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Saudi Journal of Kidney Diseases and... 2019Despite the high number of Arab children, there are a scanty available data about different types of pediatric glomerular diseases in all Arab countries. Very few Arab...
Despite the high number of Arab children, there are a scanty available data about different types of pediatric glomerular diseases in all Arab countries. Very few Arab countries have a national renal biopsy registry. In this review, we performed a systematic review analyzing the incidence of pediatric glomerular disease in all Arab countries. Relevant manuscripts in all 22 Arab countries found through searches of Medline, Science Direct, Embase and Google Scholar were evaluated. The period was from January 1990 to March 2018. A total of 17 manuscripts containing 3083 renal biopsies from seven countries were analyzed. Male-to-female ratio was 1.3:1. Saudi Arabia revealed the most published studies with seven papers. The average period of the study was 8.63 years. Retrospective studies represent 94.12%. Minimal change disease (MCD) (29.25%), focal and segmental glomerulosclerosis (FSGS) (22.34%), mesangioproliferative glomerulonephritis (14.78%), membranoproliferative glomerulonephritis (6.9%), IgA nephropathy (3.98%), and membranous glomerulopathy (2.65%) were the top types of primary glomerular diseases. The most common types of secondary glomerular diseases were lupus nephritis (36.1%), postinfectious glomerulonephritis (17.62%), congenital nephrotic syndrome (6.08%), Alport syndrome (4.71%), Henoch-Schönlein purpura (1.49%), and amyloidosis (1.36%). In conclusion, MCD and lupus nephritis are, respectively, the most common types of primary and secondary glomerular diseases in children of all evaluated Arab countries. FSGS is the predominant pattern of primary glomerular diseases in Asian Arab countries. The trend of all types of glomerular diseases has not changed in the past 28 years except a noted significant reduction in FSGS. Arab countries are strongly recommended to establish a renal biopsy registry.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Incidence; Infant; Infant, Newborn; Male; Middle East; Nephritis; Nephrosis
PubMed: 30804262
DOI: No ID Found -
Hellenic Journal of Cardiology : HJC =... 2019Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant cardiac involvement. In this study, we compared prognosis in patients with different categories of cardiac amyloidosis using meta-analysis and present a systematic review.
METHODS
A systematic literature search was performed through Jan 1, 2018, and two reviewers independently extracted data and assessed risk of bias. We extracted MACE and death endpoint events and hazard ratios from regression models and performed a meta-analysis of the multiple prognosis association studies.
RESULTS
We observed that there were significant MACE differences between patients diagnosed with transthyretin amyloidosis and light-chain amyloidosis (OR: 2.09; 95% CI: 1.06-4.12; P = 0.03), and the same is true in the sub-comparison between AL and mATTR or wtATTR (AL vs. mATTR: OR: 1.72; 95% CI: 1.06-2.82; P = 0.03; AL vs. wtATTR: OR: 1.48; 95% CI: 0.85-2.58; P = 0.17). However, no significant difference was observed between two transthyretin types (P = 0.17). Overall death rate evaluated showed that compared with transthyretin-related amyloidosis, light-chain type showed a significant difference (P < 0.05). The prognostic analysis showed that types of amyloidosis, LVEF, NYHA, restrictive filling pattern, E-wave deceleration time, E/E' ratio, and low QRS voltage were predictors of cardiac-related mortality.
CONCLUSION
Patients diagnosed with light-chain amyloidosis has a poor prognosis compared with transthyretin-related amyloidosis, while no difference was proved in prognostic analysis between wild-type and mutated TTR amyloidosis. Some clinical factors related to the death prognosis, such as the LVEF, restrictive filling pattern, E-wave deceleration time, and E/E' ratio are important prognostic factors.
