scholarly journals A Case of Essential Thrombocythemia and IgA Nephropathy with Literature Review of the Concurrence

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Shoja Rahimian ◽  
Timothy Johnson ◽  
Ronald Herb
Keyword(s):  
Renal Function ◽  
Literature Review ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasms ◽  
Immunoglobulin A ◽  
Glomerular Disease ◽  
Common Finding ◽  
Glomerular Diseases ◽  
Pathological Review ◽  
History Of

Myeloproliferative neoplasms such as essential thrombocythemia (ET) have been associated with glomerular disease on rare instances. A case of ET associated with immunoglobulin A nephropathy (IgAN) is described in a 57-year-old man with a history of hypertension. Progressively worsening renal function was noted in the patient along with unexplained mild thrombocytosis. Pathological review of renal biopsy identified IgAN concurrently with newly diagnosed JAK2-mutated ET. The patient was started on aspirin therapy and closely monitored for his renal function. A literature review of the association of ET and renal disease revealed nine cases of ET associated with IgAN, focal segmental glomerulosclerosis, and fibrillary glomerulonephritis. Comparison of the pathological features of the renal biopsies within the cases noted mesangial proliferation as a common finding, which has been described to be potentiated by platelet-derived growth factor (PDGF). This commonality may represent a link between ET and glomerular disease which deserves further attention in future cases. Improved management of such cases depends on the recognition of the combined occurrence of ET and glomerular diseases and uncovering the shared pathogenesis between platelets and glomeruli.

scholarly journals The Rationale and Evidence for SGLT2 Inhibitors as a Treatment for Nondiabetic Glomerular Disease

2021 ◽  
Vol 1 (1) ◽  
pp. 21-33
Author(s):  
Kana N. Miyata ◽  
Shao-Ling Zhang ◽  
John S.D. Chan
Keyword(s):  
Glucose Transporter ◽  
Mesangial Cells ◽  
Systemic Blood Pressure ◽  
Glomerular Disease ◽  
Renal Tubules ◽  
Protective Effects ◽  
Glomerular Diseases ◽  
Urinary Glucose ◽  
Proximal Tubular ◽  
History Of

Background: Recent studies show that sodium-glucose cotransporter 2 inhibitors (SGLT2i), originally approved for glycemic control in patients with type 2 diabetes, also exert renoprotective effects independently from effects on dysglycemia. Moreover, recent work indicates that SGLT2i treatment may be effective in patients with nondiabetic chronic kidney disease, including primary and secondary glomerular diseases. Summary: SGLT2i lower blood glucose by blocking glucose resorption in the early renal proximal tubule through the glucose transporter, SGLT2, leading to enhanced urinary glucose excretion. Recent studies indicate that SGLT2i may have pleiotropic effects on cells other than proximal tubular cells. SGLT2i reduce the glomerular workload by decreasing the intraglomerular pressure, thus ameliorating hyperfiltration, if present, and may also decrease systemic blood pressure. SGLT2i may also act directly on endothelial cells, possibly via modulating the effects of adhesion molecules and reducing inflammatory cytokines and reactive oxygen species. SGLT2i may have direct anti-inflammatory and antifibrotic effects on renal tubules. Some reports suggest direct protective effects on podocytes and mesangial cells as well. Here, we provide a review of the potential mechanisms of renoprotection, therapeutic utility, and potential side effects of SGLT2i in patients with nondiabetic glomerular diseases, based on data from studies carried out in cells, experimental animals, and humans. Key Messages: SGLT2i may be a promising addition to the glomerular disease treatment armamentarium. However, it is unclear at what point of the natural history of specific glomerular diseases (whether this is immune or nonimmune mediated) SGLT2i can be beneficial. Additionally, further studies are needed to assess the long-term efficacy and safety of SGLT2i in patients with nondiabetic glomerular diseases.

