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A 14-year-old Japanese girl was admitted to our institution for the evaluation of renal dysfunction. Her serum creatinine was 1.1 mg/dL, proteinuria was 1.5 g/day, the urine sediment contained numerous erythrocytes per high-power field, and she was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Proteinuria was first noted at the age of 12 years. Renal biopsy showed crescentic glomerulonephritis with slight immunoglobulin A (IgA) deposition. A diagnosis of ANCA-associated vasculitis was made. Immunosuppressive therapy was initiated, including steroid pulse therapy and intravenous cyclophosphamide pulse therapy, but hemodialysis was required after 6 years. Eight months after the patient became anuric and her MPO-ANCA titer became negative, living-related donor kidney transplantation was done from her mother. ANCA became slightly positive 2 years later, but the patient remains stable without proteinuria or hematuria at 4 years after surgery. This case suggests that kidney transplantation can be performed successfully for a patient with refractory childhood-onset ANCA-associated vasculitis, and that remission of vasculitis associated with ANCA negativity at transplantation may contribute to a better renal prognosis in this patient.

Background. Renal hyperparathyroidism (RHPT) is a serious complication of long-term dialysis treatment. Two intervention methods can be administered to treat RHPT, namely percutaneous ethanol injection therapy (PEIT) and a parathyroidectomy (PTx). PEIT is associated with a significant adverse event, adhesion formation. This study was performed to investigate the effect of PEIT on subsequent PTx. Methods. A total of 80 subjects were included in the study. The patients had a diagnosis of RHPT for which surgery was indicated. They were divided according to whether they underwent PEIT (PEIT group) or not (non-PEIT group). The outcomes of PTx following PEIT were evaluated. Results. There were 19 patients in the PEIT group and 61 in the non-PEIT group. The operation time was significantly longer in the PEIT group but no significant differences in the amount of bleeding or frequency of recurrent nerve paralysis were observed. The intact PTH levels immediately following surgery were slightly higher in the PEIT group. The postoperative intact PTH levels were found to be significantly higher in those who received two or more courses of PEIT. The number of patients with an intact PTH level >60 pg/ml on postoperative Day 1 was significantly higher in the PEIT group. Conclusions. These findings suggested that PEIT prior to PTx can affect the subsequent surgical outcome due to associated adhesions and dissemination. For patients with a possibility of either a decreased efficacy or a lack of efficacy for PEIT, it is therefore important to consider PTx from the very beginning of the treatment.

Background: Several thiocholine alkanoyl esters were newly synthesized and explored as substrates for the assay of human serum cholinesterase after being subjected to the Ellman reaction (Arch Biochem Biophys 1958;74:443-50 and Arch Biochem Biophys 1959;82:70-7)., Methods: We synthesized thiocholine ester iodides by the method of Renshow et al. (J Am Chem Soc 1938;60:1765-70). We examined solubility in H(2)O, substrate specificity serum for cholinesterase, (spontaneous) self-hydrolysis, storage stability, and reaction conditions for measurement of the activity of the enzyme., Results: Isobutyryl and cyclohexane-carboxyl esters showed the best efficiency for the specific and stable assay of human serum cholinesterase. Aqueous solubility of each was >10 mmol/L, and the reactivity with acetylcholinesterase was negligible. For isobutyryl and cyclohexane-carboxyl esters, respectively, spontaneous hydrolysis in the aqueous phase was approximately 1/25 and approximately 1/175 slower than the enzymatic hydrolysis, and assays with these substrates were linear to 1800 and 3000 U/L, respectively. The K(m) values of these acylthiocholines with human cholinesterase were almost equivalent (6.9 x 10(-3) mmol/L). The substrates were stable in aqueous solution and in the solid state as the iodides for at least 5 years at 5 degrees C., Conclusions: The isobutyrate and cyclohexane-carboxylate of thiocholine are suitable for the specific assay of human serum cholinesterase.

After preincubation of human lactate dehydrogenase (EC 1.1.1.27) isoenzymes 1 through 5 with lactate at pH 9.4, 9.8, 10.25, and 10.6 at 30 degrees C for 10 min, the reduction of NAD+ was measured at the same pH values and temperature during the interval 1-2 min after adding the coenzyme. Relative to the reference activities measured at pH 8.7 by the method of Buhl et al. (Clin Chem 23: 1289-1295, 1977), the respective activities of isoenzymes 1 through 5 thus measured were 111, 104, 96, 68, and 0% at pH 9.4; 123, 108, 88, 0, and 0% at pH 9.8; 140, 100, 0, 0, and 0% at pH 10.25; and 138, 0, 0, 0, and 0% at pH 10.6. These relations allow selective assay of the respective isoenzymes in samples containing mixtures of them. Optimal conditions for such selective assay at 37 degrees C are reported.