Topics: Adult; Aged; Aged, 80 and over; Amyloid Neuropathies, Familial; Cardiomyopathies; Case-Control Studies; Female; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Prealbumin; Prognosis; Ventricular Function, Left
PubMed: 30742933
DOI: 10.1016/j.hjc.2019.01.015 -
Saudi Journal of Kidney Diseases and... 2018According to the best of our knowledge, there is no review compiling incidence of glomerular disease in all Arab countries. Most of the Arab countries do not have a... (Review)
Review
According to the best of our knowledge, there is no review compiling incidence of glomerular disease in all Arab countries. Most of the Arab countries do not have a national renal biopsy registry. In addition, there is scanty data available on the epidemiology of glomerular diseases in Arab countries. In this review, we performed a systematic review analyzing the incidence of glomerular disease in all Arab countries. Relevant manuscripts in all 22 Arab countries found through searches of Medline, Science Direct, Embase, and Google Scholar were evaluated. The time was from January 1990 to March 2018. A total of 36 manuscripts containing 10,727 biopsies from 11 countries were analyzed. The male-to-female ratio was 1.2:1. Saudi Arabia had the largest number of published studies with 14 papers followed equally by Iraq, Jordan, and Sudan with three papers each. The average period of study was 8.17 years. Retrospective studies represented 86.11%. Focal and segmental glomerulosclerosis (FSGS) (27%), minimal change disease (14%), membranoproliferative glomerulonephritis (13%), mesangioproliferative glomerulonephritis (13%), and membranous glomerulopathy (11%) were the main types of primary glomerular diseases. The most common types of secondary glomerular diseases were lupus nephritis (LN) (58%), amyloidosis (10.19%), diabetic nephropathy (9.89%), hypertension (4.84%) and poststreptococcal glomerulonephritis (2.72%). In conclusion, FSGS and LN are the most common types of primary and secondary glomerular diseases, respectively, in all evaluated Arab countries. The trend of all types of glomerular diseases has not changed in the last three decades. We strongly recommend that each Arab country should have its own renal biopsy registry.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Arabs; Biopsy; Child; Child, Preschool; Female; Humans; Incidence; Infant; Infant, Newborn; Kidney Diseases; Kidney Glomerulus; Male; Middle Aged; Middle East; Time Factors; Young Adult
PubMed: 30588955
DOI: 10.4103/1319-2442.248285 -
BMC Cardiovascular Disorders Dec 2018Cardiac Amyloidosis (CA) pertains to the cardiac involvement of a group of diseases, in which misfolded proteins deposit in tissues and cause progressive organ damage....
BACKGROUND
Cardiac Amyloidosis (CA) pertains to the cardiac involvement of a group of diseases, in which misfolded proteins deposit in tissues and cause progressive organ damage. The vast majority of CA cases are caused by light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). The increased awareness of these diseases has led to an increment of newly diagnosed cases each year.
METHODS
We performed multiple searches on MEDLINE, EMBASE and the Cochrane Database of Systematic Reviews. Several search terms were used, such as "cardiac amyloidosis", "diagnostic modalities cardiac amyloidosis" and "staging cardiac amyloidosis". Emphasis was given on original articles describing novel diagnostic and staging approaches to the disease.
RESULTS
Imaging techniques are indispensable to diagnosing CA. Novel ultrasonographic techniques boast high sensitivity and specificity for the disease. Nuclear imaging has repeatedly proved its worth in the diagnostic procedure, with efforts now focusing on standardization and quantification of amyloid load. Because the latter would be invaluable for any staging system, those spearheading research in magnetic resonance imaging of the disease are also trying to come up with accurate tools to quantify amyloid burden. Staging tools are currently being developed and validated for ATTR CA, in the spirit of the acclaimed Mayo Staging System for AL.
CONCLUSION
Cardiac involvement confers significant morbidity and mortality in all types of amyloidosis. Great effort is made to reduce the time to diagnosis, as treatment in the initial stages of the disease is tied to better prognosis. The results of these efforts are highly sensitive and specific diagnostic modalities that are also reasonably cost effective.
Topics: Amyloid Neuropathies, Familial; Biomarkers; Cardiomyopathies; Echocardiography; Humans; Immunoglobulin Light-chain Amyloidosis; Magnetic Resonance Imaging; Predictive Value of Tests; Prognosis; Reproducibility of Results; Severity of Illness Index; Tomography, Emission-Computed
PubMed: 30509186
DOI: 10.1186/s12872-018-0952-8 -
Hematology/oncology and Stem Cell... Jun 2019Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The...
OBJECTIVE/BACKGROUND
Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.
METHODS
An electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.
RESULTS
We identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53-66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.
CONCLUSIONS
We reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.
Topics: Adult; Female; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Multiple Myeloma; Neoplasm Proteins; Sex Factors; Skin Neoplasms
PubMed: 30261180
DOI: 10.1016/j.hemonc.2018.09.003 -
American Journal of Clinical Dermatology Oct 2017Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement.... (Review)
Review
BACKGROUND
Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. Lichen amyloidosis, macular amyloidosis, and (primary localized cutaneous) nodular amyloidosis are different subtypes of PLCA.
OBJECTIVE
The aim of this study was to review the current reported treatment options for PLCA.
METHODS
This systematic review was based on a search in the PubMed database for English and German articles from 1985 to 2016.
RESULTS
Reports on the treatment of PLCA were limited predominantly to case reports or small case series. There were a few clinical trials but these lacked control groups. A variety of treatment options for PLCA were reported including retinoids, corticosteroids, cyclophosphamide, cyclosporine, amitriptyline, colchicine, cepharanthin, tacrolimus, dimethyl sulfoxide, vitamin D analogs, capsaicin, menthol, hydrocolloid dressings, surgical modalities, laser treatment, and phototherapy.
