Outcomes of the surgical management of encapsulating peritoneal sclerosis: A case series from a single center in Korea

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare but near-fatal complication of peritoneal dialysis (PD). Despite the high mortality rate of EPS, the surgical treatment strategy of severe EPS is yet to be established.METHODS: We retrospectively analyzed outcomes of patients with EPS who underwent enterolysis for intractable EPS at Seoul National University Hospital between 2001 and 2018. EPS was diagnosed based on the clinical symptoms and radiological findings of abdominal computed tomography (CT). CT scans were scored according to an EPS scoring system that assessed peritoneal thickening and calcification as well as bowel thickening, tethering, loculation, and dilatation.RESULTS: Thirteen patients (nine males and four females; age, 48 [29–63] years) underwent enterolysis for severe EPS. PD duration (11 [6–21] years) was not associated with survival. Two patients were newly diagnosed with EPS following kidney transplantation. Five patients died of infectious complications immediately after the surgery. Eight patients survived after the first surgery; however, five of them underwent reoperation but died of persistent infection, fistula formation, or adhesive bowel obstruction. Four young (< 60 years) male patients with relatively low CT scan scores (< 13) survived for > 2 years after the first surgery. Median survival duration from EPS diagnosis was 22 (1.3–184) months and that from the first surgery was 9 (0.3–153) months.CONCLUSION: The high mortality rate of EPS suggests the importance of appropriate surgical intervention in young symptomatic male EPS patients with relatively low CT scan scores.

Effect of Simultaneous Nephrectomy on Perioperative Blood Pressure and Graft Outcome in Renal Transplant Recipients with Autosomal Dominant Polycystic Kidney Disease / 대한이식학회지

BACKGROUND: For various reasons, kidney transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) often undergo native nephrectomy in preparation for the transplantation. Simultaneous nephrectomy can result in hypotensive events perioperatively and affect transplant outcome adversely. Our aim was to evaluate the effect of simultaneous native nephrectomy (SNx) on perioperative blood pressure and graft outcome compared to non-nephrectomy (NNx) in renal transplant recipients with ADPKD. METHODS: Data regarding renal function and blood pressure were collected from 42 renal transplant recipients with ADPKD. The primary outcome was graft function over 1 year post-transplant. The secondary outcomes were patient and graft survival, postoperative hypotensive events, and blood pressure control. We compared units of anti-hypertensive medication used by transplanted ADPKD patients in the SNx and NNx groups. RESULTS: Patients with SNx during kidney transplantation showed similar rates of patient and graft survival and renal function. Although they had significantly more hypotensive events during the perioperative period (69.2% vs. 37.5% in NNx, P=0.045), no harmful influence on renal function was observed. No difference in mean blood pressure during the 1-year post-transplant period was observed between the two groups; however, the SNx group required fewer units of anti-hypertensive medication. CONCLUSIONS: SNx is a relatively safe procedure. Graft outcome in the SNx group was not inferior to that of the NNx group, and patients with SNx can have well-controlled blood pressure.

Current progress in ABO-incompatible kidney transplantation

ABO-incompatible kidney transplantation (ABOi KT) was introduced to expand the donor pool and minimize shortage of kidneys for transplantation. Because improved outcomes of ABOi KT were reported in Japan in the early 2000s, the number of ABOi KTs has been increasing worldwide. In addition, a better understanding of immune pathogenesis and subsequent aggressive immunosuppression has helped to make effective desensitization protocols. Current strategies of ABOi KT consist of pretransplant antibody removal using plasmapheresis or immunoadsorption to prevent hyperacute rejection and potent maintenance immunosuppression, such as tacrolimus and mycophenolate mofetil, to inhibit antibody-mediated rejection. Recent outcomes of ABOi KT are comparable with ABO-compatible KT. However, there are still many problems to be resolved. Very high anti-ABO antibody producers are difficult to desensitize. In addition, ABOi KT is associated with an increased risk of infection and possibly malignancy due to aggressive immunosuppression. Optimization of desensitization and patient-tailored immunosuppression protocols are needed to achieve better outcomes of ABOi KT. This review provides an overview of the history, immune mechanism, immunosuppressive protocol, outcomes, current obstacles, and future perspectives in ABOi KT.

