Isolated noncompaction of the left ventricular myocardium is a rare cardiac disorder due to an arrest in myocardial morphogenesis. It is characterized by prominent and excessive trabeculation in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Echocardiographic findings are important clues for the diagnosis. Clinical symptoms include signs of left ventricular systolic dysfunction even to the point of heart failure, ventricular arrhythmias, and embolic events. We describe two cases of isolated noncompaction of the myocardium, with ventricular tachycardia in one, and chest pain due to microvascular dysfunction in the other.
Adult* ; Arrhythmias, Cardiac ; Chest Pain ; Diagnosis ; Echocardiography ; Heart Failure ; Humans ; Morphogenesis ; Myocardium* ; Tachycardia, Ventricular

Renal biopsy is an essential diagnostic tool for detecting acute and chronic kidney rejection as well as recurrent and de novo nephropathies in renal allograft recipients. However, a well-known complication of percutaneous renal biopsy is arteriovenous fistula (AVF). Most post-biopsy AVFs are asymptomatic and regress spontaneously but some AVFs result in hypertension, hematuria, and renal insufficiency. Whether post-biopsy AVF superimposed on transplant renal artery stenosis (TRAS) also regresses spontaneously is unknown. We present a case of acute renal insufficiency in a 51-year-old female renal allograft recipient with post-biopsy AVF and TRAS. Percutaneous angioplasty with stent implantation was performed for the TRAS and transcatheter arterial coil embolization therapy applied for AVF. The patient's renal function returned to baseline levels and is currently being followed up for 6 months.
Acute Kidney Injury ; Angioplasty ; Arteriovenous Fistula ; Biopsy ; Female ; Hematuria ; Humans ; Hypertension ; Kidney ; Rejection (Psychology) ; Renal Artery ; Renal Artery Obstruction ; Renal Insufficiency ; Stents ; Transplantation, Homologous ; Transplants

Transplant renal artery stenosis (TRAS) is a common surgical complication after kidney transplantation (KTP) and is the cause of allograft dysfunction. TRAS is a potentially curable cause of refractory hypertension and allograft dysfunction which accounts for approximately 1% to 5% of cases of post-transplant hypertension. Acute cellular rejection (ACR) is also common after KTP, which is the main cause of allograft dysfunction. Although the incidence of ACR has declined with the advent of new immunosuppressive drugs, it is still around 15% worldwide. Although each disease is frequently seen individually, seeing both together is rare. A 42-year-old man with end stage renal disease underwent KTP, and the donor was his younger brother. Four months after KTP, his serum creatinine was increased to 2.1 mg/dL, and renal biopsy showed interstitial lymphocytic infiltration and tubulitis. With the diagnosis of acute T-cell mediated rejection, steroid pulsing therapy was started, but it was resisted. Therefore thymoglobulin 60 mg (1 mg/kg/day) was administered for 6 days, but serum creatinine was 1.8 mg/dL. Abdomen magnetic resonance angiography showed TRAS, stenosis at the anastomosis site and lobar artery in the lower pole. Percutaneous transluminal angiography was performed successfully. After balloon angioplasty, the stenotic lesion showed a normal size and blood flow. The patient's renal function returned to normal levels and he is currently being followed up for 9 months.
Abdomen ; Adult ; Allografts ; Angiography ; Angioplasty, Balloon ; Arteries ; Biopsy ; Constriction, Pathologic ; Creatinine ; Diagnosis ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Kidney Transplantation ; Magnetic Resonance Angiography ; Renal Artery Obstruction* ; Renal Artery* ; Siblings ; T-Lymphocytes ; Tissue Donors ; Transplantation*

ABO-incompatible kidney transplantations have been performed successfully in Korea without splenectomy using plasmapheresis, anti-CD20 monoclonal antibody infusions and other immunosuppressants. However, there is no report of a case of ABO-incompatible kidney transplantation in a Jehovah's Witness. Hence, we report our experience of successful ABO-incompatible kidney transplantation without blood products in a Jehovah's Witness. The recipient was treated with six sessions of plasmapheresis and he received intravenous rituximab before transplantation. Immunosuppressive regimen consisted of tacrolimus, mycophenolate mofetil and steroid. The replacement fluid for plasmapheresis was 5%% albumin solution instead of fresh frozen plasma. We measured the clotting factors before and after plasmapheresis and used cryoprecipitate to prevent bleeding.
Antibodies, Monoclonal, Murine-Derived ; Hemorrhage ; Humans ; Immunosuppressive Agents ; Kidney ; Kidney Transplantation ; Korea ; Living Donors ; Mycophenolic Acid ; Plasma ; Plasmapheresis ; Rituximab ; Splenectomy ; Tacrolimus ; Transplants ; Wit and Humor as Topic

