Bartter syndrome is characterized by markedly reduced or absent salt transport by the thick ascending limb of Henle. The phenotype of Bartter syndrome is renal salt wasting, hypokalemic metabolic alkalosis, increased renin-angiotensin-aldosterone system, with normal or low blood pressure. Most of the cases have been noted in the pediatric age group and adult-onset cases are rare. Nephrocalcinosis is common in antenatal Bartter syndrome. We report a case of adult-onset Bartter syndrome associated with nephrocalcinosis.
Alkalosis ; Bartter Syndrome* ; Extremities ; Humans ; Hypokalemia ; Hypotension ; Nephrocalcinosis* ; Phenotype ; Renin-Angiotensin System

PURPOSE:BK virus associated nephropathy (BKVAN) affects 1-10% of kidney transplant (KT) patients and it produces a progressive destruction of allograft. Reducing immunosuppression is the only way to save the graft, while it needs tight monitoring of the graft rejection and graft survival is poorer in advanced case. Leflunomide has immunosuppressive effect and also antiviral activity. Addition of leflunomide may improve BK virus clearance and graft survival. METHODS:6 KT patients with biopsy proven BKVAN (Histological pattern B) were treated with leflunomide and reduced immunosuppression. All patients were monitored with serial determination of viral load in blood and graft function. RESULTS:BKVAN was diagnosed at 14 months (7-28) post transplant, at that time median serum creatinine concentration was 2.8 mg/dL (1.8-3.6). 12.5 months (6-16) later of leflunomide treatment, median serum creatinine was 2.3 mg/dL and no graft loss was found. CONCLUSION:Leflunomide therapy with reduced immunosuppression may be effective in the treatment for BKVAN.
Biopsy ; BK Virus ; Creatinine ; Graft Rejection ; Graft Survival ; Humans ; Immunosuppression ; Isoxazoles ; Kidney ; Kidney Transplantation ; Transplantation, Homologous ; Transplants ; Viral Load ; Viruses

BACKGROUND/AIMS: Diabetic nephropathy is a growing cause of end-stage renal failure, and renal transplantation is considered the best option for survival in patients who experience such renal failure. Patients with diabetes are older and frequently have comorbidity, and only a minority of these patients is considered for renal transplantation. The survival rate of patients having diabetes treated with transplantation has improved, although the long-term prognosis has not been determined. This study examined the clinical outcome of renal transplantation in patients having diabetes compared to that in nondiabetic patients. METHODS: We compared diabetic (n=70) and nondiabetic (n=355) groups of patients for whom medical records were available for more than 3 months at our hospital from March 1998 to August 2008. RESULTS: The recipients were significantly older in the diabetic group (50.6+/- 10.5 vs. 39.8+/- 10.8 years), while donor age was significantly younger in that group (33.2+/- 9.0 vs. 37.5+/- 1.3 years). Cardiovascular events occurring after transplantation were more frequent in the diabetic group (11/70 vs. 10/355). Kaplan-Meier curves for cumulative survival of the renal allograft and patient survival revealed no difference between the two groups. The allograft survival rate in the diabetic group was 100% at 5 years and 79% at 10 years posttransplantation. In the nondiabetic group, the renal allograft survival rate was 98% at 5 years and 75% at 10 years. The patient survival rates did not differ significantly: 100% vs. 99% at 5 years and 91% vs. 91% at 10 years for the diabetic and nondiabetic groups, respectively. CONCLUSIONS: In our study, the long-term survival of renal transplantation in patients with diabetes equaled that of nondiabetic patients. Graft survival was also comparable between the two groups. Therefore, kidney transplantation may be another therapeutic option for end-stage diabetic nephropathy.
Comorbidity ; Diabetic Nephropathies ; Graft Survival ; Humans ; Kidney ; Kidney Failure, Chronic ; Kidney Transplantation ; Medical Records ; Prognosis ; Renal Insufficiency ; Survival Rate ; Tissue Donors ; Transplantation, Homologous ; Transplants

