Renal Lesion Growth in Children With Tuberous Sclerosis Complex

Ewalt, David H.; Sheffield, Eugene G.; Sparagana, Steven; Delgado, Mauricio R.; Roach, E. Steve

doi:10.1016/s0022-5347(01)63072-6

Cited by 274 publications

(101 citation statements)

References 31 publications

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“…Clinically detectable renal cystic disease occurs in approximately 50% of patients with TSC associated with either the TSC1 or TSC2 gene [5,6,7]. TSC patients can have a severe very early-onset polycystic phenotype (fig.…”

Section: Renal Cystic Disease In Tscmentioning

confidence: 99%

“…A cross-sectional study of TSC patients revealed an increase in angiomyolipomas during childhood and adolescence that then stabilized throughout adulthood [13]. In a longitudinal study of children with TSC, 55% of children (mean age 6.9 years) had some type of renal abnormality, and at follow-up 80% (mean age 10.5 years) had abnormalities [6], with the most common form of involvement being angiomyolipomas. Based on their findings, the authors concluded that renal involvement begins in infancy and increases with age.…”

Section: Renal Angiomyolipomas In Tscmentioning

confidence: 99%

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Tuberous Sclerosis Complex Renal Disease

Dixon

Hulbert

Bissler

2010

Nephron Exp Nephrol

138

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Although not as common as other genetic renal diseases such as autosomal dominant polycystic kidney disease, patients with tuberous sclerosis complex frequently have significant renal involvement. Recent revelations in the cell biology of these renal disease manifestations as well as effective therapies for tuberous sclerosis complex-related renal issues have heralded hope of improved renal survival and improved quality of life for the TSC patient. This review specifically addresses some of the major renal manifestations of this disease.

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Section: Renal Cystic Disease In Tscmentioning

confidence: 99%

Section: Renal Angiomyolipomas In Tscmentioning

confidence: 99%

Tuberous Sclerosis Complex Renal Disease

Dixon

Hulbert

Bissler

2010

Nephron Exp Nephrol

138

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“…Renal angiomyolipomas are the most common renal manifestations and are present in approximately 75–80% where as renal cell carcinoma is the least. Single or multiple renal cysts are found in 14–53% cases 6–8. Tuberous sclerosis leading to chronic renal failure is rare 16 17.…”

Section: Discussionmentioning

confidence: 99%

“…Renal angiomyolipomas are the most common renal manifestation where as renal cell carcinoma is the least common. Single or multiple renal cysts are found in about 14–53% of cases 6–8. Polycystic kidney disease is present in less than 2% of cases and is a relatively rare manifestation of this disease.…”

Section: Introductionmentioning

confidence: 99%

Polycystic kidney disease and chronic renal failure in tuberous sclerosis

Dhakal¹,

Dhakal²,

Bhandari

2013

BMJ Case Reports

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Tuberous sclerosis is a rare genetic disease which leads to formation of benign tumours in the brain and other organs of the body. It is a multisystem disease with various clinical manifestations. Renal angiomyolipomas are the most common renal manifestations whereas renal cell carcinoma is the least. Renal cysts are found in around 20% of the patients but polycystic kidney disease is present in less than 2% cases and is relatively rare manifestation of the disease. We present a case of tuberous sclerosis in a 60-year-old man who presented to the medicine outpatient department for routine evaluation of his hypertension. He was diagnosed as tuberous sclerosis. His ultrasound and CT scan of abdomen revealed polycystic kidney disease. His kidney function test and urine analysis were suggestive of chronic kidney disease.

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“…However, in light of the aeromedical risk, we decided to repeat echocardiography every 3 yr in addition to annual physical examination in order to exclude such evolution. Renal lesions can develop even in patients without initial renal abnormalities, and repeated sonography every 2 -3 yr is indicated in patients without cysts or AML at initial study (10,3). Annual sonographic evaluation of the kidneys should reveal an AML or a cyst before it will present clinically and will be preformed as part of the pilot's follow-up.…”

Section: Aeromedical Considerationsmentioning

confidence: 99%

Tuberous Sclerosis in a Military Pilot

Wand¹,

Blum²,

Shachar³

et al. 2009

aviat space environ med

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Tuberous sclerosis (TS) is a multisystem disorder characterized by hamartomas in various organs. It usually manifests itself during infancy or childhood with neurological features, including mental retardation and seizures. We present the case of a military pilot who was diagnosed with TS at 22 yr. of age after he had completed flight training. Suspicion of TS arose from evaluation of a chronic rash around the pilot's nose, and diagnosis was confirmed based on the presence of multiple calcified nodules on CT imaging of the brain. No neurological abnormalities were found. The primary aeromedical concerns were the risk of seizures or development of tumors at sites that might lead to sudden incapacitation. Hamartomas can be reliably detected at an early stage by means of annual history, physical examination, and imaging of tumor-prone organs. After review of the literature and consultation with medical specialists, we assessed the risk of adult-onset seizures in a TS patient without preexisting neurological findings as scarce. The pilot was therefore granted a waiver limited to flying a two-pilot helicopter with a program of tight medical follow-up.

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Renal Lesion Growth in Children With Tuberous Sclerosis Complex

Cited by 274 publications

References 31 publications

Tuberous Sclerosis Complex Renal Disease

Tuberous Sclerosis Complex Renal Disease

Polycystic kidney disease and chronic renal failure in tuberous sclerosis

Tuberous Sclerosis in a Military Pilot

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