Materiały źródłowe

• #1 Primary and secondary hyperoxaluria: Understanding the enigma

  https://www.wjgnet.com/2220-6124/full/v4/i2/235.htm

  Hyperoxaluria is characterized by an increased urinary excretion of oxalate. […] The disease spectrum extends from recurrent kidney stones, nephrocalcinosis and urinary tract infections to chronic kidney disease and end stage renal disease. […] Conservative treatment for both types of hyperoxaluria includes vigorous hydration and crystallization inhibitors to decrease calcium oxalate precipitation. […] Pyridoxine is also found to be helpful in approximately 30% patients with primary hyperoxaluria type 1. […] Clinical differentiation between primary and secondary hyperoxaluria and further between the types of primary hyperoxaluria is very important because of implications in treatment and diagnosis. […] Hyperoxaluria continues to be a challenging disease and a high index of clinical suspicion is often the first step on the path to accurate diagnosis and management.

• #2 Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-022-00661-1

  Primary hyperoxaluria (PH) is an inherited disorder that results from the overproduction of endogenous oxalate, leading to recurrent kidney stones, nephrocalcinosis and eventually kidney failure; the subsequent storage of oxalate can cause life-threatening systemic disease. […] Management of patients with PH and kidney failure is also extremely challenging. […] In response to these developments, members of OxalEurope a network of European scientists and physicians who specialize in PH and the metabolic workgroup of the European Rare Kidney Disease Reference Network (ERKNet), formed a workgroup. Our goal was to update the 2012 guidelines and formulate new clinical practice recommendations for the diagnostic approach to patients with a suspicion of PH and the management of all types of PH with various stages of kidney dysfunction.

• #3 Hyperoxaluria and oxalosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hyperoxaluria/symptoms-causes/syc-20352254

  Hyperoxaluria can be caused by a change in a gene, an intestine disease or eating too many foods that are high in oxalate. […] It’s important to stay away from high-oxalate foods if you have diet-related or enteric hyperoxaluria. Your doctor also may tell you to limit these foods if you have primary hyperoxaluria.

• #4 Hyperoxaluria and oxalosis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/hyperoxaluria-and-oxalosis/

  Hyperoxaluria occurs when you have too much oxalate in your urine. […] The long-term health of your kidneys depends on early diagnosis and prompt treatment of hyperoxaluria. […] Oxalosis happens after the kidneys fail in people who have primary and intestinal causes of hyperoxaluria, and excess oxalate builds up in the blood. […] Eating large amounts of foods high in oxalate can increase your risk of hyperoxaluria or kidney stones. […] Avoiding high-oxalate foods is particularly important if you have enteric hyperoxaluria. […] Untreated primary hyperoxaluria can eventually damage your kidneys. […] Oxalosis in its late stages can cause a variety of complications outside the kidney, including bone disease, anemia, skin ulcers, heart and eye problems, and in children, a failure to develop and grow normally.

• #5 Hyperoxaluria: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/21117-hyperoxaluria

  You cant prevent primary hyperoxaluria because its in your genes. If someone in your family has primary hyperoxaluria, your healthcare provider can screen you for this gene mutation. If you receive a diagnosis of primary hyperoxaluria, treatment can begin before any symptoms or kidney damage occurs. […] If you receive a diagnosis of enteric or dietary hyperoxaluria, you should follow your healthcare providers treatment plan to reduce complications from the condition.

• #6 Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-022-00661-1

  We aim to make clinical practice recommendations for worldwide application and have therefore added statements for countries with restricted financial and medical means. […] The diagnostic work-up for patients with estimated glomerular filtration rate (eGFR) 30ml/min/1.73m2 and suspected primary hyperoxaluria (PH) should include at least two urine oxalate assessments (preferably from a 24h urine collection). […] If genetic assessment reveals a mutation consistent with vitamin B6 (VB6; also known as pyridoxine) non-responsive PH1, RNA interference (RNAi) therapy is indicated. […] We recommend adapting fluid management to optimize urinary oxalate excretion as determined by a morning spot urine analysis. […] We recommend starting pyridoxine supplementation in all patients suspected to have PH and in all patients with genetically proven PH1.

