Estimating baseline creatinine levels based on the kidney parenchymal volume.

BACKGROUND: Acute kidney injury (AKI) diagnosis often lacks a baseline serum creatinine (Cr) value. Our study aimed to create a regression equation linking kidney morphology to function in kidney donors and chronic kidney disease patients. We also sought to estimate baseline Cr in minimal change disease (MCD) patients, a common AKI-predisposing condition. METHODS: We analyzed 119 participants (mean age 60 years, 50% male, 40% donors) with CT scans, dividing them into derivation and validation groups. An equation based on kidney parenchymal volume (PV) was developed in the derivation group and validated in the validation group. We estimated baseline Cr in 43 MCD patients (mean age 45 years, 61% male) using the PV-based equation and compared with their 6 month post-MCD onset Cr values. RESULTS: In the derivation group, the equation for the estimated glomerular filtration rate (eGFR) was: eGFR (mL/min/1.73m2) = 0.375 × PV (cm3) + (- 0.395) × age (years) + (- 2.93) × male sex + (- 13.3) × hypertension + (- 14.0) × diabetes + (- 0.210) × height (cm) + 82.0 (intercept). In the validation group, the eGFR and estimated Cr values correlated well with the measured values (r = 0.46, p = 0.01; r = 0.51, p = 0.004, respectively). In the MCD group, the baseline Cr values were significantly correlated with the estimated baseline Cr values (r = 0.52, p < 0.001), effectively diagnosing AKI (kappa = 0.76, p < 0.001). CONCLUSIONS: The PV-based regression equation established in this study holds promise for estimating baseline Cr values and diagnosing AKI in patients with MCD. Further validation in diverse AKI populations is warranted.

Membranous nephropathy with thrombotic microangiopathy-like lesions successfully treated with tocilizumab in a patient with idiopathic multicentric Castleman disease.

Idiopathic multicentric Castleman disease (iMCD) is a systemic lymphoproliferative disease involving multiple organs, including the kidneys. Membranous nephropathy (MN) has been rarely reported as a complication of iMCD. We herein report the case of a 48-year-old man with a 9-year history of iMCD that was complicated by treatment-resistant nephrotic syndrome due to MN. The first renal biopsy performed at the age of 45 years showed diffuse and global MN with a mild glomerular endothelial injury. He was treated with combined therapy of corticosteroids, immunosuppressants, and tocilizumab, an anti-interleukin-6 (IL-6) receptor monoclonal antibody, which was administered every 2-3 weeks. However, nephrotic syndrome persisted, and renal impairment slowly worsened. Serial biopsy performed at 3 years after the first biopsy confirmed advanced lesions of both MN-related and glomerular endothelial injuries. Modification of the therapeutic strategy to weekly administration of tocilizumab gradually led to the remission of proteinuria, allowing the termination of corticosteroids. Thus, the present case suggests a close link between excessive IL-6 actions and the development of glomerular lesions in iMCD. Successful treatment by strict inhibition of IL-6 actions, in this case, may provide a clue for deciding the therapeutic strategy for severe renal complications associated with iMCD.

Proteinuric glomerulopathy in an adolescent with a distal partial trisomy chromosome 1.

We report a case of distal partial trisomy 1 from q32.1 to 41 that have exhibited proteinuric glomerulopathy. The patient was a 17-year-old adolescent with clinical features of low birth weight, mild mental retardation and mild deafness, from the birth. He exhibited non-nephrotic range proteinuria with the mild obesity since the age of sixteen. Image studies did not reveal morphological abnormalities of the kidneys. Renal biopsy findings showed no definitive evidence of primary glomerular diseases, and were characterized by a very low glomerular density, glomerulomegaly and focal effacement of podocyte foot processes. Therapies with dietary sodium restriction, body weight reduction and the administration of angiotensin receptor blocker markedly reduced his proteinuria. It was likely that mismatch between congenital reduction in the nephron number and catch-up growth of the whole body size played a major role in the development of glomerular hyperperfusion injury. At present, the direct contribution of genetic factors due to this chromosomal disorder to such a substantial reduction in the nephron number remains uncertain.