Volume 75 (2011), No. 7/2011(Supplement 1)
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Nephrology Education
Successful treatment of renal AA amyloidosis in familial Mediterranean fever with pegylated alpha-2a interferon
S.J. Vandecasteele, P. De Paepe and A.S. De Vriese
Page No. 1
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S1-S3)
Successful treatment of renal AA amyloidosis in familial Mediterranean fever with pegylated alpha-2a interferon
S.J. Vandecasteele1, P. De Paepe2 and A.S. De Vriese1
Department of 1Nephrology and Infectious Diseases and 2Pathology, AZ Sint-Jan Brugge-Oostende AV, Brugge, Belgium
Renal AA amyloidosis is a severe consequence of chronic inflammatory diseases such as familial Mediterranean fever (FMF). FMF is caused by mutations in the MEFV gene, resulting in defective control of granulocyte-mediated inflammation. Interferon-alpha is known to induce MEFV expression in monocytes and granulocytes in vitro. We present the first case of colchicine-resistant FMF in which a durable disease remission and regression of renal amyloidosis was induced by chronic treatment with pegylated interferon-alpha.Correspondence to:
S.J. Vandecasteele, MD, PhD
AZ Sint-Jan Brugge-Oostende AV
Rudderhove 10
8000 Brugge, Belgium
Email: [email protected]
Nephrology Education
Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange
R.T. Krmar, U. Holtbäck, T. Linné, U.B. Berg, G. Celsi, M.P. Söderberg, A. Wernerson, A. Szakos, S. Larsson, L. Skattum and P. Bárány
Page No. 4
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S4-S10)
Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange
R.T. Krmar1, U. Holtbäck1, T. Linné1, U.B. Berg1, G. Celsi1, M.P. Söderberg2, A. Wernerson2, A. Szakos2, S. Larsson3, L. Skattum4 and P. Bárány5
1Karolinska Institute, Department for Clinical Science, Intervention and Technology, Division of Pediatrics, 2Department of Pathology, 3Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Huddinge, 4Institute of Laboratory Medicine, Section of Microbiology, Immunology and Glycobiology, Lund University, Lund and 5Karolinska Institute, Department of Clinical Science, Intervention and Technology, Division of Renal Medicine, Karolinska University Hospital, Huddinge, Sweden
We describe the clinical course of a female adolescent who was followed because of isolated microhematuria and hypocomplementemia before admission to hospital with a sudden onset of acute renal failure. At presentation, she exhibited complement consumption through the complement alternative pathway (AP) while other serologic tests were negative. Renal biopsy revealed dense deposit disease (DDD) with a crescentic pattern. Intravenous methylprednisolone, followed by plasma exchange (PE), and intravenous cyclophosphamide pulses were started shortly after admission. C3NeF and anti-factor H antibody tests were negative. Serum factor H and I levels were normal as well as factor H activity. Screening for mutation in the factor H gene revealed the H402 allele variant. Clinical remission, defined as normalization in renal function and in the activity levels of the complement AP, was noted at one month post-presentation and throughout the follow-up. A repeat renal biopsy showed the disappearance of crescent formation, whereas electron microscopy revealed no regression in dense transformation of the lamina densa. In summary, our patient was successfully treated with immunosuppressant and PE. The absence of known factors associated with DDD suggests that, in this particular case, other regulatory mechanisms of complement AP might have been involved in the disease process.Correspondence to:
R.T. Krmar, MD, PhD
Karolinska Institute
Department for Clinical Science, Intervention and Technology
Division of Pediatrics
Karolinska University Hospital, Huddinge
141 86 Stockholm, Sweden
Email: [email protected]
Nephrology Education
Cocaine-associated acute severe hyponatremia
M.R. Karim, M. Jawairia, S. Rahman, L. Balsam and S. Rubinstein
Page No. 11
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S11-S15)
Cocaine-associated acute severe hyponatremia
M.R. Karim, M. Jawairia, S. Rahman, L. Balsam and S. Rubinstein