• #7 Improving Treatment Options for Primary Hyperoxaluria

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9329168/

  One PH1-specific treatment option is the oral application of VB6. […] Vitamin B6 should be administered primarily to any patient with suspected PH1 up until a genetic diagnosis is made. […] A prospective study reported that patients with missense mutations indeed showed a better VB6 sensitivity. […] The potential long-term benefits for patients of the substrate reduction therapy based on RNAi, namely, better preservation of renal function and, at best, prevention of progression to ESKD and systemic oxalosis, remain to be confirmed. […] Thus, the genotype, which should be known, could be the decision marker. […] Therefore, VB6 should be administered as the first-line drug treatment in patients with sensitive genotypes. […] In all other patients, either those that cannot be treated with VB6 or in those for whom VB6 medication did not reach relative normalization, RNAi medication should be considered. […] If both RNAi medications fail singularly, combined administration of GO and LDHA blockers may be considered. […] When kidney function declines, renal replacement therapy and transplantation may be necessary.

• #8 Hyperoxaluria | UK Kidney Association

  https://www.ukkidney.org/rare-renal/rare-disease-groups/hyperoxaluria

  The Hyperoxaluria Rare Disease Group aims to: […] Increase the knowledge and understanding of Primary Hyperoxaluria and Oxalosis in order to improve its clinical management. […] The Hyperoxaluria Rare Disease Group (RDG) is working with international partners with the aim of finding new and improved treatments, and to empower patients. […] Our aim is to improve diagnoses and patient outcomes in hyperoxaluria, across the UK. […] We can help the NHS use new and high-cost drugs in a cost-effective way. […] Making genetic testing quicker and easier.

• #9

  https://link.springer.com/article/10.1007/s40265-022-01735-x

  The standard treatment of care has included, classically, hyperhydration, crystallization inhibitors, and vitamin B6 (VB6), the latter in PH1. […] Hyperhydration remains the hallmark of conservative treatment for all stone diseases, here including all types of PH. […] Of paramount importance for both the idiopathic kidney stone formers, and patients with PH is treatment with anti-lithogenic substances, for example, citrate and magnesium. […] Vitamin B6 is a well-established treatment option for patients with PH1 addressed below within the pharmacological options. […] One PH1-specific treatment option is the oral application of VB6. […] Vitamin B6 should be administered primarily to any patient with suspected PH1 up until a genetic diagnosis is made. […] In general, response to VB6 treatment seems to depend on the underlying variants.

• #10 Managing Primary Hyperoxaluria (PH) | Uncovering PH

  https://www.uncoveringph.com/managing-ph.html

  Currently, there is no FDA-approved therapy that addresses all subtypes of primary hyperoxaluria. […] Avoidance of oxalate-rich foods is recommended. […] Aggressive hydration is recommended: Adults/older adolescents: 4 liters water/day; School-age children: 2-3 liters water/day; Infants/small children: 1-1.5 liters water/day. […] Dialysis is not able to sufficiently remove all endogenously overproduced oxalate. Even in patients receiving a combination of daily HD and PD, a weekly elimination of less than half of endogenously produced oxalate can be achieved. […] The goal is to keep plasma oxalate levels below plasma calcium oxalate supersaturation (30-45 mol/L) to prevent systemic oxalosis in patients awaiting organ transplant. […] A survey showed that 94% of patients hope for new therapies that would prevent dialysis, organ transplant, and oxalosis, and/or improve chances of a normal life span. […] 53% of patients with PH and 36% of caregivers are concerned about kidney stones, procedures, and therapies. […] 65% of patients with PH and 24% of caregivers are worried about kidney failure. […] 71% of patients with PH and 52% of caregivers are fearful of organ transplant.

