Disseminated aspergillosis and Cushing's syndrome in a community hospital.
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Aged↗
Biomedical subjects
M J Ahern
Publications and source records attributed to M J Ahern.
Felty's syndrome and HLA-DR antigens.
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Felty Syndrome↗
Adult onset Still's disease or coxsackie polyarthritis?
The clinical manifestations and laboratory findings of two patients with a presumptive diagnosis of coxsackie B4 virus infection are described. A striking feature was the similarity with adult onset Still's disease, with spiking fever, evanescent macular rash, and severe polyarthritis. This latter feature persisted for many weeks and required steroids to control the symptoms. Review of the literature has supported the proposition that many cases of adult onset Still's disease may be due to coxsackie B4 or other viral infection and it is suggested that these agents should be actively sought in future cases.
Adult↗
Studies on the interaction of rheumatoid factor with monosodium urate crystals and case report of coexistent tophaceous gout and rheumatoid arthritis.
Gout and classical rheumatoid arthritis rarely coexist. We report a patient with strong evidence for both these diseases. Possible reasons for the negative correlation between these diseases are summarised. One hypothesis suggests inhibition of surface activity of monosodium urate crystals (MSU) by binding of rheumatoid factor (RF). This was studied with a purified monoclonal rheumatoid factor (mRF) with specificity for IgG. The mRF bound preferentially to MSU coated with IgG in contrast with the IgM control. Inhibition of the neutrophil chemiluminescence (CL) response to IgG-coated MSU was observed at concentrations of mRF that had no effect on the CL response to uncoated crystals. Neutrophil activation was not altered by coating crystals with an IgM control at the same concentration. These data suggest that RF may bind to antigenic determinants on exposed Fc of adsorbed IgG and block the interaction of crystal-bound IgG with Fc receptors. Although crystal coating by RF may modify the expression of gouty arthritis, it is unlikely to be the sole explanation for the dissociation between gout and RA.
Arthritis, Rheumatoid↗
D-penicillamine withdrawal in rheumatoid arthritis.
Thirty-eight patients with rheumatoid arthritis in remission on penicillamine were entered into a prospective, randomised, placebo controlled study to determine the effects of gradual penicillamine withdrawal, to find a serological marker capable of predicting relapse, and to assess the effects of reintroduction of penicillamine. 80% of patients attempting gradual penicillamine withdrawal flared. There was no single serological marker capable of predicting outcome consistently. Decreasing SH levels were highly specific for recurrence of active synovitis but were insensitive. Reintroduction of penicillamine was successful. The implications of these findings, particularly concerning duration of therapy with disease modifying drugs, are discussed.
Arthritis, Rheumatoid↗
Central nervous system toxoplasmosis in homosexual men.
Many opportunistic infections have been associated with an acquired immunodeficiency state in which cellular immune status has been altered. Two homosexual patients are described who presented with fever, peripheral eosinophilia, and a travel history to Haiti and were found to have central nervous system toxoplasmosis. Despite definitive diagnosis and appropriate therapy, both died. Techniques for diagnosis of central nervous system toxoplasmosis are discussed, and the importance of brain biopsy in this clinical situation is stressed. Eosinophilia may serve as an early diagnostic marker for disseminated toxoplasmosis in homosexual patients.
Adult↗
Ankylosing spondylitis and adenocarcinoma of the lung.
A man with long standing and severe ankylosing spondylitis, treated with radiotherapy, developed a primary adenocarcinoma of the lung in an area of apical fibrosis. The significance of this is discussed and the literature reviewed.
Adenocarcinoma, Papillary↗
Granulomatous hepatitis and Seatone.
Granulomatous hepatitis developed shortly after a patient with mild chronic polyarthritis started taking Seatone, a proprietary product. No other likely cause for her liver disease could be determined.
Anti-Inflammatory Agents↗
Toxic epidermal necrolysis after mithramycin.
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Adult↗
Cytomegaloviral infection presenting as a solitary pulmonary nodule.
Cytomegaloviral infection presenting in an immunologically compromised host as a solitary pulmonary nodule has not previously been reported. A patient with a renal transplant and with no pulmonary symptoms was noted to have a single nodule on a chest roentgenogram. At autopsy, this proved to be secondary to cytomegaloviral infection. Differential diagnostic considerations in the immunosuppressed patient are discussed.
