hungary. Former studies confirmed forced vital capacity (FVC) decline as an important measure of disease progression and mortality. Available IPF treatments decrease FVC decline and can prevent the mostly deadly acute exacerbations of the disease. Additionally palliative treatments including supplementary oxygen and in selected patients lung transplantation can be offered. This is the first of a series of three articles about IPF. ]

Clinical Oncology

FEBRUARY 10, 2018

[Solid organ transplantation and malignancies]

VÉGSŐ Gyula, MÁTHÉ Zoltán

[Recent breakthroughs in the fi eld of organ transplantation and oncology have challenged existing views, and necessitate the revision of several tumor-related issues in transplantation. The need for expanding the donor pool raises the question of how and when it is plausible to transplant the organs of a donor with a history of cancer, such that the risk of tumor inoculation and manifestation due to the graft would be minimal for the recipient. Another point to consider is whether it is acceptable to transplant a recipient with a history of a malignant tumor, and if yes, how much tumor-free survival time is required as a minimum before the transplant. Transplanted patients live longer as a result of modern immunosuppressive therapy. However, the risk of malignant tumors increases proportionally to the length of the immunosuppressed state: their incidence may be as much as 20-30% in the long term. The signifi cance of „de novo” posttransplant tumors is highlighted by the fact that they are among the leading causes of death in transplant patients. Taken together, malignant diseases pose a serious problem from several aspects, the solution for which requires close teamwork of experts in oncology and transplantation, and the integration of up-to-date knowledge in the process of making a therapeutic decision, tailored individually for the patient.]

Clinical Oncology

DECEMBER 05, 2017

[Complex therapy of bladder cancer]

LANDHERR László

[Bladder cancer is the most common malignancy involving the urinary system. Urothelial (formerly called transitional cell) carcinoma is the predominant histologic type in the developed countries, where it accounts for approximately 90 percent of all bladder cancers. The optimal management of nonmuscle invasive urothelial cancer is highly important. For patients with muscle invasive cancer the gold standard treatment is the cystectomy. If the patient unable or unwilling to undergo radical cystectomy with urinary diversion, complete TURBT combined with radiation therapy plus chemotherapy may offer an alternative bladder-sparing approach. Patients with muscle invasive disease and regional lymph node metastasis limited to the pelvis (N1-N3), but without more distant lymph node or visceral metastasis may be treated with six cycles of cisplatin-based chemotherapy followed by cystectomy or a combined-modality approach. In metastatic cases the combination chemotherapy may prolong survival and often provides palliation of symptomatic disease. Checkpoint inhibition immunotherapy has substantial clinical activity in post-chemotherapy patients and is the preferred therapy for patients who have progressed after platinumbased therapy or is not suitable for them.]

Clinical Oncology

DECEMBER 05, 2017

[Inhibition of proteasome in cancer therapy]

KOPPER László

[The ubiquitin-proteasome pathway is the most important element in the regulation of intracellular protein metabolism. Its main function is the degradation of the unnecessary proteins either as part of normal metabolic balance or in case of misfolding or part of the deregulation as in cancer cells using proteolytic enzymes. The importance of this pathway has been acknowledge by Nobel prize. In certain diseases as in several malignancies, where the ubiquitin-proteasome pathway is not able to remove the proteins due to dysfunction or accumulation in a high quantity. The unregulated accumulation of proteins could lead to cell death. This phenomenon was proven by the appearance of proteasome-inhibitors targeting mainly myeloma. It should be mentioned that clinical aspects myeloma has been discussed in an excellent review by Mikala and his colleagues in Klinikai Onkológia.]

Clinical Oncology

DECEMBER 05, 2017

[Hematopoetic stem cell transplantation for pediatric non-hematological solid tumors]

HAUSER Péter, KRIVÁN Gergely

[High-dose chemotherapy followed by stem cell rescue or allogeneic stem cell transplantation in the treatment of solid tumor with non-hematologic origin applied for more than three decades. High-dose chemotherapy with stem cell rescue is the part of standard fi rst line therapy for several chemosensitive tumors with unfavorable outcome (neuroblastoma, Ewing-sarcoma, medulloblastoma), and also successfully applied in certain therapy-resistant or recurrent solid tumors (germ cell tumors, Wilmstumor). Allogeneic transplantation with reduced intensity conditioning is still not successful in terms of survival in pediatric solid tumors with non-hematological origin. In present paper results of autologus and allogeneic stem cell transplantation in different pediatric solid tumor are discussed.]

Hypertension and nephrology

APRIL 20, 2018

[Dietary treatment of dialysis patients]

ZAKAR Gábor

[Adequate nutritional indices and intake are the corner stone of long term success of renal replecement therapies (hemo- and peritoneal dialysis, transplantation) characterized by favourable survial rates and a good quality of life. There has been no major change in basic principles of nutritional prescription (protein, energy, fluid intake, restriction of sodium, potassium and phosphorous), increasing emphasis has been placed on the reduction of calcium load and ”native” vitamin-D therapy in these patients. Less avareness has been put however in the past ten years (according to recent metaanalyses) on the role and replacement of the full scale of vitamins, in spite of their occasionally altered metabolism and replacement-requirements in ESRD patients. Usually there is a need for their replacement, but some of them are represented in abundant, sometimes toxic amounts in commercially available multivitamin preparates. With in the scope of general aspects of nutrition in ESRD patients, the article gives a detalied overview of their multivitamin recommendations and alternatives of a specified substition.]

Clinical Neuroscience

MARCH 30, 2018

A case with angiographic demonstration of isolated anterior spinal artery occlusion

DOGAN Burcu Vasfiye, KARA Batuhan, SELCUK Hatem Hakan, DIRICAN Ceyhan Ayten, KOKSAL Ayhan

Anterior spinal artery syndrome (ASAS) is a rare syndrome which occurs due to thrombosis of anterior spinal artery (ASA) which supplies anterior two thirds of the spinal cord. A 27-year-old female patient was admitted to emergency clinic with sudden onset neck pain, sensory loss and weakness in proximal upper extremities which occurred at rest. Thrombophilia assessment tests were negative. Echocardiography was normal. Serum viral markers were negative. In cerebrospinal fluid (CSF) examination, cell count and biochemistry was normal, oligoclonal band was negative, viral markers for herpes simplex virus (HSV) type-1 and type-2, Brucella, Borrellia, Treponema pallidum, Tuberculosis were negative. Diffusion restriction which reveals acute ischemia was detected in Diffusion weighted MRI. Digital subtraction angiography (DSA) was performed. Medical treatment was 300mg/day acetilsalycilic acid. Patient was discharged from neurology clinics to receive rehabilitation against spasticity.