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Original article at: http://www.nature.com/ki/journal/v76/n3/abs/ki2009136a.html Clinicopathological characteristics and outcomes of patients with crescentic lupus nephritis Feng Yu 1,2,3, Ying Tan 1,2,3,
Gang Liu 1,2,3, Su-xia Wang 1,2,3,
Wan-zhong Zou 1,2,3
and Ming-hui Zhao 1,2,3 1 Renal Division,
Department of Medicine, Peking University First Hospital, Beijing, PR China;
2 Institute of Nephrology, Peking University,Beijing,
PR China; 3 Key Laboratory of Renal Disease, Ministry of Health of China,
Beijing, PR China There are few clinicopathologic and outcome data on patients with crescentic lupus nephritis, therefore, we determined
factors of the disease by retrospectively reviewing the records of 327
patients diagnosed with lupus nephritis. Of these, 152 cases were regrouped
as class IV-G, including 33 patients with crescentic
glomerulonephritis. Significantly, all patients
with crescentic glomerulonephritis
had acute kidney injury as compared with only about a quarter of the patients
without the disease. On pathological evaluation, activity scores, chronicity indexes, relapse rates, and the frequency of
positive serum anti-neutrophil cytoplasmic
antibody (ANCA) were each significantly higher, whereas complete remission
rates and renal outcomes, over a mean follow-up of 4 years, were
significantly poorer in patients with crescentic glomerulonephritis. Our study shows that crescentic glomerulonephritis
was not rare in patients with lupus
nephritis and that their long-term outcome was poor. The precise role of ANCA
in the pathologic course of crescentic lupus
nephritis remains to be determined. Kidney
International (2009) 76, 307–317; doi:10.1038/ki.2009.136. KEYWORDS: anti-neutrophil cytoplasmic
antibodies; crescentic glomerulonephritis;
lupus nephritis Correspondence: Ming-hui Zhao,
Renal Division, Department of Medicine, Key Laboratory of
Renal Disease, Ministry of Health of PR China. E-mail:
mhzhao@bjmu.edu.cn |
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Original article at: http://www.nature.com/ki/journal/v76/n5/abs/ki2009243a.html The A Working Group
of the International IgA Nephropathy Network and
the Renal Pathology Society: Daniel C. Cattran 1,w, Rosanna Coppo
2,w, H. Terence Cook 3,w, John Feehally
4,w, Ian S.D. Roberts 5,w, Ste´phan
Troyanov 6,w, Charles E. Alpers 7, Alessandro Amore 2,
Jonathan Barratt 4, Francois Berthoux 8,Stephen
Bonsib 9, Jan A. Bruijn
10, Vivette D’Agati
11, Giuseppe D’Amico 12, Steven Emancipator 13,
Francesco Emma 14, Franco Ferrario 15, Fernando C. Fervenza 16, Sandrine Florquin
17, Agnes Fogo 18, Colin C.
Geddes 1 9, Hermann-Josef Groene 20,
Mark Haas 21, Andrew M. Herzenberg 22,
Prue A. Hill 23, Ronald J. Hogg 24,
Stephen I. Hsu 25, J. Charles Jennette 26,
Kensuke Joh 27, Bruce A. Julian 28,Tetsuya
Kawamura 29, Fernand M. Lai 30,
Chi Bon Leung 31, Lei-Shi Li 32, Philip K.T. Li 31,
Zhi-Hong Liu 32,Bruce Mackinnon 19,
Sergio Mezzano 33, F. Paolo Schena 34, Yasuhiko Tomino
35, Patrick D. Walker 36,Haiyan Wang 37, Jan
J. Weening 38, Nori
Yoshikawa 39 and Hong Zhang 37 IgA nephropathy is
the most common glomerular disease worldwide, yet there is no international
consensus for its pathological or clinical classification. Here a new
classification for IgA nephropathy is presented by
an international consensus working group. The goal of this new system was to
identify specific pathological features that more accurately predict risk of
progression of renal disease in IgA nephropathy,
thus enabling both clinicians and pathologists to improve individual patient prognostication.
In a retrospective analysis, sequential clinical data were obtained on 265
adults and children with IgA nephropathy who were followed
for a median of 5 years. Renal biopsies from all patients were scored by
pathologists blinded to the clinical data for pathological variables
identified as reproducible by an iterative process. Four of these variables:
(1) the mesangial hypercellularity
score, (2) segmental glomerulosclerosis, (3) endocapillary hypercellularity,
and (4) tubular atrophy/interstitial fibrosis were subsequently shown to have
independent value in predicting renal outcome. These specific pathological
features withstood rigorous statistical analysis even after taking into
account all clinical indicators available at the time of biopsy as well as
during follow-up. The features have prognostic significance and we
recommended they be taken into account for predicting outcome independent of
the clinical features both at the time of presentation and during follow-up.