Adult↗
Antibiotic treatment of renal carbuncle.
Renal carbuncles in seven young males were successfully treated with long-term administration of penicillinase-resistant antibiotics. Selective renal arteriography provided an accurate means of diagnosis and permitted a trial of medical therapy. All patients experienced a prompt and sustained clincial remission; surigical exploration was thus obviated in all but one instance, in which post-treatment radiographic changes persisted.
Adolescent↗
Pharmacokinetics of cefamandole in patients undergoing hemodialysis and peritoneal dialysis.
The pharmacokinetics of cefamandole nafate, a new parenteral cephalosporin derivative, were evaluated in 11 patients with chronic renal failure (creatinine clearance less than 5 ml/min), including five patients during hemodialysis, four patients during routine peritoneal dialysis, and two patients during the interdialytic period. Peak serum levels of cefamandole were comparable to those observed in patients with normal renal function. Clearance of the drug during the interdialytic period and during hemodialysis and peritoneal dialysis was minimal, with a resultant significant prolongation of serum half-life. The nondialyzability of cefamandole is in contrast with reported studies of cephalothin, where significant reduction of the serum half-life was achieved during hemodialysis but not peritoneal dialysis. The concentration of cefamandole in the peritoneal dialysate after parenteral administration was observed to be bactericidal for many gram-negative pathogens and, with the exception of Streptococcus faecalis, most gram-positive organisms found in bacterial peritonitis in patients with severe renal failure. The present data suggest that if stable bactericidal serum levels of cefamandole are to be maintained during hemodialysis and peritoneal dialysis, a parenteral loading dose must be administered followed by one-half the loading dose every half-life.
Cephalosporins↗
Neutropenia during high dose intravenous oxacillin therapy.
Five patients who developed neutropenia following intravenous administration of high dose oxacillin for serious Staphylococcus aureus infection are described. Neutropenia was reversible with cessation of intravenous oxacillin therapy. Two patients were continued on oral oxacillin without untoward effects.
Adolescent↗
Scleroderma in South Australia: epidemiological observations of possible pathogenic significance.
BACKGROUND: Scleroderma is an autoimmune disorder of unknown cause. Previous epidemiological studies have suggested some regional clustering and associations with occupations involving exposure to silica dusts and hydrocarbons. AIMS: To determine: (i) prevalence and incidence of scleroderma in South Australia (SA), a state with a stable population living in well-defined urban, rural and industrial regions, (ii) hospital separation rates, (iii) cumulative survival rates, (iv) gender and disease subclassification, (v) geographical residency and occupations, (vi) familial associations and (vii) age at onset versus age-specific incidence rate. METHODS: Creation of the South Australian Scleroderma Register (SASR) to identify demographics and clinical and serological features of all scleroderma patients resident in SA. RESULTS: Scleroderma occurs in South Australia with a point prevalence of 23 per 10(5) and an incidence of approximately 1/15th of this. However, this prevalence is high compared with other regional world studies. Scleroderma is predominantly a female disease, with most patients having the limited form of scleroderma characterized by the presence of the anticentromere antibody and a mean survival of 27.6 years. In contrast, diffuse scleroderma is a less benign disease occurring at an early age of onset and has a mean survival of 9.6 years. Scleroderma occurs throughout SA without regional localization. Weak associations are seen in males, but not females, with occupations involving possible environmental exposure to silica or hydrocarbons. Scleroderma rarely occurs in families. CONCLUSION: No strong genetic or environmental influences were found to account for the relatively common occurrence of scleroderma in SA. The age at onset versus age-specific incidence curve suggests that scleroderma can be considered as a stochastic illness involving a number of random events occurring in a predisposed population. These random events may involve mutations of pivotal somatic genes.
Age of Onset↗
Scleroderma renal crisis: poor outcome despite aggressive antihypertensive treatment.
