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Extern conference

“Prolonged fever”. Extern conference. Supervised by Prof. Achra Sumboonnanonda MD Rattanavalai Chantorn MD Paisarn Parichatiganond MD. Patient profile. 12 years old Thai girl CC: Low grade fever for 1 month PTA

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Extern conference

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  1. “Prolonged fever” Extern conference Supervised by Prof. Achra Sumboonnanonda MD Rattanavalai Chantorn MD Paisarn Parichatiganond MD

  2. Patient profile • 12 years old Thai girl • CC: Low grade fever for 1 month PTA • Hx: 4 mo PTA She had persistent erythematous rash on both cheeks and active hair loss.She came to a local hospital and was diagnosed as dermatitis. • 1Mo PTA. She had low grade fever relieved by antipyretic drug.

  3. She had no other symptoms except rash on her face that sometimes aggravated by sun exposure and increase excessivehair loss • 3 wk PTA she became more malaise, pallor and went to private clinic. She received parenteral fluid and oral medications, the symptoms were partial improved. • 2 wk PTA she still had persistent fever then she went to a local hospital.

  4. At the hospital PE: erythematous rash on malar area The rash became worse and she developed painful ulcer on her lips and oral mucosa,She also had erythematous macules on her soles and swelling of her face

  5. Investigation at the hospital -BUN/Cr 22/0.7 mg/dl -CBC: Hb 8 g/l, Hct 27 %, WBC 4,470 /mm3 (N75%, L20 %) Plt 196,000/mm3 -Stool exam : WNL -UA : WNL -Urine culture: > 105Streptococcus Gr. D, Enterococci spp

  6. Rx :Ceftriaxone 2 gm OD x 9 d then Cefotaxime 1 gm IV q 6 hrs x 3d Doxycycline x 7d Gentamycin 100mg IV OD x 7d Symptoms persisted then the patient was referred to “Siriraj hospital” • Past history : healthy • Family history : no family history of atopy • Drug history: analgesic drug allergy

  7. Physical examination • V/S: T 38.3 oC, P110/min, R20/min, BP118/60 mmHg • BW 37.5 kg(P25-50), HT 155 cm(P50-75) • GA: Irritable, look weak, not cooperative, mildly pale, no jaundice, no dyspnea, dry lips, good skin turgor, no sunken eye balls , capillary refill <2 sec, no eschar • HEENT: findings as figures

  8. Bilateral scaly erythematous to brownish patches at malar eminence, nasal ridge and nasolabial folds,scaly edematous erythematous painful lips

  9. Round shape erythematous macule with central hyperpigmentation

  10. single erythematous patch with peripheral hyperpigmentation on scalp

  11. Painful shallow ulcer at hard palate with oral thrush

  12. RS: normal • CVS: normal • ABDOMEN: normal • Extremities: Bilateral symmetrical partially blanchable erythematous to purplish macules and papules on both palms and soles, no sign of joints inflammation • NS: normal • No lymphadenopathy

  13. CBC Hb 8.6 g/dl, Hct 26.9 %, MCV 71.7 fl, RDW 14.9 %, WBC 2,840 /mm3 (N 72.2, L 20.1), Platelets 198,000/mm3 HCMC RBC, no hemolytic blood picture Urinalysis pH 7.0, Sp.Gr. 1.015, Protein +++, Occult blood +, Bilirubin neg, Acetone neg, WBC 0-1/HF, RBC 1-2/HF, no cast and bacteria Investigation

  14. Blood chemistry BUN 13.0 mg/dl, Cr 0.5 mg/dl Na 135 mEq/dl, K 4.5 mEq/dl, Cl 95 mEq/dl, HCO3- 19 mEq/dl CXR WNL U/C, H/C : pending

  15. Problem list • Prolonged fever for 4 weeks • Active hair loss for 4 months • Abnormal skin manifestation on scalp, face, ears, lips, mouth and extremities • Oral thrush • Anemia: Hypochromic,microcytic • Leukopenia and lymphopenia • Proteinuria

  16. Children withprolonged fever • Fever in this patient can be defined as fever of unknown origin(FUO) • The Petersdorf and Beeson criteria for FUO, definition in 1961are:   ▪  a body temp ≥ 38.3°C for at least 3weeks; and  ▪  failure to establish a diagnosis after 1week of investigation.

  17. Differential diagnosisof FUO • Infection • Autoimmune disease • Neoplasm • Miscellaneous (drug-related fever, factitious fever, etc.)

  18. Relation between infection and autoimmune disease • Clinical symptoms of infection may be indistinguishable from those of autoimmune disease • Immunosuppressive therapy for autoimmune disease may lead to increased susceptibility to infection

  19. Infectious cause of FUO in children • Salmonellosis • Tuberculosis • Rickettsial disease • Bacterial endocarditis • Infectious mononucleosis

  20. Criteria of SLE • Malar rash • Discoid rash • Photosensitivity • Oral ulcer • Arthritis • Serositis • Renal disorder: persistent proteinuria • Neurologic disorder: seizure, psychosis • Hematologic disorder: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia • Immunologic disorder: anti-dsDNA, anti-Sm, antiphospholipid antibody • ANA positive ACR 1982, updating classification criteria 1997

  21. Characteristic of fever in active SLE disease • Non-shaking fever • Manifestation of active SLE: such as • Acute cutaneous LE • Arthritis • Hypertension, Edema • Leukopenia with lymphopenia, Thrombocytopenia

