Table 1.

Clinical data for the cases

Case	Current Age (Years “y”)	Gender	Affected Relatives	Age at Diagnosis	UTI	HTN	Renal Stones	Medical Treatment	Urology Intervention	Note
Case 1	10 y	female	Father, Two sisters, brother	Birth	Yes	No	Bilateral	Tiopronin and potassium citrate	Percutaneous Nephrolithotomy Shock wave lithotripsy	Short Factor V deficiency in affected family members
Case 2	21 y	male	sister	Birth	No	No	Bilateral	Potassium citrate Tiopronin	Right nephrectomy Percutaneous Nephrolithotomy
Case 3	23 y	Female	brother	12 years	No	No	Right side	Potassium citrate Tiopronin	ESWL
Case 4	10 y	Female	none	3 years	Yes	Yes	Bilateral	Tiopronin potassium citrate & Amlodipine Lisinopril	Percutaneous Nephrolithotomy
Case 5	29 y	Female	Paternal Cousin	18 months	No	Yes	Bilateral	Tiopronin Potassium Citrate Enalapril	Percutaneous Nephrolithotomy
Case 6	15 y	Male	Grandmother	10 years	No	Yes	Bilateral	Penicillamine and potassium citrate	Percutaneous Nephrolithotomy	Vitiligo
Case 7	11 y	Female	Grandmother Brother, sister	10 years	no	No	Unilateral	Penicillamine and potassium citrate	Waiting for Percutaneous Nephrolithotomy
Case 8	16 y	Male	Grandma Brother and sister	14 years	no	no	unilateral	Penicillamine and potassium citrate	none	Right-sided undescended testis

UTI Urinary tract infection and HTN Hypertension