dr AMNA DALEEL PPT 2024.pptx PGR FCPS PART

long case presentation of nephrotic syndrome and theoretical explanation.

1. CASE PRESENTATION Dr AMINA DALEEL History taken on 10 th of June

2. History Patient profile:- Name: Miss Lubna Age: 28 years sex: female Marital status: married Occupation: housewife Address(R/O):Gujranwala Mode of admission(MOA): 0PD Date of admission(DOA): 10th of June 24 Time:11:00 am

3. PRESENTING COMPLAINTS B/L PEDAL SWELLING …..ON AND OFF LAST 2 MONTHS RECURRENT RT AND LEFT FLANK PAIN …SINCE 01 MONTH PAIN LEFT FLANK ---- 3 days

4. HOPC: According to my patient, she was doing well 2 months back when she traveled to Karachi from Gujranwala by air and suddenly developed massive B/L pedal swelling that is not painful, limited to feet settled spontaneously within a day. Then one month back (10 th May) she suddenly developed right flank pain of severe intensity, non-radiating, non-shifting continuous for many hours, pricking in character with no aggravating factor, and wasn’t relieved by multiple pain relieving injectables from a local hospital. Associated with pain was mild B/L pedal swelling and frothy urine.

5. In the same duration , she visited NHMC OPD got some home medication with some investigations advised , and went back home with follow-up advice after one week . Pedal swelling settled a lot with medications and then one month later on 8 th June(3 days back) she developed sudden severe left- flank pain severe In intensity continued for many hours, pricking in character non-radiating, non-shifting, with no aggravating factor not relieved by any pain relieving injectable. Associated history of frothy urine , and loss of appetite . HOPC:

6. No associated history of dysuria, hematuria, frequency of micturition, polyuria , oliguria , nocturia , the passage of gravel in urine , nausea, or vomiting No history o f fever and local skin changes i.e pigmentation No associated history of shortness of breath , cough , nocturnal dyspnea , chest pain, palpitations, or nocturnal dyspnea , weight loss , altered bowel habits , arthritis , or hematemesis , No History of Abdominal swelling , or yellowing of skin. HOPC:

7. Possible differentials?

8. Differential diagnosis 1 . kidney disease i.e (Nephrotic/nephritic) 2. CCF 3. LIVER DISEASE 4. Thyroid disorder 5. Malnutrition(protein-losing enteropathy) 6. DVT 7. Cellulitis 8. Varicose veins

9. Systemic review General weight gain/loss- sleep(disturbed sleep cycle)++ fever- Cvs Breathlessness - Palpitations - chest pain - Edema feet + Git Nausea - Vomiting - Abdominal pain+ Heartburn - Dysphagia - Diarrhea - Constipation - Hematemesis- Melena - Jaundice -

10. Respiratory system Cough - Sputum - Hemoptysis - Skin Rash ( BUTTERFLY RASH )- Itch - Colored spots - CNS : weakness - numbness- Tingling- headaches - Vomiting - Giddiness - Blackouts - Fits- Visual loss- Systemic review

11. Locomotor system Joint pain - Stiffness - Restriction of movements - Endocrine system Polyuria - Polydipsia - Heat or cold - intolerance Weight gain or loss - Systemic review

12. Past medical history Past surgical history Stroke – Fits – IHD- HTN- DM- ASTHMA- TB- HEPATITIS – INSIGNIFICANT

13. Drug history There was no history of taking Penicillins , Gold, NSAIDS, or CAPTOPRIL and OCPS, No history of any other medication Family history NO HX OF CARCINOMA in; PARENTS Sibling Children Rest of family history was insignificant

14. Personal history Allergy - Sleep has been disturbed for the last few months. Appetite - Blood transfusion - Recent teeth extraction - Addiction - Smoking - Alcohol - Travel history – No history of abortions Socioeconomic history Economic status: satisfactory Upper middle class Any pet at home:- nil

15. Menstrual history Age of menarche: 17 year Duration of period: 7 days Length of cycle:25 to 28 days Regularity of cycle: Regular Dysmenorrhea- Menorrhagia- Oligomenorrhea- Intermenstrual bleeding - Post-coital bleed - Menopause age. N/A Post menopausal bleed/discharge history

