Pulmonary Calciphylaxis Associated with Acute Respiratory and Renal Failure Due to Cryptogenic Hypercalcemia: An Autopsy Case Report

Metastatic calcification is rare; it is found during autopsy in patients who underwent hemodialysis. Diffuse calcium precipitation of small and medium-sized cutaneous vessels, known as calciphylaxis, can result in progressive tissue necrosis secondary to vascular calcification. This condition most commonly involves the skin; however, a rare occurrence of visceral calciphylaxis has been reported. Here we report on an autopsy case. Despite a thorough evaluation, and even performing an autopsy, the underlying cause of acute-onset hypercalcemia, resulting in the production of pulmonary calciphylaxis and metastatic renal calcification associated with acute respiratory and renal failure, could not be determined. Metastatic calcification often lacks specific symptoms, and the degree of calcification is a marker of the severity and chronicity of the disease. This unusual autopsy case emphasizes the importance of rapidly progressing visceral calciphylaxis, as well as its early detection.

The Patency Rate of Hemodialysis Vascular Access and the Analysis of Patency-related Factors: Comparision of Native Arteriovenous Fistula with Arteriovenous Graft, Single Center Study / 대한신장학회지

PURPOSE: As the proportion of patients with diabetes and old age increases, the use of arteriovenous graft (AVG) is increasing. However, there are few data about the comparison of the patency rate of native arteriovenous fistula (AVF) to that of AVG in Korea. We compared the outcome of native AVF to that of AVG with analysis of the factors affecting the patency of the permanent vascular access in use. METHODS: A retrospective database of all vascular access related procedures performed from January 1, 2003 to December 31, 2003 was established. We evaluated the primary unassisted and cumulative access patency rate with analysis of patency-related factors. We also evaluated the primary failure rate of AVF and AVG. RESULTS: 196 new vascular access surgeries were performed during the period. 14 cases were excluded due to loss of follow-up. 142 native arteriovenous fistulas (78%) and 40 grafts (22%) were constructed. The primary failure rate was similar between AVF and AVG group. The primary unassisted (78%, 72%, 68% vs. 62%, 41%, 22% at 1, 2, 3 year; p<0.001) and cumulative patency rate (93%, 88%, 85% vs. 84%, 74%, 73% at 1, 2, 3 year; p=0.087) were better in AVF group than in AVG group. The factors affecting the patency of vascular access were the type of vascular access and planned construction of permanent vascular access. CONCLUSION: Patency of native AVF as the permanent vascular access was better than that of AVG. Planned construction of permanent vascular access improved the patency of the access.

The Clinical Outcome of Acute Renal Failure Requiring Hemodialysis after Coronary Intervention in Patients with Chronic Kidney Disease / 대한신장학회지

PURPOSE: Acute renal failure requiring dialysis (ARFD) after coronary intervention is a rare but serious complication, and it is associated with poor prognosis. This study investigated the clinical characteristics and risk factors of ARFD after coronary intervention in patients with chronic kidney disease (CKD). METHODS: This study included 178 adult patients with CKD (baseline serum creatinine of >1.3 mg/dL) undergoing coronary intervention at Samsung Medical Center from April 2003 through June 2007. We retrospectively evaluated the incidence, clinical outcome, and risk factor of ARFD via medical records. ARFD was defined as a decrease in renal function necessitating hemodialysis in the first 7 days after percutaneous coronary intervention. The estimated glomerular filtration rate (eGFR) was obtained using the Levey Modification of Diet in Renal Disease (MDRD) formula. RESULTS: Ten of the 178 patients (5.6%) developed ARFD with a subsequent in-hospital mortality rate of 10% (n=1). According to CKD stage, incidence of ARFD was 0/136 patients (baseline eGFR 30 to 90 ml/min/1.73m2 4/32 (15 to 29,) and 7/10 (<15) respectively (0% vs 9.4% vs 70%, p<0.0001). Multivariate analysis found eGFR (OR=0.5, 95% CI 0.3 to 0.9, p=0.02) to be only independent predictor of ARFD. Of those who developed ARFD (n=10), 2 patients stopped dialysis, 8 had required permanent dialysis. CONCLUSION: Incidence of ARFD increased according to the severity of renal dysfunction. The majority of ARFD patients had required permanent dialysis.