Kidney transplantation (KTP) lowers the mortality and morbidity of patients with end-stage renal disease. Post-transplantation infection and antibody mediated rejection (AMR) are the most common complications. Hepatitis B surface antigen (HBsAg) positive carrier donors and high anti A/B antibody titer ABO incompatible KTP could lead to recipient hepatitis B virus (HBV) infection and AMR. Here, we report a case of successful KTP in a 41-year-old male with a high titer of ABO incompatible and HBsAg positive donor. He underwent seven rounds of plasmapheresis, low dose intravenous immunoglobulin and rituximab treatment to inhibit antibody production and remove antibodies from the serum, after which he was administered anti-viral agent for HBV prophylaxis. The recipient maintained successful allograft function for 6 months after transplantation; therefore, we report that desensitization and anti-viral treatment achieved successful outcome in a 1:512 anti A/B antibody titer ABO incompatible and hepatitis B carrier donor KTP.
Adult ; Allografts ; Antibodies ; Antibody Formation ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Humans ; Immunoglobulins ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Male ; Mortality ; Plasmapheresis ; Rituximab ; Tissue Donors

BACKGROUND/AIMS: Heart rate variability (HRV) is a method for evaluation of autonomic nervous system activity by expressing the balance of sympathetic and parasympathetic tones. Some studies of HRV in patients with end-stage renal disease (ESRD) have been performed in Korea. However, few have examined kidney transplantation (KT) patients. Therefore, we investigated autonomic nervous system activity by means of HRV in patients with KT due to ESRD. METHODS: We compared the pattern of cardiac sympathetic and parasympathetic activity by time- and frequency-domain analysis of HRV with 24-h Holter monitoring of 23 KT and 56 dialysis patients. Patients underwent KT between January, 2008 and June, 2011. RESULTS: The mean ages of KT and dialysis patients were 54.2 +/- 12.3 and 53.7 +/- 12.6 years, respectively. The KT group showed increased time- and frequency-domain HRV (including HRV index), very low frequency (VLF), means and standard deviations of all normal R-R intervals for all 5-min segments of the entire recording (SDNNi), low frequency (LF), LF in normalized units (LF norm), and LF to high-frequency power ratio, compared with the dialysis group. CONCLUSIONS: Autonomic tone in patients with KT is higher than that in patients with ESRD on dialysis.
Autonomic Nervous System ; Dialysis ; Electrocardiography ; Electrocardiography, Ambulatory ; Heart ; Heart Rate ; Humans ; Kidney ; Kidney Failure, Chronic ; Kidney Transplantation ; Korea

Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by the formation of hamartomas in multiple organs. TSC can show lesions including facial angiofibroma, shagreen patch on the skin, cortical tuber, subependymal nodule, astrocytoma in the brain, cardiac rhabdomyoma, and renal angiomyolipoma. In particular, renal angiomyolipoma may be a cause of end-stage renal disease (ESRD). On the other hand, sirolimus has regulatory effects on cellular growth and proliferation via its inhibitory effect on a protein, mammalian target of rapamycin. We report on a case of an 18-year-old male who underwent renal transplantation due to ESRD induced by TSC. Sirolimus played a role in successful treatment of TSC and effective immunosuppression for transplantation.
Adolescent ; Angiofibroma ; Angiomyolipoma ; Astrocytoma ; Brain ; Hamartoma ; Hand ; Humans ; Immunosuppression ; Kidney Failure, Chronic* ; Kidney Transplantation ; Male ; Rhabdomyoma ; Sirolimus* ; Skin ; Tuberous Sclerosis*