16. GPE:- An ill-looking young lady lying in the bed. She’s of normal height and built and was fully conscious and well-oriented in time and place. PULSE: 98 bpm .. REGULAR, GOOD VOLUME BP : 120/90 mmHg TEMPT: 98 F R/R: 16 b/m Spo2 : 97% at room air BSR: 102mg/dl

17. PALLOR –(CKD) ABSENT CYANOSIS - JAUNDICE –(CLD) ABSENT CLUBBING - KOILONYCHIA - SPLINTER HEMORRHAGES- LEUCONYCHIA – HALF AND HALF NAILS (CKD) ABSENT Tremors absent OSLERS NODES - HEBERDONS NODES - BOUCHARDS NODES - HAND DEFORMITIES - PALMAR SWEATING –(DCLD) ABSENT PALMAR ERYTHEMA – (DCLD) ABSENT DUPUTRYNs CONTRACTURES –(DCLD) ABSENT GPE:- Hands

18. FACIAL APPEARANCE NORMAL PERIORBTAL EDEMA – ( CCF, nephrotic syndrome ) PROPTOSIS - SKIN RASH –( SLE) PAROTID GLAND: NOT ENLARGED THYROID EXAMINATION NORMAL NECK VEINS: NOT ENGORGED JVP NOT RAISED(CCF,CLD) LYMPH NODES - AXILARRY NODES - INGUINAL NODES - ANKLE EDEMA: PRESENT ++PITTNG ( NEPHROTIC AND CCF, CLD) DEHYDRATION - GPE:- Face & NECK

19. LOCAL EXAMINATION OF B/L FEET INSPECTION B/L FEET SWELLING LIMITED TO FEET ERYTHEMA – NO VISIBLE DISCHARGE No skin changes like pigmentation PALPATION NOT WARM TO TOUCH NOT PAINFUL B/L FEET INVOLVED FORM PIT ON PRESSING (PITTING EDEMA)++ NO SUCH SIGNS OBSERVED

20. ABDOMINAL EXAMINATIONS : INSPECTION: SHAPE OF ABDOMEN: Normal MOVEMENTS OF ABDOMINAL WALL: Normal (abdominothoracic) UMBILICUS : CENTRAL PULSATION : NOT VISIBLE SCAR ..-absent STRIAE - absent Spider nivae : absent PROMINENT VEINS -absent HERNIAL ORIFICES – were intact

21. PALPATION Non-tender , no viscera was palpable KIDNEYS (MURPHY^S RENAL PUNCH)-was present +++ URNARY BLADDER - PERCUSSION There was no dullness and fluid thrill . Auscultation:- Bowel sound 3 to 5 /min of normal intensity Bruit was absent ABDOMINAL EXAMINATIONS : GROIN AND GENITALIA EXAM AND DRE - NOT DONE

22. CVS S1+ S2 +0 PALPATON: trachea central, chest expansion normal >5cm, vocal fremitus is equal on both sides. PERCUSSION: Normal finding. Auscultation: NVB, no added sound, vocal resonance equal on both sides GCS:15/15 RES CNS GPE:-

23. Differential diagnosis? 1 . CCF 2. LIVER DISEASE 3. NEPHROTIC SYNDROME 4 . Thyroid disorder 5. Malnutrition(protein-losing enteropathy) 6. DVT 7. Cellulitis

24. INVESTIGATIONS

25. Viral markers

26. 18 may 24

27. Another CT abdomen with contrast was done Showed left renal vein thrombosis and infective changes. Meanwhile on the 13 th of June renal biopsy was sent .

28. Treatment INJ Meropenem 1gram iv TDS was started and given for 5 days (Pt developed a few episodes of fever during hospital admission and was treated on line of pyelonephritis) Antibiotics from 10 th June to 15 June

29. Antifungal .. Inj Diflucan 100 mg iv bd Treatmen t

30. Antimicrobial .. injection flagyl 500 mg iv tds ..2 doses were given Fluids injections normal saline 1000cc at 100 ml/ hr Treatmen t

31. Analgesic ; Injection spasfon 40 mg Iv Tds Injection tramadol 10 to 20 mg Iv Tds Tab baclin ½ BD Tab gabica 50 mg HS OD Wintogen ointment L/A Antipyretics ; injection provas 1 gm iv tds Treatmen t:

32. Antithrombin;; injection clexane 40 mg s/c OD Oral anticoagulants ; tab apixaban 10 mg bd tab xeralto 15 mg bd for 21 days STEROIDS : injection SOLUMEDRAL 50 mg Iv OD Treatmen t: FOR HYPOALBUNEMA Injection albumin 20% OD for 3 days

33. Classification on basis of etiology

34. 1. Primary Glomerular disease Minimal change nephropathy Focal segmental glomerulosclerosis Membranous GN (MOST COMMON CAUSE N ADULTS >40 YR)

35. Secondary glomerular disease ASSOCIATED WTH SYSTEMIC DISEASE Diabetic nephropathy Amyloidosis Drugs: penicillamine, gold, mercury, cadmium Allergic reactions: allergy to poison Ivy, pollens, bee stings, cow milk.

36. Membranous GN Causes; 1… IDIOPATHIC MOSTLY 2… INFECTIONS : Hepatitis B and C, Syphilis, malaria, leprosy, filariasis hydatid cyst, endocarditis 3… TUMORS carcinoma of Renal cell, bronchogenic, thyroid, breast, melanoma 4 … AUTOIMMUNE DISEASE 5… DRUGS:- gold, penicillamine, captopril, NSAIDS 6… MISCELLANEOUS : Sarcoidosis Sickle cell disease, Crohn’s disease, Fanconis syndrome guillain Barre syndrome

37. CLINICAL FEATURES Edema Malnutrition Features of underlying causes like SLE and DM Hypercoagulibility Infections

38. INVESTIGATIONS URINE ANALYSIS …PROTEINURIA 24 HOURS URINARY PROTEIN …..>3G/DAY SERUM ALBUMIN ….less than <3g/dl and total protein < 6 mg/dl Low-density lipoprotein : LDL INCREASED BUT HDL usually normal Raised ESR due to increased serum fibrinogen activity

39. Cause-related: Blood sugar for diabetes HBA1C ANA for SLE Hep B serology Serum complements Serological testing for syphilis, HIV INVESTIGATIONS Age and risk-appropriate cancer screening An elevated titer of circulating PLA2R antibodies , diagnostic for primary membranous nephropathy, eliminated the need for biopsy Renal biopsy

40. When not to do a Biopsy ? Kidney biopsy is usually performed in adults with new-onset idiopathic nephrotic syndrome of a primary renal disease that may require immunosuppressant therapy is suspected Chronically and significantly decreased GFR indicates irreversible kidney disease mitigating the usefulness of kidney biopsy In the setting of long-lasting DM+ type 1 or 2, proteinuric renal disease is rarely biopsied unless atypical features such as significant glomerular hematuria or cellular casts are also present or if other reason to suspect additional renal lesions.

41. Treatment Secondary cause must be considered before consideration of treatment. Primary disease treatment depends on the risk of renal disease progression. Most have a good prognosis with conservative management, including antiproteinuric therapy with ACE inhibitors or ARBS of blood pressure over 125/75 mmHg.

42. Spontaneous remission may develop even with heavy proteinuria in about n 30 % of cases. Then the use Of immunosuppressive agents should be limited to those at high risk of progression and with salvageable renal function Treatment

43. Treatment Patients with nephrotic syndrome despite 6 months of conservative management and serum creatinine less than 3 mg/dl may elect therapy with rituximab or with corticosteroids and cyclophosphamide for 6 months Calcineurin inhibitors with or without corticosteroids may be considered well Patients with primary membranous nephropathy are excellent candidates for renal transplant

44. Edema : Dietary salt restriction Combination of loop and thiazide diuretics for refractory fluid retention . Treatment

45. Protein loss : Dietary protein restriction Anti- Proteinuric therapy ACE inhibitors ARBS Mineralocorticoid receptors antagonists SGLT2 inhibitors Treatment

46. Treatment Hyperlipidemia (hypertriglyceridemia and hypercholesterolemia) Dietary modification and exercise Lipid-lowering drugs Treatment

47. Hypercoagulable state: Warfarin therapy for at least 3 to 6 months Treatment

48. Outpatient therapy mostly For edema refractory to medications or worsening kidney function required admission . Treatment