Division of Nephrology and Hypertension, Nassau University Medical Center, East Meadow, NY, USA
Cocaine is an indirect sympathomimetic agent that acts in the nervous system to block the presynaptic reuptake of serotonin and catecholamines (dopamine, norepinephrine and epinephrine) and to increase their bioavailability at the post-synaptic receptors. The association of cocaine and hyponatremia has not been reported in adults. We describe a case of acute severe hyponatremia that followed smoking cocaine. We propose that cocaine, via its effect on the neurotransmitters, stimulates antidiuretic hormone release and leads to a syndrome of inappropriate antidiuretic hormone secretion.Correspondence to:
S. Rubinstein, MD
Division of Nephrology and Hypertension, Nassau University Medical Center
2201 Hempstead Turnpike, Box 49,
East Meadow, NY 11554, USA
Email: [email protected]
Nephrology Education
Oxalate nephropathy in a diabetic patient after gastric by-pass
D.-A. Moutzouris, G. Skaneli, V. Margellos, T. Apostolou, C. Petraki and N. Nikolopoulou
Page No. 16
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S16-S19)
Oxalate nephropathy in a diabetic patient after gastric by-pass
D.-A. Moutzouris1, G. Skaneli1, V. Margellos1, T. Apostolou1, C. Petraki2 and N. Nikolopoulou1
Departments of 1Nephrology and 2Renal Pathology, “Evangelismos” General Hospital, Athens, Greece
We report a case of 48-year-old woman with history of diabetes and hypertension, who presented with acute to chronic kidney injury. Sixteen months before presentation, she had undergone Roux-en-Y gastric by-pass (RYGB) for morbid obesity. Kidney biopsy showed lesions consistent with oxalate nephropathy and deposition of calcium oxalate crystals. An extensive workshop excluded other causes of kidney injury. The patient subsequently required dialysis with no improvement of renal function on follow-up. The mechanism by which patients develop hyperoxaluria after RYGB remains obscure; it is suggested that RYGB provokes fat malabsorption, which results in increased load of free fatty acid in the intestine. Thus, calcium binds to free fatty acids provoking reduced synthesis of calcium oxalate. Consequently, increased quantity of oxalate remains free and is absorbed in the intestine causing hyperoxaluria. Similar to our case, oxalate nephropathy after RYGB is seen in patients with diabetes, hypertension and chronic kidney injury. Treatment includes low-fat, low-oxalate diet along with administration of calcium supplements. Unfortunately, prognosis is rather poor with the majority of patients eventually requiring permanent dialysis. Therefore, patients with history of chronic kidney disease undergoing RYGB should be closely monitored, particularly those with long standing history of diabetes and hypertension.Correspondence to:
D.-A. Moutzouris, MD
2, Miaouli Str, Neo Psychico
Athens 15451, Greece
Email: [email protected]
Nephrology Education
Sequential rupture of triceps and quadriceps tendons in a dialysis patient using hormone supplements
I. Soo, J. Christiansen, D. Marion, M. Courtney and V.A. Luyckx
Page No. 20
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S20-S23)
Sequential rupture of triceps and quadriceps tendons in a dialysis patient using hormone supplements
I. Soo1*, J. Christiansen2*, D. Marion1, M. Courtney1,3 and V.A. Luyckx1,3
1Department of Medicine, 2University of Alberta Medical School, and 3Division of Nephrology, Edmonton, Alberta, Canada
*Joint first authors
Spontaneous rupture of tendons is rare, and typically occurs in large weight bearing tendons such as the quadriceps, Achilles and patellar tendon, in the context of various chronic diseases including end-stage renal disease. In general, tendon rupture in dialysis patients is associated with hyperparathyroidism, long duration of dialysis, steroid and quinolone use. We present a case of a young man on chronic dialysis who presented with sequential rupture of triceps and quadriceps tendons requiring surgical repair, several months after initiating use of multiple hormone supplements including human growth hormone and androgens. The supplements were obtained over the internet with the aim of improving his kidney function. Although this patient did have hyperparathyroidism, it is likely his PTH elevation was exacerbated by use of human growth hormone, and tendon rupture risk increased by concurrent use of an androgen supplement. This case highlights the fact that dialysis patients do utilize alternative remedies and that there may be unexpected, dialysis-specific complications associated with their use.Correspondence to:
V.A. Luyckx, MBBCh
260 HMRC
Edmonton, Alberta, T6G 2S2, Canada
Email: [email protected]
Nephrology Education
Efficacy of diffusion-weighted magnetic resonance imaging in detecting infected cysts in a case of polycystic kidney disease
K. Katano, Y. Kakuchi, A. Nakashima, S. Takahashi and M. Kawano
Page No. 24
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S24-S26)
Efficacy of diffusion-weighted magnetic resonance imaging in detecting infected cysts in a case of polycystic kidney disease
K. Katano1, Y. Kakuchi1, A. Nakashima1, S. Takahashi1 and M. Kawano2
1Department of Internal Medicine, Saiseikai Kanazawa Hospital, and 2Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Ishikawa, Japan
Hepatic and/or renal cyst infection is a major complication in patients with polycystic kidney disease. In many cases, drainage of infected cysts is necessary, although accurate detection of infected cysts from among the numerous hepatic or renal cysts present is often difficult, because the findings of infected cysts on computed tomography and T1- and T2-weighted magnetic resonance imaging resemble those of normal cysts. We describe here a case of polycystic kidney disease complicated by hepatic cyst infection. On diffusion-weighted magnetic resonance imaging (DWMRI), which is occasionally used in the diagnosis of cerebral abscesses, infected hepatic cysts showed higher signal intensity than other cysts, facilitating differentiation of the cysts requiring drainage from numerous other cysts. Infected cysts showed a marked decrease of the apparent diffusion coefficient (ADC) values compared with those of normal cysts. DWMRI was very effective in detecting infected cysts in our patient and may be of value in other such cases with polycystic kidney disease.Correspondence to:
K. Katano, MD, PhD
Department of Internal Medicine
Saiseikai Kanazawa Hospital
13-6, Akatsuchimachi, Kanazawa,
Ishikawa, 920-0353, Japan
Email: [email protected]
Nephrology Education
Unusual acute promyelocytic leukemia following de novo renal transplant: case report and literature review
C.-P. Lai, C.-C. Chang, C.-Y. Chung and M.-L. Chen
Page No. 27
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S27-S31)
Unusual acute promyelocytic leukemia following de novo renal transplant: case report and literature review
C.-P. Lai1, C.-C. Chang1, C.-Y. Chung2 and M.-L. Chen3
1Nephrology Division, 2Hemotology Division, and 3Department of Pathology, Changhua Christian Hospital, Changhua, Taiwan
Abstract. Acute promyelocytic leukemia (APL) with transcripting three isoforms of mRNA from PML-RAR alpha fusion genes following renal transplantation has never been reported in the literature. A 39-year-old man received a cadaveric renal allograft for IgA chronic kidney disease in 2006 and was consecutively immunosuppressed by tacrolimus, mycophenolate mofetil, and prednisolone. In 2008, he presented with gum bleeding and weight loss. Following bone marrow biopsy, we diagnosed acute promyelocytic leukemia. Molecular analysis demonstrated atypical presence consisting of three isoforms of mRNA from PML-RAR alpha fusion genes. The patient was administered with three courses of consolidation chemotherapy plus atretinoin, resulting in complete remission and did not jeopardize his allograft function. This novel finding suggests that the leukemogenesis of APL may be polyclonal, sharing similar progenitor targeting on complicated karyotypes, responding well to current chemotherapy.Correspondence to:
C.-C. Chang, MD
Department of Internal Medicine
Changhua Christian Hospital
#135 Nan-Siau Street, Changhua, 500 Taiwan
Email: [email protected]
Nephrology Education
Collapsing glomerulopathy in a patient with mixed connective tissue disease
S.I. Rifkin, H. Gutta, R. Nair, C. McFarren and D.E. Wheeler
Page No. 32
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S32-S36)
