Senior-Boichis syndrome

ORPHA: 84081

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Senior-Boichis syndrome, sourced from HPO and Orphanet clinical annotations.

Renal hypoplasiaRenal corticomedullary cystsHypertensionHepatic fibrosisCholestasisPortal hypertensionHepatosplenomegalyPolydipsiaElevated circulating hepatic transaminase concentrationElevated circulating alkaline phosphatase concentrationIncreased total bilirubinHyperechogenic kidneysReduced renal corticomedullary differentiationMalformation of the hepatic ductal plateReduced number of intrahepatic bile ductsAbnormal urinary electrolyte concentrationChronic kidney diseaseThickening of the tubular basement membraneAbnormal renal interstitial morphologyTubular luminal dilatationAgitationAggressive behaviorCirrhosisAscitesAnemiaEsophageal varixAbnormal cerebral white matter morphologyDiffuse cerebral atrophyCongenital hepatic fibrosisStage 5 chronic kidney diseaseAttention deficit hyperactivity disorderCarotid artery dilatationRenal atrophySuicidal ideation

Classification & Codes

Orphanet Code

ORPHA:84081
Senior-Boichis syndrome
OrphanetORPHA:84081
Treatments0 drug(s)
Symptoms on record34 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO