Biomedical subjects

B J Linehan

Publications and source records attributed to B J Linehan.

3 recordsLinked to original sources

PubMed1986

Cyclic eosinophilic myositis and hyperimmunoglobulin-E.

A 40-year-old man had regular cyclic episodes of weight gain and eosinophilic myositis associated with hyperimmunoglobulin-E and hypereosinophilia for 9 years. During the episodes his body weight increased up to 10.8%; eosinophil counts reached 41.3 X 10(-9) cells/L; and IgE levels reached 18 000 IU/mL. These values changed regularly in a definite sequence relative to the clinical state. Attempts to document a parasitic cause were unsuccessful, and several courses of anthelmintic therapy were ineffective. An oral dose of prednisone, 10 mg/d, begun in July 1982 resulted in an immediate lessening of the severity of the episodes and a progressive lengthening of the cycle from 35 to 170 days. No further episodes have occurred since March 1984. The patient is fit and well on prednisone therapy, 12.5 mg on alternate days. This apparently unique syndrome has a benign course and is a cyclic disease involving skeletal muscle as the target organ.

Adult↗

PubMed1979

Hereditary acanthocytosis associated with the McLeod phenotype of the Kell blood group system.

Some boys with X-linked chronic granulomatous disease (CGD) have red cells of the rare McLeod phenotype in the Kell blood group system. Only one example of this phenotype has previously been described in a non-CGD subject. We have studied a 10-year-old boy and a maternal brother who do not have CGD and whose red cells are of the McLeod type . The boy presented as a haematological problem with red-cell abnormalities. These were acanthocytosis, anisocytosis and 'tailing' in the osmotic fragility curve, changes now known to occur with the McLeod phenotype. Subsequent studies revealed his rare blood group. A family study has established that an uncle also has acanthocytic red cells and the McLeod phenotype. In addition the boy's sister, mother and maternal grandmother all show red-cell mosaicism with double populations of McLeod acanthocytes and normal red cells of common Kell type. The gene that determines inheritance of the McLeod phenotype is X-linked and the mosaicism present in female carriers is believed to result from X chromosome inactivation by the Lyon effect. The study provides further evidence that the McLeod phenotype arises by inheritance of a variant X-linked modifying gene and not through inheritance of a variant gene at the Kell autosomal locus. It also represents the first occasion that a person of rare blood group has been recognized because of an associated anomaly in red cell morphology.

Acanthocytes↗

PubMed1975

Survey of HBag hepatitis infection in a semi-closed community.

The presentation of one individual with HBAg-positive hepatitis in a semi-closed community led to the testing of 262 intellectually-handicapped persons and staff-members and to the detection of 12 others who were HBAg-positive. Only the presenting case and one other became unwell with hepatitis. Three other HBAg-positive individuals became negative within three months, none showing clinical or biochemical evidence of hepatitis. The remaining eight persons were still HBAg-positive six months later. Four of these had biochemical abnormalities suggesting they had suffered anicteric hepatitis while the remainder, three of whom had Down's syndrome, had no such changes and are therefore considered to be carriers of the infective agent.

Carrier State↗