Karyomegalic interstitial nephritis: diagnosed only when suspected

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Monica Sivakumar ◽  
Balan Louis Gaspar ◽  
Balakrishnan Narasimhan

Abstract Background Karyomegalic interstitial nephritis (KIN) is an uncommon cause of chronic interstitial nephritis that eventually progresses to end-stage renal disease. Overall less than 50 cases have been reported in the literature. Case presentation We describe an asymptomatic 25-year-old gentleman with a family history of chronic interstitial nephritis who came to check the status of his kidney functions. On evaluation, he was found to have chronic interstitial nephritis which could not be attributed to a specific etiology. Renal biopsy confirmed the diagnosis of KIN. Conclusion KIN remains underdiagnosed. It is important to recognize this entity because of the familial nature, a wide range of differential diagnoses, and prognostic implications. A high index of clinical suspicion is necessary to perform renal biopsy which remains the gold standard for the diagnosis of KIN.

2017 ◽  
Vol 7 (2) ◽  
pp. 121-129 ◽  
Author(s):  
Gagandeep Kaur ◽  
Babitha Bijin ◽  
Kamron Saleem ◽  
Benjamin Sarsah ◽  
Bijin Thajudeen

Amyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria. There are only a limited number of cases reported in the literature. We present a 64-year-old Hispanic female with a history of hypertension who was referred for chronic kidney disease management. The review of her laboratory tests revealed a serum creatinine of 1.5–1.8 mg/dL and microalbuminuria (in the presence of a bland urine sediment) in the past year. She denied any history of diabetes, rheumatologic disorders or exposure to intravenous contrast, nonsteroidal anti-inflammatory drugs, herbals, and heavy metals. Serological workup was negative. A renal biopsy showed diffuse infiltration of glomerulus with pale eosinophilic material strongly positive for Congo red stain and a similar eosinophilic material in the interstitium, muscular arteries, and arterioles. Electron microscopy showed marked infiltration of the mesangium, capillary loops, and interstitium with haphazardly arranged fibrillary deposits (9.8 nm thick). Liquid chromatography tandem mass spectrometry confirmed leukocyte cell-derived chemotaxin 2 (LECT2) amyloid deposition. LECT2 amyloidosis (ALECT2) should be suspected in renal biopsy specimens exhibiting extensive and strong mesangial as well as interstitial congophilia. Individuals with LECT2 renal amyloidosis have a varying prognosis. Therapeutic options include supportive measures and consideration of a kidney transplant for those with end-stage renal disease.


Author(s):  
Channa Jayasumana ◽  
Carlos Orantes ◽  
Marc E. De Broe

Chronic Interstitial Nephritis in Agricultural Communities (CINAC) has been increasingly recognized since the early 1990s. It has been called epidemic chronic kidney disease unknown cause (CKDu) in Sri Lanka, and meso-American nephropathy in Central America. CINAC occurs regionally in the Tropics, predominantly in rural/agricultural zones. It is increasingly recognized, but also believed to be increasing in incidence. Men are affected up to three times more often than women. Its incidence increases with age, and a number of other epidemiological factors impact on it. In some areas, such as the North Central Province of Sri Lanka and regions of Central America, it drives extreme rates of CKD and end-stage renal failure. Clinically, it has the non-specific characteristics of other slowly-evolving chronic interstitial nephritis (Chapter 86). Perhaps distinctive is an inconsistent history of episodes of dysuria, sometimes loin pain, in earlier disease. Its aetiology remains unsolved. Maps of incidence commonly show a mosaic pattern, suggesting that exposure to local factors are implicated. It has been associated with working outdoors in high temperatures, but this seems inadequate as the sole explanation. Exposure to nephrotoxins, natural or possibly as agrochemicals, seems likely.


1977 ◽  
Vol 14 (2) ◽  
pp. 121-127 ◽  
Author(s):  
R. Müller-Peddinghaus ◽  
G. Trautwein

A morphologic study of 103 dogs, including two with renal amyloidosis, showed that different types of diffuse glomerulonephritis are correlated with different age groups. Membranous and membranoproliferative glomerulonephritis were more common in middle-aged and older animals, whereas mesangial lesions were found predominantly in younger dogs and considered to be early glomerular changes. Glomerulonephritis largely occurred independently of interstitial nephritis. The incidence of interstitial lesions was 71%. Chronic interstitial nephritis was rare in dogs under 1 year old. Glomerulonephritis did not seem to induce interstitial nephritis. Glomerulonephritis occurred not only in kidneys with severe interstitial damage, but also in those with slight damage. This indicated that glomerulonephritis occurred independently of interstitial nephritis. In end-stage kidneys with severe fibrosis, mesangial changes seemed to predominate.