Comparison of Safety and Efficiency of Hemodialysis Using Heparin-bound Hemophan with those of Saline Flushing Hemodialysis and Hemodialysis Using Low Dose Heparin in Patients at Risk of Bleeding / 대한신장학회잡지

OBJECTIVE: Although hemodialysis using heparin bound Hemophan (HBH-HD) has been reported to be a possible modality in patients at risk of bleeding, the efficiency and safety of HBH-HD is not certain. Therefore, we prospectively compared the safety and efficiency of HBH-HD with those of saline flushing HD (SF-HD) and HD using low dose heparin (LDH-HD) in 13 HD patients at risk of bleeding in a cross-over design. METHODS: The safety and efficiency were evaluated by measuring activated partial prothrombin time (aPTT) before and during HD, hemostasis time after needle removal, total blood compartment volume (TBCV) loss of dialyzer, urea clearance (K) and Kt/V. RESULTS: There was no difference in compression time needed to achieve hemostasis at puncture site after needle removal between HBH-HD, SF-HD and LDH-HD. During HBH-HD, there was a slight increase in aPTT at 15 min (50.6+/-4.5 sec), compared to predialysis levels (40.9+/-4.7 sec). In this cross- over study, aPTT during dialysis session was markedly higher in LDH-HD than those in HBH-HD or SF-HD (p<0.05). The loss of TBCV of the dialyzer was greater in SF-HD than HBH-HD or LDH-HD (17.4+/-1.9% vs. 12.4+/-1.4% vs. 10.1+/-1.8%). However, there was no difference in K (212.0+/-30.7 vs. 217.2+/-36.9 vs. 221.6+/- 29.5 mL/min) and Kt/V (1.22+/-0.12 vs. 1.24+/-0.16 vs. 1.26+/-0.18). CONCLUSION: We concluded that the safety and efficiency of HBH-HD are not different compared to SF-HD or LDH-HD and HBH-HD could an alternative hemodialysis method in patients at risk of bleeding.

Estimation of GFR Using Iohexol Plasma Clearance in Korean without Renal Disease / 대한신장학회잡지

BACKGROUND: Plasma clearance of iohexol (Omnipaque(r)) which used widely in radiologic procedure is considered as useful method for estimation of GFR because iohexol is neither reabsorbed nor secreted from tubule after filtered as inulin and its extrarenal clearance is negligible. Plasma clearance of iohexol can be calculated from two compartment model or one compartment model with Brochner-Mortensen (B-M) modification which convenient and reliable. But there were controversies about sufficient sampling numbers and times for B-M modification of iohexol clearance. METHODS: Nineteen healthy Korean without renal disease underwent measurement of iohexol clearance. Iohexol was given as a single iv dose, and 14 blood sample were drawn up to 300 min. A reference GFR was iohexol clearance calculated from two-compartment model using 14 samples (CL-T). From 8, 3 and 2 samples clearances were calculated by B-M modification (CL-M8, 3 and 2 respectively). The accuracy of estimates was evaluated as percent of estimates falling within 10% above or below the reference GFR. Accuracy of CCr and equations for GFR estimation were also compared. RESULTS: CL-T, CL-M8, CL-M3 and CL-M2 were not different (101.9+/-24.0, 101.9+/-18.7, 101.7+/-18.6, 101.9+/-19.5 mL/min/1.73 m2 respectively). Accuracy of CL-M8, 3 and 2 were not different (74%, 84% and 79% respectively, p>0.05). MDRD equation had higher accuracy (47%) compared with other equations. CONCIUSION: These results indicate that sampling number for measuring iohexol plasma clearance using simplified method might be reduced to only two without accuracy loss in Korean without renal disease.

Hemodialysis Using Heparin Bound Hemophan Hemodialysis in ESRD Patients at High Risk for Bleeding: A Seven-Year Experience / 대한신장학회잡지

