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Volume 66, No. 4/2006(October)
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Originals
Acute renal cortical necrosis in a series of young men with severe acute pancreatitis
A.V.M. Alfonzo, J.G. Fox, C.W. Imrie, G. Roditi and B. Young
A.V.M. Alfonzo, J.G. Fox, C.W. Imrie, G. Roditi and B. Young
223
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Abstract
Diffuse renal cortical necrosis from any cause is rare in the Western World. Over the last 5 decades, there have been isolated case reports of acute cortical necrosis as a consequence of acute pancreatitis, but the long-term outcome of these patients has not previously been reported. Here, we report 3 young men, aged 16 – 21 years, who have presented over the past 19 years with severe acute pancreatitis associated with oliguric acute renal failure. They were all found to have diffuse renal cortical necrosis and consequently made no renal recovery. Despite the appreciable mortality associated with acute pancreatitis complicated by acute renal failure, they all survived the initial illness and all have successfully undergone renal transplantation. We present a detailed account of each patient including diagnostic strategies and discuss the possible factors contributing to a favorable outcome in these patients.Correspondence to:
Dr. A. Alfonzo
Renal Unit, Glasgow Royal Infirmary
Castle Street
Glasgow G4 0SF, Great Britain
Email: annette.alfonzo@northglasgow.scot.nhs.uk
A.V.M. Alfonzo, J.G. Fox, C.W. Imrie, G. Roditi and B. Young
1Renal Unit, 2Lister Department of Surgery, 3Imaging Department, Glasgow Royal Infirmary, Glasgow, and 4Department of Pathology, Western Infirmary, Glasgow, Great Britain Diffuse renal cortical necrosis from any cause is rare in the Western World. Over the last 5 decades, there have been isolated case reports of acute cortical necrosis as a consequence of acute pancreatitis, but the long-term outcome of these patients has not previously been reported. Here, we report 3 young men, aged 16 – 21 years, who have presented over the past 19 years with severe acute pancreatitis associated with oliguric acute renal failure. They were all found to have diffuse renal cortical necrosis and consequently made no renal recovery. Despite the appreciable mortality associated with acute pancreatitis complicated by acute renal failure, they all survived the initial illness and all have successfully undergone renal transplantation. We present a detailed account of each patient including diagnostic strategies and discuss the possible factors contributing to a favorable outcome in these patients.Correspondence to:
Dr. A. Alfonzo
Renal Unit, Glasgow Royal Infirmary
Castle Street
Glasgow G4 0SF, Great Britain
Email: annette.alfonzo@northglasgow.scot.nhs.uk
Originals
Low-dose prednisolone ameliorates acute renal failure caused by cholesterol crystal embolism
M. Nakayama, M. Nagata, T. Hirano, K. Sugai, R. Katafuchi, S. Imayama, N. Uesugi, T. Tsuchihashi and H. Kumagai
232
36$
Abstract
Aims: The prognosis of renal cholesterol crystal embolism (CCE) is poor. Although various treatments for CCE have been attempted, there is no optimal therapy. We tested the effect of low-dose prednisolone (PS) on CCE-related acute renal failure (ARF). Patients and methods: 7 patients (mean age 69 years) diagnosed with CCE-related ARF were treated with oral PS at 15 – 20 mg/day for 2 – 4 weeks, which was then tapered at 5 mg/day over 2 – 4 weeks, followed by 5 mg/day maintenance dose. Recurrent ARF during PS tapering was treated with a larger dose of PS. Results: Inciting factors were identified in four patients: coronary angiography (n = 3) and cerebral angiography (n = 1). On admission, serum creatinine (SCr) was 2.1 ± 0.3 mg/dl (mean ± SEM). SCr and eosinophil count before treatment were 4.2 ± 0.4 mg/dl and 682 ± 73/ml, respectively. PS therapy improved ARF in all cases at week 2 (SCr 3.8 ± 0.5 mg/dl) parallel to a decrease in eosinophilia (116 ± 30/ml), and at week 4 (3.1 ± 0.4 mg/dl and 134 ± 20/ml, respectively). At last follow-up, renal function was improved or maintained in 5 patients compared with that at week 4 post-treatment. One patient died of lung cancer. Another required LDL apheresis and hemodialysis but died due to CCE-related multi-organ failure. A third patient had recurrent ARF and was re-treated with a larger dose of PS, which resulted in an immediate decrease in SCr. However, the patient developed acute renal dysfunction due to congestive heart failure, and required hemodialysis. Conclusions: Low-dose PS improved CCE-related ARF, probably through amelioration of inflammatory reaction surrounding affected renal vessels.Correspondence to:
M. Nakayama, MD
Division of Nephrology and Clinical Research Institute
Department of Internal Medicine
National Kyushu Medical Center Hospital
1-8-1 Jigyohama, Chuo-ku, Fukuoka 810-8563, Japan
Email: mnakayama@qmed.hosp.go.jp
M. Nakayama, M. Nagata, T. Hirano, K. Sugai, R. Katafuchi, S. Imayama, N. Uesugi, T. Tsuchihashi and H. Kumagai
Divisions of 1Nephrology, 3Dermatology, 4Pathology and 5Hypertension and Clinical Research Institute, Department of Internal Medicine, National Kyushu Medical Center Hospital, Fukuoka and 2First Department of Internal Medicine, Fukuoka Red Cross Hospital, Fukuoka, Japan Aims: The prognosis of renal cholesterol crystal embolism (CCE) is poor. Although various treatments for CCE have been attempted, there is no optimal therapy. We tested the effect of low-dose prednisolone (PS) on CCE-related acute renal failure (ARF). Patients and methods: 7 patients (mean age 69 years) diagnosed with CCE-related ARF were treated with oral PS at 15 – 20 mg/day for 2 – 4 weeks, which was then tapered at 5 mg/day over 2 – 4 weeks, followed by 5 mg/day maintenance dose. Recurrent ARF during PS tapering was treated with a larger dose of PS. Results: Inciting factors were identified in four patients: coronary angiography (n = 3) and cerebral angiography (n = 1). On admission, serum creatinine (SCr) was 2.1 ± 0.3 mg/dl (mean ± SEM). SCr and eosinophil count before treatment were 4.2 ± 0.4 mg/dl and 682 ± 73/ml, respectively. PS therapy improved ARF in all cases at week 2 (SCr 3.8 ± 0.5 mg/dl) parallel to a decrease in eosinophilia (116 ± 30/ml), and at week 4 (3.1 ± 0.4 mg/dl and 134 ± 20/ml, respectively). At last follow-up, renal function was improved or maintained in 5 patients compared with that at week 4 post-treatment. One patient died of lung cancer. Another required LDL apheresis and hemodialysis but died due to CCE-related multi-organ failure. A third patient had recurrent ARF and was re-treated with a larger dose of PS, which resulted in an immediate decrease in SCr. However, the patient developed acute renal dysfunction due to congestive heart failure, and required hemodialysis. Conclusions: Low-dose PS improved CCE-related ARF, probably through amelioration of inflammatory reaction surrounding affected renal vessels.Correspondence to:
M. Nakayama, MD
Division of Nephrology and Clinical Research Institute
Department of Internal Medicine
National Kyushu Medical Center Hospital
1-8-1 Jigyohama, Chuo-ku, Fukuoka 810-8563, Japan
Email: mnakayama@qmed.hosp.go.jp
Originals
Decreased B cells and IgA-secreting B cells partially explain the high prevalence of IgA deficiency in dialysis patients
M.-C. Kuo, J.-M. Chang, J.-C. Tsai, H.-C. Chen, W.-C. Tsai, Y.-H. Lai and S.-J. Hwang
M.-C. Kuo, J.-M. Chang, J.-C. Tsai, H.-C. Chen, W.-C. Tsai, Y.-H. Lai and S.-J. Hwang
240
32$
Abstract
Aims: We previously reported 2 hemodialysis (HD) patients with recurrent infections and selective immunoglobulin A deficiency (IgAD). We further demonstrated that serum IgA levels were lower and the prevalence of IgAD was higher in uremic patients. The exact mechanisms of IgAD in uremic patients largely remained unclear. In some patients, it was caused by anti-IgA antibody neutralization and subsequent destruction. We performed the present study to survey if there is any defect in IgA production. Materials and methods: 288 patients were initially included for examination of serum immunoglobulins. 16 normal persons, 16 dialysis patients without IgAD, and 12 dialysis patients with IgAD were enrolled after the initial examination. Blood was drawn into heparinized tubes. WBC counts and lymphocyte percentage were examined by a CBC counter. Lymphocytes were separated by the Ficoll-Paque method. Flow cytometry was utilized to isolate the B cell and IgA-secreting B cell after staining with CD19 phycoerythrin and FITC-conjugated rabbit anti-human IgA antibody. Results: There is no significant difference between WBC counts or total lymphocyte counts of these 3 groups. However, we found a lower percentage of total lymphocyte counts in dialysis patients, either with or without IgAD. The total B cell numbers were lower in dialysis patients with IgAD. In addition, there were fewer IgA-secreting B cells in dialysis patients with IgAD. Conclusion: Decreased B cell and IgA-secreting B cell counts are seen in uremic patients with IgAD. This, in turn, indicates that there might be a defect of IgA production in some patients, rather than IgA destruction by anti-IgA antibodies as seen in some other patients. Further study is needed to investigate the mechanisms of decreased B cells and IgA-secreting B cells.Correspondence to:
S.-J. Hwang, MD Division of Nephrology Department of Internal Medicine Kaohsiung Medical University Hospital Kaohsiung Medical University 100 Shih-Chuan 1st Road, Kaohsiung 807, Taiwan
Email: sjhwang@kmu.edu.tw
M.-C. Kuo, J.-M. Chang, J.-C. Tsai, H.-C. Chen, W.-C. Tsai, Y.-H. Lai and S.-J. Hwang
1Division of Nephrology and 2Division of Rheumatology, Department of Internal Medicine, Department of Medicine, Kaohsiung Medical University Hospital, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Taiwan Aims: We previously reported 2 hemodialysis (HD) patients with recurrent infections and selective immunoglobulin A deficiency (IgAD). We further demonstrated that serum IgA levels were lower and the prevalence of IgAD was higher in uremic patients. The exact mechanisms of IgAD in uremic patients largely remained unclear. In some patients, it was caused by anti-IgA antibody neutralization and subsequent destruction. We performed the present study to survey if there is any defect in IgA production. Materials and methods: 288 patients were initially included for examination of serum immunoglobulins. 16 normal persons, 16 dialysis patients without IgAD, and 12 dialysis patients with IgAD were enrolled after the initial examination. Blood was drawn into heparinized tubes. WBC counts and lymphocyte percentage were examined by a CBC counter. Lymphocytes were separated by the Ficoll-Paque method. Flow cytometry was utilized to isolate the B cell and IgA-secreting B cell after staining with CD19 phycoerythrin and FITC-conjugated rabbit anti-human IgA antibody. Results: There is no significant difference between WBC counts or total lymphocyte counts of these 3 groups. However, we found a lower percentage of total lymphocyte counts in dialysis patients, either with or without IgAD. The total B cell numbers were lower in dialysis patients with IgAD. In addition, there were fewer IgA-secreting B cells in dialysis patients with IgAD. Conclusion: Decreased B cell and IgA-secreting B cell counts are seen in uremic patients with IgAD. This, in turn, indicates that there might be a defect of IgA production in some patients, rather than IgA destruction by anti-IgA antibodies as seen in some other patients. Further study is needed to investigate the mechanisms of decreased B cells and IgA-secreting B cells.Correspondence to:
S.-J. Hwang, MD Division of Nephrology Department of Internal Medicine Kaohsiung Medical University Hospital Kaohsiung Medical University 100 Shih-Chuan 1st Road, Kaohsiung 807, Taiwan
Email: sjhwang@kmu.edu.tw
Originals
Severe vitamin D deficiency in chronic renal failure patients on peritoneal dialysis
H. Taskapan, F.F. Ersoy, P.S. Passadakis, P. Tam, D.E. Memmos, K.P. Katopodis, C. Ozener, F. Akcicek, T. Camsari, K. Ates, R. Ataman, J.G. Vlachojannis, N.A. Dombros, C. Utas, T. Akpolat, S. Bozfakioglu, G. Wu, I. Karayaylali, T. Arinsoy, C.P. Stathakis
247
40$
Abstract
The aim of this study was to evaluate the prevalence of vitamin D deficiency in chronic renal failure (CRF) patients on peritoneal dialysis (PD) and to correlate the findings with various demographic and renal osteodystrophy markers. Method: This cross-sectional, multicenter study was carried out in 273 PD patients with a mean age of 61.7 ± 10.9 years and mean duration of PD 3.3 ± 2.2 years. It included 123 female and 150 male patients from 20 centers in Greece and Turkey, countries that are on the same latitude, namely, 36 – 42° north. We measured 25(OH)D3 and 1.25(OH)2D3 levels and some other clinical and laboratory indices of bone mineral metabolism. Results: Of these 273 patients 92% (251 patients) had vitamin D deficiency i.e. serum 25(OH)D3 levels less than 15 ng/ml, 119 (43.6%) had severe vitamin D deficiency i.e. serum 25(OH)D3 levels, less than 5 ng/ml, 132 (48.4%) had moderate vitamin D deficiency i.e. serum 25(OH)D3 levels, 5 – 15 ng/ml, 12 (4.4%) vitamin D insufficiency i.e. serum 25(OH)D3 levels 15 – 30 ng/ml and only 10 (3.6%) had adequate vitamin D stores. We found no correlation between 25(OH)D3 levels and PTH, serum albumin, bone alkaline phosphatase, P, and Ca × P. In multiple regression analyses, the independent predictors of 25(OH)D3 were age, presence of diabetes (DM-CRF), levels of serum calcium and serum 1.25(OH)2D3. Conclusion: We found a high prevalence (92%) of vitamin D deficiency in these 273 PD patients, nearly one half of whom had severe vitamin D deficiency. Vitamin D deficiency is more common in DM-CRF patients than in non-DM-CRF patients. Our findings suggest that these patients should be considered for vitamin D supplementation.Correspondence to:
