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Biomedical subjects

S Skrede

Publications and source records attributed to S Skrede.

133 records · Page 8Linked to original sources
PubMedDate not supplied

Accumulation of 7 alpha-hydroxy-4-cholesten-3-one and cholesta-4,6-dien-3-one in patients with cerebrotendinous xanthomatosis: effect of treatment with chenodeoxycholic acid.

Evidence was recently presented that an essential part of the accumulation of cholestanol in patients with cerebrotendinous xanthomatosis is due to acceleration of a novel pathway, involving 7 alpha-hydroxylated intermediates in bile acid biosynthesis as precursors (J. Clin. Invest. 1985; 75:448-456). Such intermediates accumulate in patients with cerebrotendinous xanthomatosis due to lack of the mitochondrial 26-hydroxylase involved in the major pathway for bile acid biosynthesis. The new pathway may involve the following steps: 7 alpha-hydroxycholesterol----7 alpha-hydroxy-4-cholesten-3-one----cholesta-4,6- dien-3-one----4-cholesten-3-one----cholestanol. Accurate methods have been developed for assay of 7 alpha-hydroxy-4-cholesten-3-one and cholesta-4,6-dien-3-one in serum, based on isotope dilution-mass spectrometry. The serum levels of 7 alpha-hydroxy-4-cholesten-3-one as well as those of cholesta-4,6-dien-3-one were found to be markedly elevated in the three patients with cerebrotendinous xanthomatosis. Treatment of two of the patients with chenodeoxycholic acid reduced the serum levels of the two steroids by more than 80%. The concentration of cholestanol was reduced by 72% in one patient and by 48% in the other. The possibility is discussed that accumulation of cholestanol in patients with cerebrotendinous xanthomatosis is secondary to accumulation of 7 alpha-hydroxy-4-cholesten-3-one and cholesta-4,6-dien-3-one.

Brain Diseases↗
PubMedDate not supplied

The role of viral infection in acute peripheral facial palsy.

Thirty-three patients with acute non-traumatic peripheral facial palsy were studied. In one patient, varicella-zoster virus was isolated from CSF. Antibody against the same virus was present in CSF, and rising titre was demonstrated in serum. In two cases, herpes virus hominis was isolated from the nasopharynx. CF-antibody tests indicated recent viral infection in 7 other cases. One additional patient had clinical signs of herpes zoster oticus. In most of these 11 patients, but also in the majority of the remaining 22 patients, an acute phase reaction was present, and serum and CSF immunoglobulins were increased. Thus, an active or recent infection (probably viral) seemed to precede or coincide with the facial palsy in most cases in both groups.

Adolescent↗
PubMedDate not supplied

Immunodeficiency syndromes with otorhinolaryngological manifestations.

Among patients with recurrent, protracted or chronic infections of the respiratory tract involving the middle ear, 18 were found to have immunodeficiencies. In 10 of the patients, deficiency of immunoglobulins belonging to the IgG, IgA and IgM classes was found. Seven patients had an isolated IgA deficiency. One patient had a combined immunodeficiency with defects of the T-cell system and the B-cell system. One patient had an isolated T-cell deficiency.

Adolescent↗
PubMedDate not supplied

Cochlear hearing loss and viral infection.

A group of about 100 consecutive patients admitted to an otorhinolaryngological outpatient department for treatment of cranial nerve affections underwent detailed neurological, audiological and laboratory examinations. Of 34 patients with hearing loss, objective signs of viral infection were found in 12. The hearing loss was sensorineural of the cochlear type, in 7 cases of the high-frequency type, in 5 cases of the low-frequency (Menière) type. Within one year after the onset of the disease the hearing loss had disappeared in all but 2 cases. Reversible dysfunction of two or more cranial nerves was found in 7 of 8 cases examined. In the cerebrospinal fluid the cell count and the total protein and gamma-globulin values were increased in most patients. Our findings indicate a reversible sensorineural hearing loss to be part of a viral-induced meningoencephalitis and cranial polyneuropathy. The mechanism of the reversible low- and high-frequency hearing loss is discussed.

Adult↗