Developments in the Immunotherapy of Glomerular Disease

2002 ◽  
Vol 15 (6) ◽  
pp. 472-489
Author(s):  
Patrick H. Nachman ◽  
Jeffrey Martin
Keyword(s):  
Alkylating Agents ◽  
Clinical Manifestations ◽  
Calcineurin Inhibitors ◽  
Immunosuppressive Drugs ◽  
Glomerular Disease ◽  
Drug Induced ◽  
Glomerular Diseases ◽  
Care Givers ◽  
Clinical Syndromes ◽  
History Of

Glomerular diseases span a broad spectrum of clinical syndromes, with varied clinical manifestations, underlying etiologies, and pathogenic mechanisms. They can be secondary to underlying infectious, toxic, environmental, or drug exposures, or present as “primary entities.” In the latter case, most glomerular diseases are thought to be due to autoimmune dysregulation, and their treatment is primarily immunosuppressive. The armamentarium for immunomodulation includes corticosteroids, alkylating agents, anti-metabolites, calcineurin inhibitors, and new biological agents designed to block specific inflammatory pathways. The choice of therapy for an individual patient must be based on the specific character of the glomerular disease and its acuity and severity, as well as the patient’s comorbidities, history of prior exposure to immunosuppressive drugs, and risk factors for developing complications of the disease or its treatment. The complexities of such therapy can best be addressed by an experienced team of care givers in which the clinical pharmacist can help minimize, if not eliminate, potential sources of drug induced toxicities and adverse effects. This article will describe the major agents and modalities used in the management of the most common glomerular diseases.

scholarly journals Running interferon interference in treating PV/ET: meeting unmet needs

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 463-468
Author(s):  
Elizabeth A. Traxler ◽  
Elizabeth O. Hexner
Keyword(s):  
Risk Assessment ◽  
Essential Thrombocythemia ◽  
Proinflammatory Cytokine ◽  
Unmet Needs ◽  
Myeloproliferative Neoplasms ◽  
Individual Case ◽  
Treatment Goals ◽  
Interferon Treatment ◽  
Molecular Context ◽  
History Of

Abstract Enthusiasm about interferons for the treatment of myeloproliferative neoplasms has recently arisen. How does a nontargeted therapy selectively target the malignant clone? Many foundational questions about interferon treatment are unanswered, including who, when, and for how long do we treat. Using an individual case, this review touches on gaps in risk assessment in polycythemia vera (PV) and essential thrombocythemia (ET) and the history of treatment with interferons. How is it that this proinflammatory cytokine effectively treats ET and PV, themselves proinflammatory states? We summarize existing mechanistic and clinical data, the molecular context as a modifier for treatment response, the establishment of treatment goals, and the challenges that lie ahead.

scholarly journals MOLECULAR GENETIC ABNORMALITIES IN THE GENOME OF PATIENTS WITH Ph-NEGATIVE MYELOPROLIFERATIVE NEOPLASIA AFFECTED BY IONIZING RADIATION AS A RESULT OF THE CHORNOBYL NUCLEAR ACCIDENT

2020 ◽  
Vol 25 ◽  
pp. 362-373
Author(s):  
L. Poluben ◽  
◽  
L. Neumerzhytska ◽  
S. Klymenko ◽  
P. Fraenkel ◽  
...  
Keyword(s):  
Ionizing Radiation ◽  
Radiation Exposure ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasms ◽  
Molecular Genetic ◽  
Jak2 V617f ◽  
Primary Myelofibrosis ◽  
Genomic Research ◽  
History Of

Objective. to determine the frequency of major somatic mutations in the JAK2, MPL and CALR genes in the genome of patients with Ph-negative myeloproliferative neoplasms that occur in individuals who have been exposed to ionizing radiation as a result of the Chornobyl accident. Materials and methods. Molecular genetic analysis of genomic DNA samples isolated from blood was performed in 90 patients with Ph-negative myeloproliferative neoplasia (MPN) with a history of radiation exposure and 191 patients with spontaneous MPN utilizing allele-specific polymerase chain reaction (PCR). Results. The presence of major mutations in the genes JAK2, CALR and MPL was revealed in patients with MPN with a history of radiation exposure with a frequency 58.9 % (53 of 90), 12.2 % (11 of 90), and 0 % respectively, and without exposure with frequency 75.4 % (144 of 191), 3.1 % (6 out of 191) and 1.6 % (3 out of 191) respectively. Mutations JAK2 V617F in patients with spontaneous MPN were observed in each clinical form: polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). CALR mutations were detected exclusively in patients with PMF and ET, significantly more often in groups with a radiation exposure history (18.9 % and 33.3 %, vs. 4.2 % and 6.5 %) than without one. At the same time, the occurence of MPL mutations was determined only in patients with spontaneous MPN in 1.6 % of casees. Triple negative mutation status of genes JAK2, MPL and CALR prevailed in the group of patients with MPN with a history of radiation exposure and was 27.8 %, against 16.2 % in patients without radiation exposure (p = 0.05). Conclusions. Genomic research of patients with Ph-negative MPN revealed features of molecular genetic damage in those patients who were exposed to IR as a result of the Chornobyl accident and those with spontaneous MPN. The data obtained by determining of JAK2, MPL and CALR genes mutational status in the genome of patients with MPN is necessary to expand the understanding of the mechanism of leukogenesis, especially caused by radiation. Key words: myeloproliferative neoplasia, polycythemia vera, essential thrombocythemia, primary myelofibrosis, JAK2 V617F, MPL and CALR, ionizing radiation.

