CASE CONFERENCE Ischemic Stroke with Nephrotic Syndrome

Chief complaint - Rt. Side weakness and numbness onset > 내원 1 일 전 Present illness 평소 특이 병력 없던 환자로 내원 하루 전부터 발생한 우측 하지의 weakness 및 이상 감각을 호소하여 본원 한방병원으로 입원 2 개월전부터 소변에서 거품뇨가 자주 관찰되었다고 말하였으나 evaluation 하지는 않은 채로 지내왔으며 한방 병원에서 입원 치료 도중 발견된 단백뇨에 대한 further evaluation 위하여 신장내과로 전과됨 Youn H.D. M/45

Past medical history - DM / Hypertension / Tb / Hepatitis (-/-/-/-) Onset Acute cerebral infarction, Parietal lobe, Left - Op Hx (-) - Drug allergy (-) Family history (-) Personal history Alcohol (-) Smoking : 20-pack-year quit 5 years ago

Review of Systems General : fatigue (+) fever (-) chills (-) night sweat (-) weight gain (+) +6Kg during recent 1 week [60 kg  66 kg] Skin : rash (+) - both lower extremity, almost healed state Head / Neck : headache (-) sore thraot (-) Respiratory : cough (-) sputum (-) dyspnea (-) cyanosis (-) Cardiac : orthopnea (-) chest pain (-) palpitation (-) GI : A/N/V/D/C (-/-/-/-/-) melena (-) abdominal pain (-) Musculoskeletal : numbness of Rt. Lower extremity (+)

Physical Examination Vital sign 110/70 mmHg – 75/min – 22/min – 38.3 °C General appearance - Alert mentality - Chronically ill-looking appearance Skin - desquamated skin pigmentation : purplish color on sole, both Head & Neck - No cervical / supraclavicular lymph node enlargement - Neck vein engorgement (-)

Eyes and ENT - Isocoric pupils with pupilary light reflex (++/++) - Whitish sclera - Pinkish conjunctivae Chest - Clear breath sounds without crackle or wheezing - Regular heart beats without murmur Abdomen - Soft and flat abdomen - Normoactive bowel sound - No tenderness or rebound tenderness - No palpable abdominal mass

Back and Extremities - CVA tenderness ( - / - ) - Pretibial pitting edema ( + / + ) Neurology - Motor power Sensory intact VV IV + V

Initial Lab Results CBC/DC 4,060/mm 3 – 11.0 g/dL – 32.0 % - 129,000/mm 3 Chemistry TB/DB 0.60 / 0.05 mg/dL ALP 56 U/L LD 562 U/L Ca 6.2 mg/dL Prot/Alb 3.4 / 0.9 g/dL AST/ALT 22/13 U/L P 2.5 mg/dL BUN/Cr 8 /1.1 mg/dL Na/K/Cl 127/3.0/107 mmol/L UA RBC 2~4 WBC 2~4 Prot 3+ Lipid profile T-cholesterol 226 mg/dl HDL 21 mg/dl LDL 145 mg/dL TG 126 mg/dl

Chest X-ray

Initial Problem Lists I.Proteinuria Pitting edema Hypercholesterolemia Hypoalbuminemia II.Acute cerebral infarction III.Fever History of skin rash?

Assessment & Plan I.Nephrotic syndrome - 24 hrs urine protein excretion - renal biopsy for specific diagnosis - seroloy for chronic GN - ACE inhibitor - ARB - dietary modification II.Acute cerebral infarction - risk factor evaluation and correction - antiplatelet agent with or without anticoagualtion III.Fever of Unknown Origin

Nephrotic syndrome 24 hrs Urine Excretion Protein 7.1 g/day CrCl ml/min Hypercholesterolemia Hypoalbuminemia Generalized edema  Renal biopsy ( ) : Membranous glomerulonephritis - Diffuse ribbon like thickenings of the capillary walls with 2 global sclerosis and frequent short spike formations(silver stain) - The interstitium and tubules show patchy chronic inflammatory cell infiltration with some atrophic tubules - IF) G(+) Cq1(+) C3(+) (4/4) Diffuse fine granular deposits along the capillary walls

Serology ANAPositive : speckled type ANCANegative C mg/dl ↓ C 47.2 mg/dl ↓ CryoglobulinNegative Hepatitis B / C markerNegative VDRLNegative

Kidney US RK 11.7 cm LK 12.6 cm - Increased Both renal echo

Cerebral infarction Risk factor - smoking (-) - cardiac thromboembolic risk : Echocardiography – no cardiac thrombus or valve disease - EKG : within normal limits no history of AF or paroxysmal AF - hypercoagulable state PT/INR 13.9 sec / 1.07 aPTT 45 sec Antithrombin-III 66% Fibrinogen 429 mg/dl  possibly associated with nephrotic syndrome

Brain MRI & MRA Acute focal infarction at the left parietal lobe Unremarkable brain MRA and both carotid bifurcation area

Fever Broad-spectrum antibiotics : Ceftriaxone  Piperacillin/tazobactam Painful lymphadenopathy, Rt. Axilla - axillary sono-guided needle aspiration Non-specific reactive lymphadenopathy, both axillae and cervical regions(right supraclavicular zone) LN FNA : some large and small lymphocytes without malignant cells CRP 0.2 ESR 26 leukocytosis (-) Culture all negative Negative serology of Toxoplasma, EBV, CMV, HIV Spontaneous regression of fever and LN tenderness

Fever recurrence Associated symptoms and signs S > chilling and febrile sense (+) sputum / cough (-/-) O> clear lung sound, pharyngeal injection (-) purplish rash on palm and sole with mild tenderness Luekosytosis (-) CRP 0.1 mg/dl ESR 30 mm/hr BT 38~39.5 ℃ A > FUO probably due to non-infectious cause P > skin biopsy anti-pyrectics

Conclusion Acute ischemic CVA associated with underlying nephrotic syndrome - no obvious underlying cause except hypercoagulable state : increased level of fibrinogen - established risk factor of stroke Treatment Plan - general mangement of nephrotic syndrome - Steroid or immunosuppressant - antiplatelet agent - Need of anticoagulation therapy ?