Hungarian Radiology

[What we need to know on pharmacoeconomics]

SZÁNTÓ Dezső

APRIL 20, 2004

Hungarian Radiology - 2004;78(02)

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Hungarian Radiology

[Necrotising sarcoid granulomatosis: imitator in the chest]

AGÓCS Ágnes, ZIBOTICS Hilda, GREXA Erzsébet

[INTRODUCTION - Both intra and extrapulmonary manifestation of necrotising sarcoid granulomatosis is very rare. It is characterised by variable pulmonary radiological picture, typical histology and benign clinical course CASE REPORT - A young male patient was admitted to our hospital due to a mass lesion in the right apex of the lung suspicious of tuberculosis discovered on plain chest X-ray. Antituberculotic therapy resulted no change of radiological picture. Cytological sample taken by ultrasound guided fine needle aspiration contained no tumor cells. Finally necrotising sarcoid granulomatosis was confirmed by histological examination of the mass removed by atypical surgical resection. CONCLUSION - Intrapulmonary necrotising sarcoid granulomatosis has a variable picture. It has no typical radiological pattern or localisation, therefore it is impossible to diagnose by X-ray morphology. Final diagnosis is based on histology.]

Hungarian Radiology

[RSNA 2003]

MESTER Ádám

Hungarian Radiology

[Long live May Day!]

LOMBAY Béla

Hungarian Radiology

[Opsismodysplasia A report of two cases]

AL Kaissi A, CHEHIDA FB, NESSIB N, GHACHEM MB, KOZLOWSKI K

[INTRODUCTION - Opsismodysplasia is a rare, severe, neonatal dwarfism usually associated with fatal outcome in the first few years of life. Up to, 2003 about 15 cases have been reported. CASE REPORT - We describe two brothers six and four years old with opsismodysplasia, who presented to the paediatric orthopaedic clinic with the diagnosis of short stature and genu varum deformity. CONCLUSION - Paediatric specialists should be aware, that in rare instances, with improving medical care, they may see children with severe bone dysplasias which usually do not reach age in which paediatric orthopaedic services are required.]

Hungarian Radiology

[Lajos Kósa, Zoltán Novák, Edgár Székely: X-ray atlas of the pediatric lung diseases]

LOMBAY Béla

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Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Lege Artis Medicinae

[Second game, 37th move and Fourth game 78th move]

VOKÓ Zoltán

[What has Go to do with making clinical decisions? One of the greatest intellectual challenges of bedside medicine is making decisions under uncertainty. Besides the psychological traps of traditionally intuitive and heuristic medical decision making, lack of information, scarce resources and characteristics of doctor-patient relationship contribute equally to this uncertainty. Formal, mathematical model based analysis of decisions used widely in developing clinical guidelines and in health technology assessment provides a good tool in theoretical terms to avoid pitfalls of intuitive decision making. Nevertheless it can be hardly used in individual situations and most physicians dislike it as well. This method, however, has its own limitations, especially while tailoring individual decisions, under inclusion of potential lack of input data used for calculations, or its large imprecision, and the low capability of the current mathematical models to represent the full complexity and variability of processes in complex systems. Nevertheless, clinical decision support systems can be helpful in the individual decision making of physicians if they are well integrated in the health information systems, and do not break down the physicians’ autonomy of making decisions. Classical decision support systems are knowledge based and rely on system of rules and problem specific algorithms. They are utilized widely from health administration to image processing. The current information revolution created the so-called artificial intelligence by machine learning methods, i.e. machines can learn indeed. This new generation of artificial intelligence is not based on particular system of rules but on neuronal networks teaching themselves by huge databases and general learning algorithms. This type of artificial intelligence outperforms humans already in certain fields like chess, Go, or aerial combat. Its development is full of challenges and threats, while it presents a technological breakthrough, which cannot be stopped and will transform our world. Its development and application has already started also in the healthcare. Health professionals must participate in this development to steer it into the right direction. Lee Sedol, 18-times Go world champion retired three years after his historical defeat from AlphaGo artificial intelligence, be­cause “Even if I become the No. 1, there is an entity that cannot be defeated”. It is our great luck that we do not need to compete or defeat it, we must ensure instead that it would be safe and trustworthy, and in collaboration with humans this entity would make healthcare more effective and efficient. ]

