Search PubMedSearch

Biomedical subjects

R Rankin

Publications and source records attributed to R Rankin.

At least 19 recordsLinked to original sources

Renal insufficiency is a component of COACH syndrome.

Two sisters, ages 23 years and 6 years, respectively, were found to have congenital ataxia, bilateral coloboma, mental retardation and abnormal liver function. Magnetic resonance imaging showed cerebellar vermis hypoplasia in the younger girl and liver biopsy showed hepatic fibrosis in the older sister. This combination of findings suggested a diagnosis of COACH syndrome which is characterized by hypoplasia of cerebellar vermis, oligophrenia, congenital ataxia, coloboma, and hepatic fibrosis. COACH syndrome is a newly recognized condition. So far, five cases have been reported from three sibships. We report two additional cases from one sibship and suggest that several other cases may already exist in literature that were not recognized as having COACH syndrome. The occurrence of multiple cases in single sibships suggests autosomal recessive inheritance. In addition to previously described findings typical of COACH syndrome, the older of our patients showed progressive renal insufficiency with fibrocystic changes on renal biopsy. Renal function has not been described consistently in previous reports of COACH syndrome but has been abnormal in all cases in which it has been investigated. We suggest that renal insufficiency should be considered a common manifestation of COACH syndrome.

Abnormalities, Multiple

Miliary tuberculosis presenting as infection of a pacemaker pulse-generator pocket.

A seventy year old woman had a permanent VVI pacemaker inserted in 1983 for complete heart block and presented ten years later with purulent discharge from the generator pocket. During a prolonged pyrexial illness, she developed renal and respiratory failure and her illness was complicated by recurrent ventricular fibrillation. The patient died on her 31st hospital day. Subsequent histological and microbiological investigation revealed widespread miliary tuberculosis which included involvement of myocardial tissue, great vessels and the pacemaker pocket. To our knowledge, this is the first reported occurrence of miliary tuberculosis involving a permanent pacemaker system. Furthermore, the granulomatous myocarditis which occurred as part of the miliary picture is a rare occurrence and possibly explains the recurrent ventricular fibrillation.

Aged

The frequency of venous thrombosis in cemented and non-cemented hip arthroplasty.

We randomised 250 patients undergoing unilateral, elective hip arthroplasty for osteoarthritis to receive either a cemented or a non-cemented Mallory Head prosthesis. Aspirin was used as prophylaxis against thromboembolism during the first half of the study and adjusted-dose warfarin during the second half. Postoperatively, all patients were asked to have bilateral venography and 80% agreed. All were evaluated clinically for pulmonary embolism. There was no difference in the frequency of deep-venous thrombosis between the two groups (50% cemented nu 47% non-cemented, p = 0.73; 95% CI of the difference -13.6% to 19.3%). Three of the 64 patients (5%) in whom venography had demonstrated isolated distal thrombi developed pulmonary emboli.

Aged

The role of colour flow Doppler ultrasonography in the investigation of disease in the eye and orbit.

Colour flow Doppler ultrasonography is a relatively new technique in which colour-encoded representations of flowing blood are imposed over a conventional grey-scale ultrasound image. This allows interrogation of a relatively large volume of tissue to determine the presence and direction of blood flow. The waveform tracings are then evaluated by means of conventional Doppler imaging. We demonstrate examples of normal vascular structures in the orbit and describe the application of this technique in the investigation of vascular disease, such as carotid-cavernous fistula and ischemic disease. The technique can also be used to evaluate ocular membranes and masses. Colour flow Doppler imaging promises to be an important advance in ophthalmic ultrasonography.

Blood Flow Velocity

Cutaneous malignant melanoma, Scotland, 1979-89. The Scottish Melanoma Group.

