BACKGROUND: Anti-angiotensin II type 1 receptor antibodies (AT1R-Abs) have been demonstrated to increase the risk of antibody-mediated rejection. We report a case of AT1R-Ab mediated rejection which caused early critical cortical infarction.
METHODS: A 52-year-old man with end-stage kidney disease underwent preemptive kidney transplantation (KT) from his wife. He had no immunologic risk except ABO incompatibility. Proper desensitization treatment were applied prior to KT. On postoperative day 1, he showed stable clinical course with adequate urine output, but there was no decrease in serum creatinine level and imaging studies showed hypoperfusion in the transplanted kidney.
METHODS: Allograft biopsy revealed total cortical infarction with severe necrotizing vasculitis, but the medullary area was preserved. Serum AT1R-Ab concentration was elevated from 10.9 U/mL before KT to 19.1 U/mL on 7 days after KT.
METHODS: He was treated with plasmapheresis, intravenous immunoglobulin, rituximab, high-dose methylprednisolone, and bortezomib.
RESULTS: The treatment showed a partial response, and he was discharged with 7.3 mg/dL creatinine level. At 4 months, his creatinine plateaued at 5.5 mg/dL and AT1R-Ab decreased to 3.6 U/mL.
CONCLUSIONS: This case highlights the risk of early active antibody-mediated rejection by preformed AT1R-Ab, suggesting its ability to exhibit atypical histopathologic findings, such as total cortical infarction.

BACKGROUND: Sickle cell disease (SCD) is a well-known multisystem illness characterized by vascular injury due to vasoocclusion and hemolysis, as well as infectious complications and iron overload, all of which contribute to high morbidity and mortality rates among children. In these patients, some authors have previously described iron cortical deposition in the kidney. We here report the first case in the literature of a girl affected by SCD showing an anomalous metal and rare element retention in the renal cortex.
METHODS: A 10-year-old white girl affected by SCD underwent a routine magnetic resonance imaging investigation that evidenced a reduced signal intensity in the renal cortex, compatible with hemosiderin precipitation. Histologic and elemental analyses of the hepatic and the renal biotic samples, performed with inductively coupled plasma mass spectrometry, revealed that concomitant with the high iron deposition, toxic and potentially carcinogenic elements such as nickel, magnesium, rubidium, and gadolinuim were anomalously retained particularly in the kidney.
CONCLUSIONS: The finding of rare and toxic elements in the kidney of SCD patients might be linked to the development of specific neoplastic transformations already described in this patient cohort. To be confirmed, our speculations need to be demonstrated in large sampling of patients.

Estimation of the postmortem interval (PMI) is a practical task in daily forensic casework. Researches on PMI is an important practical project in forensic field. Estimation of the time since death is influenced by internal and external, antemortem and postmortem factors, thus the old methods have limitations. Fourier transform infrared (FTIR) spectroscopy has been applied to study the pure protein, nucleic acid and carbohydrate and to detect the changes in complex cells and tissues. At present because the powerful software has could be used to achieve the spectrum transformation, smoothing, baseline correction and normalization, it is possible to analyze the samples quantitatively with the FTIR which has been applied in the biology and clinical medicine. This paper has reviewed the mechanism of FTIR and its application in biomedicine. The postmortem FTIR spectral changes were also discussed, which showed its potential for estimating PMI.

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Karyomegalic interstitial nephritis is a rare, but perhaps an \"underdiagnosed\" condition. Peculiar nuclear changes characterize it, involving mainly tubular cells along with glomeruli and blood vessels. Herein, 3 bioptically proven new cases of patients with chronic renal failure are discussed. The first case had a recently diagnosed karyomegalic nephritis which, to date, still does not require dialysis. The other 2 (brother and sister) required dialysis 4 and 1 years after diagnosis. Karyomegalic changes were found not only in the skin and duodenal biopsies of the male, in skin and liver biopsies of the female and in the urine cells of both patients, but also in several organs (brain, thyroid, lung, esophagus, arteries) as shown at the autopsy of the female. There was a fatal outcome for both patients. The data reported in this study emphasize the usefulness of pathologic investigation of both tissue and urine samples in the identification of this disease. Moreover, as karyomegalic interstitial nephritis is strongly suspected to have a genetic background, its identification may well not only be of clinical relevance, due to its ominous outcome, but may also bear eugenetic value.

Lobar dysmorphism is one of a number of normal anatomic variants that may simulate renal mass lesions on cross-sectional imaging. The authors report the US and CT features in two patients with this unusual entity.

Light and electron microscopic as well as immunofluorescent findings in kidney biopsies of 9 patients suffering from nephronophthisis are reported. The lightmicroscopic picture is unspecific except the cysts at the cortico-medullary junction which are present in 70% of cases. The immunofluorescent findings are either negative or unspecifically positive. On electron microscopy, the tubular basement membranes exhibit characteristic but not specific changes, i.e. homogeneous thickening, splitting, reticulation, thinning, complete loss, granular disintegration and collapse. These findings suggest that the \"Anlageschwäche\" of the nephron especially of the tubular basement membrane may be the primary lesion in nephronophthisis.

In addition to the triad in the Hermansky-Pudlak syndrome (tyrosine-positive oculocutaneous albinism, mild bleeding tendency with a normal platelet-count and widespread accumulation of ceroid-like pigment in various organs), we document severe pulmonary fibrosis, pseudomelanosis coli and deeply pigmented renal cortex. In the liver, innumerable number of pigment-laden Kupffer cells and macrophages in the Glisson capsule were seen. Interestingly, many intralysosomal accumulations of the pigment within the hepatocytes were found by electron microscopy, suggesting that these configurations possibly resulted from a dysfunction of the lysosome itself, especially with regard to loss of digestive and secretory activity. The triad and other complications may also be resultants of a lysosomal dysfunction.

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