Pneumomediastinum: A Rare Presentation of Inflicted Injuries in Infants

2020 ◽  
Author(s):  
Adam Lee ◽  
Adam Bajinting ◽  
Abby Lunneen ◽  
Colleen M. Fitzpatrick ◽  
Gustavo A. Villalona
Keyword(s):  
Literature Review ◽  
Retrospective Review ◽  
Review Of The Literature ◽  
Rare Presentation ◽  
Spontaneous Pneumomediastinum ◽  
Nonaccidental Trauma ◽  
Comprehensive Literature Review ◽  
Healthy Infants ◽  
History Of

AbstractReports of incidental pneumomediastinum in infants secondary to inflicted trauma are limited. A retrospective review of infants with pneumomediastinum and history of inflicted trauma was performed. A comprehensive literature review was performed. Three infants presented with pneumomediastinum associated with inflicted trauma. Mean age was 4.6 weeks. All patients underwent diagnostic studies, as well as a standardized evaluation for nonaccidental trauma. All patients with pneumomediastinum were resolved at follow-up. Review of the literature identified other cases with similar presentations with related oropharyngeal injuries. Spontaneous pneumomediastinum in previously healthy infants may be associated with inflicted injuries. Clinicians should be aware of the possibility of an oropharyngeal perforation related to this presentation.

scholarly journals Sustained Opening of the Blood-Brain Barrier with Progressive Accumulation of White Matter Hyperintensities Following Ischemic Stroke

2019 ◽  
Vol 9 (1) ◽  
pp. 16 ◽  
Author(s):  
Imama Naqvi ◽  
Emi Hitomi ◽  
Richard Leigh
Keyword(s):  
Ischemic Stroke ◽  
White Matter ◽  
Acute Stroke ◽  
Blood Brain Barrier ◽  
White Matter Hyperintensities ◽  
Brain Barrier ◽  
Common Finding ◽  
Blood Brain ◽  
History Of ◽  
Iv Tpa

Objective: To report a patient in whom an acute ischemic stroke precipitated chronic blood-brain barrier (BBB) disruption and expansion of vascular white matter hyperintensities (WMH) into regions of normal appearing white matter (NAWM) during the following year. Background: WMH are a common finding in patients with vascular risk factors such as a history of stroke. The pathophysiology of WMH is not fully understood; however, there is growing evidence to suggest that the development of WMH may be preceded by the BBB disruption in the NAWM. Methods: We studied a patient enrolled in the National Institutes of Health Natural History of Stroke Study who was scanned with magnetic resonance imaging (MRI) after presenting to the emergency room with an acute stroke. After a treatment with IV tPA, she underwent further MRI scanning at 2 h, 24 h, 5 days, 30 days, 90 days, 6 months, and 1-year post stroke. BBB permeability images were generated from the perfusion weighted imaging (PWI) source images. MRIs from each time point were co-registered to track changes in BBB disruption and WMH over time. Results: An 84-year-old woman presented after acute onset right hemiparesis, right-sided numbness and aphasia with an initial NIHSS of 13. MRI showed diffusion restriction in the left frontal lobe and decreased blood flow on perfusion imaging. Fluid attenuated inversion recovery (FLAIR) imaging showed bilateral confluent WMH involving the deep white matter and periventricular regions. She was treated with IV tPA without complication and her NIHSS improved initially to 3 and ultimately to 0. Permeability maps identified multiple regions of chronic BBB disruption remote from the acute stroke, predominantly spanning the junction of WMH and NAWM. The severity of BBB disruption was greatest at 24 h after the stroke but persisted on subsequent MRI scans. Progression of WMH into NAWM over the year of observation was detected bilaterally but was most dramatic in the regions adjacent to the initial stroke. Conclusions: WMH-associated BBB disruption may be exacerbated by an acute stroke, even in the contralateral hemisphere, and can persist for months after the initial event. Transformation of NAWM to WMH may be evident in areas of BBB disruption within a year after the stroke. Further studies are needed to investigate the relationship between chronic BBB disruption and progressive WMH in patients with a history of cerebrovascular disease and the potential for acute stroke to trigger or exacerbate the process leading to the development of WMH.

