Pediatrics 5th year, 9th lecture (Dr. Adnan)


Published on

The lecture has been given on Dec. 19th, 2010 by Dr. Adnan.

Published in: Health & Medicine
  • Be the first to comment

No Downloads
Total views
On SlideShare
From Embeds
Number of Embeds
Embeds 0
No embeds

No notes for slide

Pediatrics 5th year, 9th lecture (Dr. Adnan)

  1. 1. Glomerulonephritis Dr. Adnan MH Hamawandi Professor of Pediatrics College of Medicine University of Sulaimany
  2. 2. Definition <ul><li>Inflammation and proliferation of cells within the renal glomerulus which may occur as primary condition or associated with a systemic disorder. The majority of cases are immunologically mediated. GN may present with asymptomatic hematuria, acute nephritic syndrome (oliguria, hypertension and hematuria) or a mixed picture of nephritic and nephrotic. </li></ul>
  3. 3. Etiology <ul><li>Primary GN </li></ul><ul><li>Immune complex –Postinfectious, Mesangial IgA nephropathy </li></ul><ul><li>(Berger’s disease), Membaneous and membanoproliferative GN </li></ul><ul><li>Antiglomerular basement membrane antibody mediated GN </li></ul><ul><li>Pathogenesis unknown </li></ul><ul><li>GN associated with Systemic diseases </li></ul><ul><li>Immunologically mediated </li></ul><ul><li>Vasculitic conditions-Henoch Shonlein purpura, polyarteritis nodosa </li></ul><ul><li>SLE and other connective tissue diseases </li></ul><ul><li>Infections-SBE, Shunt nephritis, malaria </li></ul><ul><li>Hereditary-Alport’s disease </li></ul><ul><li>Others-Diabetes mellitus </li></ul>
  4. 4. Acute post-infectious GN <ul><li>Typically occurs 7-14 days after group A β -hemolytic streptococcal throat or skin infection( nephritogenic strains), or infection by other organisms like staphylococci, salmonella, mycoplasma, and viruses. </li></ul><ul><li>It can occur at any age but predominantly affects school-aged children. </li></ul>
  5. 5. Clinical features <ul><li>Dark “smoky” urine . </li></ul><ul><li>Puffy face with fluid retention, usually mild causing only a degree of edema, but when severe can result in heart failure. </li></ul><ul><li>Oliguria , in severe cases acute renal failure. </li></ul><ul><li>Hypertension , occasionally acute causing encephalopathy and seizures. </li></ul><ul><li>Non-specific: malaise, abdominal pain and fever. </li></ul><ul><li>The acute phase usually resolves within 1-2 months after onset but urinary abnormalities may persist for more than 1 year. </li></ul>
  6. 6. Investigations <ul><li>Urinalysis: gross hematuria, granular & RBC cast, and variable proteinuria. </li></ul><ul><li>Blood urea and creatinine are elevated according to the degree of impairment in renal function which may range from minimal to severe. </li></ul><ul><li>Electrolytes show variable degrees of hyperkalemia, hyperphosphatemia, hypocalcaemia and acidosis. </li></ul><ul><li>Hypocomplementemia ( Low C 3 component of complement) </li></ul><ul><li>Evidence of recent streptococcal infection. Throat swab, ASO titer, and anti DNase B antibody titer . ASO titer may not rise, particularly after skin infections. </li></ul>
  7. 7. Management <ul><li>Acute post-infectious GN is usually mild and requires no specific treatment other than monitoring of daily weight, BP, and fluid balance to limit the risk of fluid overload. </li></ul><ul><li>There is no real evidence that bed rest is of benefit. </li></ul><ul><li>Salt and fluid restriction can control mild hypertension. Diuretics are helpful if BP is not controlled by salt restriction. Antihypertensive drugs are needed for more severe cases. </li></ul><ul><li>Although it does not seem to alter the course of acute GN, a 10 day course of penicillin is usually given to limit the spread of nephritogenic strains of streptococci. </li></ul><ul><li>In few patients the disease is fulminant with rapid progression to renal failure. In such cases dialysis may be needed. </li></ul>
