Renal cysts and diabetes syndrome (RCAD): Causes, symptoms, treatment Health ConditionsHealth ConditionsAlzheimer's & DementiaAnxietyArthritisAsthma & AllergiesBreast CancerCancerCardiovascular HealthCOVID-19Dermatology & SkincareDiabetesEnvironment & SustainabilityExercise & FitnessEye HealthHeadache & MigraineHealth EquityHIV & AIDSHuman BiologyInflammatory Bowel DiseaseLeukemiaLGBTQIA+Men's HealthMental HealthMultiple Sclerosis (MS)NutritionParkinson's DiseasePsoriasisSexual HealthWomen's HealthDiscoverNewsLatest NewsOriginal SeriesMedical MythsHonest NutritionThrough My EyesNew Normal HealthPodcastsHow to understand chronic painWhat is behind vaccine hesitancy?The amazing story of hepatitis C, from discovery to cureNew directions in dementia researchCan psychedelics rewire a depressed, anxious brain?Why climate change matters for human healthToolsGeneral HealthDrugs A-ZHealth HubsHealth ToolsBMI Calculators and ChartsBlood Pressure Chart: Ranges and GuideBreast Cancer: Self-Examination GuideSleep CalculatorHealth ProductsAffordable Therapy OptionsBlood Pressure MonitorsDiabetic SuppliesFitness TrackersHome GymsGreen Cleaning ProductsHow to Shop for CBDQuizzesRA Myths vs FactsType 2 Diabetes: Managing Blood SugarAnkylosing Spondylitis Pain: Fact or FictionConnectAbout Medical News TodayWho We AreOur Editorial ProcessContent IntegrityConscious LanguageNewslettersSign UpFollow UsMedical News TodayHealth ConditionsDiscoverToolsConnectSubscribe What is renal cysts and diabetes syndrome Medically reviewed by Kelly Wood, MD — By Peter Brown on August 29, 2022Renal cysts and diabetes syndrome (RCAD) refers to a rare type of diabetes that typically affects younger people. It occurs due to a gene change and can result in diabetes symptoms and the development of kidney problems. This condition belongs to a rare group of inherited types of diabetes known as maturity onset diabetes of the young (MODY). The condition, also known as MODY 5, occurs due to a gene alteration that results in renal cysts and affects insulin production. This hormone helps maintain blood glucose levels. When the body is unable to produce sufficient insulin, this can result in high blood sugar, which may lead to health complications. In this article, we discuss renal cysts and diabetes syndrome, including its causes, symptoms, and treatments. Definition Share on PinterestDragoljub Bankovic/EyeEm/Getty ImagesRCAD describes a rare type of diabetes that also results in the formation of cysts in the kidneys. It occurs due to a gene change that affects not only insulin production but also kidney development. Health experts may also refer to RCAD as MODY 5 or HNF1B MODY. This is because it refers to a rare group of diabetes called MODY, in which genetic variations limit the body’s ability to produce insulin. As the name implies, MODY usually affects children, adolescents, and young adults, often manifesting before the age of 25 years. The prevalence of MODY is roughly 1 in 23,000 children, and it may account for about 5% of all diabetes cases in the United States. However, experts believe that doctors mistakenly diagnose 90% of people with MODY with another type of diabetes. Evidence suggests that MODY 5 is one of the more common subtypes, accounting for 5–10% of MODY cases. Causes and risk factors Health experts may refer to all types of MODY as monogenic diabetes. This is because these conditions are the result of an alteration in a single gene. MODY follows an autosomal dominant inheritance pattern, meaning that only one copy of an altered gene is necessary to develop the condition. Typically, MODY occurs due to inheriting an altered gene from a parent. If a person inherits this genetic change, they will usually develop MODY before the age of 25 years, regardless of their weight, lifestyle, and racial and ethnic background. However, MODY 5 can arise from new mutations in a gene and occur in people with no family history of MODY. In people with RCAD, the condition develops due to a mutation in the hepatic nuclear factor 1B gene, also known as the HNF1B gene. This gene is responsible for producing proteins that act as transcription factors, helping control the activity of other genes. In particular, HNF1B helps regulate genes that play a role in the development of beta cells and the kidneys. Beta cells are a type of special cell present in the pancreas that secretes insulin. Due to the genetic change, the HNF1B gene produces an altered transcription factor that is unable to function correctly. This results in the beta cells being less able to produce sufficient levels of insulin to help control blood sugar levels. The HNF1B gene also plays a role in the development of other body systems, including the kidneys. The variation in the proteins that the gene produces can increase the risk of renal problems, such as the development of fluid-filled sacs known as cysts. Symptoms Individuals with RCAD may experience mild symptoms of diabetes. These can include:frequent urinationincreased thirstdehydration In addition to diabetes symptoms, RCAD often affects other organs and manifests with extra-pancreatic symptoms. As the name suggests, these usually include structural changes to the kidneys that can affect their function and increase the risk of kidney conditions. A person will also likely present with the following clinical features:early onset non-insulin-dependent diabeteskidney cystskidney diseasealterations in the genital tracthigh levels of uric acid and gout Diagnosis Initially, a person may present with symptoms that suggest diabetes. A doctor will ask questions about the person’s symptoms and family history before performing a blood sugar test to confirm high blood sugar levels. They will then request additional tests, such as an autoantibody test, to help determine the type of diabetes. Depending on these results, the doctor may suggest genetic testing, which