Tuberous Sclerosis with Renal Angiomyolipoma and Chronic Renal Failure-

Tuberous Sclerosis with Renal Angiomyolipoma and Chronic Renal Failure-

— A Case Report —Mei-Ling Tsai Yung-Chuan Hsu Boon-Sang LeeDepartment of DermatologyFar Eastern Memorial HospitalPan-Chiao, Taipei, Taiwan, R.O.C.A 53-year-old female patient, was admitted on account of fever andabdominal pain for 2 days. Physical examination revealed mutiple pinkishto skin-colored, firm papules located on the nose, nasolabial fold andcheeks, and several skin-colored, conical papules around the nails of thefingers and toes were also found. Histopathology of a papule on the noserevealed a picture of angiofibroma. Laboratory data showed anemiaurinary-tract infection and chronic renal failure. Abdominal sonogram andCT scan showed multiple renal angiomyolipomas that almost occupied thebilateral kidney. She was diagnosed as tuberous sclerosis with renalangiomyolipoma and chronic renal failure. The association between renalangiomyolipoma and renal failure is extremely rare. We herein report sucha case.key words: Renal angiomyolipoma, Tuberous sclerosis, Renal failure.Accepted for publication November 11, 1994Reprint requests: Mei-Ling Tsai, M.D. Department of Dermatology, Far Eastern MemorialHospital, Pan-Chiao, Taipei county, Taiwan, R.O.C.

Very rare, it may be caused by minor bruises when the daughter of TS merged with RAML worked five years agoThe lesion is small, the development is very slow, and more than half of the right kidney has hemorrhage and removed, and the third daughter has renal dysfunctionThe TS patient cannot live to a full life due to repeated seizures, so it is not ruled out that her daughter also has nephropathyYears 2,3. This article reports on the possibility of tuberous sclerosis.In addition, in physical examination of renal angiomyolioma and chronic renal failure, the patient is sane,Patients, and refer to related literature to discuss renal blood vessel comprehension and good conversation. Blood pressure, breathing and heartClinical diagnosis, pathological changes and treatment of myolipomas | The jump is normal, and there is a large left abdomen on palpation

Treatment method.Lump, and extends to the pelvic cavity. Skin check with Chinese Journal of Pizhi, Volume Thirteen, Issue One, March 84~42~

Tuberous sclerosisNow, there are many large skin slice specimens from the nose, both sides of the nose and Shuangdun, taken from the small pimples of the nose.Histopathological examination of small, painless and itchy, pink to light-skinned tissues shows no special changes in the epidermal layer Small papules (Fig.1); there is a large metaplasia on the left cheek, blood vessel hyperplasia is found in the upper dermis, and the hair follicleDark red plaques about 6×4 cm in size, with obvious proliferation and thickening of collagen fibers around the lesion.There are several raised and scattered small papules (Fig. in line with angiofibroma)2); There is about 3×2 male diagnosis behind the left ear (Fig.8). According to the Institute of Clinical and PathologyThe surface texture is deepened, and it is pink to light brown. It is diagnosed as tuberous sclerosis with renal blood.Colored plaques; there are several tube myolipomas and chronic renal failure on the side edges of fingernails and toenails.There are two skin color nodules (Fig.3).The patient was treated with antibiotics after hospitalization,The laboratory tests are as follows: Hgb 4.3 The symptoms of urethritis are quickly improved, and thegm/dl, Hot 13.1%, serum biochemical blood test improved his anemia. Because the patient is about to enter urineThe check showed BUN 71 mg/dl, Creatinine uremic stage (uremic stage), and the patient is recommended to act6.3 mg/dl, urinalysis showed that WBC 50 venous shunt (AV shunt) was ready to receive blood60/HPF, RBC 2-3/HPF, Protein dialysis treatment, but the patient refused. 10 of 83100 mg/dl, fecal occult blood reaction is negative, follow the patient monthly, and soon after the patient is discharged, i.e.There was no special abnormality in the chest X-ray, and it was collected for 24 hours due to deterioration of renal function.Urine Ccr 9.43 ml/min, APTT and hemodialysis treatment, now in stable condition.The PT is normal, and the arterial blood gas analysis is lightDegree of metabolic acidosis combined with respiratory compensation. XThe light examination revealed that the skull was oval ordiscussRound sclerosis lesions (Fig. 4), palms on both sidesBone, proximal phalanx and metatarsal bones have periosteum thickening and calciumPhenomenon (Fig.5). Head computer tomography tuberous sclerosis is the legacy of a developmental abnormalityIt was found that there are several calcification-transmitted diseases of varying sizes around the ventricle, and the genetic form is autodominant inheritance.Click (Fig.6). The patient’s abdominal ultrasound examination is transmitted, and the three main points of clinical diagnosis are intellectual disability and epilepsy.There are many uniform high-density retinal tumors and angiofibromas in the kidneys on both sides, and there are also brain,Tone lesions, combined with several renal cysts. Diseases of the skin, kidneys and other organs of the bodyThe fish’s abdominal computer tomography showed renal tumors on both sides, but the clinical manifestations were serious or mild.The viscera are almost divided by the lobular fat-rich tumors. 12.3. In recent years, due to diagnostics