The Impact of ABCB1 Gene Polymorphism on Steroid Responsiveness in Acute Rejection in Kidney Transplantation / 대한이식학회지

BACKGROUND: Steroid pulse therapy has been used for patients with acute rejection after kidney transplantation. The ABCB1 gene codes for P-glycoprotein, a transporter that is involved in the metabolism of steroids. However, the role of ABCB1 polymorphisms has not been investigated in patients with acute rejection after kidney transplantation. METHODS: Among 763 patients that received kidney or simultaneous pancreas-kidney transplantation at Seoul National University Hospital between May 1996 and July 2009, 684 patients agreed to genetic sampling for polymorphisms. Acute rejection was defined as biopsy-proven, acute cellular rejection with increased serum creatinine, or in the context of delayed or slow graft function. Steroid-resistance was defined as no improvement in serum creatinine, need for additional OKT3 or ATG treatment, or repeated acute rejection within 30 days. Three polymorphisms of ABCB1 gene (C1236T, C3435T, G2677T/A) were assessed. RESULTS: C allele frequency of C3435T was 59.3% and of C1236T 40.1%. Patients who were steroid-resistant (n=37) had higher serum creatinine at kidney biopsy compared to those who were steroid-sensitive (n=49, P<0.001). The frequency of ABCB1 gene polymorphisms (C1236T and C3435T) did not differ significantly between patients who were steroid-sensitive and those who were resistant. An association with G2677T/A could not be analyzed due to a high failure rate of genotyping. CONCLUSIONS: ABCB1 gene polymorphisms (C1236T and C3435T) were not associated with steroid resistance in patients with acute cellular rejection after kidney transplantation.

Impact of Combined Acute Rejection on BK Virus-Associated Nephropathy in Kidney Transplantation

BK virus-associated nephropathy (BKVAN) is one of the major causes of allograft dysfunction in kidney transplant (KT) patients. We compared BKVAN combined with acute rejection (BKVAN/AR) with BKVAN alone in KT patients. We retrospectively analyzed biopsy-proven BKVAN in KT patients from 2000 to 2011 at Seoul National University Hospital. Among 414 biopsies from 951 patients, biopsy-proven BKVAN was found in 14 patients. Nine patients had BKVAN alone, while 5 patients had both BKVAN and acute cellular rejection. BKVAN in the BKVAN alone group was detected later than in BKVAN/AR group (21.77 vs 6.39 months after transplantation, P=0.03). Serum creatinine at diagnosis was similar (2.09 vs 2.00 mg/dL). Histological grade was more advanced in the BKVAN/AR group (P=0.034). Serum load of BKV, dose of immunosuppressants, and tacrolimus level showed a higher tendency in the BKVAN alone group; however it was not statistically significant. After anti-rejection therapy, immunosuppression was reduced in the BKVAN/AR group. Renal functional deterioration over 1 yr after BKVAN diagnosis was similar between the two groups (P=0.665). These findings suggest that the prognosis of BKVAN/AR after anti-rejection therapy followed by anti-BKV therapy might be similar to that of BKVAN alone after anti-BKV therapy.

Clinicopathologic Features of IgA-Dominant Postinfectious Glomerulonephritis

BACKGROUND: IgA-dominant acute postinfectious glomerulonephritis (APIGN) is a recently recognized morphologic variant of APIGN, but its clinicopathologic features were not clearly characterized. We will present demographic, clinical and renal biopsy findings from seven patients with IgA-dominant APIGN with a literature review. METHODS: All renal biopsy specimens (n=1,119) processed by the Department of Pathology in Hanyang University Hospital from 2005 to 2009 were reviewed. Seven patients with IgA-dominant APIGN were identified, and their clinical data analyzed. RESULTS: All patients had renal failure, hematuria and proteinuria. One was diabetic, and none of the patients had previous renal diseases. Three had clinical infections at the time of presentation: 2 with methicillin-resistant Staphylococcus aureus and one with rickettsial infection. Light microscopically diffuse endocapillary proliferative and exudative glomerulonephritis was found in all cases. Immunofluorescence microscopy showed granular IgA deposits along peripheral capillary walls and in mesangium. Ultrastructurally, subepithelial 'humps' with mesangial deposits were noted. End-stage renal disease developed in two patients, chronic renal failure was stationary in two, and azotemia improved in three. CONCLUSIONS: Various infections including rickettsiosis preceded IgA-dominant APIGN in both diabetics and nondiabetics. Because the prognosis of IgA-dominant APIGN is poor, early diagnosis based on renal biopsy is required.

