Dhanalakshmi.R II yr MD Stanley medical college

Transcription

1 Dhanalakshmi.R II yr MD Stanley medical college

2 10 Yr old female child - abdominal pain & vomiting for 1 day. Diffuse in nature, moderate in intensity, not related to food intake & radiates to back. No H/o fever, abdominal distension, diarrhea & no urinary symptoms. No h/o previous hospital admission, drug intake & trauma.

3 O/E Child conscious, oriented, HR: 90/min, RR:16/min, BP:110/70 mmhg. P/A Soft, not distended, No guarding, rigidity, Tenderness in epigastrium, Bowel sounds heard, No organomegaly.

4 Differential diagnosis acute gastritis acute pancreatitis Increasing abdominal pain & shifted to PICU where she resorted to following posture.

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6 S.Lipase mu/ml (30-160) S. Amylase 865 mu/ml(30-110). Lipid profile, Ca & LFT - normal. USG - abdomen shows bulky pancreas. CT - abdomen Pancreatitis

7 Gastroenterologist opinion Pancreatitis. Endoscopy- Ascaris worm in duodenum. Conservative management & deworming

8 On day 4 of hospitilisation, she developed palpable purpuric rashes over lower limbs.

9 After 2 days - generalised edema & oliguria. Urinanalysis - hematuria (3+) and proteinuria (3+). Pain & swelling of both ankle joints. Hypertension (+) controlled with nifedipene.

10 Dermatologist opinion - Leucocytoclastic vasculitis (? HSP) Skin rash, edema, hypertension, arthritis gradually resolved over next 4-5 days. Persistent proteinuria (3+) and hematuria (3+). Nephrologist opinion - renal biopsy

11 CBC Hb-12.1, TC-9600, platelet- 2,60,000 & PCV-36% RFT - normal. S.Electrolytes - normal Repeat USG-abdomen normal. Urinalysis - Hematuria(3+), Proteinuria (3+). Serum C3, C4 normal.

12 day albumin Nil Nil Spot PCR 3.5

13 Light microscopy Increase in mesangial cellularity No endocapillary proliferation,neutrophilic infiltrates, necrotising lesions or crescent formation No spikes or double contours Immunofluorescence IgA & C3 deposits

14 Diffuse staining for IgA and granular C3 deposits

15 Final diagnosis HSP with initial atypical presentation of acute pancreatitis.

16 Prednisolone ( 1 mg/kg/day) 2 weeks. Proteinuria resolved over 2nd week of steroids. 6 wks no proteinuria & hematuria.

17 Cakir et al. Acute pancreatitis as an atypical presentation of Henoch-Schonlein purpura and cystic fibrosis. Turk J Med Sci. 2009; 39 (5): yr old male child -3 days h/o abdominal pain, diagnosed as acute pancreatitis had purpuric rash after 2 nd day of hospitilisation. The clinical picture consistent with HSP, with pancretitis & GI involvement. Soyer et al. Acute pancreatitis: a rare presenting feature of Henoch Schonlein purpura. J Paediatr Child Health ; 44(3): yr old female child presented with acute pancreatitis had characteristic HSP rash after 2 day of hospitilisation. Although pancreatitis occurs after the onset of rash, it may also occurs as presenting feature.

18 Cheung et al. Pancreatitis associated with Henoch-Schonlein purpura. J Paediatr Child Health Jun;37(3): yr old male child presented with acute pancreatitis with development of HSP rash 10 days later. Garner et al. Acute pancreatitis as a complication of anaphylactoid (Henoch-Schönlein) purpura. Arch Dis Child December; 52(12): yr old child admitted with joint pain & skin rash had abd pain after 2 days, with increased s. amylase levels. Improved after conservative treatment for acute pancreatitis.

19 Idiopathic 22% Association with systemic disease 20% HUS, SLE Trauma- 18% Structural( pancreatic divisum)- 10% Medications- 10% Valproate, L-asparginase, Infections Ascariasis AIDS Enteroviruses( Coxsackie & Echovirus), EBV, Mumps,Hepatitis Malaria Gall stones, post ERCP, familial, hypercalcimea, hyperlipidemia, diabetic ketoacidosis, PEM, CF, HSP Ref: Sleisenger & Fordtran s textbook of gastrointestinal and liver disease

20 The most common vasculitis of childhood which involves Skin, GI tract, Joints & kidney. IgA containing immune complex deposit in association with inflammatory reaction of the vessels. Epidemiology 14-20/1,00,000 children per year M:F : 1 Children > adults Peak age group yrs Common in winter months Ref: Nelson textbook of paediatics & Brenner & Rector s,the kidney

21 Skin - 100% Palpable purpura lasting for 3-10 days Symmetrical, gravity dependent areas. Joints 75% Arthritis or arthralgia, self limited & oligoarticular No erosion or deformity GI - 80% Abd pain, vomiting, diarrhea, paralytic ileus, malena, intussusception rarely perforation. Renal - 50% Hematuria, proteinuria, hypertension, frank nephritis, nephrotic syndrome.

22 The typical glomerular pathology is mesangial & endocapillary proliferative GN with variable crescent formation. In severe cases there will be inflammatory infiltrates & necrotising features. Immunoelectron microscopy predominance of IgA with some C3 & IgG in the deposits.

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24 Clinical diagnosis Mild leucocytosis, normal platelet. Serum IgA are often elevated but are not routinely measured. USG- abd for bowel wall edema and intussuception. Biopsy of skin & renal tissue- IgA deposition.

25 GI involvement Perforation Renal disease - long term complication. Serial monitoring of blood pressure and urinalyses for 6 mo after diagnosis, those who presented with hypertension or urinary abnormalities.

26 Supportive Role of steroids-controversial in HSP nephritis Empiric use of prednisone reduces abdominal and joint pain.

27 Childhood HSP- excellent prognosis. Recurrences 30% of children within 4-6 months of diagnosis. Chronic renal disease develops in 1-2% of children with HSP, 8% of those with HSP nephritis go on to have ESRD.

28 Abdominal pain in HSP is explained by bowel wall edema and vasculitis but it may also be due to acute pancreatitis. Even if rare, atypical presentation of HSP initially with acute pancreatitis should be remembered in order to plan the specific treatment and avoid unnecessary surgical interventions.

29 Thank you

30 Obstruction of papilla of vater, invasion of CBD or pancreatic duct. Children rare, smaller hepatobiliary network. Prognosis excellent.

31 Ronkainen et al. Prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement. J Pediatr. 2006;149:241-7 Gibson et al.corticosteroid Therapy for Henoch Schönlein Purpura. Pediatrics 2008;121: Benefit in renal & abd inv Dudley et al. Randomised placebo controlled trial to assess the role of early prednisolone on the development and progression of Henoch-Schonlein purpura nephritis.j Am Soc Nephrol.2007;18 :47A No difference