The Scottish Melanoma Group (SMG) was established in 1979 to assess mortality from and incidence, features, pathological data, and management of cutaneous malignant melanoma in Scotland. Incidence during the first five years and five-year survival have already been reported. We now have data about incidence and mortality over eleven years in relation to anatomical site and pathological types. From 1979 to 1989, 1354 male and 2459 female patients with primary cutaneous malignant melanomas were first diagnosed in Scottish residents. The incidence rate per 100,000 population per year has increased from 3.4 in 1979 to 7.1 in 1989 for men, and from 6.6 to 10.4 for women. The overall increase over eleven years is 82% (7.4% per year). The greatest rates of increase are seen in lesions of the superficial spreading histogenetic type, arising on the female leg and the male trunk. Following public education programmes started in 1985, the proportion of all melanomas less than 1.5 mm thick has shown a sustained and significant increase. Mortality data for 1661 patients for whom a minimum of five-year follow-up is available shows five-year survival of 71.6% overall (77.6% for women, 58.7% for men). The survival advantage for women persists when appropriate statistical adjustment is made for thickness, ulceration, and histogenetic type. These data are useful in designing public education programmes aimed at both primary and secondary prevention of melanoma and in auditing changes in trends that might result from such education.

Adolescent

A velocity evaluation phantom for colour and pulsed Doppler instruments.

We describe a phantom designed to evaluate the velocity measurements made with colour and pulsed Doppler instruments. Using a belt to translate a large volume of semi-rigid material through the entire Doppler sample volume eliminates many of the problems associated with flow and string phantoms. A servo-motor with feedback circuitry ensures accurate control of the belt velocity with an uncertainty in the mean velocity of 0.14%. The phantom provides velocities with typical variations of 0.07 cm/s. We have demonstrated the usefulness of this phantom by evaluating the linearity and accuracy of three pulsed Doppler instruments over belt velocities ranging from 0 to 80 cm/s. In addition, the measurements show the effects of the wall filter at low belt velocities. Using this phantom, we have quantified the accuracy, linearity and precision of the velocity measurements made by three colour Doppler instruments. The results also show regions where the colour instruments are aliased and where the wall filter dominates.

Calibration

Dipetalonema viteae and Brugia pahangi transplant infections in gerbils for use in antifilarial screening.

Transplanted infections of Dipetalonema viteae and Brugia pahangi have been evaluated as tools for experimental chemotherapy. Attempts were made to establish these filariae in similar pharmacokinetic sites within the same host, so that direct comparisons of in vivo drug susceptibilities could be made. Unfortunately, it was not possible to establish B. pahangi in the subcutaneous tissues, the preferred site of D. viteae. Therefore, intraperitoneal B. pahangi and subcutaneously implanted D. viteae in gerbils were used for the study. D. viteae infections were significantly enhanced by concomitant infections with B. pahangi, while B. pahangi infection rates were unaffected by the presence of D. viteae. Experiments with amoscanate, CGP6140 and Mel W demonstrated the importance of employing both B. pahangi and D. viteae for antifilarial discovery work and the fundamental effect of parasite location on drug efficacy. D. viteae rapidly migrate from the peritoneal cavity of gerbils following implantation; twenty one hours after infection 73% of transplanted worms were found in the subcutaneous tissues. It was shown that the migration response could be used as a stringent parameter for demonstrating antifilarial activity. D. viteae were exposed to antifilarial drugs for 24 hours in vitro, washed and implanted into the peritoneal cavity of gerbils. At autopsy, 5 days later, 10(-8)M ivermectin and milbemycin D had prevented migration; CGP6140, amoscanate, suramin, flubendazole and furapyrimidone were also detected at less than 10(-6)M using this parameter. In all cases the migration response was more sensitive to drugs than parasite kill. Ivermectin's ability to inhibit worm migration through the tissues is discussed, with respect to the role of itinerant males in the reproductive cycle of Onchocerca volvulus.

Animals

The potential of mice as animal models for antifilarial screening.

