Clinical quiz. Addison's disease.
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Biomedical subjects
Publications and source records attributed to P D Thomson.
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In a decade from 1980, 11 children aged 3 to 11 years presented with Takayasu's arteritis (TA). All were severely hypertensive. Operative correction was offered to 10 of 11 children presenting with renovascular hypertension (RVH) including cardiac failure alone in 1 and both renal and cardiac failure in 8, a result of TA involving renal arteries by stenosis or occlusion. Nine patients had renal autotransplantation to an heterotopic site in the pelvis. Seven of 12 kidneys were salvaged by autotransplant with relief of RVH. Renal artery stenosis was successfully corrected by this procedure in 5 patients. Autotransplantation failed in 4 patients, 1 of whom subsequently had a successful allograft transplant. One patient was treated primarily by cadaver allograft transplantation. One patient whose autotransplant failed had a functioning contralateral kidney and is well with controlled RVH. One patient died prior to any treatment. Patient survival improved with the use of total lymphoid irradiation in the most recent 7 patients.
Lipid peroxides (conjugated dienes) have been shown to increase in the plasma of thermally injured animals and in humans. The purpose of this study was to determine if conjugated diene-rich fractions extracted from the plasma of burned rats 3 hours after injury would alter humoral immune function in unburned animals. Plasma diene extracts from burned or normal rats were dissolved in ethanol and given intraperitoneally to Long-Evans rats. Fifteen minutes after diene injection, the rats were immunized with sheep erythrocytes. Five days later, serum and spleen cells obtained from these immunized rats were tested for IgM immunity against sheep erythrocytes. Both plaque-forming cells from spleen and hemagglutination titers in serum were significantly enhanced in the group treated with dienes obtained from the thermally injured rats. These data suggest that conjugated diene-bearing lipid fractions from burn plasma may modulate immune function and support the concept that humoral immunity is enhanced after thermal injury.
The predictive value of varying levels of antibody activity, its class and antigen specificity in sera of 81 recipients of cadaver renal allografts was evaluated. Recipients for transplantation were selected on the basis of a negative dye uptake T-cell crossmatch, after which the more sensitive 51Cr release technique was employed in a blind study using unseparated donor target cells. Recipient sera with peak panel reactivity and current samples were evaluated before and after reduction with dithiothreitol to destroy the IgM subclass. Double absorption with pooled platelets allowed antibodies against HLA class I antigens to be distinguished from those against HLA class II/non-HLA antigens. Optimal levels of cytotoxicity were established, giving a sensitivity of 73%. Data were assessed in terms of positive predictive value, and showed that conventional T-cell crossmatching is adequate for the primary transplant group, but more sensitive ancillary tests are indicated for regrafts. In this category of patients, IgG antibodies, whether against HLA class I antigens or HLA class II/non-HLA antigens, were highly predictive of early graft loss (positive predictive value 50%-100%). Using this protocol for patient selection, 1-month graft survival would have improved from 73% to 96%.
Six children presented with severe hypertension caused by Takayasu's arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12-18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.
We describe the clinical, radiographic and histological features of skeletal involvement in four patients with end-stage renal failure due to primary oxalosis. The clinical features were unrelenting bone pain, and in two patients multiple fractures. Radiographic features were, in chronological order: (1) radiodense metaphyses and other red marrow bone; (2) cortical defects in metaphyses; (3) spontaneous fracture-separations of epiphyses of long limb bones which healed poorly. The fractures occurred through crystal deposits, and fracture displacement was associated with extrusion of crystalline material from bone. On histological examination crystals were found to replace metaphyseal bone. Pericrystalline giant cell granulomata replaced bone marrow. Erosion surfaces near granulomas were increased. Subperiosteal and intra-osseous tophi of calcium oxalate were seen. Calcium oxalate appears to precipitate with greater facility than does physiological mineral. Bone showed the features of mixed uraemic osteodystrophy in all four patients. We conclude that: (1) the fractures occurred through heavy crystal deposits; (2) ununited fractures and intra-osseous and subperiosteal tophi contributed to the pain; (3) spontaneous fractures are of poor prognostic significance. We recommend that unstable fractures be internally fixed.
