[A case of eosinophilic fasciitis with peripheral nerve disorder].
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Biomedical subjects
Publications and source records attributed to C Terai.
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The mortality rate from tetanus is still high if the disease is accompanied by signs of autonomic nervous system dysfunction. We treated a 75-year-old woman with tetanus and autonomic dysfunction with continuous high spinal anesthesia for 23 days. She recovered. Spinal anesthesia may be a useful adjunct for the treatment of severe tetanus.
We have previously reported the establishment and characterization of B cell lines from patients and family members with various types of adenine phosphoribosyltransferase (APRT) deficiencies. These cell lines contain, at the APRT locus, three different alleles (APRT*1, APRT*Q0, and APRT*J) that are clearly distinguishable from each other. From five genetically heterozygous cell lines with two different genotypes (APRT*1/APRTQ0 and APRT*1/APRT*J), we have selected 48 clones resistant to 2,6-diaminopurine. Resistance to this adenine analogue is a characteristic of cells having defects in both of the APRT alleles in individual cells. The mutant clones from a cell line from a complete-type heterozygote had APRT activities close to zero (mean = 0.04 nmol/min per milligram protein) in the cell extracts, while 15 clones from four cell lines from the four Japanese-type heterozygotes had significant enzyme activities (mean = 3.88 nmol/min per milligram protein). Kinetic studies on two of the mutants from two Japanese-type heterozygous cell lines have shown that affinity to substrate 5-phosphoribosyl-1-pyrophosphate was reduced, indicating that APRT in those clones reflected the characteristics of the Japanese-type enzyme. The data presented here indicate that clones we obtained are genetic/artificial mutants, each having a genetic mutation in a single allele (APRT*J or APRT*Q0) and an artificially produced mutation in the other previously functional allele (APRT*1). The present procedure provided the only diagnostic method for Japanese-type APRT heterozygotes (APRT*1/APRT*J).
Adenine phosphoribosyltransferase (APRT) deficiency leading to 2,8-dihydroxyadenine (DHA) urolithiasis has been considered a rare cause of urolithiasis and renal insufficiency. We have examined samples from 19 Japanese families with DHA lithiasis. In 79% of the families, patients only partially lacked hemolysate APRT activities, clearly contrasting with the complete deficiency in all the patients from non-Japanese families so far reported. All patients with DHA lithiasis were homozygotes for defective APRT genes, whether the deficiency was complete or partial. In family studies we found two symptomatic and four asymptomatic homozygous family members. The segregation figures are compatible with the hypothesis of a simple autosomal recessive mode of inheritance. By analyzing the data stored by a large clinical laboratory in Japan, we estimated that 0.00368% of the general population has DHA lithiasis. These data indicate that more than 1% of the general population possess mutant alleles of the APRT gene as heterozygotes. Our present studies indicate that most of the patients with this disease are undiagnosed in Japan, and probably in other countries also.
In an attempt to evaluate subclinical lupus nephropathy, we determined the level of urinary albumin by radioimmunoassay in 27 patients with systemic lupus erythematosus (SLE) who had no evidence of clinical renal involvement. The ratio of urinary albumin to urinary creatinine (Ualb/Ucreat X 100) was significantly higher in patients with SLE (2.56 +/- 2.71) than in normal controls (0.83 +/- 0.72). A significant decrease of urinary albumin level in response to steroid therapy was demonstrated in 5 patients examined repeatedly before and during steroid treatment. In one patient who had two episodes of exacerbations of the disease the urinary albumin level increased parallel with a worsening of the serological data and the appearance of clinical proteinuria followed. It is concluded that many SLE patients even without clinical renal involvement have pathologic albuminuria and the determination of the urinary albumin level by radioimmunoassay in these patients is useful for the management of the disease.
The reactivity of glomerular C3b receptors (CR1) measured by the number of sheep erythrocytes bearing C3b (EAC) that adhered to glomeruli in frozen sections, was compared with the reactivity of erythrocyte CR1, which was determined by immune adherence hemagglutination (IAHA), in 22 patients with renal and non-renal diseases. Among seven patients with primary glomerulonephritis whose erythrocytes were positive for IAHA, the reactivity of glomerular CR1 was high in five. In the remaining two, the reactivity of glomerular CR1 was low, accompanied by severe sclerotic glomerular changes. Erythrocytes from five of six patients with systemic lupus erythematosus were IAHA negative, and their glomeruli failed to produce adherence of EAC, even in three cases in which there were no detectable C3 deposits or histopathological changes. In the other nine patients without appreciable glomerular changes, the reactivity of glomerular CR1 was low in three along with negative erythrocyte IAHA, whereas the remaining six exhibited high CR1 reactivity both in glomeruli and on erythrocytes. The results indicate a close correlation between the expression of CR1 in the glomerulus and on erythrocytes.
An autopsy case of clostridial gas gangrene occurring in a 54-year-old man with colon adenocarcinoma, liver cirrhosis, and diabetes mellitus is reported. The patient died 4 days after the onset of symptoms with episodes of vomiting and abdominal pain. Gangrene of both hips and perineum, hemolysis, renal failure, and disseminated intravascular coagulation were the dominant clinical features. Clostridium septicum was isolated from the subcutaneous tissue fluid. Adenocarcinoma of the ascending colon with ulceration found at autopsy was supposed to be an entry of the organism. Histologically, lesions of subcutaneous tissue and muscles were characterized by the absence of inflammatory infiltrates in spite of extensive necrosis. A summary of 35 cases of gas gangrene hospitalized to the Osaka University Hospital for the past 16 years indicates that clostridial gas gangrene patients with underlying diseases such as malignant neoplasm, diabetes, liver cirrhosis or immunodeficiency have a relatively poor prognosis.