Behcet's disease is a chronie inflammatory symptom complex consisting of recurrent oral ulcer, genital ulcer, ocular lesion or skin lesion. Intestinal involvement in Behcet's disease most commonly affects the ileocecal region in the patient with gestrointestinal symptoms, But esophageal involvement in Behcet's disease is very uncommon, most commonly affects the midesophagus with localized ulcerative lesion. A 38-Year-old woman with refractory and recurrent oral and genital ulcer was admitted because of aggravation of pharyngodynia and dysphagia. Gastrofiberscopic examination showed multiple, small sized ulcers on oral cavity and mid-to-distal esophagus. She was treated with steroid.
Adult ; Deglutition Disorders ; Esophagus ; Female ; Humans ; Mouth ; Oral Ulcer ; Skin ; Ulcer

It has been well known that long-term immune suppression in renal transplant patients increases the possibility of complications. Infectious disease is one of the representative complications. We experienced a case of nocardiosis with cytomegalovirus infection after third renal transplantation in China. Nocardiosis is an important opportunistic infection in immunosuppressed patients, lymphoma, sarcoidosis, and organ transplant patients. CMV can cause severe hepatitis, pneumonitis, enteritis, endometritis, and encephalitis. It can depress bone marrow, and impair the immune system so as to increase other bacterial infection and trigger rejections. Third renal transplantation causes long-term immune suppression or over-immune suppression on transplant patients. Very few cases of third renal transplantation have been reported in Korea. We reduced the dose of immune- suppressants, and treated it successfully with ganciclovir and Trimethoprim/Sulfamethoxazole (Bactrim(R)).
Bacterial Infections ; Bone Marrow ; China* ; Communicable Diseases ; Cytomegalovirus ; Cytomegalovirus Infections ; Encephalitis ; Endometritis ; Enteritis ; Female ; Ganciclovir ; Hepatitis ; Humans ; Immune System ; Kidney Transplantation* ; Korea ; Lymphoma ; Nocardia Infections* ; Opportunistic Infections ; Pneumonia ; Sarcoidosis ; Transplants

Surgical clips can migrate into the biliary tract and act as a nidus for stone formation. We report a case of common bile duct stone developed due to a surgical clip in a 48-year-old man. Endoscopic retrograde cholangiogram revealed a common bile duct stone a with metallic clip in it. He had laparoscopic cholecystectomy 10 years ago. The stone was removed endoscopically. The use of resorbable clips during laparoscopic cholecystectomy is recommended to avoid this type of complication.
Cholangiopancreatography, Endoscopic Retrograde ; Cholecystectomy, Laparoscopic/adverse effects/*instrumentation ; Choledocholithiasis/*etiology/radiography/therapy ; *Foreign-Body Migration ; Humans ; Male ; Middle Aged ; Surgical Instruments/*adverse effects

Although malignant melanoma involving the stomach is commonly mentioned in association with autopsies, endoscopic experience in this gastric malignancy is still limited, and few cases have been illustrated in the literature. This clinical condition is even rarer in Asians who are much less vulnerable to malignant melanoma than Caucasians. We experienced two cases of gastric metastases of malignant melanoma which presented as indigestion and epigastric pain. In the first case, a 75-year-old man visited because of indigestion persisting for 2 months. He had a history of multiple gastric ulcer and was treated at a local medical center, but symptoms did not improved. Endoscopic finding revealed multiple and elevated lesions with central black pigmented bases. In the second case, a 47-year-old man visited because of epigastric soreness and intermittent pain for 1 month. Metastatic melanoma in the stomach was noticed by endoscopic examination. Both patients had multiple metastatic lesions in the liver, thyroid, and brain. These two cases were diagnosed as having metastatic malignant melanoma in the stomach of unknown origin. Therefore, we report these cases with a brief review of the related literature.
Aged ; Asian Continental Ancestry Group ; Autopsy ; Brain ; Dyspepsia ; Humans ; Liver ; Melanoma* ; Middle Aged ; Neoplasm Metastasis ; Stomach ; Stomach Ulcer ; Thyroid Gland