CONCLUSION
No definitive recommendation of preferable treatment procedures can be made based on the analyzed literature. Randomized controlled trials are needed to offer patients an evidence-based therapy with high-quality standardized treatment regimens for PLCA.
Topics: Amyloidosis, Familial; Bandages, Hydrocolloid; Dermatologic Agents; Dermatologic Surgical Procedures; Europe; Humans; Laser Therapy; Phototherapy; Practice Guidelines as Topic; Skin; Skin Diseases, Genetic
PubMed: 28342017
DOI: 10.1007/s40257-017-0278-9 -
Current Medical Research and Opinion Jun 2017A systematic literature review on systemic light chain (AL) amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and... (Review)
Review
INTRODUCTION
A systematic literature review on systemic light chain (AL) amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps.
METHODS
MEDLINE, Embase and Cochrane databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic and PRO categories and additional searches were conducted in clinical conference proceedings, and using Google and Google Scholar. After review, there were 65 articles included for data extraction.
RESULTS
AL amyloidosis is a rare disorder without any FDA or EMA approved indications for drug therapy. Using off-label therapies, there is a high rate, 42-64%, of non-response or progression, and an associated high mortality. Toxicities during therapy are common with estimates of up to 30-40% of patients experiencing severity of grade 3 or higher. Patients with AL amyloidosis report severe psychological distress, anxiety and clinical depression.
CONCLUSIONS
There is a deficiency in the literature on the economic costs associated with AL amyloidosis, and information on costs has been derived from studies that examined multiple myeloma or other disease or treatment components common to AL amyloidosis.
Topics: Amyloidosis; Cost of Illness; Costs and Cost Analysis; Humans; Immunoglobulin Light-chain Amyloidosis; Multiple Myeloma
PubMed: 28277869
DOI: 10.1080/03007995.2017.1297930 -
Rheumatology International Mar 2015The objective of the study was to develop evidence-based and practical recommendations for the detection and management of comorbidity in patients with rheumatoid... (Review)
Review
The objective of the study was to develop evidence-based and practical recommendations for the detection and management of comorbidity in patients with rheumatoid arthritis (RA) in daily practice. We used a modified RAND/UCLA methodology and systematic review (SR). The process map and specific recommendations, based on the SR, were established in discussion groups. A two round Delphi survey permitted (1) to prioritize the recommendations, (2) to refine them, and (3) to evaluate their agreement by a large group of users. The recommendations cover: (1) which comorbidities should be investigated in clinical practice at the first and following visits (including treatments, risk factors and patient's features that might interfere with RA management); (2) how and when should comorbidities and risk factors be investigated; (3) how to manage specific comorbidities, related or non-related to RA, including major adverse events of RA treatment, and to promote health (general and musculoskeletal health); and (4) specific recommendations to assure an integral care approach for RA patients with any comorbidity, such as health care models for chronic inflammatory patients, early arthritis units, relationships with primary care, specialized nursing care, and self-management. These recommendations are intended to guide rheumatologists, patients, and other stakeholders, on the early diagnosis and management of comorbidity in RA, in order to improve disease outcomes.
Topics: Amyloidosis; Anxiety; Arthritis, Rheumatoid; Cardiovascular Diseases; Comorbidity; Delphi Technique; Depression; Diabetes Mellitus; Disease Management; Humans; Infections; Lung Diseases; Neoplasms; Obesity; Osteoporosis; Practice Guidelines as Topic; Rheumatology; Smoking
PubMed: 25543267
DOI: 10.1007/s00296-014-3196-7 -
Clinical and Experimental Medicine Feb 2015Conventional radiographic skeletal survey has been for many years the gold standard to detect the occurrence of osteolytic lesions in patients with multiple myeloma... (Review)
Review
Conventional radiographic skeletal survey has been for many years the gold standard to detect the occurrence of osteolytic lesions in patients with multiple myeloma (MM). However, the introduction of more sensitive imaging procedures has resulted in an updated anatomic and functional Durie and Salmon "plus" staging system and has remarkably changed the diagnostic and prognostic approach to this tumor. It is now established that (18)fluorine-fluorodeoxyglucose ((18)F-FDG) positron-emission tomography (PET) combined with low-dose computed tomography (CT), shortly designated PET/CT, exhibits a higher screening and diagnostic sensitivity and specificity over the skeleton X-ray. In patients with monoclonal gammopathy of undetermined significance and in those with smoldering MM, PET/CT is consistently unable to detect focal and/or diffuse marrow abnormalities. Conversely, based on a systematic review of 18 studies comprising almost 800 MM patients, PET/CT was able to detect MM osteolytic lesions with a sensitivity of approximately 80-90% and a specificity of 80-100%. Importantly, a poor degree of concordance has also been emphasized between PET/CT and