A prospective trial compared spray thrombin to a bovine collagen haemostatic agent on skin graft donor sites. Twenty donor sites had half of the area covered with the collagen sheet and the other half sprayed with thrombin. Each treatment was covered with a surgical sponge, pressure was applied for 10 min and then the sponges and collagen were removed. A second sponge was then placed on each wound for 2 min. These sponges were tested for oxyhaemoglobin by spectrophotometry. The results showed that the half of 18/20 donor sites covered with collagen lost more blood than the half treated with spray thrombin. These results suggest that thrombin is superior to collagen sheets for donor sites treated in this manner.
A retrospective review of all patients admitted for acute care over the past 15 years was undertaken to identify readmission for reconstructive surgery. Of 3167 survivors 629 or 19.9 per cent were readmitted for at least one reconstructive procedure. Those patients requiring readmission for reconstructive surgery were younger and had larger total body surface burns compared to the group that were not readmitted for reconstructive surgery. The percentage of patients now being readmitted for reconstructive surgery is decreasing significantly and covers all size burns. The most common area of reconstruction, by frequency, involved the hand and wrist, arm and forearm, face, and neck. These data indicate that the goal of reducing the incidence of burn scar contractures in this patient population is being achieved. However, further work needs to be done to reduce the frequency of reconstructive surgery and to identify those variables which predispose patients to burn scar contractures which are amenable to intervention.
The effect of various volumes of blood and serum on the in vitro antibacterial activity of 0.2% nitrofurazone soluble dressing (NSD) was studied. The antibacterial activity of NSD was tested with an agar well diffusion technique. Zones of inhibition against susceptible strains of Escherichia coli and coagulase-positive Staphylococcus aureus were measured with a micrometer. Bacterial concentrations of 10(5) and 10(8) colony-forming units per milliliter were tested to evaluate a possible effect of inoculum size. Wells contained full-strength (undiluted) NSD or 75%, 50%, or 25% NSD dilutions (w/w) in blood, serum, or 0.9% sodium chloride injection. The mean decrease in inhibition zone size produced by blood and serum was only 7.2%. The diminution of activity, albeit small, was statistically significant. The zones produced were still much larger than those associated with clinical cure. Therefore, the impact of blood and serum on nitrofurazone's in vitro antibacterial activity can best be described as a slight reduction in rather than an elimination of effectiveness. This small reduction in activity is unlikely to be clinically important in patients with burns or other surface wounds that contain blood or serum. An effect of inoculum size was demonstrated for both organisms. Blood and serum produced a small but significant reduction in NSD's antibacterial activity in vitro. Controlled clinical studies are needed to ascertain the clinical importance of these findings.
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Oligohydramnios and neonatal renal failure is reported in a premature infant whose mother had used enalapril in weeks 32-35 of her pregnancy. The infant recovered adequate renal function after peritoneal dialysis. Review of the literature supports our view that angiotensin-converting enzyme inhibitors should not be used in pregnancy; if they are, both mother and fetus should be monitored with extreme care.
It has been suggested that there may be a consistent pattern of congenital renal abnormalities related to a particular sensory level in patients with spina bifida. In assessing 163 spina bifida patients by reviewing intravenous pyelograms (without knowledge of clinical details) and correlating with the level of sensory loss (without knowledge of radiological findings), the prevalence of renal abnormalities was similar, but a consistent pattern was not confirmed.
Animal models and human studies have shown that conjugated dienes rise in the plasma after thermal injury. These dienes may serve as a marker of oxygen radical-mediated tissue injury. Twelve burn patients were randomized to receive the antioxidant enzyme polyethylene glycol-conjugated superoxide dismutase (PEG-SOD). Patients received either 500 or 1000 units per kilogram of PEG-SOD intravenously within 6 h of injury. Plasma samples were collected and conjugated diene levels were compared to diene levels of burn patients not treated and to diene levels from normal volunteers. Conjugated diene levels were increased in burn patients. PEG-SOD in either dose initially decreased conjugated diene levels in the plasma of both treatment groups. By 72 h, the diene levels increased in the 500 unit/kg group, but remained at near control levels in the 1000 unit/kg group for up to 200 h after injury. These data suggest that PEG-SOD is capable of preventing conjugated dienes formed as the result of oxygen radical production. It appears that 1000 units/kg is more effective than 500 units/kg in preventing conjugated diene formation.
