?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: 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.
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 it's 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
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
Ibrahim Lodhi has sinced written about articles on various topics from Religion, Nutrition and Pets. is a Gold Medalist in (Anatomy & Histology), Fellow Nephrology in Aga Khan University Hospital, Karachi ' Pakistan. For more Dissertation or seeking. Ibrahim Lodhi's top article generates over 90500 views. to your Favourites.