Soft tissue sarcoma, aplastic anaemia, and exposure to pesticides.
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Biomedical subjects
Publications and source records attributed to L E Hughes.
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Breast pain (mastalgia) and macroscopic breast cysts present commonly. Mastalgia may be improved by dietary manipulation to reduce saturated fat or supplement essential fatty acid intake. Fatty acid profiles were measured in women with mastalgia and breast cysts, before and during treatment with evening primrose oil, a rich source of essential fatty acids. The fatty acid profiles of both groups of patients were abnormal, with increased proportions of saturated fatty acids and reduced proportions of essential fatty acids. Treatment with evening primrose oil improved the fatty acid profiles towards normal, but this was not necessarily associated with a clinical response.
Between 1971 and 1991, details of 67 women with perianal Crohn's disease were recorded prospectively using the Cardiff classification. Two groups were identified according to the presence (n = 29) or absence (n = 38) of anorectal Crohn's fistula involving the vagina. Patients in both groups were of a similar age and had had Crohn's disease for a similar period before diagnosis of perianal involvement. The incidence of associated perianal lesions, superficial ulcers, cavitating ulcers, other fistulas and strictures was not significantly different between the two groups. A greater proportion of patients with anorectal-vaginal fistulation (n = 15) had distal intestinal Crohn's disease (rectal or contiguous colorectal) compared with women with no vaginal fistulation (n = 14). A range of therapies was used to manage women with perianal Crohn's disease, from local surgery to a defunctioning stoma and/or proctectomy. Only 13 of 38 women with perianal Crohn's disease but no vaginal fistula required a defunctioning stoma or proctectomy, whereas 18 of 29 with anorectal-vaginal fistulation underwent these procedures (P < 0.05). A vaginal fistula has a considerable adverse effect on the outcome of perianal Crohn's disease.
Assessment of the efficacy of therapeutic approaches to anal lesions of Crohn's disease is frustrated by the lack of precise definition of its various manifestations. A classification that is clinical and based on anatomic and pathologic aspects is presented; it has been derived from a 20-year prospective study of anal Crohn's disease in Cardiff. Conceptually, the classification is analogous to the TNM system for cancer. The main classification (U.F.S.) defines the presence of Ulceration, Fistula/abscess, and Stricture, qualified by numeric values reflecting severity (0 = not present, 1 = limited clinical impact, and 2 = severe). A subsidiary classification (A.P.D.) defines Associated conditions, Proximal intestinal involvement, and Disease activity. In addition, the classification may be used in a detailed form for research or comparative purposes or in a simple form defining only the dominant lesions for routine clinical use. General use of the classification would make it possible to compare in detail incidence, management, and results of treatment in different centers.
The typical macroscopic features of Crohn's disease have been well described and are widely regarded as sufficient to diagnose the disease at laparotomy. We report six patients undergoing laparotomy for symptomatic Crohn's disease, shown radiologically, who were found to have macroscopically normal small bowel despite careful examination of the bowel by an experienced surgeon. In four cases resection was deferred, but all subsequently deteriorated and required further surgery. Minor abnormalities found by balloon examination of the terminal ileum prompted resection in two further patients. Histology showed an unusually superficial distribution of inflammation, which could explain the negative findings at laparotomy. We conclude that normal laparotomy findings alone do not exclude a diagnosis of clinically important small bowel Crohn's disease. Crohn's disease should be considered in patients with persisting symptoms after negative laparotomy.
Mastalgia commonly presents to medical practitioners. The majority of patients can be managed by exclusion of cancer and reassurance. In some the severity of pain affects the quality of life and drug treatment should be considered. Since its inception 324 patients with cyclical mastalgia and 90 with non-cyclical mastalgia have received a therapeutic trial of drug treatment in the Cardiff Mastalgia Clinic. Overall 92% of those with cyclical mastalgia and 64% with non-cyclical mastalgia obtained a clinically useful response to therapy. Danazol was the most effective drug, with bromocriptine and evening primrose oil having equivalent efficacy. Many fewer adverse events were complained of by patients treated with evening primrose oil than danazol or bromocriptine.
Benign breast disorders can be considered from four points of view: clinical presentations, clinical significance, management, and pathogenesis. Understanding the pathogenesis is important for rational management and for assessing clinical significance. Clearly understood nomenclature is also important. Clinicians have tended to concentrate on the condition usually known as fibrocystic disease, a clinical condition (painful nodularity), but a terminology which relates to a histological picture of fibrosis, cyst formation, and epithelial hyperplasia, now known to occur in both asymptomatic and symptomatic breasts. To address these problems the aberrations of normal development and involution (ANDI) concept has been proposed as a framework for benign breast disorders which is comprehensive, accurate in terminology, and based on pathogenesis. For each disorder, there is a spectrum from normal through mild abnormality (aberration) and in some cases (only) to disease. This concept encompasses pathogenesis, clinical and histological significance, and general principles of management. It has proved particularly useful in giving an understanding of benign breast disorders to doctors in training.
