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A child with oedema

A child with oedema. Constantinos J. Stefanidis “A. Kyriakou” Children's Hospital Athens, Greece. What is the diagnosis ?. Urine protein 4+ Urine blood 2-3+. John B. 4 year old boy with a two day history of puffiness around the eyes. Hct 42 % Na 134 mEq/l K 4.2 mEq/l

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A child with oedema

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  1. A child with oedema Constantinos J. Stefanidis “A. Kyriakou” Children's Hospital Athens, Greece

  2. What is the diagnosis ? Urine protein 4+ Urine blood 2-3+ John B. 4 year old boy with a two day history of puffiness around the eyes. Hct 42 % Na 134 mEq/l K 4.2 mEq/l Urea 22 mg/dl Creat. 0.5 mg/dl Family doctor diagnosed an “allergic reaction” Alb. 2.2 g/L Chol. 270 mg/dl Physical examination BP 105/75mmHg HR 85/min Feet: mild pitting oedema Abdomen: N Chest: N Genitalia: N

  3. Oedema Low plasma albumin <25 g/L Severe proteinuria >40 mg/m2/hr or Pr : Cr >200 mg/mmol (1.8 mg/mg) John should be admitted at the Hospital ? Idiopathicnephrotic syndrome (NS) • NS may be accompanied by: • haematuria, • arterial hypertension and • decreased GFR • (NS with a nephritic course)

  4. All children with newly diagnosed NS should be admitted at the Hospital. The goals are: • Removal of fluid overload. • Reduction and disappearance of proteinuria. • Prevention of complications (infection, thrombosis).

  5. Removal of fluid overload. • Reduction and disappearance of proteinuria. • Prevention of complications(infection, thrombosis). Why John has oedema ??? Management

  6. Intravascular space Interstitial space Massive protein loss Hypoalbuminaemia Oedema Hypovolaemia Renin-ATII-aldosterone axis and ADH Increased reabsorption of Na and H20 Deterioration of oedema Hypovolaemia “Underfill” theory of oedema formation

  7. IntravasularspaceinpatientswithNS Donckerwolcke RA et al Kidney Int 1997 • Sodium retention seems to occur in early relapse of the NS • No evidence of reactive stimulation of vasoactive axis of renin-ATII-aldosteroneand/orADH before the establishment of overt hypoalbuminaemia Schrier RW et al Kidney Int 1998

  8. Intravascular space Interstitial space * Primary Na retention Intravascular volume expansion Oedema * Collecting duct of patients with NS are 'resistant' to the action of the atrial natriuretic peptide ??? “Overfill” theory of oedema formation Transcapillary movement of fluid Schrier RW et al Kidney Int 1998

  9. Hypoalbuminaemia Oedema Hypovolaemia Primary renal Na and H20 retention Intravascular volume expansion Oedema Transcapillary movement of fluid Donckerwolcke RA et al Kidney Int 1997 In most patients with NS: • In the early stages the 'underfill' mechanism operate. • In a later period a new steady state will be reached with a normal or expanded blood volume('overfill’ mechanism).

  10. John should have an albumin infusion??? Laboratory findings of hypovolemia : • High hematocrit • Low urine Na (1-2 mmol/L) • UK/UK+Na x 100% < 20% ? No because he has no signs of hypovolemia i.e.: • Abdominal pain • Hypotension • Oliguria • Evidence of renal failure

  11. John should have diuretics??? Diuretics: In significant oedemaand absence of hypovolemia Frusemide and spironolactone 1mg/kg/day. Hypertension Correction of hypervolemia or hypovolemia. Nifedipine 0,5-1 mg/kg/day and /or atenolol 0,5-1 mg/kg/day.

  12. Removal of fluid overload. • Reduction and disappearance of proteinuria. • Prevention of complications(infection, thrombosis). For how long John should have prednisolone ? Management

  13. Initial steroid therapy. The protocol of the International Study of Kidney Diseases in Children From the late 60's until 80's the ISKDC provided a classification of NS Prednisone (pz): 60 mg/m2/day (or 2mg/kg ideal body weight). Not exceeding a total dose of 80mg/day for one month. The 2nd month 40 mg/m2 of pz in a single dose every 48 hrs. (Total treatment period of 2 months). France: One month course of daily , followed by 2 months of alternate day pz (2mg/kg). Then pz is decreased by 0.5mg/kg every 2 weeks. (Total treatment period of 4-5 months). Germany: 6 weeks course of daily, followed by 6 weeks of alternate day pz. (Total treatment period of 3 months).

  14. Definitions Early non-responder: proteinuria does not normalize within 4 weeks of daily pz therapy Relapse: Proteinuria > 40mg/d/m2 (or Albustix 2+ or >) on three consecutive days. Frequent relapses: >2 relapses within 6 months of initial response. Steroid dependence: 2 consecutive replapses occuring during pz treatment or within 14 days of its cessation.

  15. Initial steroid therapy and frequency of relapses (%) Prednizone 60 mg/m2/24hrs + 40 mg/m2/48hrs Brodehl J Clin Nephrol 1991

  16. NS with minimal changes (90%) Focal segmental glo- merulosclerosis (8%) Diffuse mesangial proliferative glomeruglomerulo-nephritis (2%) J Pediatr 1981 Steroid sensitivity rather than histology is the major determinant of prognosis. Webb N et al. Am J Kidney Dis 1996 International Study of Kidney Diseases in Children

  17. John has hematuria should he have a renal biopsy ? The frequency of relapses alone is not an indication for biopsy. Webb N et al. Am J Kidney Dis 1996 23% of children with MCNS and 67 % with FSGS had microscopic hematuria ISKD J Pediatr 1981 • Pretreatment indications • Age • < 6 months or > 12 years • Nephritic findings • (macroscopic hematuria or microscopic and hypertension) • Renal failure • Post treatment indication of renal biopsy • Steroid resistance • Frequent relapses before cyclosporin

  18. Removal of fluid overload. • Reduction and disappearance of proteinuria. • Prevention of complications (infection, thrombosis). John should have antibiotics and/or anticoagulation treatment ??? Management

  19. What about his diet and activity ??? Antibiotics In the oedematous child with gross ascites oral penicillin 125-250 mg BID. Prevention of thrombosis by correction of hypovolemia. Diet Salt restriction. Normal protein intake. Calorie control. Activity The child should be mobilized.

  20. What parents should know about NS ? Parents should have a booklet with information about the disease. Parents should know that NS is a chronic disease and they should get prepared for possible relapses. Parents should be informed that chickenpox and measles are major threats and should go to the Hospital if their child is exposed. Children with NS should receive immunization as normal unless they have been taking pz daily for more than one week. Life vaccines can be given only if the child is on a low dose pz.

  21. Key points for clinical practice Steroid-sensitive NS, the most frequently form of childhood NS is a relatively mild form of the disease virtually without long term impairment of glomerular filtration rate. Childhood NS is a chronic disease and cannot be left untreated. Steroid-sensitive NS tends to relapse.This requires clear therapeutic strategies to try and keep the patients in long lasting remissions and to minimise the adverse effects of long-term corticosteroid therapy. Relapses should be detected at home before the onset of symptomatic NS by daily 'dipstix' for urinary protein. Ehrich JHH Drukker A Rec Adv Pediatr 1999

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