  8. 8. Management-cont. <ul><li>The child can be discharged home once the renal function is seen to be improving. Outpatient follow up should continue until urinalysis, BP, and renal function are normal. </li></ul><ul><li>Complete recovery occurs in more than 95% of cases, second attacks are rare. Renal function usually returns to normal within 10-14 days but microscopic hematuria may persist for >1 year. </li></ul>
  9. 9. Henoch Shonlein purpura <ul><li>Is an IgA mediated vasculitis of small vessels with unknown etiology. </li></ul><ul><li>The hallmark of the disease is the skin rash beginning as pinkish maculopapular or urticaria that progress rapidly into clinicaly palpable petichial and purpuric rash that evolve from red to purple to rusty brown before they fade. The lesions tend to appear in crops on the extensor surfaces of the extremities involving the buttocks. </li></ul><ul><li>Arthritis occur in more than 2/3 of cases, involve large joints and resolve without sequel. </li></ul>
  10. 10. H-S pupura <ul><li>Edema and damage to the GI tract vasculature may lead to intermittent colicky abdominal pain , diarrhea (with or without blood), hematmesis, and rarely intussusception. </li></ul><ul><li>Renal involvement occur in 25% of cases, usually as hematuria alone, rarely nephrotic syndrome may occur. </li></ul><ul><li>CNS, testicular, eye, and other organ involvement are rare but can be potentially serious. </li></ul><ul><li>Analgesia for pain can be enough, but short course steroids can improve GI and CNS complications. </li></ul>
  11. 11. Hemolytic Uremic syndrome <ul><li>Is characterized by microangiopathic anemia , thrombocytopenia , and acute renal failure . </li></ul><ul><li>Typical HUS (postdiarrheal) is associated with infections, specially verotoxin producing E.coli(O157:H7), but can follow shigella dysentry, viruses, and streptococcus pneumonae. The renal damage is primarily glomerular with thrombotic microangiopathy causing multisystem disease. </li></ul><ul><li>Atypical HUS is rare and may be idiopathic, inherited or associated with drugs. The renal damage is arteriolar with intimal and subintimal edema, necrosis, and proliferation. </li></ul>
  12. 12. Clinical features <ul><li>The majority of cases are postdiarrheal, the diagnosis should be considered in any child who develops pallor, oliguria or hematuria following a diarrheal illness, particularly if the diarrhea is bloody. </li></ul><ul><li>It occurs in summer epidemics typically in infants and young children, is equally common in boys and girls. </li></ul><ul><li>Bruising and petichiae are secondary to thrombocytopenia and jaundice may develop. </li></ul><ul><li>Hypertension if present is mild. </li></ul><ul><li>CNS involvement is rare and recurrences are unusual. </li></ul><ul><li>Atypical HUS is uncommon and tends to occur in older children There is no prodromal diarrhea. Hypertension is severe, CNS involvement is common and the prognosis is poor. </li></ul>
  13. 13. Investigation <ul><li>Complete blood count and film shows thromocytopenia and microangiopathic hemolytic anemia( helmet cells, burr cells and fragmented RBCs) and leukocytosis. Reticulocyte count is moderately elevated and Coombs test is negative. </li></ul><ul><li>Findings on Urinalysis are surprisingly mild and usually consist of low grade microscopic hematuria and proteinuria. </li></ul><ul><li>Raised FDPs. </li></ul><ul><li>Blood urea and creatinine are elevated. </li></ul><ul><li>Stool culture can recover the offending organism. </li></ul>
  14. 14. Management <ul><li>Anemia should be corrected with repeated small transfusions of packed red cells. </li></ul><ul><li>Strict fluid and electrolyte balance. Dialysis should be implemented early for fluid overload and raising urea or potassium. </li></ul><ul><li>Hypertension should be treated vigorously with antihypertensive agent, fluid restriction and diuretics. </li></ul><ul><li>Plasmapharesis and FFP can help in some cases but they may </li></ul><ul><li>exacerbate the disease in cases caused by st. pneumoniae . </li></ul><ul><li>Antithrombotic therapy has no proven therapeutic benefit in HUS. Nephronoprotection in the early phase of HUS may be possible by prevention of dehydration with IV fluids. </li></ul>