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A Lady with Renal Stones

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A Lady with Renal Stones A lady with renal stones Dr KC Lo, Dr KY Lo, Dr SK Mak KWH History 53/F NSND, NKDA Good past health Complained of bilateral loin pain for few years No urinary symptoms/UTIs No haematuria Not on regular medications/vitamins No significant family history History Attended private practitioner in Feb, 2006: Blood test : Na/K 143/3.9 Ur/Cr 7.3/101 LFT N Urine test : RBC numerous/HPF WBC 5-8/HPF CXR unremarkable Given analgesics History Still on-and-off bilateral loin and lower chest pain Seek advice from Private Hospital: Blood test: WBC 3.2 Hb 12.9 Plt 139 Na/K 146/ 3.0 Ur/Cr 6.3/108 Ca2+/PO4 2.11/1.39 LFT unremarkable Urine test : RBC 6-8/HPF, WBC 0-1/HPF no cast KUB: bilateral renal stones (as told by patient) History ESWL done to right renal stone in 5/06, planned to have ESWL to left stone later But she then defaulted FU History This time admitted to our surgical ward complaining of similar bilateral lower chest wall pain (for six months) Had vomiting of undigested food 8 times per day for 1 day, no diarrhoea No fever Recent intake of herbs one week ago Physical exam BP 156/77 P 68 afebrile Hydration normal Chest, CVS unremarkable Local tenderness over bilateral lower chest wall Abdomen soft, mild epigastric tenderness, no rebound and guarding KUB Multiple tiny calcific densities projecting in bilateral renal areas with apparent distribution of the renal medulla bilateral medullary nephrocalcinosis CT Scan 1 yr ago in private CT Scan 1 yr ago in private Investigations WBC 3.1 HB 13.1 Plt 137 Na 137 K 2.3 Cl 112 Ur 4.7 Cr 99 Ca 2.18 PO4 0.5 LFT normal, serum albumin 43 ABG : pH 7.28 pCO2 4.4 HCO3 15.3 BE -10.2 MSU G –ve A trace RBC 1-4/HPF C/ST no growth What next? Anion gap Na – Cl – HCO3 = 137 – 112 – 15.3 = 10 Normal anion gap metabolic acidosis Expected pCO2 = 0.1333 X (1.5(15.3)+8[ ± 2]) = 3.0-4.4 kPa What next? Urine anion gap Urine Na 42 K 17.5 Cl 45 Urine anion gap = Na + K – Cl = 14.5 What other lab data would you like to know? Urine pH 7.0 Dx : distal renal tubular acidosis Investigations PTH 1.2 pmol/L (N 1.6 -6.9 pmol/L) 24hr Ur TP 1.23g/d, CrCl 42ml/min 24hr Ur Ca 2.64mmol/d (N: 2.5 -7.5 mmol/d ) UPE and SPE : no monoclonal band detected Serum Vitamin D level : 25 OH 13.4 ug/L (N >10) Investigations USG abdomen : Bilateral renal medulla appear hyperechoic with shadowing seen, consistent with medullary nephrocalcinosis, no hydronephrosis No splenomegaly Investigations Skeletal survey : No Looser ’s zone Reduced bone density in the lumbrosacral spine with loss of lumbar lordosis Herbal medicine formula Investigations The herbal formula were sent to Toxicology Reference laboratory for analysis those ingredients ( 桑寄生,泰艽,川芎,白芍, 太子參, 三七, 薑黃, 元胡, 鬱金,佛手,香附,黃著, 甘草,雞血滕,大黃, 火麻仁, 枳實, 厚樸,甜杏仁,續 斷, 絡石藤) are not known to cause metabolic acidosis and hypokalaemia In summary The patient has distal renal tubular acidosis (type 1) with bilateral medullary nephrocalcinosis What is the cause ? Major cause of Type I RTA in adult Primary Secondary Sjogren ’s Syndrome Idiopathic, sporadic Rheumatoid arthritis Familial SLE AD/AR Hypercalciuria Hyperglobulinaemia Cirrhosis Sickle cell anaemia Amphotericin B Lithium carbonate Ifosfamide Renal transplantation Obstructive uropathy On further questioning She complained of dry eyes for many years and took regular eye drops bought over the counter Difficulties in swallowing biscuits or bread for one year Schirmer’s test +ve Further Investigations ANA 1280 Anti-ds DNA <10 Ig G 16.7 (N 7.4 – 15.5) Ig A 5.10 (N 0.82 – 4.53) Ig M 0.98 (N 0.46-3.04) C3 0.65 (N 0.79-1.52) C4 0.31 (N 0.16-0.38) RF +ve TFT normal BMA and trephine : no malignancy Anti-ENA screen Anti-Ro (SS-A) +ve Anti-La (SS-B) –ve Anti-RNP –ve Anti-SM –ve Anti-Scl-70 –ve Anti -Jo-1 –ve Anti-other -ve Progress Ophthamologist : moderate dry eyes Artificial eyedrops and ointment Progress She was treated with iv potassium supplement and then Put on Potassium citrate 2g bd Sodium bicarbonate 900mg tds In summary The patient has distal renal tubular acidosis (type 1) with bilateral medullary nephrocalcinosis due to Sjogren ’s syndrome Discussion Nephrocalcinosis/nephrolithiasis in distal renal tubular acidosis Renal disease in Sjogren ’s Syndrome Aggressive treatment ? Alkali therapy in dRTA Nephrocalcinosis/ Nepholithiasis Nephrocalcinosis Deposition of calcium within the renal parenchyma Classify according to the anatomic area involved : Medullary nephrocalcinosis Cortical nephrocalcinosis Common cause of medullary nephrocalcinosis Hyperparathyoridism Nephrotoxic drug Distal RTA (amphotericin B, outdated tetracycline) Bartter syndrome Primary hyperoxaluria Bone metastasis