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Biomedical subjects

B Lombay

Publications and source records attributed to B Lombay.

17 recordsLinked to original sources

Fetal MRI : reviewing the history, indications, technique, safety and drawbacks.

The ultrasound is undoubtedly the first-line diagnostic examination to identify prenatal congenital abnormalities. Fetal-MRI (FMRI) is a non-invasive, fast, highly informative examination that can shed immense information on anatomical abnormalities and affect decision-making. If, in spite of a significant pathology, the parents insist on continuing with the pregnancy, help of other interdisciplinary specialities can be sought for postnatal care. The article briefly describes different aspects of FMRI.

Female↗

[Menkes disease].

The Menkes or kinky hair disease is a rare, sex-linked systemic disorder of the copper metabolism. It is lethal in the first three years due to cerebral and cerebellar degeneration. Apropos of their case the authors summarise the knowledge on the diagnosis, pathogenesis and therapy.

Brain↗

[Rasmussen syndrome].

Authors report about a boy with Rasmussen syndrome. This is a chronic localised encephalitis with focal epileptic seizures causing progressive neurologic deficits and mental impairment. The etiology and the clinical features of the syndrome are discussed, and the diagnostic role of the new imaging modalities is emphasized. This is the first report about this entity in Hungary.

Acyclovir↗

Neuroimaging of Rasmussen's encephalitis.

A case of Rasmussen's encephalitis is reviewed. Focal seizures and hemiparesis in a 5-year-old child progressed over a period of 2 1/2 years. Neuroimaging findings showed progressive atrophy of the right temporal lobe. The diagnosis of this syndrome is possible using a combination of the clinical symptoms and neuroimaging findings.

Atrophy↗

Obstruction of cerebral arteries in childhood stroke.

Middle cerebral artery obstruction in children is reviewed by our two cases. Ischemic childhood stroke was caused by moyamoya disease in the first, and by fibromuscular dysplasia in the second patient. In both cases transcranial Doppler sonography and cranial CT were performed, but the final diagnosis was made by angiography. The importance of angiography in childhood stroke is emphasized.

Cerebral Angiography↗

[Moyamoya disease in childhood].

The authors describe two children with moyamoya disease, a rare cerebrovascular disorder characterised by progressive stenose of circle of Willis and a compensatory vascular network in basal ganglia. Both patients were presented by transient ischemic attack. The importance of hyperventilation in provoking symptoms is emphasized and the possible etiology, signs, classification, prognosis and therapeutic approaches are discussed. This is the first report of this entity in Hungary.

Age Factors↗

Assessment of skeletal age in the first year of life on basis of the caput humeri ossification centres.

A new procedure has been worked out to estimate skeletal maturity in infancy. The size of the caput humeri ossification centres was determined with the help of a pattern set from the antero-posterior chest roentgenogram. In each month of the first year of life 100 examinations were carried out and from their data percentile lines were constructed for every month of life.

Age Determination by Skeleton↗

Multicentric Castleman disease and systemic lupus erythematosus phenotype in a boy with Klinefelter syndrome: long-term disease stabilization with interferon therapy.

An 11-year-old boy with Klinefelter syndrome had Castleman disease (CD) of plasma cell type develop. Nonregulated antibody production mimicked systemic lupus erythematosus (SLE). Hepatitis C virus (HCV) infection caused significant disease worsening. The patient was treated with a daily dosage of 2 million units/m2 of IFN-alpha. Dramatic clinical improvement and decreasing autoimmune phenomenon were observed. HCV RNA were cleared. Hypergammaglobulinemia did not change. The boy has been living for 8 years with his disease. Plasma cell type CD can mimic collagenosis. Disease worsening is caused by HCV, though it can be reversed with IFN-alpha. Klinefelter syndrome may be a genetic susceptibility factor for CD in some cases.

Castleman Disease↗