Fatal and non-fatal stingray envenomation

A fatality occurred in a previously healthy 12-year-old boy after a penetrating chest injury from a stingray barb. The injury occurred under freak circumstances. Death was a result of cardiac tamponade which was secondary to venom-induced, localized myocardial necrosis and spontaneous perforation, six days after the direct penetration of the right ventricle by the barb. Three other cases of less serious stingray envenomation are described which illustrate the significant localized morbidity that may occur without immediate wound exploration and toilet after adequate anaesthesia. We also report a study of a series of 100 minor stingray envenomations which, when treated, resulted in no morbidity. It is possible that local infiltration with 1% plain lignocaine may have a direct counteraction against stingray venom that remains in the wound area. Stingray venom has insidious, but powerful, localized tissue necrosing properties in humans.     

A new transthyretin variant (Glu61Gly) associated with cardiomyopathy.

We report the identification of a new transthyretin (TTR) gene mutation and variant protein, Glu61Gly, in a 55-year-old man with progressive cardiomyopathy, mild peripheral neuropathy and bilateral carpal tunnel syndrome. A diagnosis of TTR-associated familial amyloidosis (ATTR) was considered after an endomyocardial biopsy revealed amyloid deposits in the heart of a patient who had no family history of amyloidosis and no evidence of a plasma cell dyscrasia. Serum screening for a TTR variant by isoelectric focusing (IEF) was positive and prompted further studies to identify the genetic abnormality and to characterize the amyloidogenic protein. Direct DNA sequence analysis of all four coding regions in the TTR gene demonstrated heterozygosity in exon 3. Near equal amounts of guanine (G) and adenine (A) were observed at the second base position of codon 61. The wild-type (GAG) and mutated (GGG) sequences found in codon 61 correspond to glutamic acid (Glu) and glycine (Gly) residues, amino acids which differ in mass by -72 Da. Mass spectrometric analyses of TTR immunoprecipitated from serum showed the presence of both wild-type and variant proteins. The observed mass results for the wild-type and variant proteins were consistent with the predicted values calculated from the genetic analysis data.     

Decreased pain with lower flexural rigidity of uncemented femoral prostheses

One hundred one consecutive uncemented hip arthroplasties (87 patients) were analyzed radiographically at 1-year follow up to relate mechanical factors to hip pain as determined by clinical pain scores. The average area moment of inertia and flexural rigidity were greater for the bone than the metal prosthesis for each type of prosthesis (AML, HG, PCA). Normalization of the flexural rigidity ratio (bone to prosthesis) for patient weight yielded a Spearman correlation coefficient of 0.232, significant at P = .02, suggesting that both applied stress and bending stiffness have an effect on pain. No relationship was seen between pain and AP, or average gap between prostheses and bone.     

A juvenile animal model to study the growth potential of bowel segments in the urinary tract

A juvenile animal model has been developed to study the growth potential of ileum in the urinary tract. Patch ileocystoplasties of known surface area were constructed in dogs of Group 1. Nonrefluxing ileal nipple valves of known length were created to replace one ureter in dogs of Group 2. After the juvenile animals grew and at minimum doubled their weight, they were reoperated and augmentation surface areas were remeasured at various physiologic intravesical pressures. Nipple valve lengths were remeasured after cystograms ruled out reflux. Results show that the bowel augmentation and an identical control segment increased in surface area proportionate to animal growth and that hydrostatic dilation caused further surface area increase. Nipple valves did not grow and in fact shortened, but remained nonrefluxing. The conclusion is that in the pediatric population, consideration should be given to downscaling the size of bladder augmentations or diversions in anticipation of future bowel growth, but that one should not shorten the ileal nipple valves.     

Treatment of refractoriness to platelet transfusion by protein A column therapy

Ten thrombocytopenic patients (platelets < 10–24 × 10(9)/L) who were refractory to platelet transfusion were investigated for their responsiveness to staphylococcal protein A column therapy. Nine patients had previously been treated with steroids, intravenous immune globulin, and/or other forms of immunosuppressive therapy without improvement in their transfusion response. All patients were receiving multiple platelet transfusions without achieving 1-hour corrected count increments (CCIs) > or = 7500. Eight patients had antibodies that reacted with platelets and were directed against HLA class I antigens, ABO antigens, and/or platelet-specific alloantigens. Plasma (500-2000 mL) from each patient was passed over a protein A silica gel column and then returned to the patient. Patients received from 1 to 14 treatments. A positive response to protein A therapy was defined as at least a doubling of the pretreatment platelet count and/or two successive 10- to 120-minute posttransfusion CCIs > or = 7500. Following plasma treatments, 6 of 10 patients responded with daily platelet counts that averaged 48 +/− 11 × 10(9) per L as compared with counts of 16 +/− 7 × 10(9) per L (p < 0.0005) before treatment. Posttransfusion CCI values determined in four of these patients averaged 2480 +/− 810 and 10,010 +/− 3540 (p < 0.005) before and after treatment, respectively. In contrast, among the four unresponsive patients, platelet counts averaged 10 +/− 9 and 13 +/− 10 × 10(9) per L (p = NS), respectively, while posttransfusion CCIs were 700 +/− 1410 and 1520 +/− 2460 (p = NS), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)