• #11 Article: Secondary hyperoxaluria due to pancreatic insufficiency (full text) – October 2019 – NJM

  https://www.njmonline.nl/article_ft.php?a=2148&d=1415&i=229

  Treatment aims to reduce systemic oxalosis and to prevent oxalate nephropathy from developing. It relies primarily on decreasing enteric oxalate absorption by decreasing the amount of unbound oxalate in the enteric lumen. This can be achieved by reducing the amount of dietary oxalate and taking calcium supplements with meals. Foods with especially high oxalate content, such as rhubarb, spinach, purslane, Swiss chard, sorrel, and beetroot, should be avoided. […] Secondly, binding bile acids with cholestyramine reduces colonic permeability for oxalate, decreasing net absorption. […] Lastly, an ongoing trial investigates the potential of oral oxalate decarboxylase supplementation, which breaks down intestinal oxalate into carbon dioxide and formate. […] High urine output lowers calcium oxalate supersaturation in the tubuli, decreasing the likelihood of renal crystal formation.

• #12 Primary and secondary hyperoxaluria: Understanding the enigma

  https://www.wjgnet.com/2220-6124/full/v4/i2/235.htm

  Alkalinization of the urine is well known to prevent stone formation as citrate complexes with calcium and thus decreases the amount of calcium oxalate available for precipitation. […] Despite our knowledge of O. formigenes and its use of oxalate as an energy source, the use of probiotics to reduce urinary oxalate excretion has not been demonstrated in human studies. […] Patients reaching ESRD need optimization of renal replacement therapy to ensure adequate oxalate removal. […] Transplantation must be planned when GFR falls between 15-30 mL/min per 1.73 m2.

• #13 Article: Secondary hyperoxaluria due to pancreatic insufficiency (full text) – October 2019 – NJM

  https://www.njmonline.nl/article_ft.php?a=2148&d=1415&i=229

  Citrate also reduces calcium oxalate supersaturation by binding calcium, preventing oxalate crystal formation. […] Patients who receive a renal transplant may also require haemodialysis in the initial days after surgery to prevent recurrence of oxalate nephropathy in the renal transplant. […] In this article, we explain that fat malabsorption due to exocrine pancreatic insufficiency can cause oxalate nephropathy. Since effective management is possible, early recognition is important. […] We hope to raise awareness for oxalate nephropathy secondary to fat malabsorption.

• #14 Improving Treatment Options for Primary Hyperoxaluria

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9329168/

  The primary hyperoxalurias are three rare inborn errors of the glyoxylate metabolism in the liver, which lead to massively increased endogenous oxalate production, thus elevating urinary oxalate excretion and, based on that, recurrent urolithiasis and/or progressive nephrocalcinosis. […] Treatment possibilities are scare, namely, hyperhydration and alkaline citrate medication. […] In type 1 primary hyperoxaluria, vitamin B6, though, is helpful in patients with specific missense or mistargeting mutations. […] In those vitamin B6 responsive, urinary oxalate excretion and concomitantly urinary glycolate is significantly decreased, or even normalized. […] In patients non-responsive to vitamin B6, RNA interference medication is now available. […] Lumasiran is already available on prescription and targets the messenger RNA of glycolate oxidase, thus blocking the conversion of glycolate into glyoxylate, hence decreasing oxalate, but increasing glycolate production.

• #15 Primary and secondary hyperoxaluria: Understanding the enigma

  https://www.wjgnet.com/2220-6124/full/v4/i2/235.htm

  Elevated plasma oxalate levels lead to oxalate deposition in various organ systems. Systemic oxalosis should be prevented but the diagnosis is often delayed in more than 40% of patients. […] Screening for hyperoxaluria must be undertaken in every child with the first episode of renal stone and all adults who present with recurrent calcium oxalate stones. […] Dietary interventions do not play a major role in the management of primary hyperoxaluria as absorption of oxalate from the intestine is very small. […] On the other hand, diet modification is a very important element in the treatment of secondary hyperoxaluria where efforts should be made to reduce oxalate intake in the diet. […] Early initiation of pyridoxine treatment and compliance with the treatment regimen in pyridoxine responsive patients may help to prevent renal failure in PH1.