scholarly journals Efficacy and safety of gout flare prophylaxis and therapy use in people with chronic kidney disease: a Gout, Hyperuricemia and Crystal-Associated Disease Network (G-CAN)-initiated literature review

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Huai Leng Pisaniello ◽  
Mark C. Fisher ◽  
Hamish Farquhar ◽  
Ana Beatriz Vargas-Santos ◽  
Catherine L. Hill ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Renal Function ◽  
Kidney Disease ◽  
Literature Review ◽  
Interleukin 1 ◽  
Treatment Options ◽  
Cochrane Library ◽  
Efficacy And Safety ◽  
Clinical Trial Results ◽  
Gout Flare

AbstractGout flare prophylaxis and therapy use in people with underlying chronic kidney disease (CKD) is challenging, given limited treatment options and risk of worsening renal function with inappropriate treatment dosing. This literature review aimed to describe the current literature on the efficacy and safety of gout flare prophylaxis and therapy use in people with CKD stages 3–5. A literature search via PubMed, the Cochrane Library, and EMBASE was performed from 1 January 1959 to 31 January 2018. Inclusion criteria were studies with people with gout and renal impairment (i.e. estimated glomerular filtration rate (eGFR) or creatinine clearance (CrCl) < 60 ml/min/1.73 m2), and with exposure to colchicine, interleukin-1 inhibitors, non-steroidal anti-inflammatory drugs (NSAIDs), and glucocorticoids. All study designs were included. A total of 33 studies with efficacy and/or safety analysis stratified by renal function were reviewed—colchicine (n = 20), anakinra (n = 7), canakinumab (n = 1), NSAIDs (n = 3), and glucocorticoids (n = 2). A total of 58 studies reported these primary outcomes without renal function stratification—colchicine (n = 29), anakinra (n = 10), canakinumab (n = 6), rilonacept (n = 2), NSAIDs (n = 1), and glucocorticoids (n = 10). Most clinical trials excluded study participants with severe CKD (i.e. eGFR or CrCl of < 30 mL/min/1.73 m2). Information on the efficacy and safety outcomes of gout flare prophylaxis and therapy use stratified by renal function is lacking. Clinical trial results cannot be extrapolated for those with advanced CKD. Where possible, current and future gout flare studies should include patients with CKD and with study outcomes reported based on renal function and using standardised gout flare definition.

Non-visible haematuria in a military setting

2018 ◽  
Vol 104 (3) ◽  
pp. 177-182
Author(s):  
D O’Brien ◽  
K Houlberg
Keyword(s):  
Basement Membrane ◽  
Immunoglobulin A ◽  
Armed Forces ◽  
Common Finding ◽  
Immunoglobulin A Nephropathy ◽  
Hereditary Nephritis ◽  
Military Recruits ◽  
Thin Basement Membrane Disease ◽  
Medical Examinations

AbstractAsymptomatic non-visible haematuria is a common finding at routine military medical examinations. This article briefly reviews the possible causes, which include malignancy, structural causes, exertion haematuria, hereditary nephritis, thin basement membrane disease (TBMD), immunoglobulin A nephropathy (IgAN), tuberculosis (TB) and schistosomiasis. This paper discusses how these conditions may affect potential military recruits as well as currently serving members of the Armed Forces, and offers a general approach to the management of a patient with non-visible haematuria.

