scholarly journals Corticosteroid-responsive nephrotic syndrome in children with myelodysplastic syndromes

2002 ◽  
Vol 130 (9-10) ◽  
pp. 323-328 ◽  
Author(s):  
Radovan Bogdanovic ◽  
Milos Kuzmanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Milos Ognjanovic ◽  
Dragan Micic ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Myelodysplastic Syndromes ◽  
Rapidly Progressive Glomerulonephritis ◽  
Corticosteroid Treatment ◽  
Refractory Anemia ◽  
Al Amyloidosis ◽  
Glomerular Diseases ◽  
Medline Search ◽  
Haematological Remission ◽  
Repeated Infections

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated with steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented also hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient haematological improvement occurred. Relapse subsequently occurred that was manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease but haematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, two years after the onset of MDS. Patient 2, who had refractory anemia with clonal monosomy 19, manifested bowel disease, hepatospleno- megaly, anaemia and non-organic specific autoantibodies. Prednisone led to both clinical and haematological remission. Haematologic disease relapsed 12 months later, when nephrotic-range proteinuria, haematuria and mild azotaemia were also found. Corticosteroid treatment led to long-lasting renal and haematologic remission, maintained by a small dosage of prednisone. In both patients renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with either acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis and AL amyloidosis, were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS; (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.

Approach to the Patient with Glomerular Disease

2018 ◽  
Author(s):  
Richard J. Glassock ◽  
An S De Vriese ◽  
Fernando C. Fervenza
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Rapidly Progressive Glomerulonephritis ◽  
Chronic Glomerulonephritis ◽  
Glomerular Diseases ◽  
Diagnosis And Prognosis ◽  
Protein Excretion ◽  
Clinical Syndromes ◽  
Organ Systems ◽  
Therapeutic Decision Making

Glomerular diseases of the kidneys are associated with a limited array of clinical syndromes, including asymptomatic hematuria and/or proteinuria, acute nephritis, nephrotic syndrome, rapidly progressive glomerulonephritis, and chronic glomerulonephritis. The specific diseases that underlie these syndromes are numerous and heterogeneous. Broadly, they may be divided into primary and secondary disorders depending on whether the kidneys are the sole organs affected or whether other organ systems are also involved in the disease processes. A systematic approach involving a careful history, physical examination, assessment of renal function, and urinalysis (composition and microscopy) and protein excretion, combined with biochemical and serologic testing, can provide important clues to diagnosis and prognosis. Renal biopsy is often required for a complete and accurate diagnosis as well as a prognosis and therapeutic decision making. This review contains 4 figures, 6 tables and 92 references Key words: glomerular filtration rate, glomerulonephritis, hematuria, nephrotic syndrome, proteinuria, renal biopsy, serum complement

Approach to the Patient with Glomerular Disease

2018 ◽  
Author(s):  
Richard J. Glassock ◽  
An S De Vriese ◽  
Fernando C. Fervenza
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Rapidly Progressive Glomerulonephritis ◽  
Chronic Glomerulonephritis ◽  
Glomerular Diseases ◽  
Diagnosis And Prognosis ◽  
Protein Excretion ◽  
Clinical Syndromes ◽  
Organ Systems ◽  
Therapeutic Decision Making

Glomerular diseases of the kidneys are associated with a limited array of clinical syndromes, including asymptomatic hematuria and/or proteinuria, acute nephritis, nephrotic syndrome, rapidly progressive glomerulonephritis, and chronic glomerulonephritis. The specific diseases that underlie these syndromes are numerous and heterogeneous. Broadly, they may be divided into primary and secondary disorders depending on whether the kidneys are the sole organs affected or whether other organ systems are also involved in the disease processes. A systematic approach involving a careful history, physical examination, assessment of renal function, and urinalysis (composition and microscopy) and protein excretion, combined with biochemical and serologic testing, can provide important clues to diagnosis and prognosis. Renal biopsy is often required for a complete and accurate diagnosis as well as a prognosis and therapeutic decision making. This review contains 4 figures, 6 tables and 92 references Key words: glomerular filtration rate, glomerulonephritis, hematuria, nephrotic syndrome, proteinuria, renal biopsy, serum complement

AL Amyloidosis-Associated Hypothyroidism: Prevalence and Relationship to Nephrotic Syndrome

2004 ◽  
pp. 93-95
Author(s):  
J.S. Bhatia ◽  
V. Sanchorawala ◽  
D.C. Seldin ◽  
L.F. Casserly ◽  
J.L. Berk ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Al Amyloidosis

scholarly journals New Aspects of Pathogenesis and Treatment of Membranous Glomerulopathy After the MENTOR Study

2020 ◽  
pp. 46-53
Author(s):  
Maurizio Salvadori ◽  
Aris Tsalouchos
Keyword(s):  
Nephrotic Syndrome ◽  
Adrenocorticotropic Hormone ◽  
Glomerular Diseases ◽  
The Past ◽  
Superior Efficacy ◽  
Novel Drugs ◽  
Membranous Glomerulopathy ◽  
Blocking Mechanisms ◽  
Antigenic Epitopes