• #16

  https://link.springer.com/article/10.1007/s40265-022-01735-x

  RNA interference (RNAi) therapeutics work at the level of messenger RNA (mRNA) translation. […] A first RNAi drug, lumasiran (Oxlumo; Alnylam Pharmaceuticals, Cambridge, MA, USA), was recently approved (end of 2020) by the US Food and Drug Administration and the European Medicines Agency for the treatment of PH1 and has been available via prescription since January 2021 in some countries. […] The potential long-term benefits for patients of the substrate reduction therapy based on RNAi, namely, better preservation of renal function and, at best, prevention of progression to ESKD and systemic oxalosis, remain to be confirmed. […] If both RNAi medications fail singularly, combined administration of GO and LDHA blockers may be considered. […] When kidney function declines, renal replacement therapy and transplantation may be necessary. […] A rare disease with a severe clinical phenotype has now become treatable, but only with an early diagnosis and prompt treatment installment can chronic kidney failure be prevented.

• #17 Improving Treatment Options for Primary Hyperoxaluria

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9329168/

  Nedosiran blocks liver-specific lactate dehydrogenase A and thus the final step of oxalate production. […] Future treatment possibilities are on the horizon, for example, substrate reduction therapy with small molecules or gene editing, induced pluripotent stem cell-derived autologous hepatocyte-like cell transplantation, or gene therapy with newly developed vector technologies. […] Hyperhydration remains the hallmark of conservative treatment for all stone diseases, here including all types of PH. […] Of paramount importance for both the idiopathic kidney stone formers, and patients with PH is treatment with anti-lithogenic substances, for example, citrate and magnesium. […] Vitamin B6 is a well-established treatment option for patients with PH1 addressed below within the pharmacological options.

• #18 Hyperoxaluria and oxalosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hyperoxaluria/diagnosis-treatment/drc-20352258

  To lower the amount of calcium oxalate crystals that form in your kidneys, your doctor may suggest one or more of these treatments: […] Lumasiran (Oxlumo) is a medicine that lowers the level of oxalate in children and adults with primary hyperoxaluria. Prescription doses of vitamin B-6, also called pyridoxine, can help reduce oxalate in the urine in some people with primary hyperoxaluria. Phosphates and citrate prepared by a pharmacy and taken by mouth help keep calcium oxalate crystals from forming. […] If you have enteric hyperoxaluria, your doctor also may recommend a calcium supplement to take with meals. This could make it easier for oxalate to combine with calcium in the gut and leave the body through stool. […] If your kidneys still work well, your doctor will likely tell you to drink more water or other fluids. This flushes the kidneys, prevents oxalate crystal buildup and helps keep kidney stones from forming.

• #19 Primary hyperoxaluria and oxalate: Symptoms, causes and treatment | American Kidney Fund

  https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/primary-hyperoxaluria-and-oxalate-symptoms-causes-and-treatment

  The goal of treatment is to lower the level of oxalate in your body and prevent it from building up in your kidneys. Your doctor may suggest you: […] In some patients with PH, your doctor or dietitian may recommend that you follow a healthy eating plan that: […] Avoids foods high in oxalate, such as spinach, chocolate, potatoes and nuts. Calcium binds to oxalate to prevent oxalate from building up in the kidneys.