OBJECTIVE: Positively charged N, N-diethyl-aminoehtyl groups on Hemophan enable negative charged heparin to be bound with the dialyzer membrane and hemodialysis using heparin bound Hemophan (HBH- HD) could be a hemodialysis modality in patients at risk of bleeding. We designed simplified heparin binding technique and evaluated the bleeding risk and efficiency of HBH-HD in chronic renal failure patients at risk of bleeding. METHODS: During the period from April 1995 through April 2002, 159 patients at high bleeding risk received 1057 HBH-HD (dialyzer: GFS plus 11, Gambro). The duration of each HBH-HD was standardized to 4 hours at blood-flow rate of 200-250 mL/min. To evaluate safety of HBH-HD, we measured serum heparin concentration (HC) and activated partial thromboplastin time (aPTT) at baseline, 15, 60, 120 minutes and endpoint (240 minutes) (n= 40). To evaluate the dialysis efficiency, HBH-HD and routine hemodialysis with systemic heparinization (R-HD) were compared for total blood compartment volume (TBCV) loss, dialyzer urea clearance (K) and Kt/V in same study group patients (n=20). RESULTS: Clotting of dialyzer necessitating termination of dialysis occurred in 11 (1.0%) out of 1, 057 dialyses at 150 minutes, and clotting requiring change of blood line occurred in 64 dialyses (6.1%) between 150 and 230 minutes. There was a slight increase in the aPTT (mean+/-SD, 49.8+/-10.5 sec) and HC (0.14+/-0.06 U/mL) at 15 min, compared to predialysis levels of 44.3+/-12.9 sec and 0.11+/-0.06 U/ mL, respectively (p>0.05). But no increase in aPTT, HC was observed in measurements at 60 min, 120 min, and at the endpoint. TBCV loss was significantly higher in HBH-HD (mean+/-SD, 17.2+/-9.6%), compared to R-HD (2.8+/-1.2%) (p0.05). CONCLUSION: HBH-HD could be a safe and efficient HD technique in patients at high risk of bleeding. Extracorporeal clotting, however, should be observed carefully during HBH-HD.

A Case of Light Chain Deposition Disease without Significant Proteinuria / 대한신장학회잡지

A 67-year-old male was admitted to the hospital for evaluation of incidentally detected anemia and mild azotemia. Urinalysis showed no abnormal finding and 24 hr urine protein amount was clinically insignificant (270 mg/day). Urine and serum protein electrophoresis were negative for a monoclonal spike. However, urine and serum immunoelectrophoresis demonstrated the presence of monoclonal free kappa light chains. Renal biopsy showed the features of chronic tubulointerstitial disease and on the immunofluorescence studies, kappa light chain was in a linear pattern in basement membranes of glomeruli and tubules. Work-up for multiple myeloma including bone marrow biopsy showed results compatible with multiple myeloma. Treatment was started with vincristine, adriamycin and doxorubicin at monthly interval for three months followed by autologus peripheral blood stem cell transplantation. At follow-up 5 months after autologus peripheral blood stem cell transplantation, the patient is well with a serum creatinine of 2.3-2.6 mg/dL and 24 hr urine protein of 200-350 mg.

A Case of Postinfectious Glomerulonephritis Following Meningococcal Meningitis / 대한신장학회잡지

A 21-year-old male was presented with sudden headache, fever, petechiae and neck stiffness. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics. However the patient developed generalized edema, oliguria, azotemia, and heavy proteinuria in the recovery phase of illness. Low serum C3 level was also noted. A kidney biopsy was performed and showed the features of postinfectious glomerulonephritis and typical subepithelial humps on electron-microscopic examination. His symptoms and laboratory findings were improved, and C3 level returned to normal range after conservative treatment. We suggest that a complement deficiency should be ruled out in patients of glomerulonephritis developed during the recovery phase of meningococcal meningitis. C3 nephritic factor detection and renal biopsy should be carefully considered in these patients.

Primary Sjogren's Syndrome with Renal Pseudolymphoma Featuring Interstitial Nephritis / 대한신장학회잡지

A 65 year-old woman with Sjogren's syndrome was found to have renal mass and acute renal failure. Immunopathologic analysis of renal biopsy specimens showed polyclonal lymphocytic interstitial infiltration. Gene rearrangement study of T cell receptor showed a polyclonal pattern. The degree of azotemia and the size of pseudolymphoma diminished dramatically with steroid therapy. This is a case of proven pseudolymphoma that was found as renal mass in Sjogren's syndrome.

Effects of Low-Dose Long-Term Macrolide Therapy on the Postoperative Symptom Change, Mucociliary Clearance and Polyp Regrowth

Low-dose long-term macrolide therapy (LDLMT) has been reported as an effective treatment in chronic respiratory tract diseases such as diffuse panbronchiolitis and chronic paranasal sinusitis. The mechanism of action of LDLMT is not fully revealed. The aim of this study was to evaluate the clinical effect of LDLMT on the postoperative patients of chronic sinusitis with polyp. Thirty eight control groups were defined empirically treated with antibiotics after endoscopic sinus surgery (ESS) and 62 experimental group were defined 8 weeks LDLMT (Clarithromycin 250 mg ) after postoperative 2 weeks in ESS patients. Preoperatively, we checked mucociliary clearance with saccharine test (MCT), symptom scores and sinus CT scan and total IgE, and allerg ic skin test were evaluated for allergy. After ESS, MCT and symptom scores were checked on postoperative patients at 2, 6 and 10 weeks, respectively. Recurrence of polyp was also evaluated with endoscopic examination. The improvement rate (IR) of MCT was s ignificantly higher in the experimental group than in the control group, but the IR of symptom scores was not significant statistically. High total IgE affected the IR of MCT and symptom scores in the experimental group. Allergy proven by allergic skin test also affected the IR of MCT, but the IR of symptom scores was not affected in the experimental group. The polyp recurrence rate was significantly higher in the control group compared to the experimental group. We can conclude that LDLMT has a beneficial affect on the postoperative treatment of sinusitis patients and high total IgE and allergy may affect the effect of LDLMT in postoperative patients who have undergone ESS.