Generation (Fig.7). Fundoscopy and cardiac super-improvement will incorporate incomplete phenotypes into the statistics.No special abnormalities were found in the sound waves. The incidence of patients rejecting kidney disease has increased, about 1Dirty biopsy, but abdominal ultrasound and abdominal electricity/94075, 50% of which are newly-occurring basesBrain tomography is consistent with the mutation of renal angiomyolipoma. The incidence of this disease is similar in men and women, ignorantdiagnosis.Significant racial differences.Chinese Journal of Pizhi, Volume Thirteen, Issue One, March 8443

Cai Meizhen Xu Yongchuan Li WenshengTypical TS, the characteristic on the skin is that about 45%-80% of patients with TS will haveAngiofibroma present on the face, under the nails and RAML, the incidence of men and women is similar, it is multipleParietal fibroma (subungual, bilateral, usually small lesions, rarely renalfibroma and periungual fibrona), clinical symptoms related to viscera, but as the patient agesShark-like plaques can be seen on the patient’s trunk (the growth of shagreen, RAML will gradually increase, and the numberpatches) and ash-leaf (ash-leaf) gradually increase 3, 4, 5.8. Secondly, it is more likely to occur in TSmacules), and occasionally the kidney tumors in patients with coffee milk spots are renal cysts, but so far(café-au-lait macules) 2, 4, 5, 10. No correct statistics 4, 16. Clinically aboutIn this case, 50% of the RAML patients in the wing of the nose and cheeks did not have the characteristics of TS when they were young.There are many small papules, which are determined by pathological diagnosis to be more than 40 years old, and they are more common in women.Angiofibroma. In addition, in fingernails and toenails, most of them are single lesions, and the tumors are usually hugeThere are also several paronychia fibroids on the margin. Except for TS patients 8,14,15. According to the literature, in ADIn addition to the above skin characteristics, it is easy to merge with other Bourneville and Brissard in 1880.Anomalies in the organizational system include: in the central god, it is mentioned that RAML is often combined with TS 8, 15. thisThere are often recurrent epilepsy and intellectual tumors as a defect tumor in the system, because the tissues in the embryonic developmentIt is abnormal, and it is easy to merge with huge astrocytoma caused by mixed defect. Yellow and gray under the macro view(giant cell astrocytoma), tumors of the ventricular capsule, sometimes with internal hemorrhage and damageThe subependyinal nodule (subependyinal nodule) died, which may be indistinguishable from renal cell carcinoma at this time.As well as brain calcification lesions, the eyes are rich in fatty tissue, blood vessels, and fibers under the microscope.Omental lens tumor (retinal phacoma)The most tissue and smooth muscle components, each component tumorFor common. The heart is prone to develop rhabdomyomas with different proportions, and the blood vessel wall