Histomorphological assessment of non-neoplastic renal diseases at autopsy: an institutional experience in Southwestern Nigeria

BackgroundAutopsy remains an invaluable resource for medical education and establishing diagnosis of diseases that were missed prior to death. Many patients on admission in hospitals suffer kidney diseases that may contribute to their morbidity and/or mortality. The kidneys from autopsies provide opportunity to diagnose and understand some of these non-neoplastic renal lesions. This study aimed to present the frequency of non-neoplastic renal diseases at autopsy.Methods We conducted a five-year retrospective review of post-mortem records of deceased who had autopsy. Data such as age, sex, cause of death, and kidney lesions were extracted from the post-mortem records and clinical details were gotten from the clinical summaries in the autopsy reports. The kidneys were examined for pathological findings that were then classified into glomerular, tubulointerstitial (tubulointerstitial nephritis and other tubular lesions such as tubular necrosis, casts and fibrosis) and vascular lesions.Results A total of seventy (70) cases met the inclusion criteria with 91.4% having significant non- neoplastic renal lesions. The mean age of the deceased was 57.7years (18years – 91years). Males accounted for 65.7% of the cases. Glomerular lesions were seen in 84.3% of the cases, tubulointerstitial nephritis in 41.6% of cases, vascular lesions were seen in 30% of the cases and other tubular lesions (such as stones, casts and tubular necrosis) were seen in 52.9% of the cases. Cardiovascular diseases and infections were the major causes of death in these patients, accounting for 40% and 27% respectively. Renal diseases were attributed to immediate cause of death in 10% of the cases.Conclusion The kidney at autopsy provides a valuable renal pathology educational tool, as a wide range of medical renal lesions can be seen from kidneys examined at post mortem.

Multimodality imaging evaluation of pseudotumors in chronic renal dysfunction: exposing the masquerade!

Focal renal lesions in the background of chronic kidney disease (CKD) present a diagnostic challenge. Contrast administration is usually avoided in such a setting, undermining the usefulness of computed tomography and magnetic resonance imaging. Focal regenerating nodules may occur in the background of CKD and closely mimic renal neoplasms. The aim of the present article was to highlight the salient manifestations of such CKD pseudotumors on different imaging modalities and also to depict the differentiating features from malignancy. Radiologists must be aware of the imaging appearance of this uncommonly talked about entity so as to avoid inadvertent surgery or cause undue anxiety to the patient.

Vascular, cardiac, and renal lesions attributed to primary systemic hypertension in western pygmy marmosets (Cebuella pygmaea)

In a retrospective study of a western pygmy marmoset ( Cebuella pygmaea) colony, postmortem examination of 1/8 juvenile and 29/47 adult animals identified vascular, cardiac, and renal lesions consistent with systemic hypertension. This included frequent renal arteriolar hypertrophy, hyaline and proliferative arteriolosclerosis, fibrinoid necrosis of arterioles, glomerulosclerosis, and nephrosclerosis. Affected animals ranged from 0.6 to 12 years of age (mean 6 years) and had an observed male predominance. Genealogical relatedness was evident in several breeding pairs and spanned multiple generations. Concurrent cardiac and renal disease was commonly identified, although frequently subclinical, and both were important causes of morbidity and mortality in affected animals. Cardiomegaly and hypertrophy were typical features and were accompanied by left atrial thrombosis in 10 animals. Signs of heart failure included chronic pulmonary edema in 20 cases and body cavity effusions in 17. In the kidneys, 19 cases had glomerular disease and hypertensive vasculopathy, and 26 cases had nephrosclerosis or glomerulosclerosis. Common extrarenal secondary causes of hypertension were excluded by necropsy examination. The pathogenesis is suggested to involve primary hypertension leading to renal and cardiac disease. Elevated sympathetic activity might be an underlying factor in the frequent development of primary systemic hypertension in the pygmy marmoset, as for the owl monkey.