Lupus Cystitis and Azathioprine-iIduced Pure Red Cell Aplasia in a Patient with Systemic Lupus Erythematosus / 대한신장학회지

We report a case of lupus cystitis as the manifestation of lupus flare, and pure red cell aplasia resulting from the use of azathioprine in a patient with systemic lupus erythematosus (SLE). A 30-year-old female with a nine-year history of SLE was admitted to our hospital with complaint of anemia and azotemia. Eighteen and three months before, she had two episodes of lupus enteritis treated with high dose steroid. She had serologic evidence of an SLE flare at admission. Abdominal computed tomography revealed bilateral hydronephrosis and hydroureter with marked diffuse thickening of the urinary bladder wall, suggesting lupus cystitis. Treatment with corticosteroid led to prompt normalization of her renal function. Use of azathioprine may lead to severe anemia. The bone marrow examination revealed a decrease of erythropoiesis, suggesting pure red cell aplasia. Serologic tests for hepatitis B and parvovirus B19 were negative. There was immediate hemoglobin recovery after complete azathioprine discontinuation.

Intravenous Sodium Bicarbonate and Oral N-acetylcysteine for the Prevention of Contrast-Induced Nephropathy / 대한내과학회지

BACKGROUND/AIMS: The value of hydration with sodium bicarbonate and N-acetylcysteine (NAC) in the prevention of radiocontrast-induced nephropathy is questionable. This study investigated whether sodium bicarbonate hydration with or without NAC has a more protective role in the prevention of radiocontrast-induced nephropathy than saline hydration with or without NAC. METHODS: We prospectively studied 100 patients with significant proteinuria (> or = 500 mg/d), azotemia (serum creatinine > or = 1.5 mg/dL), or diabetes mellitus who were undergoing coronary angiography using iodixanol, a nonionic iso-osmolar contrast agent. Patients were assigned randomly to receive saline infusion (S, n = 24), saline infusion plus NAC (S + NAC, n = 20), sodium bicarbonate infusion (B, n = 25), and sodium bicarbonate plus NAC (B + NAC, n = 31). Contrast-induced nephropathy was defined as an increase of 25% or more in the serum creatinine within 48 hours of contrast exposure. RESULTS: There were no significant group differences in age, sex, and basal serum creatinine. Contrast-induced nephropathy occurred in 20 patients (20%) and its incidence was not significantly different among the groups; four from group S, five from group S + NAC, five from group B, and six from group B + NAC. The incidences were not significantly different when compared between S and B, irrespective of the use of NAC (21 vs. 20%), and when compared according to the presence of pre-existing azotemia (19 vs. 20%). CONCLUSIONS: The efficacy of sodium bicarbonate hydration in the prevention of contrast-induced nephropathy seems comparable to that of saline hydration, and it was not improved by the addition of NAC.

Cisplatin nephropathy in patients with lung cancer / 대한내과학회지

BACKGROUND/AIMS: This study characterized the cisplatin nephrotoxicity occurring in patients treated with chemotherapy for lung cancer. METHODS: In all, 124 patients with lung cancer received cisplatin 70 mg/m2 on day 1 every three weeks for up to six cycles with preventive hydration using 3 L of 0.45% saline. Acute and chronic cisplatin nephropathy were defined as an increase in serum creatinine > or =30% at 3 weeks after each cisplatin administration and an increase in serum creatinine > or = 50% after the six cycles of chemotherapy, respectively. RESULTS: Acute cisplatin nephropathy occurred in 23 of 124, 8 of 110, 6 of 92, 10 of 68, 7 of 59, and 7 of 45 patients after the 1st to 6th cycle of chemotherapy, respectively. In all, 51 patients (51.5%) experienced acute cisplatin nephropathy. Chronic cisplatin nephropathy occurred in 25 out of 45 patients (55.5%). The occurrence of chronic cisplatin nephropathy was significantly associated with that of acute cisplatin nephropathy (p<0.01). In chronic cisplatin nephropathy, the serum creatinine increased to 1.82+/-1.18 mg/dL from the basal 0.82+/-0.11 mg/dL (p<0.01). It was 1.60+/-1.05 mg/dL at the end of the follow-up period (112+/-90 days). CONCLUSIONS: Despite prophylactic hydration, the incidence of cisplatin nephropathy in patients with lung cancer is still high. Acute cisplatin nephropathy may predispose patients to chronic cisplatin nephropathy, but the latter does not seem to be progressive.

