Volume 79 (2013), No. 4/2013(April)
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Original
Interaction of malnutrition, N-terminal pro-B-type natriuretic peptide and ventricular remodeling in patients on maintenance hemodialysis
Yu-Ji Lee, Bong Gun Song, Min Su Kim, Seong Cho, Woo Jung Chun, Ju Hyun Oh and Sung Rok Kim
Page No. 253
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (253-260)
Interaction of malnutrition, N-terminal pro-B-type natriuretic peptide and ventricular remodeling in patients on maintenance hemodialysis
Yu-Ji Lee1, Bong Gun Song2, Min Su Kim1, Seong Cho1, Woo Jung Chun2, Ju Hyun Oh2 and Sung Rok Kim1
1Nephrology Division and 2Cardiology Division, Department of Medicine, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea
Aims: The aim of the present study was to investigate the interaction of nutritional status, N-terminal probrain-type natriuretic peptide (NT-proBNP) and ventricular remodeling in hemodialysis patients. Methods: NT-proBNP was measured by immunoassay. Nutritional status was assessed using the subjective global assessment (SGA) and malnutrition-inflammation score (MIS). Transthoracic echocardiographic examinations were performed on all patients. Results: 44 patients undergoing maintenance hemodialysis were enrolled in this study. Malnourished patients had higher levels of extracellular water (ECW) per kg body weight (BWt) than well-nourished patients and higher levels of NT-proBNP. MIS was positively correlated with left ventricular mass index (LVMI), log NT-proBNP and ECW/BWt, and negatively correlated with fat mass and LV systolic dysfunction. LV systolic dysfunction, LVMI and MIS were independently associated with log NT-proBNP levels. Multiple regression analysis showed that log NT-proBNP, mean arterial pressure and ECW/BWt were independently associated with LVMI. However, MIS did not have an independent relationship to LVMI. Conclusions: Malnutrition in hemodialysis patients is accompanied by volume overload and associated with increased log NT-proBNP levels independent of volume status, and these levels are independently associated with increased LVMI. This suggests a possibility that nutritional status may affect ventricular remodeling in hemodialysis patients.Correspondence to:
Sung Rok Kim, MD, PhD
Department of Medicine
Samsung Changwon Hospital
Sungkyunkwan University School of Medicine
50 Hapseong 2-dong,
MasanHoiwon-Gu, 630-723, Changwon, Korea
Email: [email protected]
Original
Comparison of bioimpedance analysis and dual-energy X-ray absorptiometry body composition measurements in peritoneal dialysis patients according to edema
Seok Hui Kang, Kyu Hyang Cho, Jong Won Park, Kyung Woo Yoon, and Jun Young Do
Page No. 261
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (261-268)
Comparison of bioimpedance analysis and dual-energy X-ray absorptiometry body composition measurements in peritoneal dialysis patients according to edema
Seok Hui Kang, Kyu Hyang Cho, Jong Won Park, Kyung Woo Yoon, and Jun Young Do
Division of Nephrology, Department of Internal Medicine, Yeungnam University Hospital, Daegu, Korea
Background: Changes in the difference between bioimpedance analysis (BIA) and dual-energy X-ray absorptiometry (DXA) body composition measurements according to edema is an important issue for peritoneal dialysis (PD) patients. Methods: We reviewed the medical records of patients to identify all adults who had undergone PD and composition measurements by both BIA and DXA techniques. The aim of this study was to evaluate any change in the difference between BIA and DXA body composition measurements according to edema. Results: We identified 409 patients whose body compositions were measured by both techniques one or more times, for a total of 1,109 pairs of measurements. The measurements were divided into 4 quartiles on the basis of the edema index. Significant correlations and intraclass correlations were noted between the two methods for lean mass (LM), fat mass (FM), and bone mineral content. Simple linear regression analyses using DXA measurements for the prediction