<prompt> A13year old boy is referred for evaluation of nocturnal enuresis and short stature. His blood pressure is normal. The hemoglobin level is 8gdl.urea 112 mgdl, creatinine 6 mgdl, sodium 119 mEq dl, potassium 4 mEql, calcium 7 mgdl, phosphate 6 mgdl and alkaline phosphatase 300 UI. Urinalysis shows trace proteinuria with hyaline casts; no red and white cells are seen. Ultrasound shows bilateral small kidneys and the micturating cystourethrogram is normal. The most likely diagnosis isAlports syndromeMedullary sponge kidney.Chronic glomerulonephritis.Nephronophthisis  <response>: Features seen in this 13 yr. old boyShort StatureNocturnal enuresisNormal B.P.Reduced hemoglobinHyponatremia N level in 136-145 meqdlHypocalcemia N level is 9-10.5 mgdlNormal potassium level N is 3.5 to 5 meqdlBL small kidneysIncreased blood urea N is 10 - 40 mgdlIncreased alkaline phosphates N is upto 130 IUIncreased serum creatinine N is 1.5 m gdlThis pt. gives a classical presentation of nephronophthisis, About other optionsAlport syndromeIt can be easily ruled out as it presents withMicroscopic hematuria first symptom Proteinuria Sensorineural hearing loss bl anterior lenticonus Medullary sponge kidney Easily ruled out as the kidneys are normal or increased is size in MSK and also the age of presentation is third or fourth decade.Chronic glomerulonephritispts of CGN usually have heavy proteinuria, frank or occult hematuria and hypertension. 