Lege Artis Medicinae

[Pregnancy in women with systemic lupus erythematosus]

KISS Emese, BHATTOA P. Harjit, BETTEMBUK Péter, BALOGH Ádám, SZEGEDI Gyula

MARCH 20, 2005

Lege Artis Medicinae - 2005;15(03)

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Lege Artis Medicinae

[CAMELOT]

MATOS Lajos

Lege Artis Medicinae

[CELIAC DISEASE AND SPLENOPULPOMA IN THE BACKGROUND OF ANE]

MEKKEL Gabriella, BARTA Zsolt, KOVÁCS Judit, TÓTH László, BURIS László, RESS Zsuzsa, GERGELY Lajos, ZEHER Margit

[INTRODUCTION - Celiac disease causes inflammation with villus destruction in the duodenal and jejunal regions. The consequent secondary malabsorption affects many physiological processes adversely causing iron/folate deficiency type anaemia most frequently. This type of anaemia can disappear with the regeneration of the mucosa by gluten-free diet without any substitution. CASE REPORT - Authors report a case of a patient suffering from iron deficiency anaemia caused by celiac disease. The anaemia did not cease by dietary restrictions and iron supplementation and repeated examinations verified a rare benign tumor of the spleen which can cause anaemia as well. CONCLUSION - In connection with this case, the authors summarise the common knowledge of celiac disease and splenopulpoma calling the attention to the benefits of screening celiac disease and the necessity of splenectomy in similar cases.]

Lege Artis Medicinae

[NON-DIFFERENTIATED COLLAGENOSIS]

BODOLAY Edit, SZEGEDI Gyula

[The authors provide a review on non-differentiated collagenosis (NDC) or, as it is called by another terminology, undifferentiated connective tissue disease (UCTD), outlining the clinical and serological alterations of the disease and give a definition of NDC. NDC is a pathological state when patients present clinical symptoms and serological alterations characteristic of a polysystemic autoimmune disease that cannot be explained by any other disease, where the symptoms however do not meet the diagnostic criteria of any other polysystemic autoimmune disease. NDC is a dynamic state and in 25-30% of the cases it may differentiate into CTD but in 40- 50% it remains in NDC stage and in 10-20% of the cases the patient may achieve remission. Differentiation is most frequent in the first two years of NDC. Patients should be treated and followed up in NDC state as well. The NDC stage is very important, since with the discovery of new autoantibodies, by employing new gene technology and by the follow-up and the treatment of the patients, our main aim is the earliest possible detection of differentiation into a definite polysystemic disease.]

Lege Artis Medicinae

[Atopic dermatitis today]

SZALAI Zsuzsanna

Lege Artis Medicinae

[THE EFFECT OF SEXUAL HORMON ALTERATIONS ON THE FREQUENCY OF OEDEMATOUS ATTACKS IN PATIENTS WITH HEREDITARY ANGIONEUROTIC EDEMA]

FELVINCI Réka, VISY Beáta, NÉMETH Éva, VARGA Lilian, JAKAB László, FARKAS Henriette

[INTRODUCTION - Changes in the body's hormonal equilibrium may alter the frequency of angioedema attacks in patients with hereditary angioneurotic edema. We assessed the relations between the angioedema attacks and puberty, menstruation, anticoncipient pill taking, pregnancy, delivery and menopausa. We also studied the possible impact of an embryo with hereditary angioneurotic edema on the frequency of attacks during pregnancy. PATIENTS, METHODS AND RESULTS - 53 female patients were included in the study. Data was surveyed by a questionnaire and detailed gynecological examination. We pointed out that the frequency of the attacks increased in 34% of the patients during puberty, in 58% of the patients at the time of menstruation and in 63% of the contraceptive pill users. In 36% of the women the frequency decreased in the postmenopausal state. In case the pregnancy affected the disease, the embryo with hereditary angioneurotic edema increased the number of attacks during pregnancy. CONCLUSION - Our analysis shows that conditions with sexual hormon alterations have an effect on the number of edematous attacks, therefore patients with hereditary angioneurotic edema need more attention both in nursing and therapy when they are in a life-period of hormonal change. Our results offer the oppurtunity of a better prediction of edematous attacks thus the oppurtunity of better therapy and better quality of life.]

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Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

Delirium due to the use of topical cyclopentolate hydrochloride

MAHMUT Atum, ERKAN Çelik, GÜRSOY Alagöz

Introduction - Our aim is to present a rare case where a child had delirium manifestation after instillation of cyclopentolate. Case presentation - A 7-year old patient was seen in our outpatient clinic, and cyclopentolate was dropped three times at 10 minutes intervals in both eyes. The patient suddenly developed behavioral disorders along with gait disturbance, and complained of visual hallucinations 20-25 minutes after the last drop. The patient was transferred to intensive care unit and 0.02 mg/kg IV. physostigmine was administered. The patient improved after minutes of onset of physostigmine, and was discharged with total recovery after 30 minutes. Conclusion - Delirium is a rare systemic side effect of cyclopentolate. The specific antidote is physostigmine, which can be used in severely agitated patients who are not responding to other therapies.

Clinical Neuroscience

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]