The differentiation of subungual haematoma from benign and malignant nevoid lesions is a common clinical problem. To determine the incidence of benign subungual naevi in the general population, the digits of the hands and feet of 1000 white patients were examined. These were consecutive patients presenting to hospital for problems unrelated to the finger or toe-nail. Eight patients were found to have pigmented subungual lesions. All proved to be haematoma, although there was no history of injury. Benign subungual naevi are rare in caucasians, so subungual nevoid lesions should be regarded as malignant until proved otherwise.
A consecutive series of 644 women who presented with breast nodularity between 1976 and 1982 have been followed up to determine their rate of subsequent breast cancer. Fifteen women have developed breast cancer, 14 of these were among 352 women with an aspirated cyst (relative risk 4.4). Women with multiple cysts had the highest risk and women with breast nodularity had no excess risk. Review of histology specimens from those women who had undergone biopsy showed an excess of florid epithelial hyperplasia in women who subsequently developed breast cancer and women with multiple aspirated cysts were more likely to have florid epithelial hyperplasia. Multiple cysts are clinical markers of histological breast proliferation and women who have had multiple breast cysts aspirated have an increased risk of breast cancer and should be advised to practice regular self examination.
Terminology in benign breast conditions has been confused by multiplicity of terms which do not relate accurately to clinical or histological patterns. Further confusion arises because terminology is not based on sound concepts of pathogenesis. The ANDI classification has been put forward as a nomenclature based on pathogenesis to replace the division of benign breast disorders into 'normal' and 'disease'. It recognizes that a spectrum exists for most conditions which extends from normal, through mild abnormality--'aberrations'--to disease. This classification allows precise definition of an individual patient problem in terms of pathogenesis, histology and clinical implications. It has proved helpful in deciding rational clinical management and in teaching the significance of benign breast disorders.
Many terms, including duct ectasia, secretory disease, periductal mastitis, plasma cell mastitis, have been used in connection with a variety of clinical conditions associated with nipple discharge, non-puerperal sepsis and nipple retraction. The confused nomenclature reflects the uncertainties regarding the singularity or inter-relationships of the main elements--dilated ducts, periductal inflammation, bacterial infection and nipple retraction. Recent clinical studies combined with new histological and bacteriological information have set the scene for better understanding of the pathogenesis and management of these clinical conditions.
Twenty-two individuals who had suffered from local recurrence of breast cancer were interviewed to determine psychosocial morbidity. Psychometric assessment using the Hamilton Anxiety Scale, the Eysenck Personality Questionnaire and the Montgomery Asberg Depression Rating Scale was conducted prior to clinical evaluation including a structured interview, the Composite International Diagnostic Interview. Lifetime and current psychiatric diagnoses were established. Ten (45%) of the recurrence group had current psychiatric illness (anxiety and depression) at the time of local recurrence, a similar prevalence to that described by others at mastectomy. Previous psychiatric illness and trait neuroticism are predictive of vulnerability to psychiatric morbidity at local recurrence. These results suggest that a significant proportion of patients with local recurrence suffer from major depressive illness.
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Two hundred women with breast cysts proven by aspiration were entered into a randomized double-blind trial of Efamol (evening primrose oil) at a dose of 6 capsules daily or equivalent placebo dose for a year. Cysts were categorized by initial electrolyte composition, and follow-up continued for 1 year posttherapy. Recurrent cyst formation in the first year was slightly (but not significantly) lower in the Efamol group compared with the placebo-treated group. The Efamol treatment was well tolerated as the dropout rate was only 7% and equal in both the active and placebo groups. The initial electrolyte composition did not predict for cyst recurrence.
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The xeromammograms of 128 women with breast cancer were coded radiologically according to Wolfe's gradings. The mastectomy specimens were then histologically examined for the presence of ductal abnormality. The ducts were found to be normal in the cases coded as normal radiologically but they were not always abnormal histologically when considered to be abnormal radiologically. 'Ductal prominence' may be due to duct ectasia, intraduct hyperplasia or periductal collagenosis.
The distribution, size and density of the apocrine glands in hidradenitis suppuritiva as compared with those in axillary hyperhidrosis and normal controls have been studied. There are no significant differences in the size or density of the apocrine glands in hidradenitis suppuritiva as compared with normal controls. The apocrine glands in axillary hyperhidrosis are significantly larger than those in axillary hidradenitis and in normal controls. A simple method that can be used to demonstrate the distribution of the apocrine glands preoperatively is described. This has proved useful in delineating the area which it is necessary to excise to obtain complete apocrine gland excision in the diseased area. The apocrine gland-containing skin in the diseased region does not always correspond exactly to the hair-bearing area in that region.