Peritoneopleural Leakage in Continuous Ambulatory Peritoneal Dialysis : Successful Treatment with Video-Assisted Thoracoscopic Talc Pleurodesis / 대한신장학회잡지

Continuous ambulatory peritoneal dialysis (CAPD) is an effective renal replacement therapy for end- stage renal disease. Hydrothorax secondary to leakage of dialysate via pleuroperitoneal communication is a rare complication of CAPD. Earlier treatments of CAPD-induced hydrothorax have included pleurodesis with tetracycline, talc, fibrin, or autologous blood and surgical treatment. These procedure have made many patients switch to hemodialysis, because of the high relapse rate of the former and the invasiveness and morbidity of the latter. The talc pleurodesis with video-assisted thoracic surgery (VATS) allows not only direct visualization of potential diaphragmatic defect but also direct application of the talc to the visceral or parietal pleura. This procedure is less invasive than thoracotomy and can perform more accurate poudrage of talc than conventional methods. We have recently managed a patient CAPD-induced massive hydrothorax using thoracoscopic talc pleurodesis. This patient was successfully returned to CAPD.

Peritoneopleural Leakage in Continuous Ambulatory Peritoneal Dialysis : Successful Treatment with Video-Assisted Thoracoscopic Talc Pleurodesis / 대한신장학회잡지

Continuous ambulatory peritoneal dialysis (CAPD) is an effective renal replacement therapy for end- stage renal disease. Hydrothorax secondary to leakage of dialysate via pleuroperitoneal communication is a rare complication of CAPD. Earlier treatments of CAPD-induced hydrothorax have included pleurodesis with tetracycline, talc, fibrin, or autologous blood and surgical treatment. These procedure have made many patients switch to hemodialysis, because of the high relapse rate of the former and the invasiveness and morbidity of the latter. The talc pleurodesis with video-assisted thoracic surgery (VATS) allows not only direct visualization of potential diaphragmatic defect but also direct application of the talc to the visceral or parietal pleura. This procedure is less invasive than thoracotomy and can perform more accurate poudrage of talc than conventional methods. We have recently managed a patient CAPD-induced massive hydrothorax using thoracoscopic talc pleurodesis. This patient was successfully returned to CAPD.

A Case of Benign Fibrous Histiocytoma of Frontoethmoid Sinus / 대한이비인후과학회지

Fibrous histiocytoma or other tumors of this group, benign or malignant, usually develop indiscriminately in any skin and soft tissues of the body. Up to the present, tumors occurring in the nasal cavity, paranasal sinuses or nasopharynx have been rarely reported. Most benign fibrous histiocytoma often develop as a painless mass lesion and mostly occur in the-sun-exposed skin and orbital tissue. However, it has also been found in the aerodigestive tract, salivary gland, and deep layers of the scalp and face. The age ranges from 1 to 70 with a male to female ratio of 2.5:1. Recently, we experienced a case of benign fibrous histiocytoma in the frontoethmoid sinus in a 63-year-old woman, whose case was initially considered as a mucocele. The authors believe this is the first reported case of benign fibrous histiocytoma originating in the frontoethmoid sinus.

A Case of Embryonal Rhabdomyosarcoma of Nasal Cavity in Adult / 대한이비인후과학회지

A rare case of the nasal cavity and paranasal sinus is encountered and we report the case with the literature review of embryonal rhabdomyosarcoma. The occurrence of embryonal rhabdomyosarcoma in nasal cavity and paranasal sinus is rather uncommon. Most embryonal rhabdomyosarcoma occurs in children younger than 12 years of age, and in adult, the incidence is only 10%, Recently, we experienced a case of adult embryonal rhabdomyosarcoma originating in the nasal cavity. The clinical course and the possible management of this unusual neoplasm are discussed.