Frequent Relapsing Nephrotic Syndrome

Case presentation of frequent relapsing nephrotic syndrome

1. CASE PRESENTATION ON Frequent Relapsing Nephrotic Syndrome FLEMIN THOMAS, 11Q0407

2. Nephrotic syndrome Important chronic disease in children . Nephrotic syndrome is a kidney disorder that causes body to excrete too much protein in urine. The glomeruli are affected by an inflammation or hyalinization that allows proteins such as albumin, antithrombin or immunoglobulins to pass through the cell membrane and appear in urine “Minimal change disease” can usually be treated with drug prednisone, but this type of nephrotic syndrome can come back. this is called “relapse”.

3. It is a nonspecific kidney disorder characterized by three signs of diseases: Large proteinuria: loss of protein through the kidneys Hypoalbuminemia : low albumin levels in the blood Edema : water to be drawn into soft tissues

4. Causes Nephrotic syndrome is caused by different disorders that damage the kidneys. This damage leads to the release of too much protein in the urine. The most common cause in children is minimal change disease. Membranous glomerulonephritis is the most common cause in adults. This condition can also occur from: Cancer Diseases such as diabetes, systemic lupus erythematosus, multiple myeloma, and amyloidosis Genetic disorders Immune disorders Infections (such as strep throat, hepatitis, or mononucleosis) Use of certain drugs(penicillin ,captopril , gold salt)

5. Nephrotic syndrome can affect all age groups. In children, it is most common between ages 2 and 6 . This disorder occurs slightly more often in males than females.

6. Symptoms Swelling (oedema) is the most common symptom. It may occur: In the face and around the eyes (facial swelling) In the arms and legs, especially in the feet and ankles In the belly area (swollen abdomen ) Other symptoms include: Foamy appearance of the urine Poor appetite Weight gain (unintentional) from fluid retention

7. PATIENT DETAILS PATIENT NAME:XYZ AGE: 5 WEIGHT : 18.1Kgs IP.NO: 204 DEPT: pediatrics (PICU) UNIT:C DOA: 02 - 01 – 2016 DOD : 5-01-2016

8. REASON FOR ADMISSION: c/o fever since 7 days , cough since 3 days , puffiness of face and leg swelling since 5 days

9. History of present illness Child was apparently alright one week back ,then he developed fever which was mild to moderate grade not associated with chills ,intermittent type. c/o facial swelling and lower limb swelling since 5 days it was insedious in oneset ,progressive. Cough since 3days ,non productive type, no h/o hurried breathing and no h/o loose stools

10. Past medical history k/c/o Nephrotic syndrome is diagnosed at the age of 1 year old and is on medication during the episodes. h/o frequent similar complaints every 6 months in one year of span.

11. FAMILY HISTORY: No h/o similar complaints in the family members No h/o TB, epilepsy in the family members. Immunization : BCG+ DPT 1 2 3+ – booster dose not given Measles vaccination+

12. GPE/SE Pulse +++/++, 110bpm BP- 90/54mmhg RR- 30cpm Afebrile CVS – s1s2+ no murmur RS- B/L AEE, B/L crepts+ CNS: intact P/A :soft, nontender no organomegaly

13. 13 LABORATORY DATA HEMATOLOGY Investigation Normal range 30-12-15 Hb (13.5-17.5g/dl) 9.8 ↓ CBC: WBC (4,500-10,500cells/ uL ) 11,600 DLC: Polymorphs (37-73%) 74 Basophils (0-1%) 00 Eosinophils (0-5%) 00 Lymphocytes (20-40%) 26 Monocytes (0-7%) 00 RBC (4.7-6.1million/ uL ) 5.01 ↓ Platelets (150,000-450,000cells/mm3) 351000 ↓

14. ELECTROLYTES Investigation Normal range 31-12-15 1-1-16 Sodium (135-147mEq/L) 128 130 Potassium (3.5-5.2mEq/L) 2.7 3.4 Chlorides (95-107mEq/L) 93 99 ESR 75mm PS study : microcytic hypochromic anemia with relative neutrophilia