H. Taskapan, MD
Associate Professor of Medicine and Nephrology
Inonu University Medical School
Department of Medicine
Division of Nephrology Malatya, Turkey
Email: hulyataskapan@yahoo.com
H. Taskapan, F.F. Ersoy, P.S. Passadakis, P. Tam, D.E. Memmos, K.P. Katopodis, C. Ozener, F. Akcicek, T. Camsari, K. Ates, R. Ataman, J.G. Vlachojannis, N.A. Dombros, C. Utas, T. Akpolat, S. Bozfakioglu, G. Wu, I. Karayaylali, T. Arinsoy, C.P. Stathakis,
1Inonu University Malatya, Turkey, 2Akdeniz University Antalya, Turkey, 3Alexandroupolis Hospital, Democritous University of Thrace, Greece, 4Scarborough Hospital Toronto, Canada, 5Hippokration Hospital, Aristotle University of Thessaloniki, Greece, 6University Hospital of Ioannina, Greece, 7Marmara University Istanbul, Turkey, 8Ege University Izmir, Turkey, 9Dokuz Eylül University Izmir, Turkey, 10Ankara University Ankara, Turkey, 11Cerrahpasa Medical School, Istanbul University Istanbul, Turkey, 12University Hospital of Patra, Greece, 13Ahepa Hospital, Aristotle University of Thessaloniki, Greece, 14Erciyes University Kayseri, Turkey, 15Ondokuz Mayis University Samsun, Turkey, 16Capa Medical School, Istanbul University Istanbul, Turkey, 17Credit Valley Hospital Toronto, Canada, 18Cukurova University Adana, Turkey, 19Gazi University Ankara, Turkey, 20Laiko General Hospital of Athens, Greece, 21Uludag University Bursa, Turkey, 22General Hospital of Veria, Greece, 23Agios Dimitrios Hospital of Thessaloniki, Greece, 24Dicle University Diyarbakir, Turkey, 25Toronto-Western Hospital Toronto, Canada The aim of this study was to evaluate the prevalence of vitamin D deficiency in chronic renal failure (CRF) patients on peritoneal dialysis (PD) and to correlate the findings with various demographic and renal osteodystrophy markers. Method: This cross-sectional, multicenter study was carried out in 273 PD patients with a mean age of 61.7 ± 10.9 years and mean duration of PD 3.3 ± 2.2 years. It included 123 female and 150 male patients from 20 centers in Greece and Turkey, countries that are on the same latitude, namely, 36 – 42° north. We measured 25(OH)D3 and 1.25(OH)2D3 levels and some other clinical and laboratory indices of bone mineral metabolism. Results: Of these 273 patients 92% (251 patients) had vitamin D deficiency i.e. serum 25(OH)D3 levels less than 15 ng/ml, 119 (43.6%) had severe vitamin D deficiency i.e. serum 25(OH)D3 levels, less than 5 ng/ml, 132 (48.4%) had moderate vitamin D deficiency i.e. serum 25(OH)D3 levels, 5 – 15 ng/ml, 12 (4.4%) vitamin D insufficiency i.e. serum 25(OH)D3 levels 15 – 30 ng/ml and only 10 (3.6%) had adequate vitamin D stores. We found no correlation between 25(OH)D3 levels and PTH, serum albumin, bone alkaline phosphatase, P, and Ca × P. In multiple regression analyses, the independent predictors of 25(OH)D3 were age, presence of diabetes (DM-CRF), levels of serum calcium and serum 1.25(OH)2D3. Conclusion: We found a high prevalence (92%) of vitamin D deficiency in these 273 PD patients, nearly one half of whom had severe vitamin D deficiency. Vitamin D deficiency is more common in DM-CRF patients than in non-DM-CRF patients. Our findings suggest that these patients should be considered for vitamin D supplementation.Correspondence to:
H. Taskapan, MD
Associate Professor of Medicine and Nephrology
Inonu University Medical School
Department of Medicine
Division of Nephrology Malatya, Turkey
Email: hulyataskapan@yahoo.com
Originals
Relation between number of prescribed medication and outcome in peritoneal dialysis patients
C.-C. Szeto, K.-M. Chow, B.C.-H. Kwan, C.-B. Leung, K.-Y. Chung, M.C. Law and P.K.-T. Li
256
32$
Abstract
Background: Many patients with end-stage renal disease need to take a large number of medications. In the present study, we studied the magnitude of problem and explored the relationship between the number of prescribed medications and the clinical outcome of a large cohort of prevalent peritoneal dialysis (PD) patients. Methods: We studied the medication list of 266 prevalent PD patients. Dialysis adequacy, residual renal function and nutritional assessment were also performed. The patients were followed for 33.7 ± 20.7 months. Results: On average, each patient required 4.7 ± 1.8 type of medications or 10.0 ± 4.9 tablets per day. 40 patients (15.0%) needed at least 7 types of medication; 33 patients (12.4%) had to take more than 15 tablets each day. There is a significant but weak correlation between the number of types of medication and the Charlson’s comorbidity score (r = 0.252, p < 0.001). Despite the large number of medication prescribed, the blood pressure control, serum cholesterol level, and the use of aspirin after atherosclerotic disease remained suboptimal in many patients. By multivariate analysis, independent factors for patient survival were Charlson’s comorbidity score, number of types of medication, duration of dialysis, overall SGA score, and mean arterial blood pressure. Each additional type of medication conferred 20% increase in risk of death (95% CI, 1.6 – 41.7%, p = 0.032), and the effect is independent on the Charlson’s comorbidity score. The actual number of pills taken by a patient did not influence survival in this model. Conclusion: Our results indicate that the number of prescribed medications is related to the clinical outcome of PD patients. The number of prescribed medication may reflect the severity of uremic complications and comorbid diseases not reflected by the Charlson’s comorbidity score. Nevertheless, dialysis physicians should carefully balance the clinical need of treating multiple medical conditions with the potential problems of a complicated therapeutic regimen.Correspondence to:
Dr. C.C. Szeto
Department of Medicine and Therapeutics
Prince of Wales Hospital
The Chinese University of Hong Kong
