Showing papers by "St Thomas' Hospital published in 1980"

A study of benign chronic bullous dermatosis of childhood and comparison with dermatitis herpetiformis and bullous pemphigoid occurring in childhood.

Abstract: Summary Eighteen patients with benign chronic bullous dermatosis of childhood were studied and the findings compared with those of dermatitis herpetiformis (twenty-two cases) and bullous pemphigoid (five cases) beginning in childhood. The patients with benign chronic bullous dermatosis of childhood had a moderately pruritic bullous eruption with maximal involvement of the pelvic and perioral regions which tended to occur at an earlier age than either dermatitis herpetiformis or bullous pemphigoid. In contrast to dermatitis herpetiformis one-third of the cases with benign chronic bullous dermaiosis of childhood went into remission. Evidence of coeliac disease was only found in the dermatitis herpetiformis group. Surprisingly both diseases shared HLA-B8. A linear BMZ band of IgA was detected on direct immunofluorescence in all but one of the cases with benign chronic bullous dermatosis of childhood and circulating antibodies were detectable in two-thirds. Routine histopathology was of little value in distinguishing between benign chronic bullous dermaiosis of childhood and dermatitis herpetiformis or bullous pemphigoid. Several paradoxes have yet to be explained before it can be determined whether benign chronic bullous dermatosis of childhood is a variant of dermatitis herpetiformis or linear IgA disease.

Cardiac involvement in secondary haemochromatosis: a catheter biopsy study and analysis of myocardium

Abstract: Although high blood transfusion regimens have improved the life expectancy of the patient with Thalassemia Major, cardiac failure and arrhythmias remain a cause of early death It is not certain whether the massive myocardial iron deposition found in such patients is preventable by intensive chelation therapy This study evaluates endomyocardial biopsy as a method of assessing myocardial iron deposition Of four patients with clinical and biochemical evidence of severe haemochromatosis, only one had a myocardial iron content comparable to that found in severe haemochromatotic myocardium The one patient with cardiac failure had an endomyocardial iron content within the normal range Studies of the iron distribution in haemochromatotic myocardium demonstrate that the subendocardial myocardium contains only half the iron content of the subepicardial layer, and there is a large sampling variation It is concluded that catheter endomyocardial biopsy is an insensitive method of determining early myocardial deposition because of the location of iron and the variability of the sampling Studies of the nature of the myocardial iron protein with CM32 cation exchange resin chromatography show that there is a large increase in the haemosiderin: ferritin ratio (5:1) in iron overload myocardium as compared with the normal heart (2:1) Similar results have been observed in the liver with iron overload, where the increase in hepatic haemosiderin was associated with greater lysosomal fragility It is possible that myocardial cell damage may also occur by the rupture of iron engorged lysosomes

Evaluation of the roles of lipoprotein lipase and hepatic lipase in lipoprotein metabolism: in vivo and in vitro studies in man.

Abstract: The roles of lipoprotein lipase (LPL) and hepatic lipase in very low density lipoprotein (VLDL) and VLDL remnant metabolism were investigated by (1) in vivo studies where the kinetics of VLDL-apo B removal were measured in patients with non-functioning lipoprotein lipase systems, and (2) in vitro studies where the relative capacities of hepatic lipase and LPL to hydrolyse the triglyceride (TG) of different lipoprotein substrates was measured. The results indicated that VLDL-apo B removal was not impaired in patients with non-functional LPL, nor ws there any apparent abnormality in the conversion of VLDL-apo B to intermediate- (IDL) and low (LDL) density lipoprotein-apo B. Post-heparin plasma hepatic lipase activity against VLDL was normal in these subjects. Purified normal hepatic lipase had a similar Km for VLDL-TG hydrolysis (1.57 mmol/l) to that of LPL (1.49 mmol/l). However, at equal lipoprotein TG concentration, hepatic lipase had increasing activity with lipoproteins of decreasing particle size, in the order chylomicrons much less than VLDL of Sf 100-400 less than VLDL of Sf 60-100 less than VLDL of Sf 20-60 less than IDL. The mean contribution of hepatic lipase to VLDL-TG hydrolysis by post-heparin plasma was 35% in normal controls, but the contribution to IDL-TG hydrolysis was significantly higher (mean - 58%). It is concluded that hepatic lipase plays a significant role in VLDL and, especially, IDL metabolism, at least in patients with non-functioning lipoprotein lipase.