• #20 Hyperoxaluria and oxalosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hyperoxaluria/diagnosis-treatment/drc-20352258

  In general, it’s more important to pay attention to your food choices if you have enteric or diet-related hyperoxaluria. Diet changes may help lower the levels of oxalate in your urine. Your health care team may suggest that you restrict foods high in oxalates, limit salt and eat less animal protein and sugar. But diet changes may not help all people with primary hyperoxaluria. Follow your care team’s suggestions. […] A treatment called dialysis that takes over some of the work of your kidneys may help. But it doesn’t keep up with the amount of oxalate your body makes. A kidney transplant or kidney and liver transplant can treat primary hyperoxaluria. A liver transplant is the only treatment that might cure some types of primary hyperoxaluria.

• #21 Enteric Hyperoxaluria | OHF

  https://ohf.org/enteric/

  Enteric Hyperoxaluria (EH) is caused by the increased absorption of oxalate from foods in the digestive tract (also called the gut or intestines). This leads to high levels of oxalate in the urine. […] To help minimize fat malabsorption which may be causing hyperoxaluria, it is recommended that you work with your physician and a registered dietitian to lower fat intake, to add calcium into your diet, and also to lower your oxalate intake from food, based upon your current diet and lab results. […] Dietary management is an important component of care and includes lowering fat intake, adding calcium into your diet, and lowering your oxalate intake. […] Speak to your dietitian about putting together a balanced diet that is appropriate for your Enteric Hyperoxaluria. This may include lowering oxalate intake, increasing calcium, and reducing fat intake.

• #22 Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-022-00661-1

  We therefore recommend that urine oxalate measurements are repeated on at least two, but preferably three occasions to confirm that levels are elevated, particularly if findings are equivocal. […] Conservative treatment is of paramount importance to prevent the formation of further stones and decline of renal function, particularly in regions with hot climates. […] New therapies, particularly those based on RNAi, have shown promise in reducing oxalate production in patients with PH1, at least in the short term. […] Lumasiran (Oxlumo; Alnylam) has received marketing authorization by the EMA and FDA as an orphan drug for the treatment of PH1. […] We therefore recommend intensive HD over PD, preferably using a high-flux dialyser with maximal blood flow. […] We propose a shared decision-making approach to dialysis, taking into account whether patients and their families could tolerate daily dialysis sessions with or without nocturnal PD.

• #23 Primary hyperoxaluria and the Kidney | PPT

  https://www.slideshare.net/kenar78/primary-hyperoxaluria-and-the-kidney

  Primary Hyperoxaluria results from a genetic defect causing overproduction of oxalate by the liver. This leads to high oxalate levels in the urine and tendency to form calcium oxalate crystals in the kidneys. If untreated, it can progress to kidney failure and systemic oxalosis. […] Combined Kidney-Liver transplantation is the best long term treatment for type I hyperoxaluria. Many patients must undergo interval dialysis treatment while awaiting transplant surgery. […] Long term adherence can improve prognosis and slow progression to ESRD. 2-3L/day of fluid intake, K citrate to alkalinize urine, and pyridoxine for type 1 are recommended. […] Nocturnal Hemodialysis performed 7 nights per week using 60L of dialysate over 8-10 hours lowered oxalate concentrations. […] Substantial additional oxalate removal can be done by adding PD to intensive HD in difficult oxalosis cases.

• #24 Hyperoxaluria and oxalosis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/hyperoxaluria-and-oxalosis/

  Treatment depends on the type, symptoms and severity of hyperoxaluria and how well you respond to treatment. […] To reduce the amount of calcium oxalate crystal formation in your kidneys, your doctor may recommend one or more of these treatments: […] Dietary changes like these may help to lower the levels of oxalate in your urine. […] Kidney dialysis may help temporarily, but it doesn’t keep up with the amount of oxalate produced. […] A kidney transplant or kidney and liver transplant can cure certain inherited types of hyperoxaluria (primary hyperoxaluria). […] How can I prevent kidney stones in the future?

