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Von Hippel−Lindau Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
RCC (>3 cm) should be removed early by nephron-sparing or partial nephrectomy or radiofrequency ablation, while lesions <3 cm require no intervention. Renal transplantation may be necessary for patients undergoing bilateral nephrectomy.
Noninfectious Pulmonary Manifestations of Renal Disease In Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Stephen T. Lawless, H. Jorge Baluarte
As with other forms of rapidly progressive renal failure, early and aggressive therapy is required. Poor results can be expected in oliguric patients or in those with renal failure severe enough to need dialysis support (e.g., >8 mg/dℓ creatinine). Initial therapy with daily 3 to 4 ℓ plasma exchanges combined with 2 mg/kg/day of azathioprine or cyclophosphamide plus 1 mg/kg/day of prednisone has been recommended, at the same time monitoring the degree of renal and pulmonary function, and level of anti-GBM antibody. Although many uncertainties exist, it appears at the present time that bilateral nephrectomy should be reserved for those patients with presumptive evidence of irreversible glomerular damage who have life-threatening pulmonary hemorrhage and who have failed to respond to a brief trial of high dose corticosteroid and/or intensive plasma exchange.
Associated disorders
Published in Steve Hannigan, Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
Treatment of Drash syndrome depends mainly on the symptoms of the disorder, although patients with progressive kidney failure can be maintained on dialysis. In this case, toxins that would normally be excreted from the body in the urine are instead removed from the blood by a machine. Some patients may have their kidneys removed (bilateral nephrectomy) and then go on to have renal replacement therapy. Those afected may also have their testes or ovaries removed to reduce the risk of malignancy. Other treatment may include management of fluid and electrolytes, treatment for high blood pressure, and chemotherapy for patients with Wilms’ tumour. Genetic counselling is recommended for those affected by this condition.
Focal Segmental Membranoproliferative Glomerulonephritis: A Histological Variant of Denys-Drash Syndrome.
Published in Fetal and Pediatric Pathology, 2021
A. B. Karmila, Y. C. Yap, M. Appadurai, L. Oh, M. Fazarina, F. Abd Ghani, H. Ariffin
The risk for WT in individuals with DDS is high. It remains debatable whether prophylactic nephrectomy should be carried out in children with DDS who have isolated nephritis prior to renal transplantation. Min et al. reported two patients with DDS and ESRF; both of whom underwent bilateral nephrectomy at the time of renal transplantation [14]. Histological analysis of the specimens showed presence of nephroblastomatosis, a pre-malignant entity, and raises the possibility that had these affected kidneys been left in situ, they may have progressed to WT. For similar concerns; Eddy et al. advocated bilateral nephrectomy at the onset of ESRF in DDS [15].
Diagnostic work up of an adult female patient with systemic AA amyloidosis revealing the cause of infantile mental retardation
Published in Amyloid, 2018
Kaya Veelken, Stefan O. Schönland, Georg Gdynia, Tim Weber, Norbert Blank, Ute Hegenbart
We report on a 44 years old female with infantile brain damage and a known restriction of kidney function. Biopsy specimen had revealed renal deposits of AA amyloid. Chronic inflammatory bowel disease was diagnosed due to unspecific abdominal symptoms and suspicious histological samples. Treatment with sulphasalazine was started. Nine years later, the patient was hospitalized upon a rapid deterioration of general condition including nausea and an increased abdominal girth. Terminal renal failure was diagnosed and emergency dialysis commenced. Subsequently, the patient was referred to our amyloidosis outpatient clinic for further investigations. Initial blood analysis showed normocytic anaemia (haemoglobin 93 g/L), elevated CRP (74 mg/L; normal <5 mg/L) and serum amyloid A protein levels (SAA) (151 mg/L; <10 mg/L). Molecular genetic testing revealed a pro-amyloidogenic allelic variant in the SAA1 gene, (Val52(GTC)/Ala57(GCG)) [3]. Considering the patient’s Greek ancestry, Familial Mediterranean fever was excluded as no mutations of the MEFV gene were found. Diagnostic work-up also revealed enormous cystic lesions of both kidneys (Figure 1(A)). Bilateral nephrectomy was performed and histological analysis exposed clear cell renal cell carcinoma and amyloid deposits (Figure 1(B,C)) (left kidney: pT2b, pN0(0/1), G2, R0, stage II; right kidney: T1a, pN0(0/1), G2, R0, stage I) with extensive tissue necrosis. Besides glomerular AA amyloidosis, cancer-free renal parenchyma showed numerous mesenchymal lesions consistent with lymphangioleiomyomatosis (LAM) (Figure 1(D)). Considering the pathomechanism of this tumour, a phacomatosis was suspected. Indeed, further molecular genetic testing revealed a pathogenic point mutation of the TSC1 gene (IVS-I1G > A) consistent with tuberous sclerosis complex (TSC).
Single-dose intravesical amikacin instillation for pyocystis in a patient with autonomic dysreflexia: A case report
Published in The Journal of Spinal Cord Medicine, 2022
Erin Sherwin, Cynthia King, Howard Hasen, Shari May
A 59-year-old African American male with T1 American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade A paraplegia secondary to a motor vehicle collision was transferred from a skilled nursing facility to the Memphis Veterans Affairs Medical Center Spinal Cord Injury unit for wound care and treatment of osteomyelitis. His past medical history was significant for neurogenic bladder and bowel, hypertension, and anuria after bilateral nephrectomy secondary to suspected renal cell carcinoma dependent on hemodialysis.