of body compositions by BIA showed that non-standardized-βs of total LM decreased as the grade of edema index increased (from 1.008 to 0.949), whereas non-standardized-βs of total FM increased as the grade of edema index increased (from 1.034 to 1.162). Bias for total LM changed from positive to negative, and this negative bias amplified as the grade of edema index escalated (from 0.406 kg to –2.276 kg). A positive bias was observed for total FM in the 1st quartile, and this positive bias increased with an increase in the grade of edema index (from 0.594 kg to 2.863 kg). Conclusion: LM measured by DXA is overestimated in PD patients with edema compared to the measurements by BIA. However, FM and bone mineral content measured by BIA are is overestimated in PD patients, compared to the measurements by DXA, especially in patients with worsening edema. The difference between the two techniques grows more prominent as the grade of edema increases. A combination of two methods will allow clinicians to conduct more accurate body composition assessments for PD patients with edema.Correspondence to:
Jun Young Do, MD
Department of Internal Medicine
Yeungnam University Hospital
317-1 Daemyung-Dong, Nam-Ku, 705-717, Daegu, Korea
Email: [email protected]
Original
Kidney disease in elderly South Africans
Ikechi G. Okpechi, Olugbenga E. Ayodele, Brian L. Rayner and Charles R. Swanepoel
Page No. 269
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (269-276)
Kidney disease in elderly South Africans
Ikechi G. Okpechi1, Olugbenga E. Ayodele2, Brian L. Rayner1 and Charles R. Swanepoel1
1Division of Nephrology and Hypertension, Department of Medicine, Groote Schuur Hospital and University of Cape Town, South Africa, and 2Department of Medicine, Ladoke Akintola University Osun State, Nigeria
Background: Life expectancy is low in many African countries due to several factors including the ongoing HIV epidemic. However, the global increase in life expectancy has translated to more elderly patients living with chronic kidney disease (CKD). The patterns of kidney disease in the elderly have never been described from sub-Saharan Africa. Methods: This study was a retrospective study of 111 elderly patients (age ≥ 60 years) who had a renal biopsy performed at the Groote Schuur Hospital in Cape Town between 1st January 2000 and 31st December 2009. Results: The mean age of patients at time of biopsy was 66.3 ± 5.7 years (males: 66.4 ± 5.6; females: 66.3 ± 5.9 years). Primary glomerular diseases were seen in 38.7%, secondary glomerular diseases in 36.0%, tubulointerstitial diseases in 17.1% and diseases classified as miscellaneous in 8.1% of all patients. Nephrotic syndrome was the most common indication for the performance of a renal biopsy (48.6%). Membranous lomerulonephritis (MGN) was the most common type of disease observed (14.4%) and was significantly more frequent in males than in females (p = 0.029). Other common histological diagnoses included diabetes nephropathy (12.6%), chronic glomerulonephritis (5.4%), and lupus nephritis (4.5%). HIV associated nephropathy (HIVAN) was only seen in 1 patient (0.9%). Conclusion: The patterns of renal disease currently seen in elderly South Africans closely resembles that reported from other countries but is at complete variance with the pattern reported in the general population of South Africa in which HIV plays a significant role.Correspondence to:
Dr. Ikechi G. Okpechi, MD, BS, PhD
Division of Nephrology and Hypertension
Groote Schuur Hospital and University of CapeTown
Observatory, 7925 Cape Town, South Africa
Email: [email protected]
Original
Factors related to the glomerular size in renal biopsies of chronic kidney disease patients
Nobuo Tsuboi, Yasunori Utsunomiya, Kentaro Koike, Go Kanzaki, Keita Hirano, Hideo Okonogi, Yoich Miyazaki, Makoto Ogura, Kensuke Joh, Tetsuya Kawamura and Tatsuo Hosoya
Page No. 277
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (277-284)
Factors related to the glomerular size in renal biopsies of chronic kidney disease patients