Role of multidetector ct in quantitative enhancement- washout analysis of solid renal masses

Background Enhancement washout technique in solid renal masses using multidetector computed tomography (MDCT) can differentiate different type of lesions. 99 Patients who are presenting with suspected renal masses or renal tumour for staging are included in this study. CT examination are carried out at urology and nephrology centre using MDCT. The attenuation values (Hounsfield Unit) will be assesed for each lesion on the pre enhanced, corticomedullary, nephrographic and delayed phases. Washout ratio will be calculated for each phase of enhancement in comparison to the unenhanced attenuation value. The characteristics of enhancement-washout will be correlated with the final histopathological diagnosis. Results Early enhancement and washout pattern was noted in 54 renal lesions (54.5%) representing 4 types of renal lesions; Oncocytoma (n = 13), clear cell renal cell carcinoma (n = 16), Chromophobe renal cell carcinoma (n = 15) and unclassified renal cell carcinoma (n = 10).Prolonged enhancement pattern was noted 45 lesions (45.4%); PRCC (n = 14), 10 case of lipid poor AML (n = 10), metanephric adenoma (n = 10) and Xp11 RCC (n = 11). High pre-contrast attenuation was noted in Xp 11RCC showing attenuation value 41.7 ± 6.823HU. The highest CMP values were noted in CCRCC (151.9 ± 20.4) followed by oncocytomas (137.6 ± 19.15HU) and then CHRCC (123.6 ± 16.6 HU)while the lowest values were noted in Metanephric adenoma)57.1 ± 17.4HU)and followed by PRCC (59.9 ± 4.8)and followed by lipid poor AML (79.17 ± 13.666) and RCC unclassified (89.06 ± 18.1). Conclusions Four-phase MDCT (the unenhanced, corticomedullary, nephrographic, and excretory phases) evaluate role of MDCT in differentiation of solid renal masses.

The molecular mechanisms of inflammation and scarring in the kidneys of immunoglobulin A nephropathy

Kidney biopsy is the cornerstone for the diagnosis of immunoglobulin A nephropathy (IgAN). The immunofluorescence technique evidences the IgA deposits in the glomeruli; the routine histology shows degree of active and chronic renal lesions. The spectrum of renal lesions is highly variable, ranging from minor or no detectable lesions to diffuse proliferative or crescentic lesions. Over the past three decades, renal transcriptomic studies have been performed on fresh or frozen renal tissue, and formalin-fixed paraffin-embedded kidney tissue specimens obtained from archival histological repositories. This paper aims to describe (1) the transcriptomic profiles of the kidney biopsy and (2) the potential urinary biomarkers that can be used to monitor the follow-up of IgAN patients. The use of quantitative Real-Time Polymerase Chain Reaction (qRT-PCR), microarrays and RNA-sequencing (RNA-seq) techniques on renal tissue and separated compartments of the nephron such as glomeruli and tubule-interstitium has clarified many aspects of the renal damage in IgAN. Recently, the introduction of the single-cell RNA-seq techniques has overcome the limitations of the previous methods, making that it is possible to study the whole renal tissue without the dissection of the nephron segments; it also allows better analysis of the cell-specific gene expression involved in cell differentiation. These gene products could represent effective candidates for urinary biomarkers for clinical decision making. Finally, some of these molecules may be the targets of old drugs, such as corticosteroids, renin–angiotensin–aldosterone blockers, and new drugs such as monoclonal antibodies. In the era of personalized medicine and precision therapy, high-throughput technologies may better characterize different renal patterns of IgAN and deliver targeted treatments to individual patients.

