Lege Artis Medicinae

[Congenital haemorrhagic diathesis in childhood- an update]

ZIMONYI Ilona1, MAROSI Anikó2, JÁNKY Ilona2

JULY 31, 1991

Lege Artis Medicinae - 1991;1(13)

[Following 164 haemophilic children in the Heim Pál Children's Hospital the authors call attention to the increased responsibility of paediatricians in diagnosis and long term care. They highlight haemarthroses leading to disableness, dental care, and the hazards of the treatment, such as changes in the immunstatus, transfer of infections, and the appearence of inhibitors. Hepatitis B profilaxis, introduced last year is mentioned as a favorable result. They call attention to the bad school results of the patients with average intelligence, the difficulties in the choice of profession and adaptation. They emphasize that the solution of the problems is an averall social duty. Finally, problems to be solved in the near future are listed. ]


  1. főorvos Heim Pál Gyermekkórház Haematológiai Osztály
  2. Heim Pál Gyermekkórház Haematológiai Osztály



Further articles in this publication

Lege Artis Medicinae

[Diagnosis and treatment of neck pain II. Treatment]


[The treatment of neck pain – such as the diagnostic procedure – requires the collaboration of the general practicioner and rheumatologic, neurologic; traumatologic and orthopedic experts. This part of the article interpretes methods and possibilities which can be made or prescribed by all the collegues for such patients. In case of acute neck pain bedrest, fixing instruments, peros medicamentation, local injections and physiotherapy are recommended. Patients suffering from chronic neck pain should be treated with active gymnastics, physiotherapic and relaxation methods rather than with oral therapy to avoid medical polypragmasy. Psychic running of these patients are emphasised. The authors conclude that general practicioners should play greater role in the treatment of neck pain, such as of arthrosis or backache.]

Lege Artis Medicinae

[The clinical pathology of prostatic carcinoma]

KISS Ferenc

[On the basis of the actual state of art, the main tasks of clinical pathologists in the diagnostics of prostatic carcinoma may be summarized as follows: Recognition and differential diagnosis of prostatic adenocarcinoma. Estimation of tumour prognosis by means of a reliable histological grading system and establishing the pathological stage. Checking the efficacy of (hormonal) treatment relying upon histological features. An increasing effort to a better understanding and diagnosis of premalignant changes (dysplasia, prostatic intraepithelial neoplasia). In favour of individual characterization of a tumour, one should utilize the attainable modern investigative methods.]

Lege Artis Medicinae

[The role of the estracyt therapy in the treatment of prostatic cancer]

HATÁR András, LENGYEL István

[The authors – after summarizing the literature data – report the experiences of 57 prostatic cancer patients treated with Estracyt. The therapy was introduced as a primary one in 22 cases, and as a secondary treatment in 35 ones. It was administered mostly in T3-4 stadium cases with proved metastases. There was a complete regression in 8, partial regression in 15, while temporary regression in 14 cases (totally 64,9%). It is suggested, that the product can be used ensuring regression by the development of hormone resistance, by anaplastic tumors, and at the evolving of the recidiva following surgery or irradiation. A complete regression can be reached relatively rarely in advanced tumors. It can be used in either primary or secondary therapy. ]

Lege Artis Medicinae

[Diagnosis and therapy of prostate cancer]


[Prostate cancer is the third most common cancer in men. The majority of patients present to a doctor at an advanced stage with a tumour that has spread beyond the organ boundaries or with regional lymph node and haematogenous bone metastases. Correct treatment is based on correct clinical staging.]

Lege Artis Medicinae

[Dental and oral symptoms in diabetes mellitus]


[Results of dental examination in 1600 diabetic patients and 623 nondiabetic textile workers were compared. Standard methods – recommended by WHO (1977) – were used to the examination. The condition of caries was assessed with DMF-T mean values, periodontal changes with the use of Russell's Periodontal Index (Pl), and the degree of oral hygiene with the OHI-S index. Statistical evaluation of the data was made with the BMDP program package using chi-square test, correlation and analysis of variance. The DMF-T index in diabetic patients was higher (p < 0.01), considering the distribution of its components the average number of carious teeth was lower (p < 0.0001), in patients above 30 years more extracted teeth (M) were found (p < 0.0001) than in the controls. Diabetics lose their teeth mainly due to periodontal disease, which is also supported by the increased Pl mean values (p < 0.001). Poor oral hygiene was accompanied by an increase of oral mucosal alterations. ]

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Over the past year, many cases with newly onset or significantly exacerbated tic disorders were observed worldwide, where some aspects of the clinical presentation or the symptomatology were atypical for established tic diagnoses. Our purpose was to describe the atypical cases and raise relevant diagnostic issues. Consecutive cases with atypical tic presentations were documented. Five atypical tic cases are described. These cases shared some common characteristics, most notably the fact that all of them had been exposed to online presentation of ticking behaviour on social media platforms prior to the de novo development or exacerbation of their tics. Even though the order of events suggests causality and therefore the diagnosis of a functional tic disorder, unambiguous criteria for classifying atypical tics as functional symptoms are lacking. Differentiating neurodevelopmental and functional tics in childhood is currently problematic. Based on the currently unresolved issues in differential diagnosis, the importance of watchful waiting and behavioural interventions is highlighted to avoid unwarranted pharmacotherapy.

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[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

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[Multiple sclerosis (MS) is typically a disease of young adults. Childhood MS can be defined in patients under 18 years of age, although some authors set the limit un­der the age of 16 formerly known as “early-onset multiple sclerosis” or “juvenile multiple sclerosis”, seen in 3-5% of all MS patients. Nowadays, owing to ever-evolving, better diagnostic tools and well-traced, strictly defined diagnostic criteria, childhood MS is showing an increasing incidence worldwide (0.05-2.85/100 000). MS is characterized by recurrent episodes of the central nervous system with demyelination separated in space and time. In childhood almost exclusively the relapsing-remitting (RR) type of MS occurs. Based on experience in adults, the goal in the pediatric population is also the early diagnosis, to initiate adequate DMT as soon as possible and to achieve symptom relief and good quality of life. Based on efficacy and safety studies in the adult population, inter­feron β-1a and glatiramer acetate were first approved by the FDA and EMA for the treatment of childhood MS also. The increased relapse rate and rapid progression of childhood MS and unfavorable therapeutic response to nearly 45% of the first DMT necessitated the testing of more effective and second-line drugs in the population under 18 years of age (PARADIGMS, CONNECT). Although natalizumab was reported to be effective and well-tolerated in highly active RRMS in childhood, evidence based studies were not yet available when our patients’ treatment started. In this article, we report on the successful treatment of three active RRMS patients with individually authorized off-label use of natalizumab.]

Lege Artis Medicinae

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[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.]