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Morbidity and Mortality Meeting

By: Dr. D.S. Merchant

Case History

• 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 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

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://articlekarma.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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