We report a patient who suffered from cutaneous vasculitis with renal involvement comprising tubulointerstitial (TI) nephritis and relatively mild glomerulitis. Renal biopsy revealed immune deposits both in mesangium and along tubular basement membrane (TBM), suggesting that immune complexes played a significant role in the development of renal damage. Although it is well known that vasculitic syndromes commonly affect glomeruli, this report is the first to demonstrate tubulointerstitial immune complex nephritis in a patient with cutaneous vasculitis.
Erythrocytes from 51 patients with systemic lupus erythematosus and 75 controls were tested for the capacity to bind aggregated human gamma-globulin labeled with radioiodine in the presence of complement. Both in patients and controls, a trimodal distribution of binding capacity was observed. Low (less than 9% of the added radioactivity), intermediate (9-17%), and high binding (more than 17%) were observed in 13, 58, and 29% in controls and in 49, 43 and 8% in lupus patients. The low binding capacity of erythrocytes persisted even after patients entered remission following steroid therapy. A genetic control of binding capacity was supported by familial surveys. Prevalence of pathological proteinuria was significantly higher in patients with low binding capacity than those with intermediate or high binding capacity (16/25 vs 7/26, P less than 0.01). These results indicate that an impaired physiological disposal of immune complexes via the erythrocyte C3b receptor in lupus patients may contribute to the development of renal involvement.
The authors examined the effects of positive end-expiratory pressure (PEEP) on cardiac function by dimensional analysis of the four heart chambers using M-mode transesophageal echocardiography (TEE). The accuracy of cardiac output (CO) calculated from TEE was confirmed by its close correlation (r = 0.97) with CO, determined by the thermodilution technique. The reliability of TEE also was confirmed by excellent correlation (r = 0.95) between left ventricular end-diastolic (LVEDD) and end-systolic (LVESD) dimensions measured by the two-dimensional precordial echocardiography and those by TEE. With 10 cmH2O PEEP LVEDD decreased from its level during zero end-expiratory pressure (ZEEP), and the calculated stroke volume also decreased. These decreases were greater during 15 cmH2O PEEP, where heart rate increased slightly but significantly. Ejection fraction (EF) and fractional shortening (FS), as a whole, did not change significantly. Mean velocity of circumferential fiber shortening (mean Vcf) significantly increased and LVESD significantly decreased with PEEP. Although systolic blood pressure (SBP) significantly decreased, the (SBP-PEEP value)/LVESD ratio was not changed with PEEP. Such measures of left ventricular systolic function as EF, FS, mean Vcf, and (SBP-PEEP value)/LVESD were not decreased. Right ventricular end-diastolic dimension decreased with PEEP. Right atrial end-diastolic dimension began to decrease immediately after PEEP was initiated, whereas left atrial end-diastolic dimension began to decrease a few seconds later, suggesting that left ventricular preload decreased as a result of a decrease in right ventricular preload. The authors therefore conclude that CO was decreased as a result of the decrease in right and left ventricular preloads.
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C3b receptor (CR1) on erythrocytes from 23 patients with systemic lupus erythematosus (SLE) and 124 normal controls was determined by immune adherence hemagglutination (IAHA) and radioimmunoassay. The binding of radiolabeled monoclonal anti-CR1 to erythrocytes and their lysate was distributed continuously in a wide range. The majority of SLE patients showed low binding by both assays. CR1 sites on erythrocytes were determined also by Scatchard plot analysis and standardized by the number of similarly determined lectin-binding sites that served as a measure of erythrocyte surface. The numbers of standardized CR1 sites were classified as high, intermediate, and low. Thirty-six percent of control subjects had high numbers of CR1 sites, 53% had intermediate numbers, and 11% had low numbers. Of SLE patients, the numbers of CR1 sites were high in 0%, medium in 52%, and low in 48%. Negative IAHA was found in 10 controls (8%), all of whom had low numbers of standardized CR1 sites. Among 13 SLE patients with negative IAHA, 11 had low numbers of CR1 sites and the remaining 2 had low intermediate numbers. IAHA, therefore, was particularly efficient in detecting the low numbers of CR1 sites in SLE, which would impair the disposal of circulating immune complexes and accelerate the development of tissue injuries.
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The effects of mild hypothermia (MH) were investigated. From 1995 to 1996, 28 adult patients with out-of-hospital cardiopulmonary arrest (CPA) had return of spontaneous circulation and survived for more than two days. Thirteen patients were in the MH group. In the MH group, core temperature was maintained between 33 and 34 degrees C for 48 h, and then re-warmed to a temperature of 37 degrees C, at a rate of no greater than 1 degrees C per day. Fifteen patients, admitted before the MH protocol was instituted, were in the control group. Despite the fact that the number of witnessed arrests in the control group were greater than in the MH group, there were both more survivors (7/13 vs. 5/15) and more fully recovered patients (3/13 vs. 1/15) in the MH vs Control groups. Eleven of 13 MH patients, as compared to 6/15 controls developed pneumonia. Our study, although preliminary, suggests that MH might confer improved outcome, as has been shown in animal models, after CPA. This treatment is associated with an increase in pneumonic complications.