Shatin, Hong Kong, China
Email: ccszeto@cuhk.edu.hk
C.-C. Szeto, K.-M. Chow, B.C.-H. Kwan, C.-B. Leung, K.-Y. Chung, M.C. Law and P.K.-T. Li
Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong, China Background: Many patients with end-stage renal disease need to take a large number of medications. In the present study, we studied the magnitude of problem and explored the relationship between the number of prescribed medications and the clinical outcome of a large cohort of prevalent peritoneal dialysis (PD) patients. Methods: We studied the medication list of 266 prevalent PD patients. Dialysis adequacy, residual renal function and nutritional assessment were also performed. The patients were followed for 33.7 ± 20.7 months. Results: On average, each patient required 4.7 ± 1.8 type of medications or 10.0 ± 4.9 tablets per day. 40 patients (15.0%) needed at least 7 types of medication; 33 patients (12.4%) had to take more than 15 tablets each day. There is a significant but weak correlation between the number of types of medication and the Charlson’s comorbidity score (r = 0.252, p < 0.001). Despite the large number of medication prescribed, the blood pressure control, serum cholesterol level, and the use of aspirin after atherosclerotic disease remained suboptimal in many patients. By multivariate analysis, independent factors for patient survival were Charlson’s comorbidity score, number of types of medication, duration of dialysis, overall SGA score, and mean arterial blood pressure. Each additional type of medication conferred 20% increase in risk of death (95% CI, 1.6 – 41.7%, p = 0.032), and the effect is independent on the Charlson’s comorbidity score. The actual number of pills taken by a patient did not influence survival in this model. Conclusion: Our results indicate that the number of prescribed medications is related to the clinical outcome of PD patients. The number of prescribed medication may reflect the severity of uremic complications and comorbid diseases not reflected by the Charlson’s comorbidity score. Nevertheless, dialysis physicians should carefully balance the clinical need of treating multiple medical conditions with the potential problems of a complicated therapeutic regimen.Correspondence to:
Dr. C.C. Szeto
Department of Medicine and Therapeutics
Prince of Wales Hospital
The Chinese University of Hong Kong
Shatin, Hong Kong, China
Email: ccszeto@cuhk.edu.hk
Originals
The effect of icodextrin and glucose-containing solutions on insulin resistance in CAPD patients
E.M. Gürsu, A. Özdemir, B. Yalinbas, R.U. Gürsu, M. Canbakan, B. Güven, E.M. Atasoyu, A.T. Keskin, A. Elçi and Y. Baru
E.M. Gürsu, A. Özdemir, B. Yalinbas, R.U. Gürsu, M. Canbakan, B. Güven, E.M. Atasoyu, A.T. Keskin, A. Elçi and Y. Baru
263
28$
Abstract
Purpose: Peritoneal dialysis patients have particular risks with respect to their lipid status and hyperinsulinemia. The aim of this study was to investigate the relation between insulin resistance and the type of the peritoneal dialysis solution. Materials: 41 randomly selected non-diabetic patient cohort who were already under treatment with continuous ambulatory peritoneal dialysis (CAPD) and 10 healthy controls participated in the study. 24 of the 41 patients were using 3 standard 1.36% glucose solutions during the day and 1 hypertonic solution with 2.27% glucose dwell during the night (glucose group: mean age 45.54 ± 16.67 years and median CAPD duration 16.5 months). The remaining 17 patients were using 3 standard 1.36% glucose solutions during the day and 1 icodextrin dwell during the night for 8 – 10 hours (icodextrin group: mean age 47.47 ± 13.15 years, median duration of icodextrin use 6 months (range 2 – 20 months), and median CAPD duration 30 months). Insulin resistance (IR) was calculated according to the homeostasis model assesment (HOMA) formula: HOMA-IR = fasting glucose (mmol/l) × fasting insulin (mU/l/22.5. The HOMA cutoff point for diagnosis of insulin resistance was established with receiver-operating characteristic (ROC) curves. The patients were called HOMA-IR(+) if their HOMA scores were higher than cutoff value. Results: There were no significant differences between age, BMI, triglyceride, total and high-density lipoprotein (HDL) cholesterol, iron and ferritin, alanine aminotransferase, fibrinogen, intact parathyroid hormone, magnesium, hemoglobin and hematocrit levels of the 2 groups. The mean glucose levels of the groups were not different but fasting insulin levels and HOMA scores of the icodextrin group were significantly lower than the glucose group (10.15 ± 6.87 vs. 18.11 ± 13.15, p = 0.028, and 2.28 ± 1.67 vs. 4.26 ± 3.27, p = 0.027, respectively). The ratio of patients with low HOMA scores (cutoff = 2.511) were significantly higher in the icodextrin group than in the glucose group (71% vs 38%, p = 0.037). Other than fasting insulin and glucose levels, significantly positive correlation was found between HOMA score and BMI in both groups. With regression analysis, we found that the main parameters effecting HOMA score were BMI (p = 0.008) and triglyceride (p = 0.029) in the glucose group, but no parameters were found to affect HOMA score in icodextrin group. Conclusion: These results suggest that insulin resistance is reduced in peritoneal dialysis patients using icodextrin-based dialysis fluid instead of glucose-based dialysis fluid.Correspondence to:
A. Özdemir, MD
Halk Cad. Arif Hikmetpasa Ap. No. 76 D: 3, Üsküdar
Istanbul, Turkey
Email: alemoz2004@yahoo.com
E.M. Gürsu1, A. Özdemir1, B. Yalinbas1, R.U. Gürsu1, M. Canbakan1, B. Güven1, E.M. Atasoyu2, A.T. Keskin1, A. Elçi3 and Y. Baru1