Collapsing glomerulopathy in a patient with mixed connective tissue disease
S.I. Rifkin2, H. Gutta2, R. Nair1, C. McFarren2 and D.E. Wheeler3
1Department of Internal Medicine, 2Division of Nephrology, and 3Departments of Pathology and Cell Biology, and Internal Medicine, University of South Florida College of Medicine, Tampa, FL, USA
Collapsing glomerulopathy (CG) is a distinct clinicopathological entity characterized by glomerular capillary collapse, podocyte proliferation, diffuse mesangial sclerosis, and podocyte maturation arrest. Initially noted primarily in HIV infected patients, a number of other diseases have now been associated with CG. Mixed connective tissue disease (MCTD) is a disease with overlapping features of systemic lupus erythematosus, progressive systemic sclerosis, and polymyositis. It was originally thought that renal involvement was a rare complication of MCTD. However, over the years, it has become clearer that renal involvement, although not always clinically apparent, is frequent. In this report we present a patient with MCTD who developed CG.Correspondence to:
S.I. Rifkin, MD
2 Tampa General Circle, Suite 6076
Tampa, Florida, 33606, USA
Email: [email protected]
Nephrology Education
Superior mesenteric artery syndrome complicating dialysis patients with peritoneal failure – report of 3 cases
D.Y.H. Yap, M.K.M. Ma, A.S.H. Lai, S.Y. Chan, W.K. Seto, M.F. Lam, T.M. Chan and K.N. Lai
Page No. 37
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S37-S41)
Superior mesenteric artery syndrome complicating dialysis patients with peritoneal failure – report of 3 cases
D.Y.H. Yap1, M.K.M. Ma1, A.S.H. Lai3, S.Y. Chan4, W.K. Seto2, M.F. Lam1, T.M. Chan1 and K.N. Lai1
1Division of Nephrology, 2Division of Hepatology and Gastroenterology, Department of Medicine, 3Department of Radiology, and 4Division of Upper Gastrointestinal Surgery, Department of Surgery, Queen Mary Hospital, University of Hong Kong, Hong Kong
We report 3 cases of superior mesenteric artery syndrome in patients previously on maintenance peritoneal dialysis converted to hemodialysis after peritoneal failure. All 3 patients presented with repeated vomiting and severe malnutrition. It is postulated that complications arising from peritoneal dialysis such as peritoneal sclerosis, adhesions and collections after CAPD peritonitis may be important contributing factors for the SMA syndrome in these 3 patients. All of them succumbed within six months of diagnosis. The first 2 patients received gastrointestinal bypass surgery and died post-operatively due to impaired wound healing and nosocomial sepsis. The 3rd patient was treated conservatively with nasoduodenal feeding but succumbed to aspiration pneumonia. It is postulated that complications arising from peritoneal dialysis including peritoneal sclerosis, adhesions and collections after CAPD peritonitis may contribute to the SMA syndrome in these patients. Our experience suggests that SMA syndrome in end-stage renal disease patients is associated with high surgical morbidity and mortality possibly related to their poor pre-morbid condition and pre-existing malnutrition. Aggressive parenteral nutrition should be considered to build up the general status before proceeding to surgical intervention.Correspondence to:
Prof. K.N. Lai
Division of Nephrology
Department of Medicine
Queen Mary Hospital
University of Hong Kong
Hong Kong, HKSAR
Email: [email protected]
Nephrology Education
Remission of membranous glomerulonephritis after pancreatectomy for pancreatic neuroendocrine neoplasm – a rare coincidence
T. Forslund, I. Kellokumpu, E. Elomaa, J. Arola and K. Nuorva
Page No. 42
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S42-S46)
Remission of membranous glomerulonephritis after pancreatectomy for pancreatic neuroendocrine neoplasm – a rare coincidence
T. Forslund1, I. Kellokumpu2, E. Elomaa3, J. Arola4 and K. Nuorva5
1Division of Nephrology, Department of Internal Medicine, 2Department of Abdominal Surgery, 3Department of Clinical Radiology, Central Finland Health Care District Hospital, Jyväskylä, 4Department of Pathology, University of Helsinki and HUSLAB, Helsinki, and 5Department of Pathology, Central Finland Health Care District Hospital, Jyväskylä, Finland