PURPOSE: The presence of C4d in peritubular capillaries (C4d (PTC)) as a diagnostic in-situ marker of acute humoral rejection and CD20 as marker of B-cell deposition in graft kidney has been reported to be related to steroid resistance and poor outcome. In this retrospective study, we evaluated the clinical significance of C4d and CD20 in allograft renal biopsies by immunohistochemistry technique. And we also evaluated the relationships between C4d and CD20 positive B lymphocytes. METHODS: We studied 22 patients who had been biopsied for suspected acute rejection. Biopsies were classified by updated Banff 97 criteria. Of the 22 cases, borderline rejection and Banff 1A were 11 cases respectively and no case had a vascular lesion. Paraffin sections were stained with monoclonal antibodies (anti-C4d and -CD20) using an immunohistochemistry technique and the results of immunohistochemistry were analyzed by clinical data. RESULTS: Of the 22 cases, 22.7% (5/22) showed diffuse and 40.9% (9/22) showed focal C4d positivity in peritubular capillaries. The grafts failed to survive in 20% (1/5) of the diffuse (P), 44.4% (4/9) of the focal, and 0% (0/8) of the negative group for 2 years since postbiopsies, however, the C4d staining was not statistically related to graft loss and graft survival rates (P=0.091, P=0.106 respectively). The C4d positivity was significantly related to the level of serum creatinine (P=0.042) and to steroid pulsing therapy resistance (P=0.030). However C4d deposition was not associated with recipient gender, age, type of donor (living vs deceased), HLA matching, induction, and Banff classification. On the CD20 immunostaining, 50.0% (11/22) showed negative reactivity, 9.1% (2/22) one nodule, 40.9% (9/22) 2 nodules. The presence and the number of CD20 positive nodules were not correlated to the C4d clinical data. But, the degree of C4d staining was statistically related with the presence of CD20 positive nodules (P=0.029). CONCLUSION: The peritubular capillary C4d is clinically important however, not likely a significant predictor of grafts survival rates in mild rejection. The clinical implication of CD20 positive B lymphocyte nodules in acute rejection was not demonstrated in this study. But, CD20 positive B lymphocyte may be a positive linkage with C4d and participate in humoral rejection.
Antibodies, Monoclonal ; B-Lymphocytes ; Biopsy ; Capillaries ; Creatinine ; Graft Survival ; Humans ; Immunohistochemistry ; Kidney ; Lymphocytes ; Paraffin ; Rejection (Psychology) ; Retrospective Studies ; Survival Rate ; Tissue Donors ; Transplantation, Homologous ; Transplants

BACKGROUND/AIMS: It is well known that the menopause is related to interference in lipid metabolism, obesity, and a hypercoagulable state. The aim of the present study was to examine the impact of the menopause in middle-aged Korean females with acute myocardial infarction (AMI). METHODS: A total of 1,781 middle-aged females (aged < 65 years) in the Korean Acute Myocardial Infarction registry were enrolled into this study between November 2005 and December 2013. The patients were divided into two groups; the pre-menopause group (≤ 55 years old) and the menopause group (56-64 years old). Major adverse cardiac events (MACE) were analyzed over a one-year follow-up period. RESULTS: The pre-menopause and menopause groups comprised 669 patients (mean age, 49.1 ± 5.6 years) and 1,112 patients (mean age, 60.6 ± 2.6 years), respectively. The incidence of hypertension (42.2% vs. 59.4%, p < 0.001), diabetes mellitus (DM) (27.4% vs. 35.7%, p < 0.001), and dyslipidemia (12.9% vs. 17.7%, p = 0.008) were more frequent in menopausal patients. Additionally, the rates of smoking (20% vs. 12.7%, p < 0.001) and familial history (12% vs. 6.8%, p < 0.001) were higher in the pre-menopause group. The cumulative rates of MACE did not show any differences between the two groups. A history of atrial fibrillation, previous AMI and DM, higher Killip class, and multi-vessel disease were independent risk factors for predicting one-year MACE. CONCLUSIONS: The survival analysis demonstrated that there was no significant difference in MACE rates between the pre-menopause and menopause groups during the one-year follow-up. Therefore, middle-aged pre-menopausal women should be treated more intensively, regardless of whether they are menopausal.
Atrial Fibrillation ; Diabetes Mellitus ; Dyslipidemias ; Female* ; Follow-Up Studies ; Humans ; Hypertension ; Incidence ; Lipid Metabolism ; Menopause ; Myocardial Infarction* ; Obesity ; Premenopause ; Prognosis ; Risk Factors ; Smoke ; Smoking