A Case of Plasmodium vivax Malaria Complicated with Acute Renal Failure, Jaundice and Thrombocytopenia / 대한신장학회지

Malaria is caused by four species of the genus Plasmodium. Plasmodium vivax malaria is the most common malarial infection in Korea and usually has benign clinical course. However, serious complications such as severe anemia, pulmonary edema, acute renal failure are reported in Plasmodium vivax malaria. We report a case of Plasmodium vivax malaria complicated with acute renal failure, jaundice and thrombocytopenia. A 56-year-old male was transferred to our hospital with acute renal failure, jaundice and thrombocytopenia. 10 days before admission, he had intermittent fever, chill, myalgia, and was treated with some medications under the impression of URI. Laboratory findings showed that hemoglobin was 11.5 g/dL, platelet 44,000/mm3, blood urea nitrogen 73 mg/dL, creatinine 4.0 mg/dL, total bilirubin 5.2 mg/dL, direct bilirubin 4.0 mg/dL. Serologic tests for leptospirosis, rickettsia, EB virus and CMV were negative. Ring form trophozoites were found in red blood cells, suggesting Plasmodium vivax malaria. Following anti-malarial therapy, acute renal failure, jaundice and thrombocytopenia were recovered to normal. From this case, malarial infection should be included as a differential diagnosis in a febrile patient with acute renal failure, jaundice and thrombocytopenia.

Neurotoxicity Induced by Cefepime in a Patient with Minimal Change Disease / 대한신장학회지

A 71-year-old woman with minimal change disease visited our clinic complaining of pleuritic chest pain. Cefepime was given under the impression that she had pneumonia. Three days after cefepime administration, she became unconscious. A brain MRI scan was non-revealing and an EEG showed triphasic waves. As there was no evidence of septic, uremic or hepatic encephalopathy, we suspected cefepime-induced neurotoxicity. Cefepime was stopped and she underwent hemodialysis to decrease the blood levels of the drug. Following hemodialysis, she regained consciousness.

A case of peripheral T-cell lymphoma with central nervous system and bilateral pulmonary involvement / 대한내과학회지

Mature T-cell non-Hodgkin's lymphoma (NHL) has more frequent extranodal involvement and is less sensitive to chemotherapy than B-cell lymphoma. Peripheral T-cell lymphoma unspecified (PTCL-U) is rarely combined with pulmonary or CNS involvement. We report a case of PTCL-U with lung and CNS involvement that was treated with high-dose chemotherapy followed by autologous stem cell transplantation (ASCT). A 62-year-old man was admitted with right-side weakness and paresthesias, and was diagnosed with PTCL-U after alung biopsy. Successful complete remission was achieved after threecycles of IMEP (ifosfamide, methotrexate, etoposide, and prednisone) chemotherapywith concurrent intrathecal chemotherapy and subsequent high-dose chemotherapy with ASCT to treat potential advanced stage PTCL-U. The non-anthracycline-containing IMEP regimen can be effective for PTCL-U, especially in cases with CNS involvement.

A Case of Klebsiella pneumoniae Liver Abscess Which Progressed to Pyomyositis and Infected Aneurysm of Aorta / 감염과화학요법

Klebsiella pneumoniae causes pyogenic infections in various sites, with the risk of which increases in patients with diabetes mellitus. Recently, K. pneumoniae has emerged as a leading cause of pyogenic liver abscess. Primary liver abscess caused by K. pneumoniae in the absence of underlying hepatobiliary disease is commonly associated with metastatic infections such as endophthalmitis, meningitis, brain abscess and infection in other sites. We experienced a case of K. pneumoniae liver abscess associated with septic metastatic lesions including pyomyositis and infected aneurysm of aorta. Despite the aggressive management with antibiotics, surgical pus drainage and aortic bypass graft, patient died of ventilator associated pneumonia and multiorgan failure. Our experience suggests that clinicians should be alert to septic metastatic infections when they treat a patient with K. pneumoniae liver abscess.

A Case of Klebsiella pneumoniae Liver Abscess Which Progressed to Pyomyositis and Infected Aneurysm of Aorta / 감염과화학요법

Klebsiella pneumoniae causes pyogenic infections in various sites, with the risk of which increases in patients with diabetes mellitus. Recently, K. pneumoniae has emerged as a leading cause of pyogenic liver abscess. Primary liver abscess caused by K. pneumoniae in the absence of underlying hepatobiliary disease is commonly associated with metastatic infections such as endophthalmitis, meningitis, brain abscess and infection in other sites. We experienced a case of K. pneumoniae liver abscess associated with septic metastatic lesions including pyomyositis and infected aneurysm of aorta. Despite the aggressive management with antibiotics, surgical pus drainage and aortic bypass graft, patient died of ventilator associated pneumonia and multiorgan failure. Our experience suggests that clinicians should be alert to septic metastatic infections when they treat a patient with K. pneumoniae liver abscess.