BACKGROUND/AIMS: Many studies have reported the correlation between the spot urine protein to creatinine (P/C) ratio and 24-hour urinary protein amounts in patients with glomerulonephritis. This correlation has also been reported in Western patients with kidney transplants, but no prior study has reported on this association in Eastern populations. We compare the correlation between the spot urine P/C ratio and 24-hour urinary protein amounts and the associating factors in Korean patients with kidney transplants. METHODS: The study included 66 patients with kidney transplants from our hospital. The subjects had urine samples evaluated between January 2005 and July 2010. We compared 24-hour urinary protein amounts with a spot urine P/C ratio collected in the morning and analyzed the factors affecting the correlation in each group. RESULTS: The 24-hour urinary protein amounts were 1.31 +/- 1.69 g/day and the spot urine P/C ratio was 1.29 +/- 1.70 in all subjects. A strong positive linear correlation was observed between the 24-hour urinary protein amounts and the spot urine P/C ratio (r = 0.95). The primary factor affecting accurate quantitation of proteinuria using the spot urine P/C ratio was gender (p = 0.003). The spot urine P/C ratio and the 24-hour urinary protein levels were 1.05 +/- 1.51 and 1.26 +/- 1.68 g/day in males (p = 0.005) and 1.57 +/- 1.88 and 1.36 +/- 1.72 g/day in females (p = 0.047), respectively. CONCLUSIONS: We determined that the spot urine P/C ratio provides an accurate estimate of 24-hour urinary protein levels in Korean patients with kidney transplants.
Creatinine ; Female ; Glomerulonephritis ; Humans ; Kidney ; Kidney Transplantation ; Male ; Proteinuria ; Transplants

Thrombotic microangiopathy (TMA) is a serious complication of solid organ transplantation. Drug-induced TMA is typically caused by immunosuppressants, particularly calcineurin inhibitors. Withdrawing the causative drug can be one of the treatments for TMA. However, the more immunosuppressants are reduced, the more risk of rejection increases. Even if TMA is successfully resolved, the outcomes of patient and graft survival would be worse than expected. Therefore, it is necessary to maintain efficient and safe immunosuppression therapy. We report on a case of de novo TMA after kidney transplantation triggered by tacrolimus and reactivated by sirolimus. Belatacept, a novel CTLA4 Ig fusion protein, was administered for maintenance immunosuppressant with mycophenolate mofetil and prednisolon. The patient had excellent early graft outcome, and there have been no adverse events so far.
Abatacept ; Calcineurin ; Graft Survival ; Humans ; Immunosuppression* ; Immunosuppressive Agents ; Kidney Transplantation* ; Kidney* ; Organ Transplantation ; Sirolimus ; Tacrolimus ; Thrombotic Microangiopathies* ; Transplants

BACKGROUND: Aortic valve stenosis induces left ventricular hypertrophy as an adaptive response to the chronic overload caused by the valve disease. Despite the fact that aortic valve replacement may lead to regression of the left ventricular hypertrophy, there is a controversy on the change of the left ventricular muscle after use of small prostheses. MATERIAL AND METHOD: We reviewed 20 patients who had undergone aortic valve replacement for aortic stenosis. There were 13 males and 7 females with a mean age of 61+/-13.8 years. A retrospective analysis of Doppler echocardiography was undertaken in preoperatively, early postoperatively(mean 10.4days), and late postoperatively(mean 29.9 months). They were divided into two groups according to the size of prosthesis used(group 1; 21 mm or smaller, group 2; 23 mm or larger). RESULT: Significant improvement of NYHA Functional class was detected in all groups. Ejection fraction was not significantly different in the group 1 between preoperative and postoperative period, however it increased significantly in the group 2 over time. But preoperative ejection fraction of the group 2 was significantly lower than that in the group 1(p=0.044). Left ventricular muscle mass index(g/m2) was not reduced significantly in the group 1 at the early postoperative period, but it was reduced significantly at the late postoperative period. In the group 2 it was reduced significantly over time. CONCLUSION: Both groups showed clinical improvement. However, the number of patients in whom 19 mm size prosthesis was used was only two. Thus, we suggest that more attention to age, BSA, and exercise should be paid in patients who will undergo aortic valve.
Aortic Valve Stenosis ; Aortic Valve* ; Echocardiography, Doppler ; Female ; Humans ; Hypertrophy, Left Ventricular* ; Male ; Postoperative Period ; Prostheses and Implants* ; Retrospective Studies