[A case of familial medullary carcinoma of the thyroid complicated with Cushing's syndrome].
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Biomedical subjects
Publications and source records attributed to Y Tofuku.
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Intermittent oral administration of high dose 1,25(OH)2vitamin D3 was conducted in 7 patients associated with treatment-resistant secondary hyperparathyroidism (2nd HPT) on hemodialysis (HD) therapy. Each patient had a long history of HD therapy (range: 101-181 months, 145 +/- 29 months). Although serum calcium levels were maintained under the upper limit of the normal range with the appropriate dose of 1 alpha(OH)vitamin D3 every day before the present therapy, 2nd HPT could not have been controlled. The dose of 2-3 micrograms of 1,25(OH)2vitamin D3 3 times a week could successfully suppress serum levels of parathyroid hormone (iPTH) in all 7 patients after 20-32 weeks. The vitamin was given in the evening before each HD session and the dose and frequency of administration were dependent of the serum calcium level in each patient. After 20 weeks the iPTH-C and iPTH-intact levels decreased significantly from 35.0 +/- 15.8 to 18.6 +/- 11.7 ng/ml and from 533.2 +/- 200.0 to 249.5 +/- 136.2 pg/ml, respectively. The frequency of harmful elevations of serum calcium levels was not significantly increased in comparison with that in the previous period of the study, because serum calcium levels were strictly monitored with frequent checks. In conclusion, we could safely obtain an effect similar to the intravenous administration of the vitamin through the intermittent administration of a high oral dose 1,25(OH)2vitamin D3 in the treatment of refractory 2nd HPT in patients on HD therapy.
We investigated the urinary acid excretion and urine anion gap (AG) (Na+ + K(+)-Cl-) during NH4Cl-induced metabolic acidosis in 38 normal subjects and 53 patients with chronic renal diseases in order to clarify the significance of the urine AG as a useful marker of the ammonium (NH4+) excretion even in a state of chronic renal insufficiency. The urine pH became higher, and the urinary excretions of titratable acid (TA) and NH4+ decreased significantly, in parallel with a reduction of the creatinine clearance (Ccr). The urinary electrolyte excretion, especially the chloride excretion, also decreased significantly as Ccr fell. As a result, the urine AG increased from negative to positive values, in proportion to the decrease in Ccr with statistical significance. The urine AG showed the most significant correlation with the urine NH4+ excretion (r = -0.707, p less than 0.001). We conclude that the urine AG provides a significant marker of the urine NH4+ excretion even in a state of moderate to severe renal insufficiency.
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We report a case of 33 years old woman who developed hemolytic anemia after blood transfusion. In this case no abnormalities were observed by the cross-match test performed before blood transfusion. She was undergone the surgical resection of liposarcoma of her right arm in June, 1984 and 8 units of the concentrated red blood cells were transfused during the operation. During the following 2 months 13 units of the concentrated red blood cells were transfused. Immediately after blood transfusion, she developed icterus and oliguria and was diagnosed acute renal failure associated with hemolytic anemia. At this time we examined the antigen type of her red blood cells and of three donors' red blood cells which had been given to her. As the result, either C (Rh-hr), Pi (P), Lea (Lewis), Fyb (Duffy) antigens were found in some of donors' RBC, but no such antigens were detected in her RBC. The half life span of RBC measured using 51Cr labeled RBC was 6.2-14.5 hours, in respect of the antigen-positive RBC, where as it was 49.4 hours in the RBC without the antigens. It is more likely that the blood transfusion was the cause of hemolytic episode in this case, because the half life span of the donors' RBC was extremely shortened, compared with that of her own blood which was 16 days.
A case of bucillamine-associated membranous nephropathy in a patient with rheumatoid arthritis was reported. A 34 year-old woman was admitted to our hospital because of proteinuria in September, 1988. Rheumatoid arthritis had been diagnosed in April, 1988, and had been treated with bucillamine (disease modifying anti rheumatic drug) and amfenac (nonsteroidal antiinflammatory drug). The renal biopsy specimens showed Stage I membranous nephropathy, on both electron and immunofluorescence microscopy. Proteinuria decreased after the drugs were withdrawn despite continued RA symptoms. These results were consistent with drug induced nephropathy. Since bucillamine has some resemblances in its structure and pharmacologic action to D-penicillamine, which sometimes induces membranous nephropathy, it is thought that the nephropathy in this case was caused by an adverse reaction to bucillamine.
A 74-year-old man was admitted to our hospital because of a treatment for his right renal tumor. The abdominal CT scanning revealed a mass in the right kidney, and a right selective renal arteriography demonstrated a hypervascular tumor. On admission, urinalysis revealed proteinuria (3-4 g/day) and microscopic hematuria, and serum electrolytes were normal. Serum creatinine and urea nitrogen levels were 1.6 mg/dl and 30 mg/dl, respectively. A percutaneous right renal biopsy specimens showed crescentic glomerulonephritis. Direct immunofluorescence studies showed strong linear staining for IgG and IgA along the glomerular capillary walls. Electron microscopy showed increased mesangial matrix and swollen epithelial cells, but no dense deposits in the para-mesangial area and in the glomerular basement membrane. The patient underwent right radical nephrectomy. Histologic examination of the resected specimen revealed renal cell carcinoma. Postoperatively, he developed rapidly progressive renal failure and the renal function could not be recovered. Using the indirect immunofluorescence technique, we could not confirm the presence of a serum anti-glomerular basement membrane antibody, although the examination could not be carried out until the initiation of hemodialysis therapy. Some cases of glomerulopathies associated with renal cell carcinoma were previously reported, but the case of crescentic glomerulonephritis was very rare.