Transplanted infections of Brugia pahangi and Dipetalonema viteae in male BALB/c and CDI mice were investigated as models for evaluating potential antifilarial compounds. The physiology and genetics of the above mouse strains are better defined than any of the rodent species currently used for primary in vivo screening, facilitating a more reproducible means for predicting the filaricidal activity of compounds. The recoveries of B. pahangi macrofilariae, implanted intraperitoneally were greater than or equal to 50% up to six weeks after implant in both CDI and BALB/c mice. The recoveries of D. viteae macrofilariae, implanted subcutaneously, were greater than 50% up to four weeks post implant but had fallen to less than 30% by six weeks. The survival of B. pahangi and D. viteae macrofilariae simultaneously implanted into mice mimicked that seen with the mono-infections, but significantly better recoveries were obtained from dual implanted CDI mice compared to the BALB/c mice when the numbers of macrofilariae implanted were varied. Standard antifilarials were evaluated against D. viteae and B. pahangi dual implanted into either CDI mice or gerbils. The mouse dual implant detected significant worm reductions against D. viteae, B. pahangi or both with all antifilarials tested except CGP 6140. Similarly under the test conditions CGP 6140 was not detected in the gerbil assay, but there were marked differences in the results obtained with the mice and gerbil models. The reasons for these differences are discussed.

Animals

S-100 protein: a prognostic indicator in cutaneous malignant melanoma?

A series of 215 cases of cutaneous malignant melanoma referred to a single department of clinical oncology between 1940 and 1969 was studied to assess the accuracy of the Breslow thickness and the role of S-100 protein in predicting the clinical prognosis. Histological examination of these tumours showed that although the Breslow thickness correlated well with prognosis, in a significant number of cases it did not reliably forecast clinical outcome. From this series, tissue from those patients who survived disease-free for more than 10 years and those who died within a year of diagnosis was stained immunohistochemically for S-100 protein. Contrary to the findings of earlier studies, strong staining for S-100 protein was associated with improved survival (P less than 0.001). A marked increase in the incidence of cutaneous malignant melanoma was noted during the period of the study.

Biomarkers, Tumor

Seminoma of testis: the value of aggressive therapy, and risk of late bilateral involvement.

The results of aggressive therapy for seminoma of the testis have improved dramatically during the past decade. A patient who eight months after orchidectomy had a pulmonary metastasis excised and who nine years later developed a seminoma in the contralateral testis is described. One consequence of the much longer survival time now attainable is that an increasing number of cases with metachronous testicular seminomas may be encountered in the future.

Adult

Malignant melanoma in Scotland 1979-1983.

1318 cases of primary malignant melanoma of the skin first presenting in Scotland in the years 1979-83 and registered with the Scottish Melanoma Group are described. The annual age-adjusted incidence rate is 4.75 per 100 000 for females and 2.77 per 100 000 for males. Incidence rose by an annual average of 2.5% over the five-year period. The female: male ratio was 2:1, and 48.5% of all cases were of the superficial spreading histogenetic variety. Five-year survival rates for the patients registered in 1979 confirm the prognostic value of the tumour-thickness (Breslow) measurement. They were 93% for patients with tumours less than 1.5 mm thick (39% of patients), 67% for those with tumours 1.5-3.49 mm thick (30%), and 37% for those with tumours thicker than 3.5 mm (31%). This study shows that methods for early recognition of thin tumours should be encouraged.

Adolescent

Inhibition of contact sensitivity reactions to DNFB by topical cyclosporin application in the guinea-pig.

Contact sensitivity skin reactions to dinitrofluorobenzene (DNFB) were inhibited by twice daily topical application of cyclosporin (CsA, 2%) in normal guinea-pigs and in those with enhanced contact sensitivity reactions following pre-treatment with cyclophosphamide. In contrast to oral administration of CsA (25 mg/kg) for 4 days, topical application of the drug over the same period did not result in systemic absorption (as measured by radioimmunoassay) or in any evidence of nephrotoxicity.

Administration, Topical