The concomitant existence of a non-malignant neuroendocrine tumor (NET) and membranous glomerulonephritis (MGN) is rare. We report a subject with kidney biopsy proven MGN and nephrotic syndrome in which a computerized scan tomography (CT) examination was performed revealing a pancreatic tumor. A pancreatectomy was performed and the tumor was shown to be a non-malignant NET with a malignant potential. Although treatment with corticosteroids was initiated remission of MGN was observed within the next month after pancreatectomy. The rapid remission observed shortly after pancreatectomy pointed to that tumor removal contributed to, and that neither spontaneous nor corticosteroid treatment alone did induce the rapid remission of the MGN. The coexistence of the two disorders NET and MGN is very rare, however. This is the first report on remission of MGN after pancreatectomy for a NET.Correspondence to:
T. Forslund, MD, PhD, Associate Professor
Division of Nephrology, Department of Medicine
Central Finland Health Care District Hospital
40620 Jyväskylä, Finland
Email: [email protected]
Nephrology Education
Severe erosive lesions in the digestive tract of patients with Henoch-Schönlein Purpura (HSP) and its impact on prognosis – presentation of two cases and statistical review of adult-onset Japanese HSP
K. Tobino, Y. Shimizu, S. Miura, K. Sugawara, K. Takeda and Y. Tomino
Page No. 47
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S47-S55)
Severe erosive lesions in the digestive tract of patients with Henoch-Schönlein Purpura (HSP) and its impact on prognosis – presentation of two cases and statistical review of adult-onset Japanese HSP
K. Tobino1, Y. Shimizu1, S. Miura2, K. Sugawara3, K. Takeda2 and Y. Tomino1
1Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine, Tokyo, 2Department of Nephrology, Aso Iizuka Hospital, Fukuoka, and 3Department of Nephrology, Matsuyama Red Cross Hospital, Ehime, Japan
Introduction: Although many pediatric patients with Henoch-Schönlein Purpura (HSP) recover spontaneously, disease activity in adult patients often cannot be controlled by treatment. Purpose: To assess the specific signs not formerly considered to be those of uncontrollable adult HSP patients. Patients and methods: Clinical records of 2 adult patients who died during HSP were reviewed and previous reports on HSP were consulted. Results: Both patients had lesions in the digestive tract diagnosed as hemorrhagic erosion in the small intestine and colon. They were elderly and showed renal dysfunction. They died from severe infection after potent immunosuppressive treatment. A univariate analysis showed that age of over 60 years, severe renal symptoms (nephrotic syndrome and/or end-stage renal failure), Birmingham Vasculitis Activity Score (BVAS) of more than 18 points, massive immunosuppression and melena had significantly higher prevalence among patients who died. Multivariate statistical analysis with theoretical quantification analysis II revealed that age of over 60 and severe renal symptoms (nephrotic syndrome and/or end-stage renal failure) contributed to poor prognosis. The presence of melena did not contribute to poor prognosis despite results of the univariate analysis and our clinical impressions. Discussion: In multivariate statistical analysis, melena was selected as a sign of severe erosive lesions in the digestive tract because some of the patients were not examined by fiberscopy. Melena is caused by various lesions in the digestive tract and each of them has different effects on prognosis. Conclusion: Elderly HSP patients with severe renal impairment should be carefully treated. Examination of the digestive tract by fiberscopy is recommended when melena is observed in these patients.Correspondence to:
Y. Tomino, MD, PhD
Division of Nephrology
Department of Internal Medicine
Juntendo University of School of Medicine
2-1-1, Hongo, Bunkyo-ku
Tokyo 113-8421, Japan
Email: [email protected]
Nephrology Education
Unilateral breast swelling as a complication of innominate vein stenosis in a hemodialysis patient
U. Yalavarthy, V.K. Agrawal and A. Showkat
Page No. 56
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S56-S59)