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Plasma aldosterone concentration (PAC) in patients with chronic renal failure was measured by radioimmunoassay with and without dichloromethane extraction before the assay. Blood samples were obtained from patients with chronic renal failure who were undergoing maintenance hemodialysis, from patients with chronic renal failure who were not undergoing hemodialysis, and from age-matched normal subjects. With the three radioimmunoassay kits tested, the direct assays without extraction in normal subjects gave PAC values comparable to those obtained after the dichloromethane extraction; in contrast, in patients with chronic renal failure, the direct plasma radioimmunoassays yielded consistently higher values than those obtained after the extraction (p less than 0.001). When various plasma steroid metabolite fractions were separated by high-pressure liquid chromatography (HPLC) with a reverse-phase column, and each fraction was assayed for PAC with the three radioimmunoassay kits, high immunoreactivities were found in the polar fractions in the plasma from patients with chronic renal failure but not in normal plasma. The ratios of the immunoreactivities of these polar fractions to that of the aldosterone fraction, determined after HPLC separation, showed a significant positive correlation (r = 0.746, p less than 0.001) with serum creatinine concentrations in plasma from patients with chronic renal failure who were not undergoing hemodialysis. These results indicate that the values for PAC are falsely elevated in chronic renal failure when PAC is measured by radioimmunoassay kits without prior extraction. Furthermore, plasma from patients with chronic renal failure contains a polar substance(s) that cross-reacts with antialdosterone antibodies. This so far-unidentified substance increases with advancing renal impairment.(ABSTRACT TRUNCATED AT 250 WORDS)
In order to examine quantitatively the effect of plasma protein binding on the peritoneal transport of beta-lactam antibiotics, we employed a kinetic model based on the pore theory of transcapillary exchange. This model incorporates the changes in the volume, osmolality, and antibiotic concentration in the dialysate, so that the apparent capillary membrane permeability (Pd) and the reflection coefficient (sigma d) of an antibiotic could be assessed. Six cephalosporins (cefatrizine, cefazolin, cefpiramide, ceftazidime, ceftriaxone, cephaloridine) were used as model compounds. While the unbound fractions of these antibiotics ranged widely from 0.08 to 0.57, including linear and nonlinear protein binding, the concentration-time profiles in plasma and the peritoneal dialysate after intravenous administration in rats could be interpreted well by our model, assuming that only the unbound antibiotic is available for the peritoneal transport. The estimated Pd values were almost the same among the drugs examined. Moreover, the Pd values of cefazolin in mice, rats, and rabbits exhibited a 0.83-power dependency on the animal body weight, indicating that Pd is significantly related to the peritoneal surface area. On the other hand, the sigma d values of cefazolin were found to be almost the same among the animal species examined. Finally, the concentration-time profile of cefazolin in the dialysate after intravenous administration in a patient with end-stage renal failure was successfully predicted using the Pd value extrapolated from those of the experimental animals.
The southwestern region of Japan is known as a very high endemic area of human T-cell lymphotropic virus type 1 (HTLV-1), the etiologic agent for adult T-cell leukemia (ATL) and probable causative agent for tropical spastic paraparesis and its Japanese version, HTLV-1-associated myelopathy (HAM). Hemodialysis (HD) patients seem to be at high risk for HTLV-1 infection even in other regions of Japan because they sometimes receive multiple blood transfusions. We examined antibody against ATL-associated antigen (ATLA-Ab) in 1,132 HD patients, including 1,066 patients in nonendemic areas (Chubu and Tokyo) and 66 in a highly endemic area (Okinawa). The HD patients in Okinawa showed the highest prevalence, 21.2% (14/66), while those in the Chubu area showed the lowest, 1.1% (10/846), and those in the Tokyo area an intermediate value, 2.7% (6/220). The prevalence of HD patients in each area was significantly higher than that of local blood donors, reflecting an increased prevalence roughly corresponding to the respective endemic rate. The average prevalence of ATLA-Ab among the HD patients was 2.7% (30/1,132), which was similar to that of HBs antigen (3.2%). In the nonendemic areas, 15 of 16 patients with ATLA-Ab had a history of blood transfusions, showing a significant correlation to the presence of ATLA-Ab (P less than 0.01), although four had family histories related to the endemic area. The relative risk of the presence of ATLA-Ab for HD patients with a history of blood transfusions was calculated as 10.3. In the endemic area of Okinawa, the relationship to blood transfusion was not so close, probably masked by the high background prevalence.
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