Genetic spectrum and clinical characteristics of 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency in China

Background Biallelic variants in HSD3B7 cause 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency, a life-threatening but treatable liver disease. The goal of this study was to obtain detailed information on the correlation between the genotype and phenotype of HSD3B7 deficiency and to report on responses to primary bile acid therapy. Methods The medical records of a cohort of 39 unrelated patients with genetically and biochemically confirmed HSD3B7 deficiency were examined to determine whether there exist genotype-phenotype relationships in this bile acid synthesis disorder. Results In all, 34 of the 44 variants identified in HSD3B7 were novel. A total of 32 patients presented early with neonatal cholestasis, and 7 presented after 1-year of age with liver failure (n = 1), liver cirrhosis (n = 3), cholestasis (n = 1), renal cysts and abnormal liver biochemistries (n = 1), and coagulopathy from vitamin K1 deficiency and abnormal liver biochemistries (n = 1). Renal lesions, including renal cysts, renal stones, calcium deposition and renal enlargement were observed in 10 of 35 patients. Thirty-three patients were treated with oral chenodeoxycholic acid (CDCA) resulting in normalization of liver biochemistries in 24, while 2 showed a significant clinical improvement, and 7 underwent liver transplantation or died. Remarkably, renal lesions in 6 patients resolved after CDCA treatment, or liver transplantation. There were no significant correlations between genotype and clinical outcomes. Conclusions In what is the largest cohort of patients with HSD3B7 deficiency thus far studied, renal lesions were a notable clinical feature of HSD3B7 deficiency and these were resolved with suppression of atypical bile acids by oral CDCA administration.

Diffusion tensor imaging based multiparametric characterization of renal lesions in infants with urinary tract infections: an explorative study

Background Conventional diffusion weighted imaging (DWI) is a promising non-invasive tool in the evaluation of infants with symptomatic urinary tract infections (UTI). The use of multiparametric diffusion tensor imaging (DTI) provides further information on renal pathology by reflecting renal microstructure. However, its potential to characterize and distinguish between renal lesions, such as acute pyelonephritic lesions, permanent renal damages or dysplastic changes has not been shown. This study aimed to evaluate the potential of multiparametric DTI for characterization of renal lesions with purpose to distinguish acute pyelonephritis from other renal lesions in young infants with their first UTI. Methods Nine kidneys in seven infants, age 1.0–5.6 months, with renal lesions i.e. uptake reductions, on acute scintigraphy performed after their first UTI, were included. The DTI examinations were performed during free breathing without sedation. The signal in the lesions and in normal renal tissue was measured in the following images: b0, b700, apparent diffusion coefficient (ADC), and fractional anisotropy (FA). In addition, DTI tractographies were produced for visibility. Results There was a difference between lesions and normal tissue in b700 signal (197 ± 52 and 164 ± 53, p = 0.011), ADC (1.22 ± 0.11 and 1.45 ± 0.15 mm2/s, p = 0.008), and FA (0.18 ± 0.03 and 0.30 ± 0.10, p = 0.008) for all nine kidneys. Six kidneys had focal lesions with increased b700 signal, decreased ADC and FA indicating acute inflammation. In three patients, the multiparametric characteristics of the lesions were diverging. Conclusion Multiparametric DTI has the potential to further characterize and distinguish acute pyelonephritis from other renal lesions in infants with symptomatic UTI.

Tibetan Medicines for the Treatment of Diabetic Nephropathy

As an important part of the traditional Chinese medicine system, Tibetan medicine has its unique treatment methods for diabetes mellitus and its complications. Diabetic nephropathy (DN) is one of the most serious diabetic microvascular diseases. Tibetan medicine believes that the occurrence of DN is closely related to renal function changes, and it can be effectively prevented and treated by improving renal lesions. In this paper, we consult ancient books of Tibetan medicine and summarize the medicines that treat kidney disease in the Tibetan medicine system. The Chinese name, English name, and Latin name of these drugs were searched as keywords in the online database. Thirty-four drugs were found for the treatment of DN. The most commonly used were Amomum kravanh, Terminalia chebula, and Tribulus terrestris, and we introduced the traditional uses and modern pharmacological activities of these drugs. The results indicate that Tibetan medicines for kidney disease could be used as potential candidate drugs for DN; they would expand the range of medications for DN and provide a new idea for the treatment of DN.