CASE A 58-year-old woman, office worker CC : fatigue and weakness

Transcription

1 CASE A 58-year-old woman, office worker CC : fatigue and weakness

2 HISTORY OF PRESENT ILLNESS About 7 days before admission, she lost her appetite and had a sensation of thirst. She had difficulties to eat foods. 2 days before admission, she felt fatigue and weakness. Weakness progressively involved extremities with diffuse mild muscle pain, limiting her ability to walk. She became bedridden and required assistance with activity of daily living. Fatigue got gradually worse, and she was brought to private clinic by her family. After that she was transferred to our hospital. She didn t have numbness nor tingling. Also, she didn t have fever, diarrhea and vomiting.

3 PAST MEDICAL HISTORY # 48 y. o. hyperthyroidism # 40 y. o. lt partial mastectomy for cancer # TAH for uterine leiomyoma # in childhood chronic otitis media with cholesteatoma # depression? Anxiety disorder from 6 months ago Medication Propylthiouracil 50 mg 1T/1 Atorvastatin calcium hydrate 5 mg 1T/1 Etizolam 0.5 mg 3T/3

4 Family history Nothing particular. Social history desk work never smoke never drink

5 Physical examination BP 110/70 mmhg, PR 80 /min, RR 20 /min, BT General:slightly sick. Cons:alert. HEENT:not anemic, not icteric, dry mouth(+), caries of the teeth(-). Neck:no lymphadenopathy, no goitor. Heart:regular rhythm without murmur. S1( ) S2( ) S3(-) S4(-) Chest:CTA. Wheeze(-). Crackle(-). Abdominal: soft & flat. Bowel sound normal. Back: CVA knock pain(-). Ext:warm. no edema. mild tenderness of the lower limbs. Skin:slight dry. no rash. no redness.

6 Neurologic examination Cranial nerves were intact grade 4-/5 weakness of bilateral upper and lower extremities Sensory examination was normal Tendon reflexes are normal

7 Laboratory studies Na (meq/l) 142 WBC (µ/l) 8300 K (meq/l) 1.8 RBC (µ/l) Cl (meq/l) 112 Hb (g/dl) 12.2 Mg (mg/dl) 3.0 Hct (%) 35.6 Ca (mg/dl) 9.2 MCV (fl) 92 BUN (mg/dl) 23 Plt (µ/l) Cre (mg/dl) 1.1 AST (IU/l) 53 TSH (µiu/l) ALT (IU/l) 27 f-t3 (pg/ml) 2.85 LDH (IU/l) 251 f-t4 (µg/dl) 1.19 T-Bil (mg/dl) 0.7 CK (IU/l) 2313 CRP 0.44

8 Laboratory studies Arterial blood gas Bilirubin (-) ph Urobilinogen (-) PCO2 (mmhg) 28.4 ketone (-) po2 (mmhg) 96.1 U-Na (meq/l) 57 HCO3 (meq/l) 15.1 U-K (meq/l) 29.8 Anion gap 15 U-Cl (meq/l) 63 U-Ca (mg/dl) 3.2 urine U-Cr (mg/dl) 65 Specific gravity ph 7.0 urinary sugar (-) proteinuria (-) urinary blood (-)

9 ECG Sinus rhythm. Prolong QT(U) complex,.

10 胸部 Xp KUB Lung field : clear. CP angle sharp. CTR : 52% Renal calculus (-). Urinary calculus (-).

11 Problem lists # weakness / bilateral upper and lower extrimities # fatigue # dry mouth # mild muscle tenderness # hypokalemia (1.8 meq/l) # CK, CRP increased # metabolic acidosis # ECG : U wave (+)

12 Differential diagnosis of weakness Vascular spinal cord disease Infection sepsis, extradural abscess, infectious myositis Autoimmune vasculitis, PM/DM,, Guillain-Barre syndrome, etc. Metabolic electrolyte abnormality, Periodic Paralysis, DM, vitb deficiency,etc. Neoplastic spinal cord neoplasm, paraneoplastic neurological syndrome Idiopathic amyloidosis, etc.

13 Major causes of hypokalemia Decreased potassium intake Increased entry into cells An elevation in extracellular ph increased availability of insulin Elevated β-adrenergic activity hypokalemic periodic paralysis marked increase in blood cell production hypothermia chloroquine intoxication increased gastrointestinal losses vomiting diarrhea tube drainage laxative abuse

14 Major causes of hypokalemia increased urinary losses diuretics primary mineralocorticoid excess loss of gastric secretions nonreabsorbable anions renal tubular acidosis hypomagnesemia amphotericin B salt-wasting nephropathies - including Batter s or Gitelman s syndrome polyuria Increased sweat losses dialysis plasmapheresis

15 hypokalemia algorithm

16 Difference diagnosis s/o hypokalemic paralysis s/o rhabdomyolysis s/o hyperthyroidism r/o periodic paralysis r/o vomiting, diarrhea r/o drug, diuretics

17 differential diagnosis of RTA RTA type Ⅰ RTA type Ⅱ RTA type Ⅳ This case K (meq/l) low low high 1.8 TTKG >2 >2 < Urine ph (acidosis 下 ) Urine NH4+ excretion Urine Ca (female 9 328) >5.5 <5.5 Not low value 7.0 low Low ~ normal low Not exam Increase Normal Increase 49mg/g creatinine FE HCO 3- (acidosis on corrected) <5% >15% <10% 1.7% Serous Aldosterone Normal ~ high Normal ~ high Low ~ Normal 84.4pg/mL (in restless ) GFR Normal Normal Slight low normal Renal calculus frequent rare rare (-) replacement dose of HCO3- small amount By case small ~ moderate amount Shohl liquid Necessary of K replacement frequent occasionally Intake restriction replacement

18 Clinical course 6 5 Potassium Citrate Shohl liquid Potassium l-aspartate mg/dl MMT : grade labial minor salivary gland biopsy 0 day1 day2 day3 day4 day5 day6 day7 day9 K MMT ph HCO

19 labial minor salivary gland There are many lymphocyte (>500/4mm2) lymphocytes invade salivary glands compatible for Sjogren synd.

20 Following examination Anti SS-A antibody >500 IU/ml Anti SS-B antibody <7.0 IU/ml anti TPO antibody <5 IU/ml anti Tg antibody <10 IU/ml ANA <40 RA quantity 44 C3 100 mg/dl C4 28 mg/dl β2 microgloblin 3.8 mg/dl Urine β2 microgloblin µg/l NAG 6.0 U/l IgG 1176 mg/dl IgA 229 mg/dl IgM 66 mg/dl Anti mitochondria 2 antibody negative RTA type Ⅰ, primary Sjogren syndrome

21 RTA RTA is a syndrome of disordered renal acidification, either primary or secondary, resulting in hyperchloremic metabolic acidosis and excessive renal loss of potassium. Patients with distal RTA (RTA type Ⅰ) commonly present with symptoms of renal stones, but first abnormality might be symptoms of hypokalemia and hypocalcemia. Thirty to forty percent of Sjogren syndrome patients have symptomatic and asymptomatic renal involvement. Renal calculi, renal tubular acidosis, and osteomalacia can occur secondary to tubular damage caused by interstitial nephritis, the most common form of renal involvement in Sjogren syndrome.