BACKGROUND: Scleroderma renal crisis (SRC) is a rare but feared complication of scleroderma. Angiotensin--converting enzyme (ACE) inhibition has significantly improved survival, but it is unknown whether prophylactic ACE inhibitors will prevent this complication. AIMS: To determine: (i) the frequency of SRC in our cohort of well-characterized scleroderma patients resident in South Australia, (ii) any predisposing clinical and serological features, (iii) median disease duration at which SRC occurs, (iv) possible precipitants, (v) disease outcome, and (vi) whether patients were taking ACE inhibitors prior to onset of SRC. METHODS: Systematic review of the clinical course of all patients registered on the South Australian Scleroderma Register. RESULTS: SRC occurred in 16 patients. This constituted 2.8% of a total scleroderma cohort and 15% of the diffuse scleroderma cohort identified in South Australia. All 16 patients had diffuse cutaneous scleroderma. SRC occurred at a median disease duration of 15 months (range 1 week-11 years). Disease outcome was poor (five deaths, three requiring long-term dialysis and only two patients regaining a normal creatinine) despite aggressive antihypertensive treatment (including ACE inhibitors) in an intensive care or specialized renal unit. Two patients were later able to discontinue dialysis. Only two patients were taking small doses of ACE inhibitors prior to the onset of their SRC. The frequency of Scl-70 was decreased in the SRC group (P = 0.003). CONCLUSION: SRC is a rare event occurring in a small proportion of patients with diffuse scleroderma. The outcome of SRC was poor despite aggressive antihypertensive treatment. It is hypothesized that prophylactic ACE inhibition in susceptible patients might prevent or ameliorate this complication.
Adult↗
The pathology of liver injury induced by the chronic administration of alcohol and 'low-dose' carbon tetrachloride in Porton rats.
We have previously established a model for micronodular cirrhosis by feeding Wistar rats alcohol, in the Lieber-DeCarli liquid diet, and exposing them to 'low-dose' carbon tetrachloride (CCl4) vapour for 10 weeks. This study reports the spectrum of liver pathology seen in male Porton rats exposed to 'low-dose' CCl4 vapour 5 nights/week, 6 h/night while being fed alcohol (300 kcal/L) in the Lieber-DeCarli diet. Micronodular cirrhosis developed in all animals after 5-7 weeks of treatment. The simultaneous administration of silymarin, a putative hepatoprotective agent, in the liquid diet, did not alleviate or prevent the chronic liver injury. The histopathological features of the liver injury are described, with particular emphasis on the presence of small epithelial cells ('progenitor or stem cell'), which appear to be playing a role in liver regeneration.
Administration, Oral↗
Disease-modifying antirheumatic drugs.
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Anti-Inflammatory Agents↗
Genomic instability in scleroderma.
Scleroderma is an enigmatic rheumatic disorder of uncertain etio-pathogenesis. Cancer has an approximately two-fold higher incidence in scleroderma patients than in the general population. There are preliminary data of acquired genetic damage in scleroderma but the significance of these observations are uncertain. To determine somatic mutation frequency at the glycophorin-A (GPA) locus in patients with limited and diffuse cutaneous scleroderma. The GPA assay measures the total somatic mutation frequency (Vf), composed of gene inactivating mutations (NO) and mutations arising from mitotic recombination (NN) in individuals heterozygous for the GPA MN blood group. Mutation frequency was determined using a validated GPA flow cytometric assay using fluorescent labeled monoclonal antibodies specific for the GPA blood groups M and N. This assay detects and enumerates progeny of red blood cell (rbc) precursor cells which have acquired genetic damage resulting in a loss of expression of one of the GPA alleles. It was found that patients with scleroderma (n = 23) had significantly elevated Vf as compared with young healthy controls (p < 0.001) and elderly controls (p = 0.03). Patients with diffuse scleroderma had higher mean Vf as compared with limited scleroderma (p = 0.055). In comparison with controls, patients with scleroderma exhibit a higher proportion of mitotic recombinant mutations than inactivating mutations (p < 0.002). There was no correlation between Vf and disease duration, age at onset or autoantibody status. We have documented evidence of acquired genetic damage at the GPA locus in scleroderma. Evidence of acquired genetic damage in this disorder may be importance in explaining both the etio-pathogenesis of scleroderma and the association of scleroderma with cancer.
Adolescent↗