Lege Artis Medicinae

[Weak-hearted composer of monumental forms - Anton Bruckner]

MALINA János

APRIL 20, 2001

Lege Artis Medicinae - 2001;11(04)

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Lege Artis Medicinae

[Conclusions of a 1980 year long-term health care prognosis]

FORGÁCS Iván

Lege Artis Medicinae

[New oral antidiabetic agents - New aspects in the management of type 2 diabetes mellitus]

GERŐ László

[During the past years several new oral antidiabetic agents have become available and more new drugs of this type are expected to appear on the market. New products appeared in both groups of insulin sensitizers and insulin secretagogues. Modern insulin secretagogues are characterized by immediate and short action. As a consequence, insulin secretion will approach the physiological secretory pattern. The rapid, short-term insulin output prevents the abnormal increase of postprandial glucose level and - at the same time - the danger of late hypoglycemia will decrease. The combination of insulin sensitizers with the new insulin secretagogues provides the possibility to apply the basisbolus therapeutical principle to type 2 diabetic patients treated with oral agents.]

Lege Artis Medicinae

[Consensus or chaos?]

GRÉTSY Zsombor

Lege Artis Medicinae

[Postgraduate training for diabetologists - Report]

GYIMESI András

Lege Artis Medicinae

[History of Doctors’ Circle]

KAPRONCZAY Károly

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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.

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Acute bilateral drop foot as a complication of prolonged squatting due to haemorrhoid

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Drop foot is defined as difficulty of dorsiflexion of the foot and ankle due to weak anterior tibial, extensor hallucis longus and extensor digitorum longus muscles. Cauda equina syndrome, local peroneal nerve damage due to trauma, nerve entrapment, compartment syndrome and tumors are common etiologies. A 32-year-old male patient was applied with difficulty in dorsiflexion of both of his toes, feet and ankles after he had squatted in toilette for 6-7 hours (because of his haemorrhoid) after intense alcohol intake 2 weeks before. Acute, partial, demyelinating lesion in head of fibula segment of peroneal nerves was diagnosed by electromyography. This case was reported since prolonged squatting is an extremely rare cause of acute bilateral peroneal neuropathy. This type of neuropathy is mostly demyelination and has good prognosis with physical therapy and mechanical devices, but surgical intervention may be required due to axonal damage. People such as workers and farmers working in the squatting position for long hours should be advised to change their position as soon as the compression symptoms (numbness, tingling) appear.

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

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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.]

Lege Artis Medicinae

[INSULIN ASPART IN THE CLINICAL PRACTICE]

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[Insulin aspart (B28 Asp-insulin), which is produced by recombinant DNA technology, is a fast-acting insulin analogue. Due to the aspartate for proline substitution at position 28 of the Bchain, the insulin molecule's tendency for selfassociation is diminished, therefore, insulin aspart rapidly dissociates into dimeric and monomeric forms and absorbs quickly and easily after subcutaneous administration. Compared to human regular insulin, insulin aspart has a faster onset of activity, a higher plasma peak and a shorter duration of action. Overall, the pharmacokinetic profile of insulin aspart better mimics the physiological postprandial insulin secretion. Therefore, insulin aspart can be used for prandial insulin substitution in order to decrease postprandial blood glucose excursion. It should be administered immediately before meals, but some observations suggest that it can also be used after finishing meal. This allows a more flexible lifestyle for patients. Insulin aspart can be used in both type 1 and type 2 diabetes. Compared to regular human insulin, a moderate decrease in the HbA1c values and fewer nocturnal hypoglycaemic events are expected from insulin aspart use. Insulin aspart is appropriate for pump treatment as well. It has recently been approved for use in pregnancy, whereas for children and adolescents the expected benefits should be weighed against the more modest clinical experience available. Similarly to other insulin analogues, results of long-term clinical investigations with insulin aspart with regard to the development of complications are not yet available.]