LAM KID

[Stop at one - Make your first break your last!]

NÉMETH Éva

DECEMBER 10, 2012

LAM KID - 2012;2(04)

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LAM KID

[Diffusely increasing neck fat mass]

LAM KID

[Adherence of Hungarian postmenopausal women with osteoporosis]

LAKATOS Péter, TÓTH Emese, LANG Zsolt, NAGY Bence, SZEKERES László, TAKÁCS István

[INTRODUCTION - Osteoporosis is defined as a loss of bone tissue and bone mass that leads to a compromised trength and quality of bones and thus to an increased risk of fractures. In many women, menopausal hormonal changes are associated with an increased bone loss. This population has postmenopausal osteoporosis. The essence of osteporosis treatment is the adequate calcium and vitamin D supplementation, which, if needed, might be combined with drug therapy to inhibit the process of bone loss. METHODS - We assessed the adherence to therapy of Hungarian patients and its effect on the risk of bone fractures, using data recorded by the National Health Insurance Fund Administration between 2004 and 2010 (n=223068, mean age: 69.9 years). We performed a statistical analyses of the available data. Medication possession ratio (MPR) for each treatment and the ratio of patients receiving continuous treatment in the study period (for 12, 18 and 24 months) were estimated. Medication persistence was investigated using Kaplan-Meier survival analysis. A multivariate Cox proportional hazard model was used to determine the factors influencing the risk of fracture. RESULTS, CONCLUSION - The results of our study show that medication adherence to treatment is low among Hungarian patients [mean MPR: 57.9%; 95% CI (57.7%- 58.0%) and persistence rate: 32.4%; 95% CI (32.2%-32.6%) in the first year]. These parameters are substantially influenced by the administration route and the frequency of treatments [mean MPR ranged 41.5%- 100% and persistence rates ranged 18.8%- 100% in the first year, differences between subgroups were significant (p<0,05)]. Our compliance as well as persistance studies showed that parenteral administration had more beneficial effects. Confirming our preliminary hypotheses, the improvement of patient compliance significantly reduced fracture risk (good compliance was defined as MPR>80%, which was associated with RR: 0.57, p<0.05 for fracture risk). Further improvement might be achieved by parenteral administration [RR for fracture risk 0.60 compared with non-compliant patients and 0.44 compared with compliant subgroups treated with oral and parenteral medications (p<0.05)].]

LAM KID

[Effect of zoledronic acid treatment on pain and quality of life in patients with metastatic bone disease suffering from breast and prostate cancer - Multicenter, prospective, observational study]

PÁPAI Zsuzsanna, LANDHERR László, SPEER Gábor

[INTRODUCTION - Metastatic bone disease is frequently associated with breast and prostate cancer. Bisphosphonate treatment of bone metastases is palliative: its primary goal is to relieve pain, while it's also important to decrease the risk of bone fractures, prolong survival and maintain physical activity of the patients. Pain is the most common symptom of bone metastases. PATIENTS AND METHODS - In total 845 patients were enrolled in our open, multicenter, prospective, observational study, the first of its kind in Hungary. The agent tested was zoledronic acid (Zometa®). Duration of the study was 20 months and its primary goal was to assess the correlation between pain and quality of life during the treatment of patients with bone metastases from breast or prostate carcinoma. RESULTS - During the 18 months of the study, the average intensity of pain, measured on the visual analog scale showed a 42% reduction (p<0.0001). By the end of the 18. month, the ratio of patients free of symptoms has increased by 15% and the number of patients with substantial complaints has decreased by 73%. CONCLUSION - Our study supports the observation published in the international literature that in patients with bone metastases from breast and prostate cancer, zoledronic acid treatment is beneficial for reducing pain and thus for improving quality of life.]

LAM KID

[The effects of targeted therapies on bones]

SZEKANECZ Zoltán

[Arthritis is associated with local as well as generalised bone loss. It is likely that similar inflammatory/immunological factors contribute to both types of bone loss. Today, the main targets of arthritis therapy are proinflammatory citokines (TNF-alpha, IL-1, IL-6, in the future IL-17) and the inhibition of B and T cells. All biological therapies have been proved to slow down the development of focal joint destruction. TNF-inhibitors in particular have been demonstrated to have a beneficial effect on generalised osteoporosis. In ankylosing spondylitis, generalised osteoporosis and locally increased bone formation occur at the same time, creating a controversial situation. Further studies are needed for a better understanding of the effects of targeted therapy on bones.]

LAM KID

[Everyday decisions regarding osteoporosis treatment]

TAKÁCS István

[Efficient, new medicines as well as recent scientific results have substantially changed the options and daily practice of osteoporosis treatment. Besides the indicated duration of therapies, their indication has also changed in several key points, and the range of possibilities for switching therapies have expanded. Despite the availability of a steadily increasing number of data, large-scale studies do not always help making therapeutic decisions, so we have to rely on professional protocols and our own experience, as well as on logical thinking. Moreover, we also have to observe financial regulations. This summary is intended to serve as a guideline for the most important decision situations from the initiation of therapy until its cessation.]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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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.

Clinical Neuroscience

[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

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[The Comprehensive Aphasia Test in Hungarian]

ZAKARIÁS Lilla, RÓZSA Sándor, LUKÁCS Ágnes

[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]

Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]