• Title/Summary/Keyword: Fibromuscular Dysplasia

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Successful Renal Autotransplantation for the Treatment of Severe Renovascular Hypertension in a 14-year-old Boy (신 자가이식을 통한 14세 소년에서 발생한 심한 신성 고혈압의 성공적인 치료)

  • Ji, Min-Chul;Park, Se-Jin;Choi, Jae-Young;Ko, Young-Guk;Kim, Myoung-Soo;Kim, Ji-Hong;Shin, Jae-Il
    • Childhood Kidney Diseases
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    • v.14 no.2
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    • pp.223-229
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    • 2010
  • Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.

Over-expression of MMP-3 in the fissured tissue of cleft lip and palate

  • Park, Young-Wook;Min, Bong-Gi;Kim, Ji-Hyuck;Kim, Soung-Min;Lee, Young-Joon;Lee, Sang-Shin;Lee, Suk-Keun;Moon, Huck-Soo;Chi, Je-Geun
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.32 no.1
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    • pp.19-26
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    • 2006
  • Objective: In order to elucidate the retrogressive degeneration of orofacial cleft, the fissured tissues of prenatal and postnatal cleft lip and palate were examined by histological and immunohistochemical methods. Design: Totally 42 cases of prenatal (n=17) and postnatal (n=25) cleft lip and/or palate were examined in comparison with 10 cases of normal lip and oral mucosa using immunohistochemical stainings of MMP-3, MMP-9, MMP-10, cathepsin G, PCNA, E-cadherin, TGase 2, HSP-70, vWF, and VEGF. Main Outcome Measures: In the fissured tissue the sebaceous glands were strongly positive for PCNA and grew into the underlying fibromuscular tissue (24/42). Some hyperplastic sebaceous glands of prenatal cleft lip produced infundibular follicular cyst (9/17). The skin and mucosal epithelia from the postnatal cleft lip and palate (10/25) showed severe basal hyperplasia (11/25) and melanocyte infiltration (7/25). Results: The immunostaining of MMP-3 and HSP-70 were strongly positive in the hyperplastic sebaceous glands and nearby atrophying muscle bundles of the fissured tissue, while MMP-9, MMP-10, and cathepsin G were almost negative. The immunoreactions of the other antibodies used in this study were similar between in the fissured tissues and in the normal controls. Conclusions: These data suggest that the over-expression of MMP-3 is closely related to the sebaceous gland hyperplasia, epithelial dysplasia, and the muscle degeneration, and that the over-expression of MMP-3 in the fissured tissue may continuously aggravate the cleft condition in the later life.

Diagnostic Criteria of $^{99m}Tc$-diethylenetriaminepentaacetic acid Captopril Renal Scan for the Diagnosis of Renovascular Hypertension by Unilateral Renal Artery Stenosis ($^{99m}Tc$-diethylenetriaminepentaacetic acid 캅토프릴 신장스캔의 단측 신동맥 협착에 의한 신혈관성고혈압 진단 기준)

  • Choi, Seung-Jin;Hong, Il-Ki;Chang, Jae-Won;Park, Su-Kil;Moon, Dae-Hyuk
    • The Korean Journal of Nuclear Medicine
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    • v.38 no.6
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    • pp.498-505
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    • 2004
  • Purpose: We compared captopril renal scintigraphic criteria for the diagnosis of renovascular hypertension by unilateral renal artery stenosis. Materials and Methods: The study group consisted of 24 patients (m/f : 16/8, age: $39{\pm}18$ years) with unilateral renal artery stenosis who underwent renal artery revascularization and captopril renal scintigraphy with $^{99m}Tc$-diethylenetriaminepentaacetic acid between May 1995 and April 2004. The blood pressure response was classified as cure/improvement or failure. We evaluated captopril-induced changes in relative function (BCfun) and renogram grade (0 to 5: 0=normal, and 5=renal failure pattern without measurable uptake) (CBren) and the difference of renograms between the normal and stenotic kidney on captopril scan (CNren). Results: light of 24 patients were cured and 11 improved and 5 patients were classified as failed revascularization. Significant predictors of a cure or improvement of blood pressure were younger age, stenosis by fibromuscular dysplasia or arteritis, BCfun, CBren and CNren. Areas under the receiver operating characteristic curve of age, BCfun, CBren and CNren were not significantly different. Positive and negative predictive values of predictors were 100% and 42% (age ${\leq}38$): 92% and 50% (BCfun ${\geq}1%$): 92% and 75% (CBren ${\geq}1$), and 90% and 60% (CNren ${\geq}1$), respectively. Conclusion: Captopril induced changes in renal function and renogram can reliably predict hypertension response to revascularization. Renogram pattern on captopril scan can diagnose renovascular hypertension without baseline data in patients with unilateral renal artery stenosis.