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Home | Reference & Education | Psychology Admitted via clinic on 25-06-2007 for Fever off and on-------- 8-9 months Abdominal Pain --------- 2-3 weeks In Gilgit she was being treated as a case of genital Tuberculosis without any objective genitourinary complaints or symptoms She took ATT for 2 months and then stopped b/c of drug induced Acute Hepatitis. Fever 8-9 months off and on High grade up to 103-104 F, no chills/rigors No urinary or chest complaints but she had upper abd. Pain, moderate in intensity, no radiation, no vomiting but nausea was present with ass. Weight loss. Clinical Examination: Alert awake oriented, toxic look Pulse Rate: 90 beats/minute regular Blood Pressure: 160/80 mmHg Resp. Rate: 20 b/minute Temp: 36.8 centigrade Pallor with Icteric Malar Flush but no rash B/L pitting pedal edema Clinical Examination: RHC tenderness, hepatomegaly 2 fingers below Rt costal margin, splenomegaly 1 finger, positive shifting dullness. S1 and S2 audible, Panystolic murmur in mitral area radiating to axilla, no gallop. Chest: B/L symmetrical shape and resp movements, equal chest expansion B/L, with NVB except at Rt basal region which had decrease air entry. Clinical Examination: CNS: Alert, OrientedX3, no focal sensory or motor deficit, plantars normal. Musculoskeletal System: No positive findings Labs: 11-06-2007 Hb: 10.2 gm/dl (NN) Hct: 27.8 Wbc: 7.4 Neutrophils: 73.8% Lymphocyte: 21.4% Plat: 30 Retic=6.8 FBS: 110mg/dl LDH: 8752 Labs PTT=25.3/12 TB=5.9 Coombs ++ INR=2.12 DB=3.7 CRP = 8.3 APTT=68.7/30 IB=2.2 D-Dimer=0.87 GGT=81 MP ve SGPT=53 MP ICT ve ALP=348 Urine DR: Dark yellow Protein +3, bil +3, Hb trace, rest normal 24 hrs urinary protein was 7080 mg/24hrs She was started on broad spectrum ABx, IV hydration and supportive management initially CXR Rt. Lower lung consolidation with possible consolidatory changes in the Lt lower lung field, findings are suspicious for pneumonia. she was already being treated for pneumonia. US Abd: Fatty infiltration of liver, sludge filled Gallbladder, mild ascites. Her CT Abd and Pelvis with contrast was done considering disseminated TB which showed mild B/L pleural effusion with mild to moderate ascites, no paraaortic lymphadenopathy Ascitic Fluid DR Glu=70 prot=814 TLC=100 N=10 L=90 RBC +++ During the hospital stay She became short of breath(14/06/2007) Her CXR showed Pulmonary edema ABG 7.49/37.8/70.8/28.9/+5.9/95.3 on 6L Fio2 Trop I x2 were negative She was treated with IV diuretics Cardiology service was involved they continued IV diuretics Echo: EF 60%, moderately dil. Lt Atrium LVDD grade II Mod-severe MR, mild TR Mild PHTN, no vegetations/clots Her Autoimmune profile was sent, and in the mean while bone marrow was done to send TB CS and cause for worsening Bicytopenia (dropping PLTs & Hb) under cover of FFPs. GI service was also involved for deranged LFTs They suggested to send autoimmune workup Which was already sent. 6-8 hrs after bone marrow Pt started having heavy bleeding from bone marrow procedure site and she was Tx with FFPs & platelets and with in next 12 hrs she started bleeding from every site (GI, oral cavity, Nose), Hematology was involved they suggested DIC workup Which was sent and which turned out to be negative Twice daily IV omeprazole was converted into infusion. Bone marrow aspirate: hypocellular/dilute specimen Few erythroid and myeloid precursors. No megakaryocyte seen Results of bone trephine (H&E) section: Erythroid hyperplasia with nuclear to cytoplasmic asynchrony. Few large cell seen ?early precursors. normal myeloid precursors. Adequate megakaryocytes. No metastatic infiltrate to granuloma seen. Final Report: Autoimmune hemolytic anemia ?cause. megaloblastic features on bone trephine can be due to folate deficiency (secondary to hemolysis). Pt was already kept on folic acid. Her bleeding continued and ENT service was involved for nasal packing She was transfused with multiple PRBC, FFPs, CryoPPT and was given factor VII (novoseven) on the advice of hematologist Multiple blood CS, Ascitic fluid CS, BM CS including AFB CS were sent which were negative. Her CCHF was sent which was also negative Her Ascitic fluid