Membranous nephropathy (MN) is the major cause of nephrotic syndrome in adults, accounting for 20% of cases with an annual incidence of 1 per 100,000 population. In the past 10 years, the role of podocytes has been identified. Environmental triggers in genetically predisposed patients can activate podocytes to exhibit antigenic epitopes, including PLA2R, THBS1, and NELL1, which become targets of specific autoantibodies with subsequent complement activation. The discovery of these mechanisms has opened a new horizon in the treatment of MN, and novel drugs are available with more specific mechanisms of action. Rituximab, a monoclonal antibody directed against CD20 expressed on B lymphocytes, has been used in several trials and appears to induce remission of nephrotic syndrome in 60% of patients (GEMRITUX trial). The recently published results of the MENTOR trial documented the superior efficacy of rituximab in patients observed for up to 24 months. In MN, the concept of targeting disease control has introduced novel therapies with specific blocking mechanisms, such as belimumab; nonspecific blocking mechanisms, such as those against adrenocorticotropic hormone; and new therapeutic options, such as ofatumumab, bortezomib, and eculizumab, which have recognised the pathological processes involved in the glomerular diseases.

scholarly journals ASSOTIATIONS OF PECULIARITIES OF HLA-PHENOTYPE AND THE SENSIBILITY TO THE CORTICOSTEROID TREATMENT IN PATIENTS WITH CHRONIC GLOMERULONEPHRITIS WITH NEPHROTIC SYNDROME

2017 ◽  
pp. 37-44
Author(s):  
M. Kolesnyk ◽  
G. Drannik ◽  
V. Driyanska ◽  
O. Petrina ◽  
M. Velychko
Keyword(s):  
Nephrotic Syndrome ◽  
Hla Antigens ◽  
Corticosteroid Treatment ◽  
Steroid Resistance ◽  
Control Group ◽  
Chronic Glomerulonephritis ◽  
Healthy Donors ◽  
Steroid Sensitive ◽  
Steroid Sensitivity ◽  
Hla Phenotype

The purpose of study was determination of HLA -antigens I and II classes as predictors of ineffectiveness of initial steroid therapy, and according prognozonegative markers of chronic glomerulonephritis with nephrotic syndrome. Methods. In 59 chronic glomerulonephritis with nephrotic syndrome patients (steroid sensitive n=33 (1 gr.) and steroid resistant’s n= 26 (2 gr.)) and 350 healthy donors( control group) studied HLA antigens I and II classes of the special anti- HLA-antigens panel (20 antigens of locus A, 31 – of locus B and 9- of locus DR). Result. In patients with chronic glomerulonephritis, nephrotic syndrome with hormone sensitivity relative risk is high at the presents of A28 (RR=8,5, r р <0,001), it made attributive risk (=0,37). In comparison with a control group, RR>2 for antigens  A11  (RR=2,23), A23 (RR=4,28),  A24 (RR=3,3),  A29 (RR=10,78) that A30 (RR=11,23); attributive risk more than 0,1 for the antigen A11 (=0,16) ; A24 (=0,13), other did not differ from control. Subzero connection is exposed for the antigens of A2 (р<0,001), А9 (р=0,007). In locus antigen B14 (RR=5,65, р =0,001) are exposed, B44 (RR=48,25, р =0,004), B51(RR=12,32, р =0,006) and attributive risk of development of disease (according =0,24, 0,12 ; 0,14); and antigens B38 and B41 (RR=11,57, р=0,05). The steroid sensitivity was associated with the antigens B5 (p=0,033), B12 (p=0,005) and B35 (p=0,021). In locus DR made etiologic faction antigens DR4 (RR=7,0 and =0,24) DRw52 (RR=7,0 and =0,25). Conclusions. For patients with chronic glomerulonephritis with a nephrotic syndrome antigens of HLA-B14,B38, B51, DRw52 are associated with steroid sensitivity. The attributive risk of steroid resistance is high for split A19+31+32, antigens B8, B55.

scholarly journals Treatment of myelodysplastic syndromes with all-trans retinoic acid. Leukemia Study Group of the Ministry of Health and Welfare

Blood ◽  
1993 ◽  
Vol 81 (5) ◽  
pp. 1152-1154
Author(s):  
R Ohno ◽  
T Naoe ◽  
M Hirano ◽  
M Kobayashi ◽  
H Hirai ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Retinoic Acid ◽  
Myelodysplastic Syndromes ◽  
Liver Function Tests ◽  
High Serum ◽  
Refractory Anemia ◽  
Prior Therapy ◽  
Trans Retinoic Acid ◽  
All Trans Retinoic Acid ◽  
Advanced Stages