is the gold standard for diagnosing MODY. Correctly identifying which type of diabetes a person has is important for optimizing treatment strategies. The MODY diagnostic guidelines recommend genetic testing if a person:has a diabetes diagnosis before the age of 25 yearshas a family history of diabetescan produce insulinhas a C-peptide level in the normal rangehas a negative result for beta-cell autoantibodies If the test identifies a mutation in the HNF1B gene, this result confirms a diagnosis of MODY 5. Treatment Unlike other types of MODY, evidence suggests that individuals with RCAD often present with some level of insulin resistance. As such, common treatment options for MODY, such as sulfonylureas, are inefficient. Instead, people may require insulin therapy to help treat the condition. It may also be advisable for a person to follow a diet plan from a dietitian to help them manage their blood sugar levels. When to contact a doctor If an individual or child has symptoms of diabetes, it is advisable to consult a healthcare professional. An endocrinologist can help diagnose diabetes and determine the type. In some cases, they may recommend genetic testing for MODY 5. This is possible if a person receives a diabetes diagnosis at a young age, has a known family history of the disease, and presents with symptoms that are not typical features of other diabetes conditions. A doctor may also be able to detect renal cysts in the womb before a baby is born, which may indicate RCAD. Summary Renal cysts and diabetes syndrome is a rare subtype of diabetes. It belongs to a group of conditions known as maturity onset diabetes of the young. It occurs due to an alteration in the HNF1B gene. This gene has a role in the development of beta cells and the kidneys. The gene mutation affects the production of insulin and the structure of the kidneys, which increases the risk of cysts. Genetic testing is necessary to confirm a variation in the HNF1B gene and diagnose RCAD. Treatment for the condition typically involves insulin therapy and following a dietary regimen to help control blood sugars. Last medically reviewed on August 29, 2022DiabetesGeneticsUrology / Nephrology 19 sourcescollapsedMedical News Today has strict sourcing guidelines and draws only from peer-reviewed studies, academic research institutions, and medical journals and associations. We avoid using tertiary references. We link primary sources — including studies, scientific references, and statistics — within each article and also list them in the resources section at the bottom of our articles. You can learn more about how we ensure our content is accurate and current by reading our editorial policy.Amaral, S., et al. (2021). MODY 5: A rare cause of diabetes and chronic kidney disease - a report of 10 cases [Abstract]. https://www.endocrine-abstracts.org/ea/0073/ea0073pep2.1Delvecchio, M., et al. (2020). Treatment options for MODY patients: A systematic review of literature. https://link.springer.com/article/10.1007/s13300-020-00864-4Diabetes: MODY. (n.d.). https://www.nationwidechildrens.org/conditions/diabetes-modyHNF1B gene. (2020). https://medlineplus.gov/genetics/gene/hnf1b/HNF1B-MODY (MODY 5). (n.d.). https://monogenicdiabetes.uchicago.edu/for-healthcare-professionals/hnf1b-mody-mody-5/HNF1B MODY & RCAD. (n.d.). https://www.diabetesgenes.org/what-is-mody/hnf1b-mody-rcad/Hoffman, L. S., et al. (2022). Maturity onset diabetes in the young. https://www.ncbi.nlm.nih.gov/books/NBK532900/Jang, K. M. (2020). Maturity-onset diabetes of the young: Update and perspectives on diagnosis and treatment. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6986955/Mateus, J. C., et al. (2020). Maturity-onset diabetes of the young type 5 a multisystemic disease: A case report of a novel mutation in the HNF1B gene and literature review. https://clindiabetesendo.biomedcentral.com/articles/10.1186/s40842-020-00103-6Mature-onset diabetes of the young type 5 (MODY 5). (n.d.). https://www.chop.edu/conditions-diseases/mature-onset-diabetes-young-type-5-mody-5Maturity-onset diabetes of the young. (2020). https://medlineplus.gov/genetics/condition/maturity-onset-diabetes-of-the-young/Maturity onset diabetes of the young (MODY). (n.d.). https://www.diabetes.org.uk/diabetes-the-basics/other-types-of-diabetes/modyMonogenic diabetes (neonatal diabetes mellitus & MODY). (2017). https://www.niddk.nih.gov/health-information/diabetes/overview/what-is-diabetes/monogenic-neonatal-mellitus-modyNkonge, K. M., et al. (2020). The epidemiology, molecular pathogenesis, diagnosis, and treatment of maturity-onset diabetes of the young (MODY). https://clindiabetesendo.biomedcentral.com/articles/10.1186/s40842-020-00112-5Renal cysts and diabetes syndrome (RCAD). (n.d.). https://www.ncbi.nlm.nih.gov/medgen/96569Renal cysts & diabetes (HNF1b). (n.d.). https://www.diabetesgenes.org/tests-for-diabetes-subtypes/renal-cysts-diabetes-hnf1b/Tan, C. S. H., et al. (2022). MODY5 hepatocyte nuclear factor 1ß (HNF1ß)-associated nephropathy: Experience from a regional monogenic diabetes referral centre in Singapore. https://journals.sagepub.com/doi/full/10.1177/23247096211065626Tshivhase, A., et al. (2021). Diagnosis and treatment of MODY: An updated mini review. https://www.mdpi.com/2076-3417/11/20/9436/htmUrakami, T. (2019). Maturity-onset diabetes of the young (MODY): Current perspectives on diagnosis and treatment.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625604/FEEDBACK:Medically reviewed by Kelly Wood, MD — By Peter Brown on August 29, 2022 Latest newsWhat sets 'SuperAgers' apart? Their unusually large neuronsOmega-3 may provide a brain boost for people in midlifeSeasonal affective disorder (SAD): How to beat it this fall and winterCDC: Monkeypox in the US 'unlikely to be eliminated in the near future'Why are more women prone to Alzheimer's? 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