ORPHA:567548
Idiopathic steroid-resistant nephrotic syndrome
Also known as: Idiopathic SRNS
Query health: suspect — Only one of 2 strategies returned hits (phrase).
Publications
133
60.6th percentile
Trials
0
Interventional, condition-specific
Researchers
722
Distinct authors in sample
Gene link
DAAM2
Strong
Readiness
3/6
Stages with a signal
Clinical definition (Orphanet)
A rare, nephrotic syndrome characterized by the triad of proteinuria, hypoalbuminemia and edema in patients who do not respond, or only partially respond, to the initial trial of corticosteroids. Patients may be multidrug resistant or may be sensitive to second-line immunosuppressive therapy.
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0031008
- OMIM:619263
- UMLS:C5543267
Additional Mondo synonyms (3)
NPHS24 · idiopathic SRNS · idiopathic steroid-resistant nephrotic syndrome
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
3/6 stages with a signal
No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.
- Gene identifiedPresent
Strong — DAAM2
- LiteraturePresent
133 matched papers (72 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPartial
None under the specific name; 134 for broader category nephrotic syndrome
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Yes — we know a specific gene responsible (DAAM2).
GenCC classification: Strong.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
133
133 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
133 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
72 in the last 10 years · high confidence · 60.6th percentile (publications denominator)
Phrase hits: 133 · MeSH hits: 0
Who's working on it?
722
Distinct author names in 133 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01Bagga A10 papers · 2017
Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. Electronic address: arvindbagga@hotmail.com.
Papers in Europe PMC - 02Hari P8 papers · 2017
Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India.
Papers in Europe PMC - 03Craig JC5 papers · 2025
The Children's Hospital at Westmead, Cochrane Kidney and Transplant, Centre for Kidney Research, Locked Bag 4001, Westmead, NSW, Australia, 2145.
Papers in Europe PMC - 04Hodson EM5 papers · 2025
Cochrane Kidney and Transplant, Centre for Kidney Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW, Australia, 2145.
Papers in Europe PMC - 05Dinda AK4 papers · 2017
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Papers in Europe PMC - 06Gulati A4 papers · 2013
Division of Pediatric Nephrology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
Papers in Europe PMC - 07Gupta A4 papers · 2018
Department of Nephrology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Papers in Europe PMC - 08Oh J4 papers · 2025
Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Papers in Europe PMC - 09Ranchin B4 papers · 2025
Pediatric Nephrology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Université de Lyon, Lyon, France.
Papers in Europe PMC - 10Schaefer F4 papers · 2025
Division of Pediatric Nephrology, University Center for Pediatrics and Adolescent Medicine, Im Neuenheimer Feld 430, 69120, Heidelberg, Germany.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 134 trials are registered for nephrotic syndrome, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
high confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
134 interventional trials matched nephrotic syndrome, the broader category — listed below. Those studies are not counted in the condition-specific total.
Broader category: nephrotic syndrome
134
Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.
Worth raising with a clinician. How we count trials.
Recruiting under the broader category
- NCT06622915·RECRUITING·Cardiac Performance Evaluation in Children With Steroid Dependent vs Steroid Resistant Nephrotic Syndrome
Conditions: Nephrotic Syndrome·Matched via name phrase
- NCT07214818·RECRUITING·SGLT2 Inhibitors in Adult Primary Nephrotic Syndrome
Conditions: Nephrotic Syndrome·Matched via name phrase
- NCT06792799·RECRUITING·Anti-CD19/BCMA CAR-NK Cells in Patients With B Cell Mediated Autoimmune Disease
Conditions: Autoimmune Diseases · Systemic Lupus Erthematosus · Multi-Drug Resistant Nephrotic Syndrome · IgAN - IgA Nephropathy·Matched via name phrase
- NCT07091175·RECRUITING·Dupilumab Therapy in Nephrotic Syndrome in Children
Conditions: Nephrotic Syndrome in Children · Nephrotic Syndrome Steroid-Dependent·Matched via name phrase
- NCT07151456·NOT YET RECRUITING·Short-term gluCOCOrticoid in Adult STEROID-sensitive Nephrotic Syndrome: The COCO-ASTEROID Study
Conditions: Idiopathic Nephrotic Syndrome·Matched via name phrase
- NCT07685093·NOT YET RECRUITING·Comparison of Efficacy and Safety of Albumin Versus Fresh Frozen Plasma in Managing Diuretic Resistant Edema in Children With Idiopathic Nephrotic Syndrome.
Conditions: Idiopathic Nephrotic Syndrome (INS)·Matched via name phrase
- NCT07116239·NOT YET RECRUITING·Edoxaban Steady-State PK/PD in Adults With Nephrotic Syndrome
Conditions: Nephrotic Syndrome · Hypoalbuminemia·Matched via name phrase
- NCT06983028·RECRUITING·Atacicept in Multiple Glomerular Diseases
Conditions: pMN · IgAN · Nephrotic Syndrome · MCD·Matched via name phrase
- NCT00977977·RECRUITING·Rituximab Plus Cyclosporine in Idiopathic Membranous Nephropathy
Conditions: Nephrotic Syndrome · Proteinuria · Autoimmune Disease · Glomerular Disease·Matched via name phrase
- NCT06553898·RECRUITING·Study of Therapeutic Efficacy of CAR-T Cell Therapy in Patients With MDR-SRNS
Conditions: Multi-Drug Resistant Nephrotic Syndrome · CAR-T Cell Therapy·Matched via name phrase
- NCT06718894·RECRUITING·Multitarget Strategy for Primary Podocytopathies
Conditions: Nephrotic Syndrome·Matched via name phrase
- NCT05588063·RECRUITING·taVNS for FRNS in Children
Conditions: Nephrotic Syndrome in Children · Minimal Change Disease · Focal Segmental Glomerulosclerosis·Matched via name phrase
- NCT03949855·RECRUITING·Belimumab With Rituximab for Primary Membranous Nephropathy
Conditions: Membranous Nephropathy · Nephrotic Syndrome·Matched via name phrase
- NCT05850546·NOT YET RECRUITING·Rituximab in the First Episode of Paediatric Nephrotic Syndrome
Conditions: Steroid-Sensitive Nephrotic Syndrome·Matched via name phrase
- NCT05786768·RECRUITING·Efficacy and Safety of Obinutuzumab Versus Rituximab in Childhood Steroid Dependant and Frequent Relapsing Nephrotic Syndrome
Conditions: Steroid-Dependent Nephrotic Syndrome · Steroid-Sensitive Nephrotic Syndrome·Matched via name phrase
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Idiopathic steroid-resistant nephrotic syndrome" OR "Idiopathic SRNS" OR "NPHS24"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Idiopathic steroid-resistant nephrotic syndrome" OR "Idiopathic SRNS" OR "NPHS24" OR "DAAM2"
Recall-expansion terms: DAAM2
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"nephrotic syndrome"
Query health: suspect — strategies attempted: phrase, recall-expansion; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T18:28:24.372Z