1Department of Internal Medicine, Haydarpasa Numune Education and Research Hospital, 2Department of Nephrology, Gülhane Military Academy of Medicine Haydarpasa Training Hospital and 3Biochemical Laboratories, Haydarpasa Numune Education and Research Hospital, Istanbul, Turkey Purpose: Peritoneal dialysis patients have particular risks with respect to their lipid status and hyperinsulinemia. The aim of this study was to investigate the relation between insulin resistance and the type of the peritoneal dialysis solution. Materials: 41 randomly selected non-diabetic patient cohort who were already under treatment with continuous ambulatory peritoneal dialysis (CAPD) and 10 healthy controls participated in the study. 24 of the 41 patients were using 3 standard 1.36% glucose solutions during the day and 1 hypertonic solution with 2.27% glucose dwell during the night (glucose group: mean age 45.54 ± 16.67 years and median CAPD duration 16.5 months). The remaining 17 patients were using 3 standard 1.36% glucose solutions during the day and 1 icodextrin dwell during the night for 8 – 10 hours (icodextrin group: mean age 47.47 ± 13.15 years, median duration of icodextrin use 6 months (range 2 – 20 months), and median CAPD duration 30 months). Insulin resistance (IR) was calculated according to the homeostasis model assesment (HOMA) formula: HOMA-IR = fasting glucose (mmol/l) × fasting insulin (mU/l/22.5. The HOMA cutoff point for diagnosis of insulin resistance was established with receiver-operating characteristic (ROC) curves. The patients were called HOMA-IR(+) if their HOMA scores were higher than cutoff value. Results: There were no significant differences between age, BMI, triglyceride, total and high-density lipoprotein (HDL) cholesterol, iron and ferritin, alanine aminotransferase, fibrinogen, intact parathyroid hormone, magnesium, hemoglobin and hematocrit levels of the 2 groups. The mean glucose levels of the groups were not different but fasting insulin levels and HOMA scores of the icodextrin group were significantly lower than the glucose group (10.15 ± 6.87 vs. 18.11 ± 13.15, p = 0.028, and 2.28 ± 1.67 vs. 4.26 ± 3.27, p = 0.027, respectively). The ratio of patients with low HOMA scores (cutoff = 2.511) were significantly higher in the icodextrin group than in the glucose group (71% vs 38%, p = 0.037). Other than fasting insulin and glucose levels, significantly positive correlation was found between HOMA score and BMI in both groups. With regression analysis, we found that the main parameters effecting HOMA score were BMI (p = 0.008) and triglyceride (p = 0.029) in the glucose group, but no parameters were found to affect HOMA score in icodextrin group. Conclusion: These results suggest that insulin resistance is reduced in peritoneal dialysis patients using icodextrin-based dialysis fluid instead of glucose-based dialysis fluid.Correspondence to:
A. Özdemir, MD
Halk Cad. Arif Hikmetpasa Ap. No. 76 D: 3, Üsküdar
Istanbul, Turkey
Email: alemoz2004@yahoo.com
Originals
Respiratory evaluation of patients on continuous ambulatory peritoneal dialysis prior to renal transplantation
G. Ulubay, S. Sezer, S. Ulasli, N. Ozdemir, O.F. Eyuboglu and M. Haberal
G. Ulubay, S. Sezer, S. Ulasli, N. Ozdemir, O.F. Eyuboglu and M. Haberal
269
28$
Abstract
Background and aim: Pulmonary function tests (PFTs) and cardiopulmonary exercise tests (CPETs) are important in predicting preoperative pulmonary complications and mortality rate in potentially renal transplant recipients. There is no adequate clinical research aimed at learning the effect of empty and full status of the peritoneal cavity on PFTs and CPET for estimating decide PFTs and CPET timing in preoperative evaluation. The aim of this study was to investigate whether PFT and CPET results are altered in patients on continuous ambulatory peritoneal dialysis (CAPD) according to the presence of dialysis solution in the abdomen. Subjects and methods: 22 subjects were included (12 male, 10 female, mean age 29.64 ± 8.29 years, CAPD duration, 37.35 ± 7.15 months). Data were collected from each patient when the peritoneal cavity was filled with solution (full status) and again when the cavity had been drained (empty status). Forced expiratory volume in 1 s (FEV1), ratio of forced expiratory volume in 1 s to forced vital capacity (FEV1/FVC), total lung capacity (TLC), and residual volume (RV) were calculated. Peak oxygen uptake (peak VO2) and exercise duration were determined by cardiopulmonary exercise testing. Results: When the peritoneal cavity was empty, mean (± SD) values for the parameters tested were % predicted FEV1: 85 ± 17%, %FEV1/FVC: 84 ± 8%, % predicted TLC: 98 ± 17%, % predicted RV: 108 ± 25%, % predicted DLCO: 90 ± 14%, peak VO2: 43 ± 11 ml/kg/min, test duration: 6.8 ± 1.6 min. When the peritoneal cavity was full, mean (± SD) values were % predicted FEV1: 86 ± 17%, %FEV1/FVC: 83 ± 7%, % predicted TLC: 91 ± 14%, % predicted RV: 95 ± 22%, % predicted DLCO: 87 ± 16%, peak VO2: 42 ± 8 ml/kg/min, test duration 6.5 ± 1.7 min. % predicted FEV1, %FEV1/FVC, % predicted DLCO and peak VO2 were not statistically significant between the mean values at empty status versus those at full status (p < 0.05 for all). There were significant decreases between the mean values for % predicted TLC and % predicted RV at full status versus empty status (p < 0.002 for TLC, p < 0.001 for RV). No statistically significant correlation was found between PFTs and % change ratio of dialysate. Conclusion: FEV1, %FEV1/FVC, % predicted DLCO and CPET test results do not differ according to abdomen status in CAPD patients suggesting that the timing of PFT maneuver does not affect preoperative transplantation evaluation. Therefore, when evaluating the results of these tests prior to transplantation period, the presence of dialysis solution in the abdomen may be ignored.Correspondence to:
G. Ulubay, MD
Baskent University Faculty of Medicine
Pulmonary Disease
Fevzi Cakmak Cad. No: 48 06490, Besevler, Ankara, Turkey
Email: gulubay66@yahoo.com
G. Ulubay, S. Sezer, S. Ulasli, N. Ozdemir, O.F. Eyuboglu and M. Haberal
1Department of Pulmonary Diseases, 2Department of Nephrology and 3Department of Transplantation, Baskent University Faculty of Medicine, Ankara, Turkey Background and aim: Pulmonary function tests (PFTs) and cardiopulmonary exercise tests (CPETs) are important in predicting preoperative pulmonary complications and mortality rate in potentially renal transplant recipients. There is no adequate clinical research aimed at learning the effect of empty and full status of the peritoneal cavity on PFTs and CPET for estimating decide PFTs and CPET timing in preoperative evaluation. The aim of this study was to investigate whether PFT and CPET results are altered in patients on continuous ambulatory peritoneal dialysis (CAPD) according to the presence of dialysis solution in the abdomen. Subjects and methods: 22 subjects were included (12 male, 10 female, mean age 29.64 ± 8.29 years, CAPD duration, 37.35 ± 7.15 months). Data were collected from each patient when the peritoneal cavity was filled with solution (full status) and again when the cavity had been drained (empty status). Forced expiratory volume in 1 s (FEV1), ratio of forced expiratory volume in 1 s to forced vital capacity (FEV1/FVC), total lung capacity (TLC), and residual volume (RV) were calculated. Peak oxygen uptake (peak VO2) and exercise duration were determined by cardiopulmonary exercise testing. Results: When the peritoneal cavity was empty, mean (± SD) values for