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Nephrotic Syndrome Case Presentation

Tuesday 4 th January 2011 Dr Matthew Home. Nephrotic Syndrome Case Presentation. Case presentation Definition Causes Guidelines References. contents. Born AGH Term (40 weeks) Apgars 7 1 / 9 5 Well since Immunisations up-to-date Four brothers (8, 11, 16, 17 yrs) all well

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Nephrotic Syndrome Case Presentation

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  1. Tuesday 4th January 2011 Dr Matthew Home Nephrotic SyndromeCase Presentation

  2. Case presentation • Definition • Causes • Guidelines • References contents

  3. Born AGH • Term (40 weeks) • Apgars71/ 95 • Well since • Immunisations up-to-date • Four brothers (8, 11, 16, 17 yrs) all well • No regular medications • NKDA Previous history

  4. S/B GP ~14/09/2010 Facial swelling – particularly periorbitally Loss of Appetite Flu symptoms (Rhinorheoa, Fevers) a few weeks before Arrange bloods and follow-up appointment Presentation

  5. Follow-up appointment not attended S/B GP again on 04/10/2010 • Abdominal pain and distension • Ankle swelling • Continued loss of appetite • “Drinking plenty of fluids” • Increased urine volume • No dysuria, haematuriaor offensive urine • Bowels - trace blood (Recent prolapse) • No rash, fever

  6. Haemoglobin  13.4 g/dL 11.5 - 17.5 White Cell Count  8.8 x109/L 3.7 - 15.5 Platelets  492 x109/L 140 - 450 Sodium 135 mmol/L 132 - 143 Potassium  4.4 mmol/L 3.2 - 5.7 Bicarbonate  24.5 mmol/l 13 - 29 Urea  2 mmol/L 1.8 - 9.6 Creatinine18umol/L 27 - 88 Chloride  106 mmol/L 96 - 114 Calcium  2.07mmol/L 2.14 - 2.62 Total Protein  35 g/l 56 - 77 Albumin  less thn 10 ALT  12 IU/L Nov-39 Alkaline Phosphatase  118 IU/L 110 - 302 Total Bilirubin  6 umol/L 0 - 34 Vitamin D 17nmol/L 60 - 150 Free T4 8.7 pmol/L 7.5 - 21.1 Ferritin 19 ng/mL 14 - 180 TSH  8.58 miu/L 0.34 - 5.6 Bloods / Urine Dip (14/09/2010)

  7. Examination • P 118, BP 109/63, SaO2 99% RA • Chest – Clear • HS - I + II + 0 • ENT - NAD • Abdomen • moderately distended (Ascites) • No palpable L/S/K/K • Peripheral odema +

  8. Haemoglobin  13.7 g/dL 11.5 - 17.5 White Cell Count  7.7 x109/L 3.7 - 15.5 Platelets  437 x109/L 140 - 450 Sodium  136 mmol/L 132 - 143 Potassium  4.1 mmol/L 3.2 - 5.7 Bicarbonate  23.8 mmol/l 13 - 29 Urea  3.2mmol/L 1.8 - 9.6 Creatinine13umol/L 27 - 88 Chloride  105 mmol/L 96 - 114 Calcium  2.21 mmol/L 2.14 - 2.62 Total Protein  47 g/l 56 - 77 Albumin  16 g/L 31 - 48 Corrected Calcium  2.69mmol/L 2.14 - 2.62 Globulin  31 g/L 23 - 41 ALT  18 IU/L 11-39 Alkaline Phosphatase  91 IU/L 110 - 302 Total Bilirubin  8 umol/L 0 - 34 Blood Culture (Aerobic): No growth (5 days) VZV IgG Antibodies  Positive Plasma Glucose  4.8 mmol/L 3.0 - 12.0 Antistreptolysin-O  1 IU/mL 0 - 200 TSH  10.72miu/L 0.34 - 5.6 Free T4  7.5 pmol/L 7.5 - 21.1 Thyroid PeroxidaseLess than 10 Bloods on admission (04/10/2010)

  9. Urine Dip Protein +++

  10. Nephrotic Syndrome Vitamin D Deficiency Diagnosis

  11. Nephroticrange proteinuria (> 1g/m2/day) • Hypoalbuminaemia(<25 g/l) • Oedema Classification • Idiopathic (primary) nephrotic syndrome • Minimal change (80-90%) • Focal segmental glomerulosclerosis (FSGS) (10-20%) • Secondary nephrotic syndrome (HSP, SLE, MPGN) • Congenital nephrotic syndrome Nephrotic Syndrome Definition

  12. Minimal-change disease (MCD) • Also known as lipoid nephrosis or nil disease • It refers to a histopathologic lesion in the glomerulus • Disorder of T cells, which release a cytokine that injures the glomerular epithelial foot processes.