scholarly journals Intravitreal Silicone Oil Migration Into the Lateral Cerebral Ventricles

2019 ◽  
Vol 3 (6) ◽  
pp. 466-473
Author(s):  
Jessica L. Cao ◽  
Andrew W. Browne ◽  
Thomas Clifford ◽  
Sumit Sharma ◽  
Vivek Patel
Keyword(s):  
Case Report ◽  
Retinal Detachment ◽  
Literature Review ◽  
Current Literature ◽  
Symptom Management ◽  
Silicone Oil ◽  
Cerebral Ventricles ◽  
Oil Migration ◽  
Intraocular Tamponade ◽  
History Of

Purpose: Silicone oil (SO) is often used as an intraocular tamponade in repairs of retinal detachments. It may be associated with complications such as cataract, glaucoma, keratopathy, subretinal migration of oil, fibrous epiretinal and sub retinal proliferations, and oil emulsification. The purpose of this report is to describe a rare phenomenon of intraocular silicone oil migration into the cerebral ventricles, which may later be mistaken for intraventricular hemorrhages on neuroimaging. Methods: Case report with literature review. Results: A patient with a history of retinal detachment repair with intraocular SO presented with headaches. Neuroimaging revealed SO migration to the cerebral ventricles. The patient was treated conservatively with symptom management and headaches resolved. Conclusions: We present a case of intraocular SO migration to the cerebral ventricles and review the current literature. We also propose two mechanisms for this phenomenon.

P0234LOW URINARY CITRATE LEVELS ARE NOT SPECIFIC OF AUTOSOMAL POLYCYSTIC KIDNEY DISEASE (ADPKD) AND ARE PRESENT WHEN RENAL FUNCTION IS IMPAIRED IN ALL NEPHROPATIES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Francisco-Jose Borrego-Utiel ◽  
Enoc Merino Garcia ◽  
Isidoro Herrera ◽  
Clara Moriana Dominguez ◽  
Victoria Camacho Reina ◽  
...  
Keyword(s):  
Renal Function ◽  
Uric Acid ◽  
Kidney Disease ◽  
Renal Impairment ◽  
Polycystic Kidney Disease ◽  
Normal Renal Function ◽  
Serum Bicarbonate ◽  
Polycystic Kidney ◽  
Glomerular Diseases ◽  
Urinary Citrate

Abstract Background and Aims In polycystic kidney disease (PKD) it is frequently found a reduction in urinary citrate that is related with degree of renal impairment but it is unknown if this alteration is specific or if it is also present in other nephropathies. Recently it has been suggested that urinary citrate could be a marker of covert metabolic acidosis and reflects acid retention in chronic kidney disease (CKD). Our aim was to compare urinary citrate in PKD with other renal diseases and to show its relation with serum bicarbonate and excretion of uric acid and calcium. Method We determined citrate, calcium and uric acid in 24-hour urine in patients with PKD and with other nephropathies with varied degree of renal impairment followed in a outpatient clinic of nephrology. Results We included 291 patients, 119 with glomerular diseases, 116 with PKD, 21 with other nephropathies, and 35 patients with normal renal function. Urinary citrate was higher in women (Females 309±251 mg/gCr vs. males 181±145 mg/gCr, p&lt;0.001) and in patients with normal renal function (normal 380±210 mg/gCr; PKD 203±166 mg/gCr; glomerular 279±282 mg/gCr; p&lt;0,001). PKD patients showed similar values of urinary citrate to patients with glomerular diseases and with other nephropathies. We observed a progressive reduction in urinary citrate parallel to degree of renal impairment, in a comparable way among patients with PKD and glomerular diseases. We did not observe a relationship between urinary citrate and serum bicarbonate levels. Calcium and uric acid elimination in ADPKD patients was similar to other nephropathies and lower to patients with normal renal function. However, serum uric acid was significantly higher in glomerular patients than other nephropathies after adjust with glomerular filtration rate and sex. Conclusion Hypocitraturia is not specific of PKD but it is also present in all nephropathies. Urinary citrate are related to degree of renal impairment and it is not related with serum bicarbonate. We think that it could be interesting to study urinary citrate as a marker of renal function and its role as prognostic factor of renal deterioration.

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