Nobuo Tsuboi1, Yasunori Utsunomiya1, Kentaro Koike1, Go Kanzaki1, Keita Hirano1, Hideo Okonogi1, Yoich Miyazaki1, Makoto Ogura1, Kensuke Joh2, Tetsuya Kawamura1 and Tatsuo Hosoya1
1Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine and 2Division of Pathology, Sendai Shakaihoken Hospital, Minato-Ku, Japan
Background: Glomerular enlargement is an important process that preserves the optimal surface area of glomerular capillaries under both physiological and pathological conditions. However, information is limited regarding how the glomerular size is defined, especially in chronic kidney disease (CKD) patients. Methods: A total of 206 renal biopsy specimens obtained from two different patient cohorts with or without a diagnosis of glomerulonephritis (non-GN group and IgAN group) were examined. The mean glomerular volume was estimated from the outer capillary area of individual glomeruli, and the clinicopathological factors at biopsy that were associated with the mean glomerular volume were analyzed in each group. Results: The mean glomerular volume showed maximal 5.8 and 7.9-fold variations between individuals in the non-GN and IgAN groups, respectively. In both groups, the body mass index and glomerular density (non-sclerotic glomerular number per renal cortical area of the biopsy) were consistently identified as independent factors that were associated with the mean glomerular volume. In addition, the multivariate analyses using the glomerular density/body mass index ratio showed a more close association with the mean glomerular volume than the analyses using each measure separately. Conclusion: These results suggest that factors presumably reflecting both body consumption and nephron number have close relationships with the glomerular size, regardless of mechanism(s) underlying the injury. The most relevant factor affecting glomerular size may be a balance between these two measures.Correspondence to:
Nobuo Tsuboi, MD, PhD
Division of Kidney and Hypertension
Department of Internal Medicine
The Jikei University School of Medicine
3-25-8 Nishi-Shinbashi, Minato-Ku, Japan
Email: [email protected]
Original
The changing pattern of glomerular disease in HIV and Hepatitis C co-infected patients in the era of HAART
Sumit Mohan, Leal C. Herlitz, Jennifer Tan, Jen-Tse Cheng, Herman L. Anderson, Michael B. Stokes, Glen S. Markowitz, Vivette D. D’Agati and Jai Radhakrishnan
Page No. 285
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (285-291)
The changing pattern of glomerular disease in HIV and Hepatitis C co-infected patients in the era of HAART
Sumit Mohan1, Leal C. Herlitz2, Jennifer Tan3, Jen-Tse Cheng3, Herman L. Anderson3, Michael B. Stokes2, Glen S. Markowitz2, Vivette D. D’Agati2 and Jai Radhakrishnan1
1Department of Medicine, Division of Nephrology, 2Department of Pathology, Columbia University College of Physicians and Surgeons, and 3Department of Medicine, Division of Nephrology, Harlem Hospital Center, New York, NY, USA
Previous reports have suggested a poor renal prognosis in patients with HIV and HCV co-infection with a preponderance of immune complex mediated glomerular disease on biopsy. Although the benefits of HAART on HIVAN are known, its impact on co-infected patients is unclear. We describe the renal biopsy findings and renal outcome in 29 co-infected patients in the HAART era and compare them to findings in 14 historical controls reported from our institution in the pre-HAART era. Our present cohort was predominantly male and Black with the majority reporting a history of intravenous (i.v.) drug use. Renal biopsy findings included 16 patients with immune complex mediated glomerular disease and 14 patients with FSGS, of which only 3 had collapsing features and/or tubular microcysts typical of HIVAN. Five patients had other biopsy diagnoses not directly related to viral infection. Median renal survival in our cohort was 15.6 months – significantly better than the 1.7 months seen our pre-HAART cohort. The modern cohort’s improved renal outcome occurred despite older patients, longer HIV infection and similar levels of renal insufficiency. Our data indicate a changing epidemiology and natural history of renal disease in the HAART era with less immune complex mediated glomerular disease and more non-collapsing FSGS of the usual type. The marked improvement is likely to be multifactorial, including use of antiretroviral and anti-HCV therapies, RAAS antagonists, earlier nephrology referral and generally improved medical care.Correspondence to:
Sumit Mohan, MD, MPH
Department of Medicine, Division of Nephrology
Columbia University Medical Center
622W 168th street, PH4-124, New York, NY 10032, USA
Email: [email protected]
Original
Immunopathologic co-localization of MPO, IgG, and C3 in glomeruli in human MPO-ANCA-associated glomerulonephritis
Soko Kawashima, Yoshihiro Arimura, Katsuko Sano, Akihiko Kudo, Yoshinori Komagata, Shinya Kaname, Hayato Kawakami and Akira Yamada
Page No. 292
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (292-301)
Immunopathologic co-localization of MPO, IgG, and C3 in glomeruli in human MPO-ANCA-associated glomerulonephritis
Soko Kawashima1, Yoshihiro Arimura1, Katsuko Sano1, Akihiko Kudo2, Yoshinori Komagata1, Shinya Kaname1, Hayato Kawakami2 and Akira Yamada1
1First Department of Internal Medicine and 2Department of Anatomy, Kyorin University School of Medicine, Tokyo, Japan
Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)- associated glomerulonephritis (GN) is characterized by pauci-immune necrotizing glomerulonephritis(NGN). Although it has been thought that MPO-ANCA is involved in the pathogenesis of capillary injuries in NGN via activation of neutrophils, recent studies suggest a possible role of other factors such as immunoglobulins precipitated on the glomeruli. Here we performed a pathological study investigating a relationship of deposition of MPO, IgG, complements with regard to MPO-positive cells and glomerular capillaries in human MPO-ANCA-associated GN. Renal specimen including 317 glomeruli obtained from 20 patients with MPOANCA- associated GN were analyzed. All of the specimens showed significant focal segmental deposition of IgG. There was a significant glomerular infiltration of MPO-positive cells along with deposition of extracellular MPO in the active lesions of segmental and global NCG, with CD34 staining being decreased in the adjacent areas. IgG deposits were almost colocalized with C3 and partly with MPO, which are also associated with a decrease in CD34 staining, suggesting that immune complex formation and the resultant capillary injuries. Actually occurred, the colocalization of MPO, IgG and C3 was seen only in the glomerular lesions with low severity and activity. These results suggest that not only MPO itself released from the neutrophils but also immune complexes composed of MPO and anti-MPO antibody may play some pathogenetic roles for the glomerular injuries especially in the early phase of human MPO-ANCA-associated GN.Correspondence to:
Dr. Soko Kawashima
First Department of Internal Medicine
Kyorin University School of Medicine
6-20-2 Shinkawa, Mitaka-shi, Tokyo, 181-8611, Japan
Email: [email protected]
Original
Henoch-Schönlein purpura-like presentation in IgA-dominant Staphylococcus infectionassociated glomerulonephritis – a diagnostic pitfall
Anjali A. Satoskar, Matthew Molenda, Patrice Scipio, Rosemary Shim, Matthew Zirwas, Reena S. Variath, Sergey V. Brodsky, Gyongyi M. Nadasdy, Lee Hebert, Brad Rovin and Tibor Nadasdy
Page No. 302
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (302-312)
Henoch-Schönlein purpura-like presentation in IgA-dominant Staphylococcus infectionassociated glomerulonephritis – a diagnostic pitfall
Anjali A. Satoskar1, Matthew Molenda2, Patrice Scipio3, Rosemary Shim3, Matthew Zirwas4, Reena S. Variath5, Sergey V. Brodsky1, Gyongyi M. Nadasdy1, Lee Hebert3, Brad Rovin3 and Tibor Nadasdy1
1Department of Pathology, Ohio State University Medical Center, Columbus, 2Department of Dermatology, Cleveland Clinic, Cleveland, 3Department of Internal Medicine; Division of Nephrology, 4Department of Internal Medicine, Division of Dermatology, Ohio State University Medical Center, Columbus, OH, and 5Southern Ohio Nephrology, Portsmouth, OH, USA