• #25 Primary hyperoxaluria and the Kidney | PPT

  https://www.slideshare.net/kenar78/primary-hyperoxaluria-and-the-kidney

  Early start to dialysis is suggested as total accumulated body oxalate determines long term outcomes. […] Preemptive liver transplant before systemic deposition is the goal before CKD. […] Future therapies include cell therapy to repopulate the liver with normal hepatocytes, gene transfer, and inhibition of glycolate oxidase.

• #26

  https://link.springer.com/article/10.1007/s40265-022-01735-x

  The primary hyperoxalurias are three rare inborn errors of the glyoxylate metabolism in the liver, which lead to massively increased endogenous oxalate production, thus elevating urinary oxalate excretion and, based on that, recurrent urolithiasis and/or progressive nephrocalcinosis. […] Treatment possibilities are scare, namely, hyperhydration and alkaline citrate medication. […] In type 1 primary hyperoxaluria, vitamin B6, though, is helpful in patients with specific missense or mistargeting mutations. […] In those vitamin B6 responsive, urinary oxalate excretion and concomitantly urinary glycolate is significantly decreased, or even normalized. […] In patients non-responsive to vitamin B6, RNA interference medication is now available. […] Future treatment possibilities are on the horizon, for example, substrate reduction therapy with small molecules or gene editing, induced pluripotent stem cell-derived autologous hepatocyte-like cell transplantation, or gene therapy with newly developed vector technologies.

• #27 Perspectives in primary hyperoxaluria — historical, current and future clinical interventions | Nature Reviews Urology

  https://www.nature.com/articles/s41585-021-00543-4

  Primary hyperoxalurias are a devastating family of diseases leading to multisystem oxalate deposition, nephrolithiasis, nephrocalcinosis and end-stage renal disease. […] Traditional treatment paradigms are limited to conservative management, dialysis and inevitably transplantation of the kidney and liver, which is associated with high morbidity and the need for lifelong immunosuppression. […] New therapeutics being developed for PHs take advantage of biochemical knowledge about oxalate synthesis and metabolism to specifically target these pathways, with the goal of decreasing the accumulation and deposition of plasma oxalate in the body. […] New therapeutics can be divided into classes, and include substrate reduction therapy, intestinal oxalate degradation, chaperone therapy, enzyme restoration therapy and targeting of the inflammasome. […] Future work includes further clinical trials for promising therapeutics in the pipeline, identification of biomarkers of response to PH-directed therapy, optimization of drug development and delivery of new therapeutics.

• #28 Secondary hyperoxaluria: Cause and consequence of chronic kidney disease | Nefrología

  https://www.revistanefrologia.com/en-secondary-hyperoxaluria-cause-consequence-chronic-articulo-S2013251424002359

  Secondary hyperoxaluria is a metabolic disorder characterized by an increase in urinary oxalate excretion. […] Treatment includes hygienic dietary measures, and medications aimed at reducing intestinal absorption by increasing fecal excretion. […] Currently, specific treatments for secondary hyperoxaluria are lacking, making early diagnosis and preventive measures against kidney failure the main therapeutic strategies. […] The therapeutic approach to secondary hyperoxaluria can be classified into three strategies: reducing the intake of oxalate and its precursors, increasing renal elimination to avoid the precipitation of oxalate crystals at the tubular level, and reducing gastrointestinal absorption of oxalate while increasing its intestinal elimination. […] Treatment and prevention focus on reducing the intake of oxalate and its precursors, promoting renal elimination by avoiding the precipitation of oxalate crystals at the tubular level, and establishing targeted measures to reduce oxalate absorption and increase its intestinal elimination.