Author(s):  
Anne M Straffen ◽  
DJS Carmichael ◽  
Angela Fairney ◽  
B Hulme ◽  
M Snell

Abnormalities of calcium homeostasis are a recognized feature of end-stage renal disease. The treatment of choice is renal transplantation, but this does not always result in normalization of the biochemical profile. Persistent hypercalcaemia is well documented and our study was undertaken to investigate the status of the calcium regulating hormones in renal patients post-transplantation. Serum calcium, parathyroid hormone, 1,25-dihydroxyvitamin D (1,25(OH)2D) and osteocalcin concentrations were measured in post-transplant patients. Twenty per cent of the patients had subnormal 1,25(OH)2D concentrations while 55% had biochemical evidence of hyperparathyroidism but only 5% were hypercalcaemic. Time elapsed since transplantation was not correlated with any of the analytes investigated and there was no relationship between persistent impairment of renal function and abnormalities of calcium homeostasis.


2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Dimitri Mikhalski ◽  
Karl Martin Wissing ◽  
Renaud Bollens ◽  
Daniel Abramowicz ◽  
Vincent Donckier ◽  
...  

Advanced atherosclerosis or thrombosis of iliac vessels can constitute an absolute contraindication for heterotopic kidney transplantation. We report the case of a 42-year-old women with end-stage renal disease due to lupus nephritis and a history of bilateral thrombosis of iliac arteries caused by antiphospholipid antibodies. Occlusion had been treated by the bilateral placement of wall stents which precluded vascular anastomosis. The patient was transplanted with a right kidney procured by laparoscopic nephrectomy from her HLA semi-identical sister. The recipient had left nephrectomy after laparoscopical transperitoneal dissection. The donor kidney was orthotopically transplanted with end-to-end anastomosis of graft vessels to native renal vessels and of the graft and native ureter. Although, the patient received full anticoagulation because of a cardiac valve and antiphospholipid antibodies, she had no postoperative complication in spite of a short period of delayed graft function. Serum creatinine levels three months after transplantation were at 1.0 mg/dl. Our case documents that orthotopical transplantation of laparoscopically procured living donor kidneys at the site of recipient nephrectomy is a feasible procedure in patients with surgical contraindication of standard heterotopic kidney transplantation.


2021 ◽  
Vol 22 (4) ◽  
pp. 2009
Author(s):  
Anne Grunenwald ◽  
Lubka T. Roumenina ◽  
Marie Frimat

The incidence of kidney disease is rising, constituting a significant burden on the healthcare system and making identification of new therapeutic targets increasingly urgent. The heme oxygenase (HO) system performs an important function in the regulation of oxidative stress and inflammation and, via these mechanisms, is thought to play a role in the prevention of non-specific injuries following acute renal failure or resulting from chronic kidney disease. The expression of HO-1 is strongly inducible by a wide range of stimuli in the kidney, consequent to the kidney’s filtration role which means HO-1 is exposed to a wide range of endogenous and exogenous molecules, and it has been shown to be protective in a variety of nephropathological animal models. Interestingly, the positive effect of HO-1 occurs in both hemolysis- and rhabdomyolysis-dominated diseases, where the kidney is extensively exposed to heme (a major HO-1 inducer), as well as in non-heme-dependent diseases such as hypertension, diabetic nephropathy or progression to end-stage renal disease. This highlights the complexity of HO-1’s functions, which is also illustrated by the fact that, despite the abundance of preclinical data, no drug targeting HO-1 has so far been translated into clinical use. The objective of this review is to assess current knowledge relating HO-1’s role in the kidney and its potential interest as a nephroprotection agent. The potential therapeutic openings will be presented, in particular through the identification of clinical trials targeting this enzyme or its products.


2001 ◽  
Vol 12 (12) ◽  
pp. 2759-2767 ◽  
Author(s):  
Gérard M. London ◽  
Bruno Pannier ◽  
Alain P. Guerin ◽  
Jacques Blacher ◽  
Sylvain J. Marchais ◽  
...  

ABSTRACT. Left ventricular (LV) hypertrophy (LVH) is a risk factor for mortality in patients with end-stage renal disease (ESRD). Whether the attenuation of LVH has a positive effect on survival of patients with ESRD has not been documented. The aim of this study was to determine the effect of parallel treatment of hypertension and anemia on LV mass (LVM) and to determine the effect of LVM changes on survival. A cohort of 153 patients receiving hemodialysis was studied. The duration of follow-up was 54 ± 37 mo. All patients had echocardiographic determination of LV dimensions and LVM at baseline and regular intervals until the end of the follow-up period. During the study, BP decreased from (mean ± SD) 169.4 ± 29.7/90.2 ± 15.6 to 146.7 ± 29/78 ± 14.1 mmHg (P< 0.001), and hemoglobin increased from 8.65 ± 1.65 to 10.5 ± 1.45 g/dl (P< 0.001). The LV end-diastolic diameter and mean wall thickness decreased from 56.6 ± 6.5 to 54.8 ± 6.5 mm (P< 0.001), and from 10.4 ± 1.6 to 10.2 ± 1.6 mm (P< 0.05), respectively. The LVM decreased from 290 ± 80 to 264 ± 86 g (P< 0.01). Fifty-eight deaths occurred, 38 attributed to cardiovascular (CV) disease and 20 attributed to non-CV causes. According to Cox analyses after adjustment for age, gender, diabetes, history of CV disease, and all nonspecific CV risk factors, LVM regression positively affected the survival. The hazard risk ratio associated with a 10% LVM decrease was 0.78 (95% confidence interval, 0.63 to 0.92) for all-causes mortality and 0.72 (95% confidence interval, 0.51 to 0.90) for mortality due to CV disease. These results show that a partial LVH regression in patients with ESRD had a favorable and independent effect on patients’ all-cause and CV survival.