Unilateral breast swelling as a complication of innominate vein stenosis in a hemodialysis patient
U. Yalavarthy1, V.K. Agrawal2 and A. Showkat1
1Division of Nephrology and 2Department of Radiology, University of Tennessee Health Science Center, Memphis, TN, USA
A 53-year-old African-American female with end-stage renal disease presented with 3 months history of right breast swelling. The patient had a right internal jugular tunneled catheter for dialysis access. Physical examination was significant for right breast swelling, peau d’orange appearance, and mild tenderness on palpation. Mammogram was normal. A CT scan of the chest with contrast demonstrated chest wall edema and prominent superficial collateral veins suggesting central vein obstruction. Right upper extremity venogram revealed right innominate vein occlusion up to its junction with the subclavian vein. Angioplasty of occluded segment was attempted and was unsuccessful, but right breast swelling significantly improved following the procedure. Pathogenesis, clinical manifestations and management of central vein stenosis is discussed.Correspondence to:
U. Yalavarthy, MD
Division of Nephrology
956 Court Avenue B224
Memphis, TN 38163, USA
Email: [email protected]
Nephrology Education
Ritonavir-induced acute kidney injury: kidney biopsy findings and review of literature
T. Shafi, M.J. Choi, L.C. Racusen, L.A. Spacek, C. Berry, M. Atta and D.M. Fine
Page No. 60
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S60-S64)
Ritonavir-induced acute kidney injury: kidney biopsy findings and review of literature
T. Shafi1, M.J. Choi1, L.C. Racusen2, L.A. Spacek3, C. Berry4, M. Atta1 and D.M. Fine1
1Department of Medicine, Division of Nephrology, 2Department of Pathology, 3Department of Medicine, Division of Infectious Diseases, and 4Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Ritonavir therapy is not generally considered nephrotoxic. We report a case of acute kidney injury secondary to ritonavir, with kidney biopsy demonstrating extensive acute tubular injury. This is the first report of a kidney biopsy and pathology in acute kidney injury associated with ritonavir. A review of published medical literature on the topic is also presented.Correspondence to:
T. Shafi, MD, MHS
Department of Medicine
Division of Nephrology
Johns Hopkins University School of Medicine
4940 Eastern Avenue
Baltimore, MD 21224-2780, USA
Email: [email protected]
Nephrology Education
Aeromonas hydrophila as a causative organism in peritoneal dialysis-related peritonitis: case report and review of the literature
V. Liakopoulos, S. Arampatzis, P. Kourti, T. Tsolkas, S. Zarogiannis, T. Eleftheriadis, M. Giannopoulou and I. Stefanidis
Page No. 65
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S65-S68)
Aeromonas hydrophila as a causative organism in peritoneal dialysis-related peritonitis: case report and review of the literature
V. Liakopoulos, S. Arampatzis, P. Kourti, T. Tsolkas, S. Zarogiannis, T. Eleftheriadis, M. Giannopoulou and I. Stefanidis
Department of Nephrology, School of Medicine, University of Thessaly, Larissa, Greece
Most episodes of peritoneal dialysis (PD)-related peritonitis could be attributed to a single organism, but in almost 10% of peritonitis episodes multiple organisms are identified. Polymicrobial peritonitis is often related to intra-abdominal pathology, and the prognosis may be poor. Aeromonas spp. have rarely been identified as the causative pathogen in PD-related peritonitis, and a very small number of cases has been reported in the literature. These rod-shaped, gram-negative microorganisms have been isolated from wastewater drainage systems, food, vegetables, and soil. Herein we report a case of polymicrobial peritonitis in a continuous ambulatory peritoneal dialysis (CAPD) patient with systemic lupus erythematosus (SLE), due to a combination of Streptococcus viridans and Aeromonas hydrophila infection. The patient was involved in gardening and was not compliant with her technique protocol. She did not wear a mask and omitted thorough hand washing. The patient was treated with i.p. vancomycin and ceftazidime and peritonitis was resolved. The patient’s technique was reassessed, and she was restrained by our PD nurses.Correspondence to:
Dr. I. Stefanidis, PD, Associate Professor of Nephrology
Department of Nephrology, School of Medicine
University of Thessaly, Neo Ktirio
Mezourlo Hill, 41110 Larissa, Greece
Email: [email protected]
Nephrology Education
Renal dysfunction and barttin expression in Bartter syndrome Type IV associated with a G47R mutation in BSND in a family
C.W. Park, J.H. Lim, D.-Y. Youn, S. Chung, M.-H. Lim, Y.K. Kim, Y.S. Chang and J.-H. Lee
Page No. 69
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S69-S74)
Renal dysfunction and barttin expression in Bartter syndrome Type IV associated with a G47R mutation in BSND in a family
C.W. Park1, J.H. Lim1, D.-Y. Youn2, S. Chung1, M.-H. Lim2, Y.K. Kim2, Y.S. Chang1 and J.-H. Lee2
1Division of Nephrology, Department of Internal Medicine, and 2Department of Biochemistry, College of Medicine, The Catholic University of Korea, Seoul, Korea
Bartter syndrome (BS) Type IV, associated with a G47R mutation in the BSND gene, is known to result in a mild renal phenotype. However, we report here on three brothers with varying degrees of renal dysfunction from mild to end-stage renal disease associated with renal barttin and ClC-K expression. The brothers had histories of polyhydramnios, prematurity, polyuria, deafness, and small body size. Laboratory findings showed hypokalemic metabolic alkalosis, normotensive hyperreninemic hyperaldosteronism, and an increased urinary excretion of sodium, potassium and chloride, consistent with BS Type IV. Microscopic examination of renal tissue showed hyperplasia of cells at the juxtaglomerular apparatus with dilated atrophic tubules and tubulointerstitial fibrosis. A weak barttin signal related to CIC-K expression in the cytoplasm of tubule cells, but not the basement membrane, was noted. A sequence analysis of the BSND gene showed that the affected males were homozygous for a missense G47R mutation in exon 1 of BSND. These findings suggest that the G47R mutation results in a dramatic decrease in barttin expression, which appears to be related to the location of CIC-K being changed from the basement membrane to the cytoplasm in the tubule and might have varying effects on renal function associated with factors other than this gene.Correspondence to:
J.-H. Lee, MD, PhD
Department of Biochemistry
The Catholic University of Korea, #505 Banpo-Dong, Seocho-Ku
Seoul, 137-040 Korea
Email: [email protected]
Nephrology Education
Tubulointerstitial nephritis and uveitis syndrome (TINU) with Fanconi’s syndrome
Y.-H. Yao, C.-C. Lin, Y.-M. Chung, A.-H. Yang, S.-Y. Li, C.-C. Lin, Y.-P. Lin, W.-C. Yang and C.-Y. Yang
Page No. 75
Abstract
Clinical Nephrology, Vol. 75 – Suppl. 1/2011 (S75-S78)
Tubulointerstitial nephritis and uveitis syndrome (TINU) with Fanconi’s syndrome
Y.-H. Yao1,4, C.-C. Lin1, Y.-M. Chung2,4, A.-H. Yang3,4, S.-Y. Li1,4, C.-C. Lin1,4, Y.-P. Lin1,4, W.-C. Yang1,4 and C.-Y. Yang1,4
1Division of Nephrology, Department of Medicine, 2Department of Ophthalmology, 3Department of Pathology, Taipei Veterans General Hospital, and 4School of Medicine, National Yang-Ming University, Taipei, Taiwan
We report a 57-year-old woman with concurrent tubulointerstitial nephritis and uveitis syndrome (TINU) and Fanconi’s syndrome. She presented with sudden onset of bilateral ocular pain, blurred vision, acute renal failure, glucosuria and proteinuria. Slit lamp examination revealed acute bilateral anterior uveitis. Tubulointerstitial nephritis was confirmed by kidney biopsy. Laboratory examination revealed normoglycemic glucosuria, proteinuria, normal anion-gap metabolic acidosis, phosphaturia, urinary uric acid wasting and kaliuresis leading to hypokalemia. Her vision and renal function improved gradually after systemic steroid therapy. There have been rare reports of TINU syndrome which had features of Fanconi’s syndrome. The prevalence of TINU syndrome may be underestimated, and its association with Fanconi’s syndrome requires further investigation.Correspondence to:
C.-Y. Yang, MD
Division of Nephrology
Department of Medicine
Taipei Veterans General Hospital, No. 201, Section 2
Shih-Pai Road, Taipei 11217, Taiwan
Email: [email protected]