thickness is curved(rhabdomyoma), the kidney is an irregular feature of RAML; while smooth muscle cells are sometimes presentOr renal cyst. The skeletal system has dense nuclei and pleomorphic nuclei, so it is often misleadingOften bone sclerosis on the skull and pubic bone is diagnosed as malignant tumor. RAML occasionally has local expansion(osteosclerosis) lesions, phalanges and tension, and even lymph nodes, but the clinical is not farCases of cystic change of bone and bone end metastasis and death are still recognizedThe phenomenon of film thickening and calcification. The lung and pancreas are also benign tumors 11, 15, 17.Viscera, thyroid, adrenal glands, and ovaries may also need to be determined to diagnose RAML mainly through the kidneysCombined with defective tumor 1,2,3,4,5,7. There is no pathological biopsy in this case, but the multiplicity of TS Chronic renal failure : a rareassociation. Postgrad Med J 63 :811-813, 1987.17. Pitts WR, Kazam E, Gray G, et al. :Ultrasonography, computerizedtransaxial tomography and pathologyof angiomyolipoma of thekidney : solution to a diagnosticdilemma. J Urology, 124 : 907-909,1980.18. Oesterling JE, Fishman EK,GoldmanSM, et al. : The management ofrenal angiomyolipoma. J Urology.135: 1121-1124, 1986.19. Jardin A, Richard F, Le Duc A, etal. : Diagnosis and treatment of renalangiomyolipoma( based on 15cases ), arguments in favor ofconservative surgery ( based on 8cases ). Eur Urol, 6 : 69-82, 1980.failure treated by cadaveric kidneytransplantation. Clinical Nephrology,4 : 109-112, 1975.7. Roach ES : Neurocutaneoussyndrome. Pediatric Clinics of NorthAmerica, 39 : 591-600, 1992.8.00 21, EK HO RSE LÍTIL É ALIENTI. 41. 4: 169-176,1979.9. O’callaghan TJ, Edwards JA, Tobin M,et al. : Tuberous sclerosis withstriking renal involvement in a family.Arch Intern Med, 135 : 1082-1087,1975.10. Habif TP : Clinical dermatology– acolor guide to diagnosis and therapy.2nd ed. St. Louis : C. V. Mosby Co.,654-655, 1990.11. Ackerman LV, Posai J : SurgicalPathology. 6th ed. St. Louis : C. VMosby Co., 792, 1981.12. Bush WH, Freeny PC, Orme BM :Angiomyolipoma : characteristicimages by ultrasound and computedtomography. Urology, 14 : 531-535,1979.13. Shawker TH, Horvath KL, DunnickNR, et al. : Renalangiomyolipoma : diagnosis by .combined ultrasound andcomputerized tomography. JUrology, 121 : 675-676, 1979.14. Lingman JE, Donohue JP, MaduraJA, et al. : Angiomyolipoma :emerging concepts in management.Urology, 20 : 566-570, 1982.15. Pode D, Meretik S, Shapiro A, etal. : Diagnosis and management ofrenal angiomyolipoma. Urology,25 : 461-467, 1985.16. Nimr AB, Patel PJ, Tongia PK, etE tEU17.094H51. Page 12 Zyrtec(cetirizine)Is More than AntihistamineWwwZYRTEC IS AN ANTIALLERGIC DRUG DEMONSTRATED TO INHIBIT MIGRATIONOF INFLAMMATORY CELLS TO THE SITE OF THE ALLERGIC REACTON.Charlesworth E.N et al. J. All. Clin Immunol.,81/1,212 Abst. 176 (1988). FADEL R. et al. Clinical Allergy, 17:373-379 (1987)MICHEL L. et al. 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