cytology was negative and so was autoimmune profile except AntiDsDNA which was 11.4 (n=0-6), Anti PLP & anticardiolipin Ab were negative C3=0.57 (n=0.88-2.01), C4=0.20 (n=0.16-0.47) She was started on pulse steroid After 3 days of bleeding and supportive transfusions she started dropping Spo2 on room air, able to maintain Spo2 at 96% on 15L Fio2 and her GCS dropped to 5/15, family was not agreed for intubation despite counseling. ABG=7.49/37.8/70.8/28.9/+5.9/95.3 on 15L Fio2 She was maintaining blood pressures initially then she started having hypotension hence was started on inotropic support but on 25 June 2007 at 1430 hrs she had a sad demise. Questions/Queries Whether she had SLE or something else? What was the cause of bleeding? Anything additional in the management of this patient which would have saved her life? What about her previous diagnosis of TB? If she would have been correctly diagnosed earlier would she able to survive? SYSTEMIC LUPUS ERYTHROMETOSIS (SLE) DEFINITION SLE is the prototype of a multisystem disease of autoimmune origin characterized clinically by acute/insidious onset chronic, remitting & relapsing in its course virtually affecting any organ of body & biochemically by presence of circulating autoantibodies against diversity of antigen. EPIDEMIOLOGY 1:2500 in general population 1:700 in women 9:1 Female to male ratio 2:1 Female to male ratio in childhood & in age group above 65 More common in African-American women. Clinical Features Constitutional Symptoms Fatique, Fever Arthralgia, Myalgia, Weight Loss Cutaneous Acute Skin Lesions Generalized, Erythema, Bullous, Butterfly Rash Subacute Erythematous Palpable Plaques Associated With Ro/Ssa Chronic Discoid Alopecia Raynaud Clinical Features 1. Renal - Acute Renal Failure Chronic Renal Failure Nephrotic Syndrome Nephritis Pyelonephritis Clinical Features .. 3. Pulmonary - Pneumonitis Pleurisy Pleural Effusion Pulmonary Embolism Pulmonary Fibrosis Alveolar Hemorrhage 4. G.I.T. Dysphagia - Mouth Ulcers - Peritonitis - Pancreatitis - Mesenteric Vasculitis - Bowel Infarction Clinical Features.. 5. Cardiac - Pericarditis - Endocarditis (Libman-Sachs) - Myocarditis - Coronary Artery Disease 6. Reticulo-Endothelial - Lymphadenopathy - Splenomegaly Diagnostic Criteria Diagnosis - Acr Criteria 1. Malar Rash 2. Discoid Rash 3. Skin Photosensitivity 4. Painless Oral Or Nasopharnygeal Ulcers 5. Non Erosive Arthritis Or Arthralgia 6. Serositis (Pleurisy, Pericarditis) 7. Renal Involvement 8. Neurologic Disorders 9. Haematologic Disorders 10.Immunologic Disorder (Le Cells, Anti-Dna, Anti-Smith, False + Ve Vdrl, Aca 11. Ana Any Four Out Of The Above Criteria Labs Laboratory 1. Leucopenia < 4,000 2. Thrombocytopenia 3. Anaemia Hemolytic, Normochromic, Normocytic 4. Markedly Elevated Esr > 100 5. Usually Normal Crp 6. Ana Present In 95% Homogeneous, Speckled 7. Anti Ds-Dna -Specific But Not Sensitive Suggest Severe Or Lupus Nephritis 8. Anti- Sm-Specific 9. Anti Ro/Ssa, La/Ssb Neonatal Lupus, Congenital Heart Block 10.Anti Ribosomal P-Lupus Cerebritis Labs.. 11. Anti-Phospholipid (Igg Or Igm) Aps 12. False Positive Vdrl Lupus Nephritis 1. Albuminuria > 0.5g/24 Hrs Or Dipstick 3+ 2. Casts (Rbc, Granular, Tubular, Mixed) 3. Haematuria (> 5rbc/Hpf) 4. Elevated Creatinine Treatment Simple Analgesics Nsaids Steroids Hydroxychloroquine Dmards - Methotrexate, Azathioprine, - Cyclosporin, Cyclophosphamide, - Mycophenolate Mofetil Complications - Opportunistic Infection - Avascular Necrosis - Premature Atherosclerosis Myocardial Infarction - RECURRENT ABORTION - NEONATAL LUPUS Prognosis Overall five years survival is more than 90 % Early mortality is due to organ failure or sepsis Late mortality is due to CVS complications Article Source: http://www.articlewheel.com
Dr. D.S. Merchant is a Gold Medalist in (Anatomy & Histology), Fellow Nephrology in Aga Khan University Hospital, Karachi ' Pakistan. For more Dissertation or seeking Dissertation help visit www.articlesbridge.com The Most popular website that offers information Research on different Disease and Case Studies. Please leave the links intact if you wish to reprint this article.
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