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Biomedical subjects

P J Holt

Publications and source records attributed to P J Holt.

At least 19 recordsLinked to original sources
PubMed1992

Regressing atypical histiocytosis, a regressing cutaneous phase of Ki-1-positive anaplastic large cell lymphoma. Immunocytochemical, nucleic acid, and cytogenetic studies of a new case in view of current opinion.

BACKGROUND: Regressing atypical histiocytosis is a rare multifocal cutaneous tumor characterized by large, spontaneously regressing, ulcerating skin nodules. Although initially self-remitting, the condition may progress to systemic lymphoma. METHODS: Using material from one patient, an attempt was made to clarify the nature of this condition with immunophenotyping, genotyping, and chromosome studies. RESULTS: Immunophenotyping studies indicated the condition was of T-cell lineage, although T-cell receptor gene studies showed polyclonal rearrangement. This case progressed to systemic lymphoma. CONCLUSIONS: The authors believe regressing atypical histiocytosis is a regressing phase of Ki-1-positive anaplastic large cell lymphoma of the skin.

Adult↗
PubMed1992

Psychiatric disorder and cognitive impairment in systemic lupus erythematosus.

OBJECTIVES: To determine the point prevalence of psychiatric disorder in patients with systemic lupus erythematosus (SLE). To investigate associations between psychiatric disorder and social stress, cognitive impairment, systemic disease activity, and corticosteroid therapy. METHODS: A cross-sectional study of 73 consecutive patients with SLE assessed using standardized psychiatric and clinical research methods. RESULTS: Current psychiatric disorder was present in 15 patients (20.5%) and was significantly associated with social stress, lack of social support, and impairment on 2 tests of cognitive function (Verbal Fluency Test and Benton Visual Retention Test, Part A, number of errors). There was no association with systemic disease activity or corticosteroid therapy. Cognitive impairment on 2 or more tests was found in 26% and was associated with clinical evidence of central nervous system (CNS) disease, but not systemic disease activity or corticosteroid therapy. CONCLUSIONS: The point prevalence of psychiatric disorder in this cohort of patients with SLE was found to be similar to that observed in patients with rheumatoid arthritis. It was independently associated with social stress and 2 indicators of cognitive impairment, but not with systemic disease activity or corticosteroid therapy. Marked cognitive impairment was present in a significant percentage of patients even when there was no overt evidence of CNS involvement.

Adult↗
PubMed1992

Corneal autoimmunity in patients with peripheral ulcerative keratitis (PUK) in association with rheumatoid arthritis and Wegener's granulomatosis.

Serum antibodies to the cornea were investigated in patients with peripheral ulcerative keratitis (PUK) in isolation or in association with a systemic disease (rheumatoid arthritis or Wegener's granulomatosis). Indirect immunofluorescence on bovine corneal sections demonstrated that antibodies bound to epithelial antigens in two distinct patterns: a lattice-like pattern, probably staining intercellular membrane antigens, and a diffuse pattern covering the entire surface of the epithelium. Both patterns were associated with PUK rather than systemic disease whilst the presence of the lattice pattern was more associated with the onset of the PUK. Immunoblotting of sera to corneal epithelial protein extracts demonstrated that a number of corneal antigens were targeted by antibodies. Two antigens, 54 kDa and 70 kDa, were of particular interest. Antibodies to the 54 kDa antigen, the major corneal-specific antigen, were also detected by enzyme-linked immunosorbent assay (ELISA). Longitudinal studies showed that these antibodies often first occurred after an episode of PUK. Antibodies to the 70 kDa antigen were related to the Wegener's granulomatosis rather than the PUK.

Adult↗
PubMed1992

Smoking habits in psoriasis: a case control study.

We have examined smoking habits in 108 patients with psoriasis, including some with palmoplantar distribution, and compared the results with matched controls from the community. There was a significant association between psoriasis, current smoking status (OR = 2.7, 95% CI 1.44-5.42, P less than 0.01) and smoking habits prior to the onset of disease (OR = 3.75, 95% CI 1.68-9.47, P less than 0.001). There was also a marked dose-response relationship; the relative risk of psoriasis in those currently smoking more than 20 cigarettes/day was significantly elevated (OR = 5.3, 95% CI 2.1-13.0, P less than 0.001). Separate analysis of patients without palmoplantar distribution of psoriasis showed a significant association with smoking prior to onset of psoriasis (OR = 3.6, 95% CI 1.5-9.8, P less than 0.001). Smoking may play a role in the aetiology of this common skin disorder.

Adolescent↗
PubMed1992

Minimizing the risk of post-operative pyoderma gangrenosum.

A 61-year-old woman with seropositive rheumatoid arthritis developed numerous ulcers due to pyoderma gangrenosum at suture entry/exit sites following an arthroplasty of the right hip when interrupted silk sutures were used to close the skin. When a subsequent arthroplasty was performed on the left hip and subcuticular Dexon sutures were used to close the skin only two small ulcers developed. Sixteen cases of pyoderma gangrenosum developing in surgical wounds have previously been reported. We recommend that surgery in patients with a history of pyoderma gangrenosum is performed when the pyoderma is clinically quiescent, and that subcuticular sutures are used for skin closure, thus avoiding puncturing the skin surface.