High-Density Lipoprotein Metabolism

Abstract: Publisher Summary Cholesterol is an essential structural component of all cell membranes. The cholesterol of extrahepatic tissues appears to be derived from receptor-mediated uptake of plasma low-density lipoprotein, to a lesser extent from endogenous synthesis, and also from direct transfer from cholesterol-rich lipoproteins. Most tissues are unable to catabolize cholesterol or excrete it from the body. The transport of cholesterol from such tissues to sites of catabolism and excretion might be a function of high-density lipoproteins (HDL). This possibility was raised when it was found that the cholesterol content of HDL could be increased by incubation with Celite dispersions bearing adsorbed cholesterol. On the basis of the foregoing observations, it seems probable that the transformation of nascent HDL to plasma HDL in vivo involves at least the following processes: the esterification of surface cholesterol and hydrolysis of surface lecithin by lecithin: cholesterol acyl-transferase (LCAT); the movement of the resultant lysolecithin to albumin; the movement of a proportion of the resultant non-polar cholesteryl ester into the inner hydrocarbon region of the phospholipid bilayer, transforming it into spherical pseudomicellar particle; the transfer of other cholesteryl ester molecules, together with apoprotein E, to triglyceride-rich lipoproteins; the transfer of apoprotein from triglyceride-rich lipoproteins to HDL; and fusion of HDL 3 so formed with aggregates of phospholipid, unesterified cholesterol and apoprotein C displaced from the surfaces of very low-density lipoproteins (VLDL) and chylomicrons during triglyceride hydrolysis.

Acanthosis palmaris: tripe palms. A distinctive pattern of palmar keratoderma frequently associated with internal malignancy.

Abstract: Summary We describe five patients who showed a distinctive pattern of late onset hyperkeratosis and acanthosis of palms and fingers. The epidermal thickening gave rise to a rugose appearance with broadened rete ridges bounded by deep sulci (tripe palms). One of these patients had malignant acanthosis nigricans, two also had carcinomata with the palmar changes as a forme fruste of acanthosis nigricans and two patients had pruriginous dcrmatoses with palmar changes of diffuse lichenification which were reversible. The relationship between palmar-plantar keratoderma and malignancy is discussed.

Rubella-Specific Serum and Nasopharyngeal Antibodies in Volunteers with Naturally Acquired and Vaccine-Induced Immunity after Intranasal Challenge

Abstract: Thirty-nine volunteers, who were either naturally immune to rubella virus or immune as a result of vaccination with RA 27/3, Cendehill, or To-336 vaccines, were challenged intranasally with high-titered RA 27/3 virus. Before and after challenge, rubella-specific IgG and IgA in serum and nasopharyngeal washings were measured by hemagglutination inhibition, neutralization, and radioimmunoassay. The reinfection rate (at least a fourfold rise in titer of serum antibody by one or more tests) was highest among recipients of Cendehill vaccine. Significant rises in titer were most frequently detected by radioimmunoassay for rubella-specific IgG. After challenge of immune volunteers, rubella-specific IgM was detected in six of the 29 with vaccine-induced immunity. Although high levels of rubella-specific serum and nasopharyngeal IgA before challenge appeared to be associated with protection in recipients of RA 27/3 vaccine, no level of any antibody tested was invariably associated with protection. For comparison, volunteers with vaccine-induced immunity challenged intranasally with the same dose of vaccine after inactivation did not show evidence of reinfection.

Plasma high-density lipoprotein metabolism in subjects with primary hypertriglyceridaemia: altered metabolism of apoproteins AI and AII.

Abstract: 1 The metabolism of the major proteins of plasma high-density lipoprotein (HDL), apoproteins AI and AII, have been studied in 10 normotriglyceridaemic subjects and in 11 hypertriglyceridaemic subjects (plasma triglyceride 45--25 mmol/l) by kinetic analysis of the plasma specific radioactivity versus time curves of the apoproteins after intravenous injection of autologous 125I-labelled high-density lipoprotein 2 The specific radioactivity versus time curves of both apoproteins (followed for 14 days) were bi-exponential in all subjects 3 The plasma apoprotein AI and AII concentrations were significantly lower in the hypertriglyceridaemic subjects than in the normotriglyceridaemic subjects Kinetic analysis showed that this was associated with a lower rate of synthesis of apoprotein AI (P < 001) and a higher fractional catabolic rate of apoprotein AII (P < 001) in the hypertriglyceridaemic group 4 There were no significant differences between the two groups in the synthetic rate of apoprotein AII, the fractional catabolic rate of apoprotein AI or the intravascular/extravascular distributions of the apoproteins 5 Thus hypertriglyceridaemia appears to be frequently associated with divergent abnormalities of the metabolism of the major high density lipoprotein apoproteins

Visual acuity, its development and amblyopia.