the parameters tested were % predicted FEV1: 85 ± 17%, %FEV1/FVC: 84 ± 8%, % predicted TLC: 98 ± 17%, % predicted RV: 108 ± 25%, % predicted DLCO: 90 ± 14%, peak VO2: 43 ± 11 ml/kg/min, test duration: 6.8 ± 1.6 min. When the peritoneal cavity was full, mean (± SD) values were % predicted FEV1: 86 ± 17%, %FEV1/FVC: 83 ± 7%, % predicted TLC: 91 ± 14%, % predicted RV: 95 ± 22%, % predicted DLCO: 87 ± 16%, peak VO2: 42 ± 8 ml/kg/min, test duration 6.5 ± 1.7 min. % predicted FEV1, %FEV1/FVC, % predicted DLCO and peak VO2 were not statistically significant between the mean values at empty status versus those at full status (p < 0.05 for all). There were significant decreases between the mean values for % predicted TLC and % predicted RV at full status versus empty status (p < 0.002 for TLC, p < 0.001 for RV). No statistically significant correlation was found between PFTs and % change ratio of dialysate. Conclusion: FEV1, %FEV1/FVC, % predicted DLCO and CPET test results do not differ according to abdomen status in CAPD patients suggesting that the timing of PFT maneuver does not affect preoperative transplantation evaluation. Therefore, when evaluating the results of these tests prior to transplantation period, the presence of dialysis solution in the abdomen may be ignored.Correspondence to:
G. Ulubay, MD
Baskent University Faculty of Medicine
Pulmonary Disease
Fevzi Cakmak Cad. No: 48 06490, Besevler, Ankara, Turkey
Email: gulubay66@yahoo.com
Review
Immunomodulation by 1,25-dihydroxyvitamin D3: therapeutic implications in hemodialysis and renal transplantation
C. Mathieu and M. Jafari
C. Mathieu and M. Jafari
275
40$
Abstract
The active metabolite of vitamin D3, 1,25-dihydroxyvitamin D3, is a secosteroid hormone that regulates calcium and bone metabolism, controls cell proliferation and differentiation, and plays an important role as an immunomodulator. Recent advances in understanding the mechanisms underlying 1,25(OH)2D3 immune actions expand the range of the therapeutic implications of 1,25(OH)2D3 and its analogs. This review will cover the current knowledge on vitamin D-mediated immunotolerance and recent advances in vitamin D-based therapies for the treatment of autoimmune disease and the prevention of graft rejection in renal transplantation. Initiation of vitamin D-based therapies at earlier stages of chronic kidney disease may impact the immune status of patients who progress to require dialysis or transplantation.Correspondence to:
M. Jafari Pharm. D.Assistant Professor, Associate Director Pharmaceutical Sciences Program College of Health Sciences, University of California 252 Irvine Hall, Irvine, CA, 92697, USA
Email: mjafari@uci.edu
C. Mathieu and M. Jafari
1Department of Endocrinology, UZ Gasthuisberg, Leuven, Belgium, 2Program in Pharmaceutical Sciences, College of Health Sciences, University of California, Irvine, CA, USA The active metabolite of vitamin D3, 1,25-dihydroxyvitamin D3, is a secosteroid hormone that regulates calcium and bone metabolism, controls cell proliferation and differentiation, and plays an important role as an immunomodulator. Recent advances in understanding the mechanisms underlying 1,25(OH)2D3 immune actions expand the range of the therapeutic implications of 1,25(OH)2D3 and its analogs. This review will cover the current knowledge on vitamin D-mediated immunotolerance and recent advances in vitamin D-based therapies for the treatment of autoimmune disease and the prevention of graft rejection in renal transplantation. Initiation of vitamin D-based therapies at earlier stages of chronic kidney disease may impact the immune status of patients who progress to require dialysis or transplantation.Correspondence to:
M. Jafari Pharm. D.Assistant Professor, Associate Director Pharmaceutical Sciences Program College of Health Sciences, University of California 252 Irvine Hall, Irvine, CA, 92697, USA
Email: mjafari@uci.edu
Case Reports
Multi-organ affecting CMV-associated cryoglobulinemic vasculitis
J. Kramer, H. Hennig, C. Lensing, S. Krüger, U. Helmchen, J. Steinhoff and C. Dodt
284
32$
Abstract
We report on a 67-year-old female patient who was admitted to our intensive care unit with acute renal failure and severe hypoxemia. Transiently, the patient had to be treated with kidney replacement therapies and artificial ventilation. The actual illness started with general weakness, recurrent bloody diarrhea and intermittent dermatitis of the lower legs. Skin symptoms were initially observed 2 years before the actual clinical findings. The bloody diarrhea was attributed to an inflammatory stenosis of the sigma. The life-threatening clinical aggravation was due to diffuse alveolar hemorrhage and alveolitis. In the search for the cause of the systemic disease, both a monoclonal g-globulinemia, causing a cryoglobulinemia type II and an acute cytomegalovirus infection were diagnosed. Additionally, the course of the disease was complicated by a secondary antibody deficiency as well as an endocarditis of the aortic valve caused by Enterococcus faecium. A cryoglobulinemic vasculitis type II was histologically found in biopsy specimen of the kidney. Thus, the present case reports on a coincidence of a monoclonal gammopathy causing a cryoglobulinemia type II with extensive organ involvement and a florid CMV infection. We hypothesize that the CMV infection has triggered the cryoglobulinemia and its particular severe organ involvement.Correspondence to:
Dr. J. Kramer
Medical Department 1
Division of Nephrology and Transplantation Unit
University Clinics of Schleswig-Holstein, Campus Lübeck
Ratzeburger Allee 160
23538 Lübeck, Germany
Email: Jan_Kramer@gmx.de
J. Kramer1, H. Hennig2, C. Lensing3, S. Krüger4, U. Helmchen5, J. Steinhoff1 and C. Dodt1
1Medical Department 1, 2Institute of Immunology and Transfusion Medicine, 3Institute of Microbiology, 4Institute of Pathology, University of Lübeck, 5Institute of Pathology, University of Hamburg, Clinic Eppendorf, Hamburg, Germany We report on a 67-year-old female patient who was admitted to our intensive care unit with acute renal failure and severe hypoxemia. Transiently, the patient had to be treated with kidney replacement therapies and artificial ventilation. The actual illness started with general weakness, recurrent bloody diarrhea and intermittent dermatitis of the lower legs. Skin symptoms were initially observed 2 years before the actual clinical findings. The bloody diarrhea was attributed to an inflammatory stenosis of the sigma. The life-threatening clinical aggravation was due to diffuse alveolar hemorrhage and alveolitis. In the search for the cause of the systemic disease, both a monoclonal g-globulinemia, causing a cryoglobulinemia type II and an acute cytomegalovirus infection were diagnosed. Additionally, the course of the disease was complicated by a secondary antibody deficiency as well as an endocarditis of the aortic valve caused by Enterococcus faecium. A cryoglobulinemic vasculitis type II was histologically found in biopsy specimen of the kidney. Thus, the present case reports on a coincidence of a monoclonal gammopathy causing a cryoglobulinemia type II with extensive organ involvement and a florid CMV infection. We hypothesize that the CMV infection has triggered the cryoglobulinemia and its particular severe organ involvement.Correspondence to:
Dr. J. Kramer
Medical Department 1
Division of Nephrology and Transplantation Unit
University Clinics of Schleswig-Holstein, Campus Lübeck
Ratzeburger Allee 160
23538 Lübeck, Germany
Email: Jan_Kramer@gmx.de
Case Reports
Vasculitis-related Wunderlich’s syndrome treated without surgical intervention
H.-Y. Chen, K.-D. Wu and Y.-M. Chen
291
28$
Abstract
Spontaneous renal or peri-renal bleeding or so-called Wunderlich’s syndrome is a rare but potentially life-threatening condition. Most reported cases are Caucasian and caused by ruptured renal tumors, either benign or malignant. The syndrome has never been documented in Orientals with underlying autoimmune diseases. We report 3 cases of spontaneous renal bleeding with concurrent systemic lupus erythematosus and vasculitis presenting with flank or abdominal pain, anemia, leukocytosis, and a high C-reactive protein. All were diagnosed by computerized tomography, magnetic resonance imaging or angiography, and treated successfully with glucocorticoids and cytotoxic agents without surgical intervention.Correspondence to:
Y.-M. Chen, MD
National Taiwan University Hospital
No. 7 Chung-Shan South Road
Taipei, 100, Taiwan
Email: ymchen@ha.mc.ntu.edu.tw
H.-Y. Chen, K.-D. Wu and Y.-M. Chen
Renal Division, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan Spontaneous renal or peri-renal bleeding or so-called Wunderlich’s syndrome is a rare but potentially life-threatening condition. Most reported cases are Caucasian and caused by ruptured renal tumors, either benign or malignant. The syndrome has never been documented in Orientals with underlying autoimmune diseases. We report 3 cases of spontaneous renal bleeding with concurrent systemic lupus erythematosus and vasculitis presenting with flank or abdominal pain, anemia, leukocytosis, and a high C-reactive protein. All were diagnosed by computerized tomography, magnetic resonance imaging or angiography, and treated successfully with glucocorticoids and cytotoxic agents without surgical intervention.Correspondence to:
Y.-M. Chen, MD
National Taiwan University Hospital
No. 7 Chung-Shan South Road
Taipei, 100, Taiwan
Email: ymchen@ha.mc.ntu.edu.tw
Case Reports
Reversible renal vasoconstriction in a patient with acute renal failure after exercise
K.J. Oh, H.H. Lee, J.S. Lee, W. Chung, J.-H. Lee, S.H. Kim and J.S. Lee
K.J. Oh, H.H. Lee, J.S. Lee, W. Chung, J.-H. Lee, S.H. Kim and J.S. Lee
297
24$
Abstract
Acute renal failure (ARF) with severe loin pain induced by anaerobic exercise is a rare condition that is accompanied by wedge-shaped contrast enhancement seen on computerized tomographic (CT) scan without evidence of rhabdomyolysis. An 18-year-old Korean male was transferred to our hospital for evaluation of mild azotemia, that developed after anaerobic exercise. The laboratory tests revealed that the serum creatinine was 2.1 mg/dl and the serum uric acid level was 1.6 mg/dl without any elevation of the serum myoglobin or creatine phosphokinase. Under the impression of exercise-induced ARF, we tried to determine the relationship between the occurrence of clinical symptoms, renal dysfunction and the characteristic CT findings by observing those changes prospectively before and after anaerobic exercise. After obtaining a written consent, the patient underwent a strenuous period of anaerobic exercise to induce the clinical symptoms. Before exercise, he was completely asymptomatic; his serum creatinine level was 0.9 mg/dl and CT scan of the kidneys showed no abnormalities. Loin pain developed 2 hours after exercise, and the serum creatinine level increased to 1.2 mg/dl 18 hours after the exercise. CT scan 18 hours after exercise showed multiple perfusion defects, and a 24-hour delayed CT scan showed multiple areas of wedge-shaped enhancement on both kidneys. These changes were completely resolved on the follow-up CT scan obtained 13 days after exercise with the return of a normal serum creatinine level. We conclude that reversible renal vasoconstriction is probably the main pathophysiologic mechanism of acute renal failure induced by anaerobic exercise.Correspondence to:
Dr. J.-H. Lee
Department of Internal Medicine
School of Medicine, Konkuk University
4-12 Hwayang-Dong, Gwangjin-Gu, Seoul 143-729, Korea
Email: nephlee@hanmail.net
K.J. Oh, H.H. Lee, J.S. Lee, W. Chung, J.-H. Lee, S.H. Kim and J.S. Lee
1Department of Internal Medicine, Gil Medical Center, Gachon Medical School, Incheon, 2Department of Internal Medicine, School of Medicine, Konkuk University, Seoul, 3Department of Imaging Radiology, Gil Medical Center, Gachon Medical School, Incheon and 4Department of Internal Medicine, College of Medicine, Seoul National University, Seoul, Korea Acute renal failure (ARF) with severe loin pain induced by anaerobic exercise is a rare condition that is accompanied by wedge-shaped contrast enhancement seen on computerized tomographic (CT) scan without evidence of rhabdomyolysis. An 18-year-old Korean male was transferred to our hospital for evaluation of mild azotemia, that developed after anaerobic exercise. The laboratory tests revealed that the serum creatinine was 2.1 mg/dl and the serum uric acid level was 1.6 mg/dl without any elevation of the serum myoglobin or creatine phosphokinase. Under the impression of exercise-induced ARF, we tried to determine the relationship between the occurrence of clinical symptoms, renal dysfunction and the characteristic CT findings by observing those changes prospectively before and after anaerobic exercise. After obtaining a written consent, the patient underwent a strenuous period of anaerobic exercise to induce the clinical symptoms. Before exercise, he was completely asymptomatic; his serum creatinine level was 0.9 mg/dl and CT scan of the kidneys showed no abnormalities. Loin pain developed 2 hours after exercise, and the serum creatinine level increased to 1.2 mg/dl 18 hours after the exercise. CT scan 18 hours after exercise showed multiple perfusion defects, and a 24-hour delayed CT scan showed multiple areas of wedge-shaped enhancement on both kidneys. These changes were completely resolved on the follow-up CT scan obtained 13 days after exercise with the return of a normal serum creatinine level. We conclude that reversible renal vasoconstriction is probably the main pathophysiologic mechanism of acute renal failure induced by anaerobic exercise.Correspondence to:
Dr. J.-H. Lee
Department of Internal Medicine
School of Medicine, Konkuk University
4-12 Hwayang-Dong, Gwangjin-Gu, Seoul 143-729, Korea
Email: nephlee@hanmail.net
Case Reports
Treatment of hirudin overdosage in a dialysis patient with heparin-induced thrombocytopenia with mixed hemodialysis and hemofiltration treatment
C. Mon, G. Moreno, M. Ortiz, R. Diaz, J.C. Herrero, A. Oliet, I. Rodriguez, O. Ortega, P. Gallar and A. Vigil
C. Mon, G. Moreno, M. Ortiz, R. Diaz, J.C. Herrero, A. Oliet, I. Rodriguez, O. Ortega, P. Gallar and A. Vigil
302
20$
Abstract
We report a patient who developed type II heparin-induced thrombocytopenia (HIT) and accidentally received a recombinant hirudin (r-hirudin) overdosage. Treatment with hemodialysis (HD) using high-flux polysulfone dialyzer and hemofiltration was performed. Length of treatment was adjusted, monitoring activated partial thromboplastin time (aPTT) to 1.5 – 2.5 times the mean of the normal range. She developed deep venous thrombosis and occlusion of vascular access. Only after cessation of heparin lock catheter, platelet count began to increase. After one year of treatment with acenocoumarol and additional low-dose r-hirudin, neither bleeding nor thrombotic episodes have been reported.Correspondence to:
C. Mon, MD
Hospital Severo Ochoa
Avda de Orellana sn
28911 Madrid, Spain
Email: cmon.hsvo@salud.madrid.org
C. Mon1, G. Moreno2, M. Ortiz1, R. Diaz3, J.C. Herrero1, A. Oliet1, I. Rodriguez1, O. Ortega1, P. Gallar1 and A. Vigil1
1Nephrology Department, 2Hematology Department and 3Intensive Care Unit, Hospital Severo Ochoa, Madrid, Spain We report a patient who developed type II heparin-induced thrombocytopenia (HIT) and accidentally received a recombinant hirudin (r-hirudin) overdosage. Treatment with hemodialysis (HD) using high-flux polysulfone dialyzer and hemofiltration was performed. Length of treatment was adjusted, monitoring activated partial thromboplastin time (aPTT) to 1.5 – 2.5 times the mean of the normal range. She developed deep venous thrombosis and occlusion of vascular access. Only after cessation of heparin lock catheter, platelet count began to increase. After one year of treatment with acenocoumarol and additional low-dose r-hirudin, neither bleeding nor thrombotic episodes have been reported.Correspondence to:
C. Mon, MD
Hospital Severo Ochoa
Avda de Orellana sn
28911 Madrid, Spain
Email: cmon.hsvo@salud.madrid.org
Case Reports
Successful treatment of respiratory dysfunction in cystinosis by nocturnal non-invasive positive pressure ventilation
M.A. Edens, W.J. van Son, M.H.G. de Greef, E.N. Levtchenko, T. Blijham and P.J. Wijkstra
M.A. Edens, W.J. van Son, M.H.G. de Greef, E.N. Levtchenko, T. Blijham and P.J. Wijkstra
306
20$
Abstract
Cystinosis is a rare metabolic disorder characterized by lysosomal cystine accumulation leading to multi-organ damage, with kidneys being clinically first affected. Longer survival of cystinosis patients due to successful renal replacement therapy, revealed previously unknown extra-renal symptoms of cystinosis, generally appearing after the first decade. Respiratory insufficiency caused by overall respiratory muscle myopathy is a severely invalidating and sometimes a life-threatening complication of cystinosis. We report a successful treatment of hypoventilation, due to diaphragm myopathy in a cystinosis patient, by nocturnal non-invasive positive pressure ventilation (NIPPV). After initiation of NIPPV the clinical condition of the patient improved and blood-gasses normalized, indicating that this treatment modality should be considered in cystinosis patients with severe respiratory insufficiency.Correspondence to:
M.A. Edens, MSc
University Medical Center Groningen
Triadegebouw, Kamer E 4.04, Hanzeplein 1
P.O. Box 30.001
9700 RB Groningen, The Netherlands
Email: m.a.edens@epi.umcg.nl
M.A. Edens, W.J. van Son, M.H.G. de Greef, E.N. Levtchenko, T. Blijham and P.J. Wijkstra
1University Department of Human Movement Sciences, 2Department of Nephrology, 3Department of Paediatrics UMC st Radbout Nijmegen, 4Center for Home Mechanical Ventilation, and 5Department of Pulmonary Diseases/Center for Home Mechanical Ventilation, University Medical Center Groningen, Groningen, The Netherlands Cystinosis is a rare metabolic disorder characterized by lysosomal cystine accumulation leading to multi-organ damage, with kidneys being clinically first affected. Longer survival of cystinosis patients due to successful renal replacement therapy, revealed previously unknown extra-renal symptoms of cystinosis, generally appearing after the first decade. Respiratory insufficiency caused by overall respiratory muscle myopathy is a severely invalidating and sometimes a life-threatening complication of cystinosis. We report a successful treatment of hypoventilation, due to diaphragm myopathy in a cystinosis patient, by nocturnal non-invasive positive pressure ventilation (NIPPV). After initiation of NIPPV the clinical condition of the patient improved and blood-gasses normalized, indicating that this treatment modality should be considered in cystinosis patients with severe respiratory insufficiency.Correspondence to:
M.A. Edens, MSc
University Medical Center Groningen
Triadegebouw, Kamer E 4.04, Hanzeplein 1
P.O. Box 30.001
9700 RB Groningen, The Netherlands
Email: m.a.edens@epi.umcg.nl
Letters to the Editor
Pseudogout: a cause of acute arthritis of the right ankle in a uremic patient
S.I. Lee, W.H. Yoo, Y.B. Jang, K.P. Kang, S. Lee, W. Kim and S.K. Park
310
12$
Abstract
S.I. Lee, W.H. Yoo, Y.B. Jang, K.P. Kang, S. Lee, W. Kim and S.K. Park
Letters to the Editor
Association between intensive intravenous iron treatment and angina pectoris in hemodialysis patients
P. Malindretos, A. Sioulis, E. Avgeriou, A. Michalaki, V. Roma and D. Grekas
P. Malindretos, A. Sioulis, E. Avgeriou, A. Michalaki, V. Roma and D. Grekas
312
12$
Abstract
P. Malindretos, A. Sioulis, E. Avgeriou, A. Michalaki, V. Roma and D. Grekas
Letters to the Editor
Editorial Announcement
-
314
8$
Abstract
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