Bilateral above the knee amputations occur rarely in the seriously burned population. In a 6-month interval, two patients were treated in our Burn Center whose trauma necessitated bilateral above-knee amputations. This led us to review our experience with such cases. A review of 5018 patients treated over a 28-year period identified six patients over a 20-year period who had bilateral above-knee amputations, four of whom survived. These patients were found to have large and deep burns, concomitant injuries and a number of complications. Recovery was time consuming and costly. Physical therapists were challenged to create adaptations of traditional methods to meet the rehabilitation needs of these patients. The patients who survived remain wheelchair bound and unemployed, but are socially active, appear relatively well adjusted and report themselves satisfied with their lives. During a period of time when technological advances in medicine are ensuring survival of victims of massive trauma, questions are raised concerning the advisability of resuscitation. Knowledge gained in the treatment of these patients who are both burned and have had bilateral above-knee amputations can be reassuring to care providers with limited experience with such massive injury.
A new system of determining susceptibility of burn wound microorganisms to topical antimicrobial therapy was studied. The new method, TOPITEST, employs a methacrylate vehicle impregnated with various antimicrobial agents and an agar overlay. Test plates can be inoculated by a direct swab culture of the wound or with micro-organisms isolated by other methods. This new technique was compared to an agar well diffusion (AWD) method using commercially compounded antimicrobials and biopsy-recovered organisms. The new test method provided comparable results to the more established AWD test when using biopsy-recovered organisms. Using the swab culture technique, however, TOPITEST had a high rate of failure to recover sufficient organisms from the burn wound for testing. The new, more standardized test provided results earlier than the AWD test, but may prove to be less cost effective.
We evaluated effluent blood from extremities of human patients undergoing reconstructive surgical treatment, which is routinely accompanied by upper-extremity exsanguination and application of a tourniquet, resulting in total interruption of arterial blood flow to one upper extremity. After tourniquet release (reperfusion), there were immediate increases in the plasma levels of xanthine oxidase activity, uric acid, and histamine in the ipsilateral limb and much smaller increases, if any, in levels of the same materials in plasma obtained from the contralateral extremity. There was no detectable xanthine dehydrogenase activity in plasma from either limb. Plasma also contained evidence of products consistent with the formation of oxygen-derived free radicals, namely, the appearance predominantly in the reperfused limb of hemoglobin and fluorescent compounds. These data indicate for the first time in humans that ischemia-reperfusion events are associated with the appearance of xanthine oxidase activity and its products in the plasma effluent.
Polycystic kidney disease was investigated in 28 families in which at least one member attended the paediatric nephrology clinic at a Johannesburg or Pretoria hospital. Twenty-five (89.3%) of the families had autosomal recessive polycystic kidney disease (ARPKD), and of these a significantly larger number than would have been expected (92%) were Afrikaans-speaking. There was a consanguineous marriage in the ancestry of 2 patients but 6 of the families (26%) had ancestors with one surname in common and 7 of the families (28%) had their origin in the western Transvaal. A point prevalence for ARPKD of 1:26,000 was estimated for the Afrikaans population (based on the age cohort 0 - 19 years) and the carrier rate for the gene was 1:83; on the basis of the live-birth rate for ARPKD of 1:11,000 the carrier rate is 1:53. These findings suggest that ARPKD may be unusually frequent in the Afrikaans-speaking population. A founder effect is probably responsible.
A 23-year-old male with an 87 per cent body surface area burn from a motor vehicle accident developed a left lower quadrant abdominal wall faecal fistula. This fistula presumably resulted from thermal injury to the level of the peritoneum. This case report describes the course and management of this unusual occurrence.