2021 ◽  
Vol 14 (6) ◽  
pp. e242553
Author(s):  
Dilpat Kumar ◽  
James Boyer ◽  
Warsha Fnu ◽  
Harry Boamah

A 46-year-old woman with a history of end-stage renal disease on chronic haemodialysis presented with 1 week of fever, chills, altered mental status and hand pain. She was febrile and ill-appearing on presentation with a pulse rate of 102 beats per minute. She had a tunnelled dialysis catheter in her right neck. Hand examination demonstrated a swollen, erythematous and tender wrist. Cardiovascular examination demonstrated no murmurs. CT of the hand showed abscesses involving the left forearm. Blood and abscess cultures grew methicillin-resistant Staphylococcus aureus (MRSA). Transesophageal echocardiography (TEE) showed a 1.0×1.0 cm mobile vegetation involving the eustachian valve (EV), confirming EV endocarditis. She remained bacteraemic for 18 days despite being on vancomycin with appropriate blood levels. Vancomycin was switched to daptomycin and ceftaroline, which cleared her cultures. Repeat TEE showed improved vegetation size. Our case highlights the rarity and management of EV endocarditis and the importance of synergy for treatment of persistent MRSA bacteraemia.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Eva Paraskevi Andronikidi ◽  
Glykeria Tsouka ◽  
Myrto Giannopoulou ◽  
Konstantinos Botsakis ◽  
Xanthi Benia ◽  
...  

Abstract Background and Aims Renal transplantation is considered the most effective and less costly modality of renal replacement therapy in patients with end stage renal disease. The disparity between kidney allografts and recipients has led to a global effort to increase the pool of kidney donors. Accordingly, fibromuscular dysplasia (FMD) is no longer considered an absolute contraindication for kidney donation. The incidence of FMD is about 2.3%-5.8% in potential kidney donors. There are few cases in the literature where renal artery stenosis in allografts with known pre-transplantation FMD became worse after transplantation, indicating the importance of a proper follow up in the recipients. This is a case of a living kidney donor with no history of hypertension, proteinuria or elevated serum creatinine, whose intra-arterial digital subtraction angiography revealed FMD lesions in the left renal artery. Method Case report Results A 54-year-old Caucasian female with medical history of hypothyroidism took the decision to offer her kidney to her 37-year-old son who was diagnosed with end-stage renal disease five years ago secondary to diabetes mellitus type I. She had no history for diabetes, hypertension and renal disease. Her vital signs on admission were heart rate of 78 beats/min and blood pressure of 130/70 mmHg. Urinalysis, biochemical profile and serological evaluations were all within normal ranges. Blood urea was 36 mg/dL and serum creatinine was 0.6 mg/dL (eGFR 97ml/min/1.73m2). The abdominal ultrasound and renogram with Tc-99m DTPA showed no remarkable findings. On intra-arterial digital subtraction angiography an abnormal succession of dilatations and multifocal stenoses of the left renal artery, characteristic of medial FMD, was found. The right renal artery was normal. Apart from a dysfunctional permanent left femoral catheter, the patient had no other vascular access for hemodialysis because of Superior Vena Cava syndrome, so he needed urgent transplantation. Taking all of these into consideration, the patient was offered renal transplantation as the best option. A left open donor nephrectomy was performed; the renal artery was divided distal to the stenotic dysplastic area. The allograft was placed at the right iliac fossa of the recipient with arterial and venous anastomosis to the extrarenal iliac vessels. Post-operatively, the recipient had a delayed graft function lasted 13 days. On renal artery Doppler in the allograft we found increased resistance index (RI) that gradually normalized without any intervention. An immunosuppressive regiment of tacrolimus, mycophenolate and prednisone was administered according to our center protocol. At discharge serum creatinine was 1.7 mg/dL (eGFR: 50ml/min/1.73m2). At the year follow-up, the donor was normotensive and had near normal renal function (Cr:1.3mg/dL, eGFR: 70ml/min/1.73m2). The recipient has a well-controlled blood pressure receiving two antihypertensive drugs and maintains a satisfactory renal function. Conclusion Few cases with FMD in renal allografts from living and deceased donors have been described. In a review of 4 studies the authors concluded that the outcome of transplantation with allografts from living donors with medial FMD was satisfactory and these allografts could be used to increase the donor pool. Furthermore, it is strongly recommended to have a thorough pre-transplantation check of the donor as well as a close monitoring of both the donor and recipient after transplantation. This case shows that allografts harvested from carefully selected donors with renal arterial FMD can be successfully used, particularly in urgent conditions. Detailed pre-tranplantation imaging of donor’s renal arteries, selection of the appropriate screening method, as well as close monitoring of both donor and recipient for early interventions after transplantation is of paramount importance.


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