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31 záznamů v PubMed Filtry

Článek

Embryonálne tumory s mnohovrstvovými rozetami--vzácne tumory centrálneho nervového systému v detskom veku
[Embryonal Tumors with Multilayer Rosettes--Rare Central Nervous System Tumors in Infants]

Klinicka onkologie. 2015 ; 28 (4) : 288-92.

Klin Onkol
ISSN 0862-495X
Zdroj

INTRODUCTION: The most recent findings show a histopathological, genetic and clinical uniformity in cases of tumors called embryonal tumors with multilayer rosettes. This group is composed of medulloepithelioma, ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes. Amplification of locus 19q13.42, which includes C19MC cluster containing genes for microRNA, and also LIN28A positivity are present in all three entities. Dysregulation of epigenetic modifiers is very important in pathogenesis of the disease. These tumors manifest in little children (median less than 3 years of age); overall survival is 5-10%. CASE REPORT: Almost three year-old boy diagnosed with brainstem tumor: meduloepithelioma, WHO grade IV confirmed by histological investigation. He presented with dysarthria, bulbar syndrome, central lesion of the facial nerve, quadriparesis with right-side dominancy. He received three induction cycles of chemotherapy from March to May 2014 (according to protocol COG ACNS0334). Only partial improvement of his clinical state was reached. Signs of an intracranial hypertension appeared resulting in VP shunt insertion; impairment of consciousness developed after the induction cycles and before any other treatment could be initiated. He underwent radiotherapy due to vital indication. After application of two fractions (boost in the center of the tumor), the patient became quickly comatose. Spinal cord metastasis was demarked by MRI scan (in the level of 3rd cervical vertebra). A bilateral infiltration in pulmonary parenchyma, according to a radiologist metastasis-wise, was detected by CT scan (histologisation of infiltration was not implemented). The patient died in August 2014--six months after manifestation of first symptoms. CONCLUSION: We reported our first documented case of a patient with tumor from embryonal tumors with multilayer rosettes group in Slovakia. Nowadays, there is no effective treatment of these tumors. Research of molecules targeting to epigenetic modifiers would be one of the possible promises for future therapy.

MeSH
lidé MeSH
magnetická rezonanční tomografie MeSH
nádory mozku patologie terapie MeSH
počítačová rentgenová tomografie MeSH
předškolní dítě MeSH
primitivní neuroektodermové nádory patologie terapie MeSH
Check Tag
lidé MeSH
mužské pohlaví MeSH
předškolní dítě MeSH
Publikační typ
anglický abstrakt MeSH
časopisecké články MeSH
kazuistiky MeSH

Článek

Dlhodobá prognóza detí s malými abnormalitami glomerulov
[Long-term prognosis in children with minor glomerular abnormalities]

Ceskoslovenska pediatrie. 1991 Mar ; 46 (3) : 129-38.

Cesk Pediatr
ISSN 0069-2328
Zdroj

The authors evaluated the course and prognosis of the disease in 109 children with minor glomerular abnormalities manifested clinically in 45.9% as nephrotic syndrome (NS), in 33% as nephritic syndrome (GN), in 11.9% as isolated haematuria (IH) and in 9.2% as Schönlein-Henoch's purpura (PSH). In NS 78% of the children had before biopsy of the kidneys frequent relapses, 22% were resistant to cortisonoids. After biopsy all children were given cortisonoids, 94% immunosuppressive treatment with cytostatics and some of the children additional treatment. The number of resistant cases declined to 10% and the mean number of relapses from four to one in 12 months. Children under five years had more relapses (P less than 0.05) but also more complete remissions (P less than 0.001) than older children. Relapses occurred up to 10.2 years after the onset of the disease (mean = 4 years). With advancing age and duration of the disease their number declined after treatment. An adverse symptom was resistance to cortisonoids and immunosuppressive treatment, major haematuria and persisting hypertension but not immunological activity (elevated level of immune complexes, reduced C3, positive immunohistochemical finding in renal tissue). The morphological finding which at the onset was slightly beyond the range of minor abnormalities had a poorer prognosis when associated with greater clinical activity. The group developed 88% complete remissions and 6% CHRI. After 22 years the probability of survival in complete continual remission is 66%, the probability in CHRI is 10% (with morphological progression). In nephritic syndrome the children were given after biopsy prednisone in 80.6% and cytostatics in 44.4%. In PSH this treatment was given to 100% and 60% of the children, in IH to 61.5% and 7.7%. On evaluation in nephritic syndrome complete remission was recorded in 47.2%, after 0.4-10.5 years since the onset of the disease; 30.6% did not improve and in 2.8% CHRI developed. In PSH remission developed in 60% after 0.8-6.9 years, no improvement was recorded in 20%, incl. 10% where CHRI developed after a resistant course of NS. In IH 84.6% of the patients did not improve, but in none the renal function deteriorated. The course was in all instances milder than in NS, most frequently only with microscopic haematuria and/or slight proteinuria, respectively minor immunological activity. In the entire group of minor glomerular abnormalities complete remission was achieved in two-thirds of the children, in one quarter the disease did not improve, incl. 4.6% where CHRI developed, always associated with progression of morphological changes.(ABSTRACT TRUNCATED AT 400 WORDS)

MeSH
dítě MeSH
glomerulonefritida * diagnóza terapie MeSH
hematurie * etiologie MeSH
IgA vaskulitida * komplikace MeSH
kojenec MeSH
lidé MeSH
mladiství MeSH
nefrotický syndrom * diagnóza terapie MeSH
předškolní dítě MeSH
Check Tag
dítě MeSH
kojenec MeSH
lidé MeSH
mladiství MeSH
předškolní dítě MeSH
Publikační typ
časopisecké články MeSH

Článek

Liecba nefrotického syndrómu cyklosporínom A
[Treatment of nephrotic syndrome with cyclosporin A]

Ceskoslovenska pediatrie. 1990 Oct ; 45 (10) : 591-5.

Cesk Pediatr
ISSN 0069-2328
Zdroj

The authors treated eight children with corticoid dependent syndrome caused by minor abnormalities of the glomeruli with Cyclosporin A. They administered Cyclosporin, 5 mg/kg/24 h., for a period of 8-16 weeks. In three patients they used Cyclosporin A alone, 5 children were given in addition 10 mg Prednisone per day. They achieved complete remission in all patients. During treatment they monitored haematological and biochemical parameters as well as Cyclosporin A levels; before treatment and after its termination they examined also immunological indicators. They did not observe any serious side effects of treatment.