whole-body magnetic resonance imaging (WB-MRI) in that when both techniques were applied to the same patients, double-positive results were recorded in approximately 30% of the cases, but in the majority of them, a higher number of lesions were revealed with PET/CT than with MRI. Double-negative results, on the other hand, were found in about 22% of the patients. Because PET/CT is able to identify tumor foci throughout the body, it can be usefully applied to the study of solitary bone plasmacytoma and extra-medullary plasmacytoma: In both conditions, the detection of additional, previously overlooked sites of skeletal involvement would falsify the diagnosis of single-district disease, upstage the tumor, and therefore require a different therapeutic approach. In addition, although PET/CT is poorly sensitive to diffuse bone marrow infiltration, it can anticipate a site of impending fracture throughout the body and can discriminate old from new pathologic fractures. MRI should, however, be preferred when vertebral bodies are suspected to be involved and the risk of vertebral fracture is to be assessed. PET/CT is a sensitive and reliable procedure to evaluate the response to chemotherapy and/or radiotherapy, which is shown by a remarkable reduction and sometimes total disappearance of FDG accumulation in the involved bony structures, although these structures remain morphologically abnormal. Conversely, an increased focal uptake of FDG in apparent remission patients often precedes clinically overt relapse. PET/CT should be preferred to other imaging techniques to assess the remission status after autologous stem cell transplantation. In patients with primary and remission-induced non-secretory MM, the use of PET/CT may help to early detect single or multiple districts of focal non-secretory relapse. Osteonecrosis of the jaw, its location, and extent in MM patients receiving bis-phosphonates are better defined by both PET/CT and contrast-enhanced MRI compared with dental panoramic views derived from cone beam CT imaging. Little is known as to the possible role of PET/CT in the assessment of disease extension, tumor load, and response to therapy in patients with Waldenström's macroglobulinemia (WM). In a study conducted on 35 WM patients, comparative PET/CT before and after therapy was able to detect positive findings in 83% of the patients, in contrast with the previous results achieved with conventional imaging that reported visceral involvement in much lower percentages. Similarly scanty are the data on the use of PET/CT in localized and systemic amyloidosis, given the small number of patients studied so far. A retrospective study has shown that, at variance from (123)Iodine-serum amyloid P component ((123)I-SAP) scintigraphy, which was found to be positive in about one-third of the patients with localized amyloidosis, an increased FDG uptake was detected at the amyloid site in virtually all of them. On the contrary, none of the patients with systemic amyloidosis showed an increased FDG uptake in sites of known deposition, whereas (123)I-SAP scintigraphy tested positive in the large majority of them. In another study, however, no such remarkable difference of positive PET/CT scans between localized and systemic amyloidosis was reported. Finally, false-positive and false-negative PET/CT findings can occur in different conditions that should be kept in mind to avoid wrong or omitted diagnoses.
Topics: Bone Marrow; Bone Neoplasms; Bone and Bones; Diagnostic Errors; Fluorodeoxyglucose F18; Humans; Magnetic Resonance Imaging; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Plasmacytoma; Positron-Emission Tomography; Prognosis; Radiopharmaceuticals; Tomography, X-Ray Computed
PubMed: 25218739
DOI: 10.1007/s10238-014-0308-3 -
Danish Medical Journal Nov 2013Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the... (Review)
Review
INTRODUCTION
Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised cutaneous amyloidosis (PLCA). The method of treatment was compared to the risk of local recurrence.
METHODS
A literature search produced 77 articles with localised cutaneous amyloidosis, 23 articles were excluded; thus, a total of 54 articles were included.
RESULTS
A total of 94 patients were included with a male:female ratio of 1.2:1.0. The median age was 57 years (range 24-87 years). The most common tumour localisation was in the head and neck region with a total of 38 lesions (34%), and 20 patients (22%) had two or more lesions in different locations. The nodular subtype was reported in 65 patients (69%). Only 29 patients received therapy with eight patients having two or more treatments (28%). Eight patients (9%) had local recurrence and all were nodular PLCA, which were mainly seen in males and localised in the face. One patient developed SA (1%); in fact, this was the only patient who was positive for monoclonal amyloid light chain amyloidosis by immunoelectrophoresis of the serum.
CONCLUSION
Our review suggests that PLCA is a benign disease that has a good prognosis and that it is associated with a low risk of developing SA (1%). The risk of developing local recurrence or developing new lesions was 9%, and no significant differences were found when compared to the primary treatment.
Topics: Amyloidosis, Familial; Disease Progression; Humans; Immunoglobulin Light Chains; Recurrence; Risk Assessment; Skin Diseases, Genetic
PubMed: 24192243
DOI: No ID Found