Adolescent↗
PubMed1992

Familial acne fulminans.

We report a patient of acne fulminans with arthralgia whose sister presented with the same disease at an identical age 10 years earlier. The two affected siblings in our family also had identical HLA phenotypes which further supported the role of genetic susceptibility in acne fulminans.

Acne Vulgaris↗
PubMed1992

Multiple glomus cell tumours--treatment by infra-red coagulation.

This report describes the successful use of infra-red coagulation in the treatment of multiple glomus tumours. Glomus cell tumours are usually solitary and an excisional diagnostic biopsy therefore provides effective treatment. Such an approach is, however, impractical in the rarer condition of multiple glomus cell tumours where up to 400 lesions have been described in one patient. We describe a case of multiple glomus cell tumours in which more than 30 tumours were treated quickly and effectively by infra-red coagulation.

Female↗
PubMed1992

Bone abnormalities and severe arthritis in pachydermoperiostosis.

Two patients with pachydermoperiostosis were studied in whom the predominant features at presentation were severe and disabling knee and ankle joint pain in association with distal long bone pain. Analysis of synovial fluid from the knee joints showed non-inflammatory changes. In one patient a bicortical iliac crest bone biopsy specimen, taken after labelling with demeclocycline, showed appositional rates which were increased in cortical bone but reduced in trabecular bone. This mismatching resulted in trabecular osteoporosis, which occurred in association with increased numbers of osteoclasts, findings which suggest differential functional changes affecting the two bony envelopes.

Adolescent↗
PubMed1992

Subacute necrotizing encephalopathy: oxidative phosphorylation defects and the ATPase 6 point mutation.

Subacute necrotizing encephalopathy (SNE) or Leigh's disease is associated with various defects in oxidative phosphorylation (OXPHOS). However, the relationships between these OXPHOS defects and nuclear DNA or mitochondrial DNA (mtDNA) mutations is still unclear. We evaluated three SNE pedigrees (two singleton cases and a pedigree) biochemically for OXPHOS abnormalities and genetically for four mtDNA point mutations. There was a complex I defect in all three pedigrees that was associated with a complex III defect in two individuals. An mtDNA mutation in the ATPase, subunit 6 gene (np 8993) was present in one SNE pedigree. This mutation was maternally inherited, heteroplasmic, produced marked clinical and biochemical heterogeneity between pedigree members, and varied along the maternal lineage at levels ranging from 0% to > 95% of the total mtDNAs. These mtDNA mutations were not present in the other two pedigrees. These observations emphasize the importance of screening for OXPHOS defects and mtDNA mutations in SNE cases.

Adenosine Triphosphatases↗
PubMed1992

Lichenoid eruption induced by low dose captopril.

A patient with congestive cardiac failure developed a rash following captopril treatment. The clinical and histological features were consistent with a lichenoid eruption. The rash spontaneously resolved without any treatment three months after captopril was discontinued.

Aged↗
PubMed1991

The cardio-facio-cutaneous (CFC) syndrome and Noonan syndrome: are they the same?

The distinction between the cardio-facio-cutaneous syndrome (CFC) and the Noonan syndrome (NS) has been based on the presence of a characteristic facies, abnormal hair and skin, and sporadic occurrence. However, all reports of the CFC syndrome comment on the similarity between it and NS, and its sporadic nature is now debatable. This report demonstrates the evolution of the clinical phenotype in a patient with the CFC syndrome and prompts us to question the validity of separating CFC from NS.

Diagnosis, Differential↗
PubMed1991

Evidence for nonrenal synthesis of 1,25-dihydroxyvitamin D in patients with inflammatory arthritis.

The extrarenal synthesis of 1,25-dihydroxyvitamin D [1,25-(OH)2D] is a characteristic of activated macrophages and has been demonstrated to occur in vitro in synovial fluid macrophages from patients with inflammatory arthritis. To examine whether such synthesis occurs in vivo, 19 patients with rheumatoid arthritis, 5 patient controls, and 5 healthy controls received a challenge oral dose of 250 micrograms 25-hydroxyvitamin D3 (25-OHD3) and the serum 1,25-(OH)2D3 response was measured. The median rise in serum 1,25-(OH)2D3 was significantly greater (22 pg/ml) in the rheumatoid patients compared to either of the control groups (8 pg/ml), although the increase in precursor 25-OHD3 was similar in all groups. The serum 1,25-(OH)2D concentration did not rise above the normal upper limit in any of the control subjects but exceeded the normal range in 8 of the rheumatoid patients. Extrarenal 1,25-(OH)2D synthesis is substrate dependent, unlike renal 1 alpha-hydroxylation, which is homeostatically controlled. Excessive 1,25-(OH)2D3 synthesis in the rheumatoid group on raising the 25-OHD3 concentration is indicative of nonrenal production of the hormonal metabolite. Further evidence for substrate-dependent extrarenal synthesis came from measurements of 25-OHD and 1,25-(OH)2D in paired serum and synovial fluid samples from 19 patients with inflammatory arthritis, including 15 with rheumatoid arthritis. Synovial fluid 1,25-(OH)2D was usually present at a lower concentration than serum 1,25(OH)2D, with which it was strongly correlated (Kendall's R = 0.46, P less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