• #29 What Is Primary Hyperoxaluria Type 1 (PH1)? Life Expectancy

  https://www.medicinenet.com/what_is_primary_hyperoxaluria_type_1_ph1/article.htm

  Primary hyperoxaluria type 1 (PH1) is an extremely rare hereditary condition that is characterized by excessive oxalate formation a waste product usually removed by the kidneys and discharged in the urine. […] A genetic mutation in PH1 causes the liver to produce excess oxalate that accumulates in the kidneys and urinary system. […] The treatment of choice for patients with PH1 is hyperhydration and intensive dialysis with or without organ transplantation and supportive/symptomatic care. […] Patients with PH1 should be aware of the need to avoid specific things in addition to their treatment to avoid further complications. To protect their kidneys, patients should avoid dehydration. […] Additionally, they should consult a doctor before taking excessive amounts of vitamin C, vitamin D, loop diuretics, nonsteroidal anti-inflammatory drugs, or any other drugs that could impair kidney function. […] An early and accurate diagnosis, followed by aggressive supportive treatment, plays a significant role in both short- and long-term outcomes. […] The only method for curing patients with primary hyperoxaluria type 1 (PH1) is an organ transplant. However, it is not recommended for everyone.

• #30 UAB named OHF Care Center for excellence in rare kidney stone disease research and clinical care – UAB News

  https://www.uab.edu/news/health-medicine/uab-named-ohf-care-center-for-excellence-in-rare-kidney-stone-disease-research-and-clinical-care

  UAB has recently been named an OHF Care Center for excellence in research and clinical care for rare kidney stone disease. […] This designation marks UABs recognition as an international research hub and multidisciplinary clinical site focusing on primary hyperoxaluria and other rare kidney stone diseases. […] Research at UAB helped lead to the development of new FDA-approved drugs for patients with primary hyperoxaluria that have changed patients care pathways to prevent more of them from needing transplants. […] In addition to offering a strong research component, OHF Care Centers are recognized for having a multidisciplinary team of experts managing a patients care. […] I think now with the designation, it really puts an emphasis on our continuing to create a robust multidisciplinary team. […] UABs primary hyperoxaluria team aims to continue to address the needs of patients living with the disease from various perspectives. […] We’re trying to get answers to questions for these individuals that nobody else is doing across the country, and we’re doing it here.

• #31

  https://dramarkulkarni.in/hyperoxaluria-and-oxalosis.php

  Hyperoxaluria is a medical condition characterized by the presence of high levels of oxalate in the urine. […] Prevention of hyperoxaluria and oxalosis involves managing underlying medical conditions that may contribute to the development of the conditions, as well as maintaining a healthy diet and lifestyle. It is also important to stay hydrated and avoid consuming large amounts of oxalate-rich foods, such as spinach, rhubarb, and beets. […] Diagnosis of hyperoxaluria and oxalosis may involve blood and urine tests, imaging studies, and a kidney biopsy. Treatment options may include medications to reduce oxalate levels in the body, dietary changes to reduce the intake of oxalate-rich foods, and in some cases, kidney transplantation.

• #32 A hidden cause of oxalate nephropathy: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-02732-6

  Oxalate nephropathy is a rare disorder that can result in acute kidney injury (AKI) and progresses to end-stage kidney disease (ESKD. […] Special attention should be given to the secondary causes of hyperoxaluria in patients with predisposing conditions such as CKD. […] Supportive management includes increasing fluid intake, decreasing oxalate in the diet, urine alkalization, and taking calcium binders that reduce precipitation of calcium oxalate stones. […] Management of secondary hyperoxaluria involves: (1) maintaining a low-oxalate and high-calcium diet, which helps prevent hyperoxaluria, (2) increasing fluid intake (3 L/1.73 m2), (3) supplementation with calcium or other oxalate binders and bile acid sequestrants, which decreases permeability of oxalate in the intestines, (4) consumption of probiotics that contain oxalate-degrading bacteria, however recent studies have not proven its definite efficacy. […] Patients with predisposing conditions such as CKD have a higher risk of developing oxalate-induced nephropathy. […] Therefore, in addition to the typical work-up, special attention should be warranted to the dietary habits of patients at high risk, such as those with underlying CKD.

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