We treated 23 patients with myelodysplastic syndromes (MDS); 2 refractory anemia (RA) with prior therapy, 11 RA with excess of blasts (RAEB), and 10 RAEB in transformation (RAEB-T), with daily oral 45 mg/m2 all-trans retinoic acid (ATRA) in a multiinstitutional prospective study. In two patients with RAEB and one with RAEB-T, a more than 1,000/microL increase of peripheral neutrophil counts was observed with some reduction of blast percentage in the bone marrow 2 to 9 weeks after the start of ATRA. However, the effect was transient and did not last for more than 5 weeks despite the continuation of ATRA therapy. In one other patient with RA, one patient with RAEB, and one patient with RAEB-T, slight increase of hemoglobin levels or reduction of blast percentage in bone marrow was noted. Toxicities attributable to ATRA were minimal and included cheilitis, xerosis, dermatitis, gastrointestinal disorders, abnormal liver function tests, and high serum triglyceridemia. Although ATRA works remarkably as a differentiation therapy in acute promyelocytic leukemia, its effect in MDS included in this study was modest. Further study of this agent alone or in combination may be warranted in less advanced stages of this disease.

scholarly journals DNA instability in low-risk myelodysplastic syndromes: refractory anemia with or without ring sideroblasts

2008 ◽  
Vol 17 (14) ◽  
pp. 2144-2149 ◽  
Author(s):  
B. Novotna ◽  
R. Neuwirtova ◽  
M. Siskova ◽  
Y. Bagryantseva
Keyword(s):  
Myelodysplastic Syndromes ◽  
Low Risk ◽  
Refractory Anemia ◽  
Dna Instability ◽  
Ring Sideroblasts

Renal medicine

2021 ◽  
pp. 353-382
Author(s):  
Gopesh K. Modi ◽  
Vivekanand Jha
Keyword(s):  
Kidney Disease ◽  
Renal Disease ◽  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Renal Stones ◽  
Renal Diseases ◽  
Acute Glomerulonephritis ◽  
Glomerular Diseases ◽  
Stage Renal Disease ◽  
End Stage

Assessing renal function, Urinalysis, Proteinuria, Hematuria, Chyluria, Imaging in renal disease, Kidney biopsy, Acute Kidney Injury (AKI), Chronic Kidney Disease (CKD), Diabetic Nephropathy, End Stage Renal Disease and Dialysis, Kidney Transplantation, Glomerular diseases, Acute glomerulonephritis, Urinary schistosomiasis (bilharzia), Infections and Kidney Disease, Rapidly Progressive glomerulonephritis, Tubulointerstitial Disease, Urinary Tract Infection, Vesico-ureteric reflux, Renal Stones, Renal Disease in Pregnancy, Renal Artery Stenosis, Renal Mass, Inherited Renal Diseases

scholarly journals Glomerular Diseases Associated with Malignancies: Histopathological Pattern and Association with Circulating Autoantibodies

Antibodies ◽  
2020 ◽  
Vol 9 (2) ◽  
pp. 18
Author(s):  
Sophia Lionaki ◽  
Smaragdi Marinaki ◽  
Konstantinos Panagiotellis ◽  
Ioanna Tsoumbou ◽  
George Liapis ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Kidney Biopsy ◽  
Rapidly Progressive Glomerulonephritis ◽  
Glomerular Diseases ◽  
Transmembrane Glycoprotein ◽  
Phospholipase A2 Receptor ◽  
Nephritic Syndrome ◽  
Acute Nephritic Syndrome ◽  
Circulating Autoantibodies ◽  
End Stage

Aim: Glomerular diseases (GD) associated with malignancies (AM, GDAM) have unique features, which are important to recognize, in the light of the progress made in cancer therapy. We aimed to describe the clinical and histopathological characteristics of patients with GDAM in relation to the presence of circulating autoantibodies, pointing to potential immune pathogenic pathways connecting cancer to GD. Materials and Methods: The included patients were studied retrospectively on the basis of a kidney biopsy proving GD and a related biopsy to establish the diagnosis of AM. We recorded patients’ demographics, serological and laboratory parameters, histopathological findings, and the type of malignancy, GD, and therapy. Results: In total, 41 patients with GDAM, with a mean age of 63.1 (±10.7) years, were studied. In 28 (68.3%) cases, GD was associated with a solid tumor, and in 13 (31.7%) patients with a lymphoid malignancy. The most frequent histopathological pattern was membranous nephropathy (43.9%). Overall, at the time of GD diagnosis, 17% of the patients were positive for antinuclear antibodies (ANA), and 12.2% for antineutrophil cytoplasmic autoantibodies (ANCA), all against myeloperoxidase (MPO). In addition, 93.3% of the patients who had membranous nephropathy were negative for transmembrane glycoprotein M-type phospholipase A2 receptor (PLA2R) antibody. Sixteen patients (39.0%) presented with acute nephritic syndrome, of whom five (31.25%) developed rapidly progressive glomerulonephritis. In a mean follow-up time of 36.1 (±28.3) months, nine (21.95%) patients ended up with end-stage kidney disease, and eight (19.5%) died. Conclusion: We found that 3.2% of patients who underwent a native kidney biopsy in our institution during the past decade, for any reason, were identified as having some type of GD associated with a malignancy. Serology indicated a significant presence of ANA or MPO-ANCA antibodies in patients with nephritic syndrome and the absence of PLA2R antibodies in patients with membranous nephropathy.

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