Renal tuberculosis Chronic pyelonephritis Sarcoidosis Cushing syndrome Sickle cell disease Hypo/hyperthyroidism Vitamin D excess Idiopathic hypercalcaemia Medullary sponge kidney Common cause of cortical nephrocalcinosis Acute cortical necrosis Alport syndrome Chronic glomerulonephritis Chronic hypercalcaemic states Ethylene glycol poisoning Oxalosis Rejected renal transplant Sickle cell disease Workup in nephrocalcinosis Blood Na, K, Cl, blood gas analysis PTH (in case of increase calcium levels) Vitamin D and metabolites Urinalysis Urine pH (measurement after each voiding, minimum 4x/day) 24hour urine (2 collections) Volume, urine pH, specific weight, Ca2+, PO4, oxalate, uric acid, citrate, magnesium Nephrolithiasis in renal tubular acidosis Frequently seen in dRTA but rare in pRTA Nephrolithiasis in distal renal tubular acidosis Hypercalciuria Persistent acidaemia was buffered by bone calcium carbonate release of calcium in urine Increase PTH increase release of bone calcium Increase circulating H+ ion concentration reduce negative charges on serum proteins more free form Ca2+ circulates more for glomerular filtration Acidosis induced inhibition of tubular calcium reasborption (unknown mechanism) Nephrolithiasis in distal renal tubular acidosis persistently high urine pH favors the precipitation of calcium phosphate Hypocitraturia acidemia enhances proximal citrate reabsorption and its metabolism Not related to low serum citrate concentration or hypokalemia Citrate is a potent stone formation inhibitor both by forming a soluble complex with calcium and by inhibiting stone growth by agglomeration of calcium crystals Often improves only modestly with alkali therapy Nephrolithiasis in distal renal tubular acidosis Amplify the acidification dysfunction By impairing the transfer of NH 3 from the LH to the collecting duct Recurrent pyelonephritis common Can be difficult to treat Renal disease in Sjogren ’s Syndrome Prevalence varied widely, ranging from 2 to 67 % Renal disease in Sjogren ’s Syndrome 20 out of 471 (4.2%) patient showed overt renal involvement : Proteinuria > 500 mg /d Serum creatinine > 140 umol/L Active urinary sediments CrCl < 50ml/min Evidence of distal RTA recurrent renal colic with imaging findings of urolithiasis or nephrocalcinosiasis Fanconi syndrome without any other identifiable cause 18 patients Bx done out of 20 such patients Clinically significant and biopsy-documented renal involvment in primary Sjogren ’s syndrome Goules : Medicine (Baltimore), Vol 79(4) July, 2000. 241-249 Renal disease in Sjogren ’s Syndrome -- Histopathology Chronic interstitial nephritis (10/18) Presence of small lymphocytes, plasma cells and monocytes in the interstitium combined with tubular atrophy and fibrosis Clinical presentation : RTA type I with/without acidaemia Impairment in urine concentrating ability Fanconi syndrome Clinically significant and biopsy-documented renal involvment in primary Sjogren ’s syndrome Goules : Medicine (Baltimore), Vol 79(4) July, 2000. 241-249 Renal disease in Sjogren ’s Syndrome -- Histopathology Glomerulonephritis (9/18) Mesangial proliferative (5/9) Proliferation of mesangial cells and matrix Clinical presentation : mild haematuria, proteinuria, hypertension Membranoproliferative (4/9) Diffuse proliferation of mesangial cells and infiltration of glomeruli by macrophages, increased mesangial matrix, thickening and reduplication of GBM Clinical presentation : Variable combinations of nephritic and nephrotic syndrome with active urine sediment Moderate proteinuria Acute decline in GFR Clinically significant and biopsy-documented renal involvment in primary Sjogren ’s syndrome Goules : Medicine (Baltimore), Vol 79(4) July, 2000. 241-249 Renal Outcome over a 15 yr FU Glomerulonephritis (8 patients): 2 progressed to ESRF Mixed monoclonal cryoglobulinaemia more frequently observed (p = 0.023) Interstitial Nephritis (10 patients): 4 patients had mild to moderate impairment in renal function, but no evidence of deterioration during follow up, no RRT required younger at disease onset (36.8 +/- 11.9 vs 46 +/- 7.07) ( p = 0.063) developed earlier during the course of disease (2.2 +/- 3.2 vs 8 +/- 5.5yr) (p = 0.001) Clinically significant and biopsy-documented renal involvment in primary Sjogren ’s syndrome Goules : Medicine (Baltimore), Vol 79(4) July, 2000. 241-249 Distal Renal Tubular Acidosis in Sjogren ’s syndrome mechanism incompletely understood immunocytochemical analysis complete absence of the H-ATPase pump in the intercalated cells in the collecting tubules that is largely responsible for distal proton secretion but mechanism of immune injury unknown high titers of autoantibody directed against carbonic anhydrase II
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