Abstract: I became intrigued with amblyopia, which is a loss of visual acuity without any obvious pathology, through my contacts with patients in the electrodiagnostic clinic. But realizing that we needed to know more about the physiology of normal visual acuity before we investigated the problem of amblyopia, we began to work in search of the physiological basis of normal acuity and then moved on to the problem of amblyopia.

Clinical Experience with Intravesical Prostaglandin E2| A Prospective Study of 36 Patients

Abstract: Summary— In a prospective study, intravesical prostaglandins were given to 36 patients who had urinary symptoms associated with a poorly functioning detrusor; 67% of these patients were found to be in chronic retention. Using standard urodynamic techniques, 72% showed objective evidence of an immediate improvement in detrusor function and there was prolonged therapeutic benefit in 39%. A prolonged response occurred only in patients who had an intact sacral reflex arc and urodynamic evidence of a pathologically enlarged bladder, and who did not have radiologically demonstrable outflow obstruction. The use of intravesical prostaglandin to stimulate the detrusor was also found to be of value as a urodynamic technique for the investigation of the lower urinary tract.

Supranuclear palsy of eyelid closure

Abstract: An inability to close the eyes voluntarily while at the same time retaining reflex blinking is described in three patients with Creutzfeldt-Jakob's disease. In one patient it is was an early symptom but in the others it formed part of a generalized spastic paralysis. By analogy with other reports it is deduced that in the case of a supranuclear palsy of this type the site of the lesion is probably in the cortical neurons rather than in the descending motor tracts.

Vitamin D prophylaxis and osteomalacia in chronic cholestatic liver disease

Abstract: Bone histology was examined in 32 patients with chronic cholestatic liver disease, of whom just over one half were receiving high-dose parenteral vitamin D therapy. Four patients had histological evidence of osteomalacia; two of these were receiving vitamin D therapy, and showed only very mild osteomalacia, while the remaining two untreated patients had more severe bone disease. Plasma 25-hydroxyvitamin D levels were normal in all vitamin D-treated patients, and serum calcium concentrations were significantly higher in the treated group. Clinical symptoms and biochemical and radiological findings were unreliable in predicting osteomalacia. It is concluded that osteomalacia is uncommon in patients with chronic cholestatic liver disease irrespective of whether or not they are receiving vitamin D therapy. However, high-dose parenteral vitamin D prophylaxis protects against vitamin D deficiency and may also prevent the development of severe bone disease.

Social factors and height gain of primary schoolchildren in England and Scotland

Abstract: Associations between height and certain social factors are known to persist throughout the primary school years. To discover whether the height differences between social groups are increasing or diminishing during this time, heights of 7569 English and Scottish 5 to 10 year olds measured in 1972 and 1973 were examined, together with information about number of siblings, father's social class and father's employment status. With the exception of five year old children of unemployed fathers, any increases in height differentials were no more than would be expected from the dependence of height gain on initial height. No evidence was found of absolute decrease in height differences over one year, for any of the three social factors considered, but children from larger sibships grew more than expected given their starting heights. Thus associations between attained height and social factors in five to ten year old children arise almost entirely before the age of five and do not alter appreciably during the primary school years.

Rubella immunity by four different techniques: results of challenge studies.

Abstract: When 42 sera with low or inconsistent levels of haemagglutination-inhibiting (HAI) antibodies were tested by single radial haemolysis (SRH) radioimmunoassay (RIA), and enzyme-linked immunoassay (ELISA), RIA was shown to be the most reliable test for detecting low levels of antibody. SRH, however, was found to be an acceptable alternative screening test for rubella antibodies and was more reliable than HAI. Although SRH plates prepared in our own laboratory failed to detect antibodies in six sera, in five of the six, antibodies were only at a low level (RIA titre 1:20-1:80). OriVir plates (Orion Diagnostica, Finland) failed to detect low levels of antibody in only three sera. There were six (14.3%) sera which were false positives in the HAI test. These women were shown to be seronegative by radioimmunoassay and, when three of these six volunteers were vaccinated, they developed a typical primary immune response which resembled that developed by 43 seronegative women following vaccination. Fifteen of the young women with consistently low HAI titres and one woman who was seronegative by HAI but seropositive by RIA and ELISA were subsequently vaccinated. Six (37.5%) of these women showed no significant rise in titre by any of the tests employed, while ten had a significant rise in titre, detected by at least one test, with a low level of rubella-specific IgM detectable in one.

The Problem of Herpetic Whitlow Among Hospital Personnel

Abstract: Five cases of nosocomially acquired herpetic whitlow are presented; three cases developed following a common exposure. All infections were acquired despite the use of surgical gloves as recommended by existing guidelines on control of nosocomial infection. In two cases the mistaken diagnosis of pyogenic paronychia led to surgical intervention, with excessive morbidity and disability. Among the five cases, morbidity ranged from several days of unemployment to permanent sequelae necessitating a career change. Revised guidelines for infection control should include the use of gloves on both hands whenever patient care involves contact with a debilitated patient with herpes simplex labialis.

National Study of Health and Growth: Respiratory Symptoms and Helght in Primary Schoolchildren

Abstract: An analysis of the relationship between the height of primary schoolchildren in England and Scotland and their respiratory symptoms as reported by their parents showed that children with morning cough, day and night cough, wheeze and asthma or bronchitis in the preceding 12 months were shorter than those without these symptoms. In subsequent analyses account was taken of differences in social and biological factors as measured by father's social class, sibship size and parent's height. The relationship between height and asthma remained statistically significant. Morning cough and day and night cough were related to short stature in children living in more deprived conditions. The number of respiratory symptoms was inversely related to height but once the children with asthma were excluded from the analysis the trend tended to disappear. There was also some evidence that children with respiratory symptoms gained less height in a year than those without. Our results indicate that, with the exception of asthma, the association between respiratory symptoms and height in primary school children is only marginal. Nevertheless in lower social classes and where sibling size is large common respiratory symptoms are related to short stature.

Steatocystoma multiplex with unusually prominent cysts on the face.

Abstract: SUMMARY A patient is described who had unusually large facial cysts which histologically were typical of steatocystoma multiplex. An excellent cosmetic result was obtained after plastic surgery.

Herpes gestationis and bullous pemphigoid: a disease spectrum.

Abstract: SUMMARY A patient with herpes gestationis has persisted with clinically active disease for 8 years post-partum. She also had Graves' disease, alopecia totalis and ulcerative colitis. The case demonstrates the close overlap between herpes gestationis and bullous pemphigoid.

Amyloid vascular disease: cord-like thickening of mucocutaneous arteries, intermittent claudication and angina in a case with underlying myelomatosis

Abstract: SUMMARY A patient with established myelomatosis and an IgG paraproteinaemia presented with intermittent claudication. Indurated cord-like structures were noted on the dorsum of the right hand and the buccal surface of the lower lip, and biopsy of the hand lesion revealed massive amyloid deposition within the wall of an artery. Cord-like thickening of mucocutaneous arteries as a presenting sign of systemic amyloidosis in the absence of glossomegaly or other cutaneous lesions has not been reported previously. The presence of intermittent claudication and angina suggested that amyloid involvement of large arteries was extensively distributed.

Indications for fluorescein angiography in disease of the ocular fundus: a review.

Abstract: It is nearly twenty years since the principle and technique of fluorescein angiography were first described; the procedure has now become standardized and has found a regular place in the routine diagnosis of fundus disorders. Much of the contribution of angiography would have been sterile were it not for the parallel development of photocoagulation as a method of treating fundus disease, with initially the xenon-arc photocoagulator and later the ruby, argon and krypton lasers adapted for retinal therapy. The two techniques of angiography and photocoagulation are often complementary, so that the assessment of fundus disease before treatment is one of the prime indications for angiography. To this must be added its undoubted value in the diagnosis of retinal and choroidal disorders where conventional methods of examination are insufficient, and its positive research value in the study of the retinal circulation and of the the retina, pigment epithelium and choroid. There are, however, a large number of situations where fluorescein angiography is quite unnecessary and wasteful of time and expense, and the purpose of this review is to highlight the abuses of the procedure as much as its uses.

Acquired von Willebrand's syndrome.

Abstract: Further data are now available on one of the four cases of acquired von Willebrand’s syndrome described earlier (Ingram et al, 1971, 1973). When tested between 1969 and 1971, E.Ca., male, born 28 June 1892, had shown, as previously reported, V1II:C in the range 7-30%; the bleeding time was > 20 min (Ivy’s method). Incubation of the patient’s plasma with normal plasma in equal volumes did not show the progressive lengthening of the partial thromboplastin time suggestive of an anti-VIII : C antibody. In 1974 E.Ca. presented an enlarged right submandibular gland, which on biopsy showed squamous carcinoma. Unfortunately, antihaemophilic cover was not provided for an excision; profuse bleeding followed, and a haematoma formed. Extensive submucous laryngeal bruising (? from anaesthetic intubation) was noted 9 d postoperatively (Mr M. 0. Carruthers). A course of radiotherapy was given to the neck (Dr T. K. Wheeler). Careful search then and regular follow-up by Dr Wheeler and by one of us to March 1979 have failed to detect the primary growth or evidence of further metastasis, and the patient has remained in good health except for recurrent epistaxis, which was his presenting symptom.