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Fanconi Syndrome
Published in Charles Theisler, Adjuvant Medical Care, 2023
Fanconi syndrome (FS), also called Fanconi’s renotubular syndrome, is a kidney disorder in which excess amounts of glucose, cysteine, galactose, fructose, bicarbonate, phosphates (phosphorus salts), uric acid, potassium, and certain amino acids are lost in the urine to be excreted. Normally, these substances are reabsorbed back into the bloodstream by the proximal tubules in the kidneys. A child with FS may experience slow growth, failure to thrive, and chronic kidney disease along with increased urine production, weakness, and bone abnormalities.1 There are hereditary and acquired forms of this disorder. In general, treatment involves maintaining fluid balance through replacement of the nutrients lost in the urine. In severe cases, some people may develop kidney failure and need a kidney transplant.1
Diabetic Nephropathy
Published in Jahangir Moini, Matthew Adams, Anthony LoGalbo, Complications of Diabetes Mellitus, 2022
Jahangir Moini, Matthew Adams, Anthony LoGalbo
The diagnosis of chronic renal failure involves measurements of BUN, electrolytes, creatinine, calcium, and phosphate. A complete blood count (CBC) is routinely done. Urinalysis includes examination of urinary sediment. Quantitative urine protein is assess in a 24-hour protein collection, or a spot urine protein: creatinine ratio. Ultrasonography is often performed, and sometimes, renal biopsy. When serum creatinine is found to be high, the disease is usually suspected. There must be a determination if kidney failure is acute, chronic, or acute superimposed on chronic. This is an acute form of disease that causes more complications of renal function in a person that already has CKD. The cause of kidney failure must be determined. The duration of the disease sometimes reveals the cause, or vice versa.
Inborn Errors of Metabolism
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Surekha Pendyal, Areeg Hassan El-Gharbawy
Liver transplant is recommended in GSD I when adenomas are large and numerous and have a high risk of becoming cancerous. Transplantation is curative for the hypoglycemia and most other complications of GSD I except for neutropenia in GSD Ib. Renal dialysis and transplant is required in some patients for advanced kidney failure.
Baseline anxiety disorders are associated with progression of diabetic kidney disease in type 2 diabetes
Published in Renal Failure, 2023
Bin Han, Ling Wang, Yueyue Zhang, Lijie Gu, Weijie Yuan, Wei Cao
We conducted a prospective, observational cohort study of patients diagnosed with type 2 diabetes in the Diabetes Center of Shanghai General Hospital Affiliated to Shanghai Jiaotong University School of Medicine in Shanghai, China. Patients were consecutively recruited on a basis of their regular hospital visits in the period of 1 May 2017 and 31 June 2020 and were followed up from the baseline to the date of occurrence of a progression event, or 31 June 2021 if no event occurred. At baseline, attending physician ascertained diagnosis of type 2 diabetes on the basis of the Chinese diabetes care guidelines by Chinese Diabetes Society [24]. Exclusion criteria were as follows: (a) age <18 years; (b) having a diagnosis of kidney failure at baseline; (c) a life-threatening comorbidity such as cancer; (d) language/hearing disorders, or dementia; (e) missing clinical information; and, (f) decline to participate in the present investigation. The study protocol adhering to the Declaration of Helsinki was approved by the Ethics Committees of Shanghai General Hospital (2019KY014). The written informed consents were obtained from all individuals prior to participation.
Incidence and risk factors for recurrent focal segmental glomerulosclerosis after kidney transplantation: a meta-analysis
Published in Renal Failure, 2023
Jiang Bai, Tianxiang Zhang, Yan Wang, Jiajing Cao, Zihui Duan, Linghui Ji, Yun Zhou, Chuan Hao, Qiang Guo
No curative therapies exist for the treatment of FSGS, and a significant proportion (40–70%) of patients with FSGS advance to kidney failure within 10–20 years after diagnosis, making FSGS one of the most common primary glomerular diseases leading to kidney failure requiring dialysis, along with diseases such as diabetic nephropathy and hypertensive nephrosclerosis [8,9]. In cases of kidney failure, kidney transplantation is usually the therapy of choice. However, primary FSGS recurrence occurs in 40%–60% of patients after kidney transplantation and varies widely depending on the study population, diagnostic criteria, and follow-up duration [10]. Patients with FSGS recurrence have a 52 percent 5-year graft survival rate compared to 83 percent in patients without FSGS recurrence, which significantly impairs the quality of life and places a heavy burden on families [10].
Shear wave elastography parameters adds prognostic value to adverse outcome in kidney transplantation recipients
Published in Renal Failure, 2023
Tian-yi Zhang, Jiayi Yan, Jiajia Wu, Wenqi Yang, Shijun Zhang, Jia Xia, Xiajing Che, Hongli Li, Dawei Li, Liang Ying, Xiaodong Yuan, Yin Zhou, Ming Zhang, Shan Mou
Kidney transplantation has become a promising choice for patients with kidney failure to prolong life and improve the quality of life. Both short- and long-term survival of kidney transplantation recipients have improved substantially during the last few decades, resulting from advances in immunosuppression, more precise cytotoxic antibody detection and better human lymphocyte match [25–27]. However, evolving challenges such as chronic allograft dysfunction, infection, and cardiovascular diseases are still a concern [28]. Beyond one year after the transplantation, chronic allograft nephropathy was characterized by microvascular and glomerular injury [29]. The use of calcineurin inhibitors (tacrolimus and cyclosporin a) could lead to luminal narrowing, glomerulosclerosis, and tubulointerstitial damage [29]. All the above histological alterations cause increase stiffness of kidney parenchyma.