Background: In children and adults, Henoch-Schönlein purpura (HSP) has a characteristic clinical presentation that includes a purpuric lower extremity skin rash, IgA-dominant glomerulonephritis, and abdominal and joint pain. A similar clinical presentation can be seen in adults who have a systemic infection with methicillin-resistant Staphylococcus aureus. It is critically important to distinguish the IgAdominant glomerulonephritis of HSP from the IgA-dominant glomerulonephritis of staphylococcal infection, because HSP may need to be treated with corticosteroids and immunosuppressives, while Staphylococcus infection-associated glomerulonephritis requires antibiotics. Design: We searched our renal biopsy database for cases of Staphylococcus infection-associated IgA-dominant glomerulonephritis, to identify those with an HSP-like presentation. Their clinical, laboratory, and biopsy findings are reviewed. Results: Between 2004 and 2011, we identified 37 patients with culture-proven Staphylococcus infection-associated glomerulonephritis. Of these, 8 (22%) had an HSP-like presentation manifested by lower extremity purpuric skin rash. Mesangial IgA and C3 deposits were consistent findings on kidney biopsy. Crescents were uncommon. Four of the 8 patients received glucocorticoid (steroid) therapy for a presumed diagnosis of HSP. Renal function worsened in 3 patients, and 1 patient ultimately improved but developed sepsis during the course. Overall, renal outcome was poor in 71% of the cases despite mild chronic renal injury in the biopsy. Conclusion: In adult patients with an HSPlike presentation, a high index of suspicion for underlying Staphylococcal infection is warranted. Blood cultures are frequently negative. Cultures from the site of infection should be performed. Steroid treatment did not improve outcomes. Renal outcomes were frequently poor.Correspondence to:
Anjali A. Satoskar, MD
Department of Pathology, Ohio State University
M018 Starling Loving, 320 West 10th Ave
Columbus OH 43210, USA
Email: [email protected]
Original
Large decrease of anti-tetanus anatoxin and anti-pneumococcal antibodies at one year after renal transplantation
Emine Nilufer Broeders, Karl M. Wissing, Lidia Ghisdal, Anna Lemy, Anh-Dung Hoang, Pierre Vereerstraeten, Françoise Mascart and Daniel Abramowicz
Page No. 313
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (313-317)
Large decrease of anti-tetanus anatoxin and anti-pneumococcal antibodies at one year after renal transplantation
Emine Nilufer Broeders1, Karl M. Wissing1, Lidia Ghisdal1, Anne Lemy1, Anh-Dung Hoang1, Pierre Vereerstraeten1, Françoise Mascart2 and Daniel Abramowicz1
1Renal Transplantation Clinic, and 2Immunobiology Clinic, Erasme Hospital and Laboratory of Vaccinology and Mucosal Immunity, Hospital Erasme, Université Libre de Bruxelles (U.L.B.), Brussels, Belgium
Aims: In kidney transplant recipients (KTR), antibody (Ab) synthesis is hampered by AZA and CsA. We here report in a prospective cohort study, the effects of mycophenolate mofetil (MMF) associated to a calcineurin inhibitor on plasma levels of anti-tetanus anatoxin Ab (TAnAb) and anti-pneumococcal Ab (PnPsAb). Methods: Serum titers of the TAnAb and the PnPsAb against serotypes 14, 19F and 23F were measured in 94 KTR on Day 0 (T0) and 1 year (T12) after renal transplantation and in 49 healthy controls. Results: 1) At T0, TAnAb were detected in only 71% of patients vs. 98% of controls (p < 0.0001) and the titers were significantly lower in KTR (1.46 UI/ml vs. 2.74 in controls, p = 0.01); they further decreased between T0 and T12 (1.46 UI/ml to 0.31, p < 0.0001). The calculated half-life (t1/2) of TAnAb was 7.7 months, as compared to more than 10 years in a normal population. 2) In KTR, PnPsAb titers decreased significantly between T0 and T12 (p < 0.005); the t1/2 of the different PnPsAb ranged from 9.2 to 11.9 months. Conclusions: In KTR treated by MMF and CNI, the TAnAbs and PnPsAbs titers decrease significantly and profoundly during the first year. Immunization pre-transplantation should be encouraged to maintain adequate post-transplant Abs levels.Correspondence to:
Emine Nilufer Broeders, MD
Renal Transplantation Clinic, Hospital ULB-Erasme
Route de Lennik, 808, 1070 Brussels, Belgium
Email: [email protected]
Nephrology Education
Recovery of kidney function following delayed use of theralite™ dialyzer in a patient with myeloma cast nephropathy
Khagendra Dahal, Shani Shastri, Uma Narayanasami, Vanesa Bijol, Karen Rider, James A. Strom and Bertrand L. Jaber
Page No. 318
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (318-322)
Recovery of kidney function following delayed use of theralite™ dialyzer in a patient with myeloma cast nephropathy
Khagendra Dahal1, Shani Shastri1, Uma Narayanasami2,3, Vanesa Bijol4, Karen Rider1, James A. Strom1,3 and Bertrand L. Jaber1,3
1Division of Nephrology, 2Division of Hematology/Oncology, Department of Medicine, St. Elizabeth’s Medical Center, 3Department of Medicine, Tufts University, School of Medicine, and 4Department of Pathology, Brigham and Women’s Hospital, Boston, MA, USA
We report the case of a 60- year- old man who presented with newly diagnosed multiple myeloma complicated by biopsy-proven acute cast nephropathy, requiring hemodialysis, plasmapheresis and chemotherapy. After remaining dialysis dependent for 5 weeks, a high cut-off (HCO) dialyzer, intended to use for the removal of plasma substances with a molecular weight of up to 45 kDa such as free light chains, was introduced to his outpatient 4-hour hemodialysis regimen with an increase in treatment frequency to 4 sessions per week. Following 6 weeks of dialysis with the HCO dialyzer, serum levels of free κ light chains declined by more than 75%. Concurrently, he recovered kidney function and discontinued dialysis. He subsequently received a successful autologous stem-cell transplant. We discuss the potential merit of using the HCO dialyzer late in the course of the care of patients with myeloma cast nephropathy who are dialysis dependent.Correspondence to:
Bertrand L. Jaber, MD, MS
St. Elizabeth’s Medical Center
736 Cambridge Street, Boston, MA 02135, USA
Email: [email protected]
Nephrology Education
Pseudo-blood leak? A hemodialysis mystery
Jose Avila, Deepali Prasad, Lawrence S. Weisberg and Richard Kasama
Page No. 323
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (323-325)
Pseudo-blood leak? A hemodialysis mystery
Jose Avila, Deepali Prasad, Lawrence S. Weisberg and Richard Kasama
Division of Nephrology, Department of Medicine, Cooper Medical School of Rowan University, Cooper University Hospital, Camden, NJ, USA
Hydroxocobalamin is a treatment for cyanide toxicity with few sideeffects. We report a case of a hemodialysis patient whose treatment was compromised by hydroxocobalamin interference with the blood leak detector.Correspondence to:
Jose Avila, MD, Cooper
Medical School of Rowan University
Cooper University Hospital
401 Haddon Avenue, Room 284, Camden, NJ 08103, USA
Email: [email protected]
Nephrology Education
Minimal change disease caused by exposure to mercury-containing skin lightening cream: a report of 4 cases
Hon-Lok Tang, Yuen-Fun Mak, Kwok-Hong Chu, William Lee, Samuel Ka‑Shun Fung, Thomas Yan-Keung Chan and Kwok-Lung Tong
Page No. 326
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (326-329)
Minimal change disease caused by exposure to mercury-containing skin lightening cream: a report of 4 cases
Hon-Lok Tang1, Yuen-Fun Mak2, Kwok-Hong Chu1, William Lee1, Samuel Ka‑Shun Fung1, Thomas Yan-Keung Chan3 and Kwok-Lung Tong1
1Division of Nephrology, Department of Medicine and Geriatrics, 2Department of Pathology, Princess Margaret Hospital, and 3Department of Medicine and Therapeutics, the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong, China