Clinical Neuroscience

Effects of valproate, carbamazepine and levetiracetam on Tp-e interval, Tp-e/QT and Tp-e/QTc ratio

YASAR Altun, ERDOGAN Yasar

Aim - To evaluate P-wave dispersion before and after antiepileptic drug (AED) treatment as well as to investigate the risk of ventricular repolarization using the Tpeak-Tend (Tp-e) interval and Tp-e/QT ratio in patients with epileptic disorder. Methods - A total of 63 patients receiving AED therapy and 35 healthy adults were included. ECG recordings were obtained before and 3 months after anti-epileptic treatment among patients with epilepsy. For both groups, Tp-e and Tp-e/QT ratio were measured using a 12-lead ECG device. Results - Tp-e interval, Tpe/QT and Tp-e/QTc ratios were found to be higher in the patient group than in the control group (p<0.05, for all), while QTmax ratio was significantly lower in the patient group. After 3 months of AED therapy, significant increases in QT max, QTc max, QTcd, Tp-e, Tp-e/QT, and Tp-e/QTc were found among the patients (p<0.05). When the arrhythmic effects of the drugs before and after treatment were compared, especially in the valproic acid group, there were significant increases in Tp-e interval, Tp-e/QT and Tp-e/QTc values after three months of treatment (p<0.05). Carbamazepine and levetiracetam groups were not statistically significant in terms of pre- and post-treatment values. Conclusions - It was concluded that an arrhythmogenic environment may be associated with the disease, and patients who received AED monotherapy may need to be followed up more closely for arrhythmia.

Hypertension and nephrology

[Hungarian dialysis statistics: changing trends in the renal epidemiology]

KULCSÁR Imre, KISS István, SZEGEDI János

[In the last 30-35 years, dialysis care in Hungary has been a major development: both the incidence and prevalence of patients have increased year by year. Over the last decade, growth has slowed and is becoming more and more stabilized (similar trends can be seen in dialysis statistics in developed countries). Behind the dialysis indication the acute kidney injury (AKI) is more common than the end-stage renal disease (ESRD). The latter incidence has been stable for last 6 years (200-230 patient/million population). The annual average growth rate of prevalent dialysis patients was only 0.9%/year in the last 6 years. Among prevalent dialysis patients, the proportion of diabetic patients has remained unchanged for 10 years (26-27%), but those have increased who had hypertension nephropahty. The average age of incident and prevalent dialyzed patients has decreased gradually over the past 8 years (between 2009 and 2017 incident rate was from 67.1 to 63.0 years, prevalent rate was from 65.6 to 61.8 years). Unfortunately, just over half of the patients who dialyzed due to chronic kidney disease (CKD) have reached dialysis day 91. This is due to the high proportion of patient who was in urgent need of dialysis. In chronic hemodialysis (HD) program, the proportion of patients treated with arterovenous fistulas (AVF) decreases, while the rate of central venous catheter (CVC) users increases. The Hungarian peritoneal dialysis program in Europe is very good. The number of prevalent patients receiving renal replaement therapy (RRT) in Hungary in 2017 was 1005 for 1 million inhabitants.]

Clinical Neuroscience

[The long-term follow-up of enzyme replacement treatment in late onset Pompe disease]

MOLNÁR Mária Judit, BORSOS Beáta, VÁRDI Visy Katalin, GROSZ Zoltán, SEBÕK Ágnes, DÉZSI Lívia, ALMÁSSY Zsuzsanna, KERÉNYI Levente, JOBBÁGY Zita, JÁVOR László, BIDLÓ Judit

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]