The value of crescents was not addressed due to their low
prevalence in the enrolled cohort. Kidney
International (2009) 76, 534–545; doi:10.1038/ki.2009.243 KEYWORDS: glomerulonephritis; IgA
nephropathy; pathology; renal
failure Correspondence:
John
Feehally, The John Walls Renal Unit, Leicester W These authors
contributed equally to the work and are named in alphabetical order. |
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Original article at: http://www.nature.com/ki/journal/v76/n7/abs/ki2008666a.html Light chain proximal tubulopathy Leal C. Herlitz 1, Joseph Roglieri
2, 1 Department of
Pathology, Columbia University, College of Physicians and Surgeons, New York,
NY, USA; 2 Northeast Nephrology Associates, Troy, NY, USA and 3 New York
Oncology Hematology P.C., Troy, NY, USA CASE
PRESENTATION A 55-year-old
Caucasian woman presented to her primary care physician with complaints of
progressive fatigue for several months, dyspnea
with minimal exertion, loss of appetite, 15-pound weight loss, and recurrent
low-grade fevers. Past medical history was significant only for migraine
headaches. The patient was taking no prescription or over-the-counter
medications. There was no history of environmental toxin exposure, recent
travel, smoking, excess alcohol consumption, or use of illicit drugs. She was
empirically treated with a course of esomeprazole
and amoxicillin. The patient returned
2 weeks later and reported no improvement in her symptoms. Laboratory studies
revealed anemia, thrombocytopenia, hypercalcemia,
and acute renal failure. The patient was admitted for further evaluation. Upon admission,
physical examination revealed a well nourished but pale female in no acute
distress. Her blood pressure was 156/70mmHg, pulse 96 bpm,
temperature 98.6 F, respiratory rate 20 breaths/min, and pulse oximetry 100% on room air. Cardiac and pulmonary
examinations were unremarkable. Abdominal examination revealed splenomegaly with a palpable liver edge 1–2cm below the right
costal margin. Laboratory testing (Table 1) was notable for a hemoglobin of
6.1 g/dl (normal range, 11.0–15.0 g/dl), platelet count 112 K/mm3 (nl 150–400 K/ mm3), creatinine
3.7mg/dl, BUN 34mg/dl, and calcium12.4mg/dl (nl
8.5–10.1mg/dl). Liver function tests, coagulation studies, and parathyroid
hormone levels were normal. Urinalysis revealed 1ž protein, 1ž glucose, and trace
blood. Negative serologies included anti-glomerular
basement membrane antibody, proteinase-3 antineutrophil
cytoplasmic antibodies, and myeloperoxidase
anti-neutrophil cytoplasmic
antibodies. Serum complement levels were mildly elevated with C3 179.0mg/dl (nl 75–135mg/dl) and C4 41.9mg/dl (nl
9–36mg/dl). Serum protein electrophoresis with immunofixation
showed no monoclonal protein. A bone marrow biopsy revealed normocellular marrow with no evidence of lymphoma or a
plasma cell dyscrasia. A noncontrast
computed tomography scan of the chest, abdomen, and pelvis was notable for splenomegaly (18x15x10cm) with a hypodense
area measuring 7x7x4 cm and mild hepatomegaly. The
kidneys measured 11.7 and 12 cm in length by ultrasound, without evidence of
obstruction. A skeletal survey showed no abnormalities. The patient was treated
with hydration and a single dose of pamidronate. At
the time of discharge 6 days later, her creatinine
had fallen to 1.9mg/dl, and her calcium had normalized. The patient was seen in nephrologic consultation 1 week after discharge, at which
time her creatinine had declined to 1.5mg/dl.
Computed tomography scan was repeated with contrast and revealed a 19x16x10cm
spleen with an ill-defined 11x11x13cm mass with areas of probable necrosis.
In light of the absence of a clear indication for splenectomy, a renal biopsy was performed to determine
the cause of the patient’s persistent renal
dysfunction. KEYWORDS: kappa light
chain; light chain Fanconi syndrome; lymphoma Kidney
International (2009) 76, 792–797; doi:10.1038/ki.2008.666 Correspondence: Glen S.
Markowitz, Department of Pathology, University, |
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Original
article at: http://ndt.oxfordjournals.org/cgi/content/full/24/11/3265 Kidney injury molecule-1 (KIM-1):
a urinary biomarker and much more Joseph V. Bonventre Renal Division, Department of
Medicine, Brigham and Women’s Hospital, The kidney injury molecule-1
(designated as Kim-1 in rodents, KIM-1 in humans) mRNA was
identified using techniques of representational difference
analysis, a PCR-based technique [1], which we carried out to find
genes whose expression was markedly upregulated
24–48 h after ischaemia in the rat [2]. Kim-1
was the gene found to be most highly upregulated
in this screen. A large pharmaceutical company consortium, using
an unbiased genomic approach to evaluate genes upregulated
with the nephrotoxin cisplatin,
determined that Kim-1 was upregulated more
than any other of the 30 000 genes tested [3]. There are a
large number of studies in animals showing robust Kim-1 protein
production in the affected segments of the proximal tubule
whenever a toxin or pathophysiological
state results in dedifferentiation of the epithelium (e.g. [4–6]).
Dedifferentiation is a very early manifestation of the epithelial
cell response to injury Keywords: acute kidney injury; TIM-1; acute
renal failure; phagocytosis; apoptosis Nephrol Dial Transplant
(2009) 24: 3265–3268, doi: 10.1093/ndt/gfp010, Advance Access publication 23
March 2009 Correspondence :Joseph V. Bonventre; E-mail: joseph_bonventre@hms.harvard.edu |