Hungarian Immunology

[Experiences on cyclosporin-A treated childhood atopic dermatitis resistant to other therapies]

SZAKOS Erzsébet, SZEGEDI Andrea, SÓLYOM Enikõ, HUNYADI János

FEBRUARY 15, 2004

Hungarian Immunology - 2004;3(02)

[BACKGROUND - Cases of six children suffering from serious form (resistant to conventional therapy) of atopic dermatitis are presented and the experiences regard of treatment with cyclosporin A (Sandimmun Neoral-microemulsion form) are summarised. PATIENTS - The average age of the 4-16 year-old children (three girls, three boys) was 9.9 years. The skin process started at infantile age. The cyclosporin A was used during 12-85 weeks, in a maximal dose of 3.5-5 mg/body weight kg/day. The patients received locally creams or ointments to moisture their skin continuously and if it was necessary, corticosteroid creams or ointments for a few days. RESULTS - The therapeutic response was excellent in five cases and poor in one case. The short time therapies resulted transient, the long time therapies long acting effect. There was no side effect indicating the stop of cyclosporin A therapy. Three children presented body weight elevation slightly higher than the age-related physiologic change. Epstein-Barr and cytomegalovirus infection with severe submandibular lymphadenopathy appeared in a patient and transient hypertrichosis in the case treated for 85 weeks. DISCUSSION - The authors propose treating severe childhood atopic dermatitis resistent to other therapeutic possibilities with cyclosporin A. The adequate follow up, monitoring of clinical and bloodchemical parameters are important.]



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Hungarian Immunology

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[INTRODUCTION - The aim of the present study was to investigate the cerebrospinal fluid and serum β- endorphin levels in several diseases characterized by central nervous system demyelinisation. PATIENTS AND METHODS - Ten patients with systemic lupus erythematosus complicated with demyelinating syndrome and ten patients with chronic progressive form of multiple sclerosis were selected. Concentrations of β-endorphin were measured using a high sensitive, specific radioimmunoassay. Statistical significance (Wilcoxon test, two variable t test) and correlations (Spearman and Pearson correlations coefficients) were calculated. RESULTS - β-endorphin concentration in the cerebrospinal fluid did not differ in multiple sclerosis and systemic lupus erythematosus patients compared to the controls.]

Hungarian Immunology

[Diagnostic value of MRI in patients with juvenile dermatomyositis]

CONSTANTIN Tamás, PONYI Andrea, BALÁZS György, SALLAI Ágnes, DANKÓ Katalin, FEKETE György, KARÁDI Zoltán

[Diagnosis of juvenile dermatomyositis is based on the presence of proximal muscle weakness, characteristic skin lesions, muscle enzyme elevation in the serum, and may requires the performance of invasive procedures such as electromyography and/or muscle biopsy. Magnetic resonance imaging (MRI) is considered to be an objective non-invasive tool to detect muscle involvement for diagnosis as well as for follow-up studies. We report a case of a 12 years old girl with definitive juvenile dermatomyositis. She received glucocorticoid therapy and achieved remission of the disease. After a long-term relapse free period, she was presented with severe proximal muscle weakness and normal creatinine kinase levels. The laboratory studies did not reveal acute inflammation or infection. In this case MRI was diagnostic to the relapse of juvenile dermatomyositis, with an increased STIR (short tau inversion recovery) signal of proximal muscles. The muscle involvement detected by MRI correlated with functional ability. After she achieved clinical remission, further follow-up MRI scans demonstrated that the affected muscles had returned to normal signal intensity. Findings of dermatomyositis on MRI scans include increased signal intensity in the affected muscles, perimuscular edema, chemical-shift artifact, and increased signal intensity in subcutaneous tissue. MRI is a sensitive technique and proposed to be a good indicator for an early diagnosis of the disease. MRI may also help to guide the muscle biopsy and may enhance the sensitivity of histological examination. After completion of therapy, MRI may be used for monitoring the progress of the disease as signal intensity of affected muscles returns to normal. MRI is also helpful, if the diagnosis is suspected but has not been formally evaluated.]

Hungarian Immunology

[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

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Clinical Neuroscience

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

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Clinical Neuroscience

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Clinical Neuroscience

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Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.