15. Albumin (3.5 -5.2gm%) : 1.5 gm% Sr. urea : 73.0gm% Sr.creatinine : 0.4mg/dl Urine routine test – albumin present

16. TREATMENT CHART BRAND NAME GENERIC NAME DOSE R FQ. 1 2 3 4 5 IVF DNS@56ml/hr with 10 cc kcl y 5cc kcl Inj.Augmentin Amoxacillin+clavulanic acid 550mg iv tid y y y y y Neb. A sthalin salbutamol qid y y y y y Syp . Pacimol DS paracetamol 5.5ml po tid y y y Y Tab. Lasilactone Furosemide+spirinolactone 20/50mg po ½-0-½ y y y y Tab.Amlong amlodipine 0.2mg po ½-½-0 y y y Inj. Levoflox Levofloxacin 36ml iv od y y y Inj.vit k Vitamin k 1 amp 1mg OD po y y y

17. DAY 1: O/E: pt is afebrile. PR:110 bpm. BP:90/60 mmhg. RS- B/L crepts+ P/A:soft, distented,no organomegaly.

18. DAY 2 c/o cough, oedema + BP- 112/80mmhg Pulse-98bpm Rr-30cpm Urine albumin+ P/A: soft ,nontender ,no organomegaly UO – 2.2ml/kg/hr

19. DAY 3 : Pulse: 98bpm. BP: 112 /80 mmHg. NFC O/E CVS- S1S2 present. RS- B/L crepts+ P/A- Soft, nontender,no organomegaly . UO- 2.1ml/kg/hr

20. DAY 4 Pulse: 120bpm BP:130/90mmhg RS-B/L crepts+ UO- 3.7ml/kg/hr P/A: soft, non tender,no organomegaly

21. Day 5 Patient stabilized NFC BP-110/90mmhg Pulse- 100bpm Edema reduced, cough reduced All vitals stabilized Adv. For discharge

22. Discharge medications Brand name Generic name dose Route frequency Nos. Tab.amlong Amlodipine 0.2mg po ½-½-0 7 Syp.pacimol paracetamol sos Advised salt restricted diet. Review after 7 days in paediatric OPD.

23. PHARMACEUTICAL CARE PLANS PUFFINESS OF FACE AND LEG SWELLING PITTING EDEMA OVER THE LEGS. ABDOMINAL DISTENTION k/c/o Nephrotic syndrome Hb - 9.8g/dl ESR-75mm/hr WBC-11,600cells/ uL Albumin -1.5gm% Sr.urea -73gm% SUBJECTIVE EVIDENCES OBJECTIVE EVIDENCES

24. ASSESEMENT: By observing the subjective and objective evidence it is diagnosed as frequent relapsing nephrotic syndrome.

25. THERAPEUTIC GOALS: To reduce the signs and symptoms such as pitting edema and facial puffiness. To reduce the cough.

26. MONITORING PARAMETERS: To monitor BP,PULSE RATE, urine output. To monitor the albumin level in blood and infections .

27. Standard recommendation Managing Children With Nephrotic Syndrome. Diet : A balanced diet, adequate in protein (1.5-2 g/ kg ) and calories is recommended. Patients with persistent proteinuria should receive 2-2.5 g/kg of protein daily. Edema: Patients with persistent edema and weight gain of 7-10 % are treated with oral frusemide (1-3 mg/kg daily ). Additional treatment with potassium sparing diuretics is not required if frusemide is used at this dose for less than one week. Patients requiring higher doses and prolonged duration of treatment with frusemide should receive potassium sparing diuretics , e.g., spironolactone (2-4 mg/kg daily). Blood pressure should be monitored frequently.

28. Immunization: Parents should be advised regarding the need for completing the primary immunization.Administration of some vaccines , e.g., hepatitis B, measles -mumps-rubella or meningococcal vaccines may rarely precipitate a relapse.

29. PATIENT COUNCELLING: ABOUT THE DISEASE: Parental motivation and involvement is essential in the long-term management of these children . Thrombosis: Children with nephrotic syndrome are at risk for venous and, rarely, arterial thrombosis Children with nephrotic syndrome are susceptible to severe infections, which need prompt treatment. ABOUT THE DRUGS: Parents should be advised regarding the need for completing the primary immunization . Drugs should be taken at the right time with right dose for patient. Extreme tiredness or weakness should be monitoring

30. ABOUT THE LIFE STYLE MODIFICATIONS: A low-salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem . Low-protein diets may be helpful. 1 gram of protein per kilogram of body weight per day. You may need vitamin D supplements if nephrotic syndrome is long-term and not responding to treatment .

31. THANK YOU