  22. The most likely diagnosis “Active SLE disease”

  23. How to recognize SLE presenting symptomsin pediatric patients ?

  24. Study from department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversitySuroj Supavekin MD*,Wanida Chatchomchuan MD**, Anirut Pattaragarn MD*, Vibul Suntornpoch MD*, Achra Sumboonnanonda MD**J Med Assoc Thai 2005; 88(Suppl 8): S115-23 • From July 1985to March 2003,101 patients • The major clinical presentation of pediatric SLE are - Renal (86.2%) - Skin and mucocutaneous (76.3%) - hematological involvement (73.4%)

  25. Pediatric Systemic Lupus Erythematosus in Siriraj HospitalSuroj Supavekin MD*,Wanida Chatchomchuan MD**, Anirut Pattaragarn MD*, Vibul Suntornpoch MD*, Achra Sumboonnanonda MD** Signs and Symptoms at Diagnosis J Med Assoc Thai 2005; 88(Suppl 8): S115-23

  26. The Results of Renal Biopsies J Med Assoc Thai 2005; 88(Suppl 8): S115-23

  27. Classification of lupus nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Class III: Focal LN Class IV: Diffused LN Class V: Membranous LN Class VI: Advanced sclerosis LN International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003

  28. Management

  29. Further investigation Work up for source of infection • KOH for oral thrush: Pseudohyphae with budding yeast • Stool concentration for parasite x 3days negative • Urine culture (10/4/50) No growth • Hemoculture (10/4/50) No growth • PPD skin test: Negative at 48, 72 hr • Consult dentist: No dental caries

  30. Further Investigation • peripheral blood smear (14/4/2550): hypochromic microcytic RBC, no anisopoikilocytosis, Plt 10-15/HPF • Reticulocyte count 0.61% • Direct coomb’s test: negative • Serum ferritin (17/4/2550): 1,537 (13-50) “Iron deficiency anemia”

  31. Further investigation Autoimmune profile • ANA Positive Positive with Fine-speckled pattern titer >1:2,560 Positive with Coarse-speckled pattern Positive with Homogeneous pattern Positive with Peripheral pattern Positive with Anti-Cytoplasmic Ab • Anti-ds DNA Positive titer > 1:160 • C3 level 36.8 (N 83-177) • C4 level 6.56 (N 15-45)

  32. Further investigation Total protein 5.4 g/dl, Albumin 1.7 g/dl Urine Creatinine 28.3 mg/dl Urine Micro-TP 148 mg/dl Urine protein/creatinine ratio 5 Urine protein 24 hr 55 mg/kg/d “Nephrotic range proteinuria” Renal biopsy

  33. Indication for kidney biopsy All patient who correlate with criteria of LN Nephrotic patient with undetermined diagnosed of Diffuse proliferative GN or Membranous GN Patient whose renal function get worse despite of receiving high dose steroid

  34. TREATMENT

  35. Patient education Avoid sunlight Avoid physical and mental stress Drug compliance

  36. Fever and malaise: Low dose NSAIDs with antimalarial drug or low dose oral corticosteroid Cutaneous lesion: Sunblock cream with topical steroid and antimalarial drug

  37. Treatment of lupus nephritis Base on renal pathology Class I: no treatment required Class II: short course treatment of low dose steroid (prednisolone 0.5-1 MKD) Class III: prednisolone 1-2 MKD(max60mg/d) +immunosuppressive drug(Azathioprine 2MKD) Class IV: prednisolone 2 MKD+pulse cyclophosphamide Class Ⅴ: prednisolone 1-2 MKD Class Ⅵ:slow renal progression,aggressive immunosuppressive drug not required

  38. Progression -Continue cefotaxime until 7days(10-13/4/50) -Cotrimazole troche to treat oral candiasis - Septic work up: all negative so non-infectious cause is most likely Medication Prednisolone (2mg/kg/d) Hydroxychloroquine (5mg/kg/d) 0.02%TA cream apply to lesions at face 0.1% TA cream apply to lesions on scalp

  39. Progression • Anemia:FeSO4 (200mg) 1 tab PO tid pc (5.6 mg/kg/day) (start 17/4/2550) • New onset HT:BP 128/86(16/4/2550) max135/73 mmHg (P95 124/81mmHg) - Enalapril (5mg) 1 tab PO OD pc • Follow up urinalysis 18/4/2550 pH 7.0, sp.gr 1.010, protein neg, WBC 0-1,RBC neg, occult blood neg, others neg

  40. RENAL PATHOLOGY

  41. Renal pathology • Mesangial hypercellularity and matrix expansion • No endocapillary proliferation • No crescent • Microthrombi in 1 arteriole Imp: Lupus nephritis class II with thrombotic microangiopathy(TMA)

  42. References • Suroj Supavekin MD,Wanida Chatchomchuan MD, Anirut Pattaragarn MD, Vibul Suntornpoch MD, Achra Sumboonnanonda MD Study from department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University J Med Assoc Thai 2005; 88(Suppl 8): S115-23 • The Subcommittee for Systemic Lupus Erythematosus Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7 • Wallco DJ, Halm BH, eds. Dubois’ lupus erythematosus. 5th edn. Baltimore: Williams and Wilkins, 1997

  43. Thank you

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