Mercury is a known cause of nephrotic syndrome and the underlying renal pathology in most of the reported cases was membranous nephropathy. We describe here 4 cases of minimal change disease following exposure to mercury-containing skin lightening cream for 2 – 6 months. The mercury content of the facial creams was very high (7,420 – 30,000 parts per million). All patients were female and presented with nephrotic syndrome and heavy proteinuria (8.35 – 20.69 g/d). The blood and urine mercury levels were 26 – 129 nmol/l and 316 – 2,521 nmol/d, respectively. Renal biopsy revealed minimal change disease (MCD) in all patients. The use of cosmetic cream was stopped and chelation therapy with D-penicillamine was given. Two patients were also given steroids. The time for blood mercury level to normalize was 1 – 7 months, whereas it took longer for urine mercury level to normalize (9 – 16 months). All patients had complete remission of proteinuria and the time to normalization of proteinuria was 1 – 9 months. Mercury-containing skin lightening cream is hazardous because skin absorption of mercury can cause minimal change disease. The public should be warned of the danger of using such products. In patients presenting with nephrotic syndrome, a detailed history should be taken, including the use of skin lightening cream. With regard to renal pathology, apart from membranous nephropathy, minimal change disease should be included as another pathological entity caused by mercury exposure or intoxication.Correspondence to:
Dr. Hon-Lok Tang
Division of Nephrology
Department of Medicine and Geriatrics
Princess Margaret Hospital
2-10 Princess Margaret Hospital Road
Lai Chi Kok, Kowloon, Hong Kong, China
Email: [email protected]
Nephrology Education
What lies beneath: Fabry nephropathy in a female patient with severe cerebrovascular disease
Elen A. Romão, Charles M. Lourenço, Wilson Marques Júnior, Arndt Rolfs, Veronica Muñoz, Osvaldo M. Vieira Neto, Marcio Dantas, Gyl E.B. Silva, Roberto S. Costa
Page No. 330
Abstract
Clinical Nephrology, Vol. 79 – No. 4/2013 (330-334)
What lies beneath: Fabry nephropathy in a female patient with severe cerebrovascular disease
Elen A. Romão1, Charles M. Lourenço2,3, Wilson Marques Júnior2,3, Arndt Rolfs4, Veronica Muñoz2, Osvaldo M. Vieira Neto1, Marcio Dantas1, Gyl E.B. Silva5 and Roberto S. Costa5
1Nephrology Division, 2Neurogenetics Division, 3Department of Neurology, Clinics Hospital of Ribeirao Preto, Faculty of Medicine of Ribeirao Preto, University of São Paulo, Brazil and 4Department of Neurology, Rostock University, Germany and 5Department of Pathology, Faculty of Medicine of Ribeirao Preto, University of São Paulo, Brazil
Fabry disease is an X-linked inborn error of metabolism, which is caused by the deficiency of α-galactosidase A, leading to progressive accumulation of neutral glycosphingolipids and a-galactosyl breakdown products in most body fluids and several tissues, resulting in the clinical manifestations. The onset of Fabry disease symptoms in females is not observed as early as in males. We report a novel presentation of Fabry disease in a female patient with medical history of relapsing strokes and brain magnetic resonance angiography showing signs of microangiopathy and multiple lacunar strokes that were first diagnosed as Moyamoya disease (a chronic progressive cerebrovascular disease). The patient subsequently displayed increased levels of serum creatinine and proteinuria. Diagnosis of Fabry disease was made by a renal biopsy and was confirmed by molecular studies showing a missense mutation: c1066C > T (het) [R356W]. The diagnosis was delayed by 21 years with respect to her first symptom (stroke), probably because her initial clinical presentation was neurological and diagnosed as Moyamoya disease. Other factors that contributed to the delay of the diagnosis were the lack of acute or chronic pain (neuropathic pain) and angiokeratomas. Some similarities in the pathogenic aspects of the patient’s vascular lesions lead us to speculate that this patient has Fabry disease, with a phenotype that had not yet been described. It is necessary to be aware of this possibility to avoid misdiagnosis of Fabry disease as Moyamoya disease.Correspondence to:
Elen A. Romao, MD
Division of Nephrology
Faculty of Medicine of Ribeirão Preto – USP.
Av. Bandeirantes, 3900, 14048-900. Ribeirão Preto, SP, Brazil
Email: [email protected]
