Wilson’s disease is a rare inherited disorder that causes copper to accumulate in your liver, brain and other vital organs. Most people with. Anexo:Enfermedades epónimas Enfermedad de Kienböck; Enfermedad de Kikuchi; Enfermedad de Kimmelstiel-Wilson; Enfermedad de Klippel-Trénaunay. Request PDF on ResearchGate | On May 1, , C. Calderón-González and others published Enfermedad renal crónica: clasificación, etiopatogenia y factores.
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Nodular glomerulosclerosis in a non-diabetic hypertensive smoker with dyslipidemia. Predosa 1W. Gomes 1M. Teles 1V.
Nodular glomerulosclerosis may be idiopathic or develop associated with diabetes mellitus, membranoprolipherative glomerulonephritis, light or heavy chain deposits, amyloidosis, fibrillary or immunotactoide disease, and Takayasu’s arteritis.
Histological features of idiopathic nodular glomerulosclerosis are similar to the Kimmelstiel-Wilson changes. Recent evidence points to the role of hyperglycemia, hyperlipidemia, hypertension and smoking in the mechanisms of this uncommon condition.
The case study of a year-old male presenting recent arterial hypertension and nodular non-diabetic glomerulosclerosis is described, and the possible role of heavy smoking in the pathogenesis of this condition is emphasized. Idiopathic nodular glomerulosclerosis ING refers to histological features of glomerular lesions similar to the Kimmelstiel-Wilson changes, in patients without evidence of underlying disease Tobacco use constitutes a major worldwide problem, which is related with increased indices of morbidity and mortality, years of potential life lost, and productivity losses 6.
Smoking is related to microalbuminuria in healthy people and may impair renal function by diverse mechanisms, including the genesis of advanced glycation end products AGEsoxidative stress, angiogenesis, intrarenal hemodynamic disorders, and hypertensive effects of hypoxia on the sympathetic and the rennin-angiotensin systems 2. ING has been associated with hypertension, smoking and hypercholesterolemia, which show similar prevalence The role of heavy smoking is highlighted in the patient here described with diagnosis of nodular glomerulosclerosis.
A year old white man presenting with peripheral oedema for 4 months was admitted because of progressive loss of renal function. There was antecedent of high blood pressure systolic: Periodic evaluations about albuminuria and creatinine enfermedxd had been unremarkable until near two years before admission.
He was a cigarette smoker pack-year: Two of his brothers had hypertension, and his father had chronic renal failure; they were heavy cigarette smokers, but the patient had no information about the exact etiology, type and severity of his father renal disease. There was no family history of consanguineous marriage or diabetes mellitus. His fasting blood glucose, glycated haemoglobin HbA1cand glucose tolerance d were all within the normal ranges.
On admission, he appeared chronically ill and body mass index was Heart and lung examinations were unremarkable. Direct ophthalmoscopy did not disclose retinopathy. Renal ultrasonography US showed normal symmetrical kidneys.
CONCEPTOS ACTUALES SOBRE NEFROPATÍA DIABÉTICA
Diagnosis of nephrotic syndrome was established and US guided kidney biopsy was done. Renal specimen contained 23 glomeruli and 3 of them were globally sclerotic; the remaining glomeruli showed focal or diffuse expansion of mesangial area in a nodular pattern. There was mild tubular atrophy, and afferent and efferent arteriolar hyalinosis Fig.
Immunofluorescence did not show immune deposits, and no amyloid was seen by Congo red stain Fig. Electron microscopy showed marked diffuse basement membrane thickening and some effaced podocytes. Peripheral capillary loops were uniformly thickened, without abnormal deposits. No fibrillary or micro tubular structure was observed Fig. This year old white male with ING was a heavy tobacco smoker and had diagnosis of arterial hypertension. Moreover, he presented with high triglycerides and cholesterol blood levels, but no disturbance in glucose metabolism was detected.
ING patients with hypertension, hypercholesterolemia and smoking may show exaggerated glomerulovascular response due to higher sensitivity to non-diabetic glycemic levels. Therefore, special attention has been paid to the follow-up of glucose tolerance to rule out eventual late-onset diabetes 2.
Three of the main risk factors involved in the pathogenesis of ING are briefly commented. The effects of uncontrolled hypertension on the progression of chronic nephropathy are well established, as well as the role played by the rennin-angiotensin-aldosterone system both on the progression of diabetic nephropathy and the development of glomerular nodular lesions.