  13. Focal Segmental Glomerulosclerosis • ?viral- or toxin-mediated damage or intrarenal hemodynamic changes such as hyperperfusion and high intraglomerular capillary pressure • Injury to podocytes • shrinkage/collapse of glomerular capillaries • scarring (glomerulosclerosis)

  14. Idiopathic NS • MCNS • FSGS • MPGN • Membranous glomerulonephritis (MGN) • IgA nephropathy • Idiopathic crescenticglomerulonephritis • Genetic nephrotic syndrome/congenital nephrotic syndrome • Finnish-type congenital nephrotic syndrome (NPHS1, nephrin) • Denys-Drash syndrome (WT1) • Frasier syndrome (WT1) • Diffuse mesangial sclerosis (WT1, PLCE1) • Autosomal recessive, familial FSGS (NPHS2, podocin) • Autosomal dominant, familial FSGS (ACTN4, α-actinin-4; TRPC6) • Nail-patella syndrome (LMX1B) • Pierson syndrome (LAMB2) • Schimkeimmuno-osseous dysplasia (SMARCAL1) • Galloway-Mowat syndrome • Oculocerebrorenal (Lowe) syndrome • Secondary nephrotic syndrome • Infections • Congenital syphilis, toxoplasmosis, cytomegalovirus, rubella • Hepatitis B and C • HIV/acquired immunodeficiency syndrome (AIDS) • Malaria • Drugs • Penicillamine • Gold • Nonsteroidal anti-inflammatory drugs (NSAIDs) • Interferon • Mercury • Heroin • Pamidronate • Lithium • Systemic disease • Systemic lupus erythematosus • Malignancy - Lymphoma, leukemia • Vasculitis -Wegener granulomatosis, Churg-Strauss syndrome, polyarteritisnodosa, microscopic polyangiitis, Henoch-Schönleinpurpura (HSP) • Immune-complex–mediated - Poststreptococcalglomerulonephritis Nephrotic Syndrome - Causes

  15. Nephrotic Syndrome - Idiopathic Typical Features Age 1-10 years Normotensive Normal Adrenal Function +/- microscopic haematuria Atypical Features <1yr, >10years Hypertensive Elevated Creatinine Macroscopic Haematuria

  16. Blood: FBC, U+E’s; Creatinine; LFT’s; ASOT; C3/C4; Varicella titres • Urine: Urine culture and Urinary protein/creatinine ratio • BP • Urinalysis including glucose • A urinary sodium concentration can be helpful in those at risk of hypovolaemia. • Varicellastatus should be known in all children commencing steroids. • Hepatitis B status may be appropriate in children at high risk. Nephrotic Syndrome - Investigations

  17. Age < 1 yr Age > 10-12 yrs Persistent hypertension Macroscopic haematuria Low C3/C4 Failure to respond to steroids within 4 weeks Nephrotic Syndrome - Referral

  18. Hypovolaemia • Despite odema may be intra-vascularly depleted • Infection • Loss of complement components • Thrombosis • Loss of proteins and exacerbated by hypovolaemia Nephrotic Syndrome - Complications

  19. Prednisolone +/- Ranitidine • Albumin where indicated + Frusemide mid-infusion • Penicillin prophylaxis • Salt/Fluid Restriction • Vaccination Treatment

  20. Remission – 3+ days of trace / - protein on dipstick testing Up to 60 - 70 % of children may have one or more relapse. Urine check twice weekly initially, then weekly Monitoring

  21. Prednisolone • Albumin • Salt Restriction • Penicillin • Varicella vaccine • Consider between relapses in children who are varicella seronegative. Treatment of Relapsing NS

  22. Referral to / Discussion with Paediatric Nephrology if Frequent relapsers Steroid dependency Steroid toxicity Referral of Relapsing NS

  23. Frequent relapsers are diagnosed if there is: • 2 or more relapses within the first 6 months of presentation • 4 or more relapses within any 12 month period • This becomes steroid dependency if the relapses are occurring during steroid tapering. • Varicella status should be repeated 6 monthly in those who are non-immune. Frequent Relapses

  24. Low Dose Alternate Day Prednisolone Levamisole Cyclophosphamide Cyclosporin Mycophenylate Mofitil (MMF) Treatment of Frequent Relapses

  25. Prednisolone 60mg/m2 • Ranitidine • Penicillin V • DalavitVit D Suppliments • HAS 20% @ 5ml/kg with Frusemide 1mg/kg half way through x3 • Urinalysis with each void • Monitor daily urine output • Daily weights – adjust fluid restriction accordingly • Fluid restrict to 600ml/day • D/W Leeds renal team Our Case - Plan

  26. Review 15/10/10 • Odemasettled • Persistent Proteinuira Pro+++ • Penicillin V stopped • Continue Prednisolone Review 22/10/10 • Odema settled • Persistent Proteinuira Pro+++ • Penicillin V stopped • Continue Prednisolone Review 29/10/10 • Persistent Pro+++ • Prednisolonechanged to 40mg/m2 - 25mg alt days Review 13/11/10 • Persistent Pro+++ • Restart Penicillin V 125mg BD • Referred to Paediatric renal team for ?renal biopsy Case progress

  27. www.gpnotebook.co.uk/ http://emedicine.medscape.com/ http://www.clinicalguidelines.scot.nhs.uk/ www.newcastle-hospitals.org.uk/ References

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