Infectious Complications in Patients with Kidney Transplantation: Follow-Up Results in Single Center / 대한이식학회지

PURPOSE: Infectious complications may remain a significant cause of morbidity and mortality following renal transplantation. This study was undertaken to investigate current status of infectious complications in renal transplant recipients. METHODS: Clinical data were retrospectively analyzed from 534 recipients who underwent kidney transplantation in Hanyang University Hospital from February 1986 to September 2006. RESULTS: 251 recipients (47%) had 390 episodes of infectious complications. Most patients (86.8%) suffer two or less episodes of infection. The skin and soft tissue (40.7%) infection was the most common over the whole post-transplant periods, and the most common causative organism was virus (55.2%). Among the bacterial infections, urinary tract (43%) was the most common site of infection. Among the viral infections, varicella-zoster virus was the most frequent. Increasing age at kidney transplantation (> or =40 years) was associated with the risk of infection within one month after transplantation. Both acute rejection and loss of allograft function were associated with the risk of infectious complications. The overall profile of infectious complications occurring after renal transplantation did not seem to be changed during the last two decades. However, the mortality rate associated with the infection showed a decreasing tendency over the past decade. CONCLUSION: Infectious complications are still important to affect the outcomes of kidney transplantation. Strategies to further reduce the infectious complications are necessary.

A Case of a Solitary Fibrous Tumor of the Pleura Presenting as Pneumonia and Acute Respiratory Failure / 결핵및호흡기질환

Solitary fibrous tumors of the pleura (SFTPs) are relatively rare tumors that originate from the mesenchymal cells of the submesothelial tissue of the pleura. Patients with SFTPs are often asymptomatic, while some patients experience pleuritic chest pain, cough and/or dyspnea. We report here on a case of SFTP, and the patient presented with septic shock and respiratory failure that required mechanical ventilation. A 68-year-old woman was admitted for the evaluation of her dyspnea and generalized edema. Chest imaging studies showed an 18 cm-sized voluminous mass occupying the right thoracic cavity with anterior displacement of hilar structures and atelectasis of the right lung. Immediately after admission, she developed pneumonia and septic shock that required antibiotics and mechanical ventilation. She displayed a partial response to medical treatment, and then complete excision of the tumor was performed and the pathologic examination revealed benign SFTP. Afterward, she fully improved without evidence of recurrence until now.

Histological Changes of Gastric Atrophy and Intestinal Metaplasia after Helicobacter pylori Eradication / 대한소화기학회지

BACKGROUND/AIMS: Long-term Helicobater pylori infection results in atrophic gastritis and intestinal metaplasia, and increases the risk of gastric cancer. However, it is still controversial that eradication of H. pylori improves atrophy or metaplasia. Therefore, we investigated histological changes after the H. pylori eradication in patients with atrophy or metaplasia. METHODS: One hundred seven patients who received successful eradication of H. pylori infection in Hanyang University, Guri Hospital from March 2001 to April 2006, were enrolled. Antral biopsy was taken before the eradication to confirm the H. pylori infection and grade of atrophy or metaplasia by updated Sydney System. After a certain period of time, antral biopsy was repeatedly taken to confirm the eradication and investigate histological changes of atrophy or metaplasia. RESULTS: Mean age of the patients was 55.3+/-11.3, and average follow-up period was 28.7+/-13.9 months. Endoscopic diagnosis included gastric ulcer, duodenal ulcer, non-ulcer antral gastritis. Atrophy was observed in 41 of 91 and their average score was 0.73+/-0.92. After the eradication of H. pylori, atrophy was improved (0.38+/-0.70, p=0.025). However, metaplasia which was observed in 49 of 107, did not significantly improve during the follow-up period. Newly developed atrophy (7 of 38) or metaplasia (18 of 49) was observed in patients who without atrophy or metaplasia initially. Their average scores were slightly lower than those of cases with pre-existing atrophy or metaplasia without statistical significance. CONCLUSIONS: After the eradication of H. pylori infection, atrophic gastritis may be improved, but change of intestinal metaplasia is milder and may take longer duration for improvement.

Water Intoxication Following Low-Dose Intravenous Cyclophosphamide

Cyclophosphamide is frequently used for the treatment of severe lupus nephritis, but is very rarely associated with dilutional hyponatremia. Recently we experienced a case of water intoxication following low-dose intravenous cyclophosphamide. Five hours after one dose of intravenous pulse cyclophosphamide 750 mg, the patient developed nausea, vomiting, and general weakness. Serum sodium concentration revealed 114 mEq/L and her hyponatremia was initially treated with hypertonic saline infusion. Then her serum sodium concentration rapidly recovered to normal with water restriction alone. During the course of intravenous pulse cyclophosphamide therapy, one must be aware of the possibility of significant water retention.