Contrary to most of the previous reports, hypertension was not severe in this patient and retinal examination was normal, suggesting a less conspicuous role of hypertension in the genesis of the nodular changes here reported. Although hypertension seemed not essential for the origin of nodular glomerulosclerosis in the present case study, smoking is a major risk factor for high blood pressure and for ING 5.
Hypertensive nephrosclerosis is considered the main precursor event for development of ING; and uncontrolled hypertension can adversely influence the outcome of glomerulosclerosis 5. In contrast with the STZ model, the animals develop marked increase in serum triglycerides and cholesterol, and lower levels of serum glucose.
These findings strongly suggest that, at least in animal models, hyperglycemia itself is not enough to cause the development of the glomerular nodular change, while hyperlipidemia constitutes an important synergistic factor.
There is growing evidence that cigarette smoking increases the risk of progression in diabetic and non-diabetic nephropathy. Unfortunately, there are no animal models of smoke-induced kidney damage to demonstrate nodular glomerulosclerosis. Furthermore, regression of these lesions with smoking cessation was never unequivocally showed.
However, as almost all reported cases have kimmelztiel-wilson described in smokers, smoking is kimmelstuel-wilson as the main risk factor for the development of ING.
Moreover, smoke of cigarettes contains free radicals capable of directly inducing oxidative stress 2which may increase the production of glomerular extracellular matrix by activation enfdrmedad TGF-and IGF receptor prosclerotic signalling pathways encermedad.
Although the age of onset of ING is more than 60 years, Kikuchi et al reported a year-old male with nodular glomerulosclerosis and hypertension.
The patient was tobacco smoker for more than 13 years, and was exposed to passive smoking for decades, because his parents were smokers at home 1. Nasr and D’Agati reported a year-old non-diabetic woman with longstanding hypertension, chronic renal kimmesltiel-wilson and albuminuria, but without dyslipidemia. She was a reformed smoker with COPD due to a pack-year: The hypertensive heavy smoker here described was also a passive smoker at home.
Epidemiological and experimental studies have disclosed relationships between smoking, hypertension and dyslipidemia, both in diabetic and in non-diabetic individuals 8,9. Smoking is associated with kimmepstiel-wilson, increased glicated haemoglobin, and metabolic syndrome 9. Nicotine can increase proteinuria, glomerular hypertrophy, and mesangial area in diabetic mice, in association with increased NADPH oxidase 4nitrotyrosine, and Akt expression kmimelstiel-wilson.
Non-diabetic mild-to-moderate hypertensive patients may have microalbuminuria MAa marker of endothelial dysfuction, and dyslipidemia may play a role in the origin of MA Data about initial MA of this patient are lacking because he was firstly followed elsewhere.
Pathology Outlines – Diabetic glomerulosclerosis
In Spain, like in Brazil, smoking poses a heavy social and economic burden despite of official envermedad against the tobacco consumption 6,which is related with diverse malignancies, bronchopulmonary and arterial diseases, dyslipidemia, and hypertension 6,9. This case study seems to strengthen the association between heavy smoking and nodular glomerulosclerosis, and may contribute to enhance the interest about further experimental studies to better clear the role of tobacco smoking on the development of ING.
Association between long-term smoking and hypertension and early-onset nodular glomerulosclerosis. Nippon Jinzo Gakkai Shi ; Idiopathic nodular glomeurulosclerosis in a non-diabetic hypertensive smoker-case report and review of literature.
Neprhol Dial Transplant ; Li W, Verani RR. Hum Pathol ; Idiopathic nodular glomerulosclerosis is a distinct clinicopathologic entity linked to hypertension and smoking. Nodular glomerulosclerosis in the nondiabetic smoker. J Am Soc Nephrol ; An Sist Sanit Navar ; Nicotine worsens the severity of nephropathy in diabetic mice: Am J Physiol Renal Physiol ; The dd between psychosocial stress, age, BMI, CRP, lifestyle, and the metabolic syndrome in apparently healthy subjects.
J Physiol Anthropol ; Microalbuminuria and its relations with serum lipid abnormalities in adult Nigerians with newly diagnosed hypertension. Ann Afr Med ; 9: Rev Paul Pediatr ; J Bras Pneumol ; 5: Case report A year old white man presenting with peripheral oedema for 4 months was admitted because of progressive loss of renal function. Discussion Kimmlestiel-wilson year old white male with ING was a heavy tobacco smoker and had diagnosis of arterial hypertension.