Dreariness and Mortality Conference .

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Morbidity and Mortality Conference. January 9, 2002 A# 50239521-3 (K1304) Carole Bibeau, MD. Initial Presentation. 69 yo WM establishing care at the WRJ VA Overall felt well ROS: DOE x 6 months, “dizziness,” easy bruising. Past Medical History Diabetes insipidus HTN CVA 1997
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Horribleness and Mortality Conference January 9, 2002 A# 50239521-3 (K1304) Carole Bibeau, MD

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Initial Presentation 69 yo WM building up care at the WRJ VA Overall felt well ROS: DOE x 6 months, "wooziness," simple wounding

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Past Medical History Diabetes insipidus HTN CVA 1997 PVOD with LE claudication Medications Desmopressin nasal shower qd Amlodipine 10 mg PO qd Benazepril 20 mg PO qd ASA 325 mg PO qd ALLS : NKDA History

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Social History Married, lived in New London, NH Tobacco: none EtOH: 1 drink/day Retired sales representative for surgical cleaning supply organization Family History Mother alive age 94, lived alone, solid Father expired age 74, obscure cause Sister alive, well No FHx CAD, DM, disease Social and Family History

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Physical Exam Gen: Looks marginally more established than expressed age VS: BP 110/60 HR 72 wt 160# HEENT: PERRL Card: RRR with incidental early beat; additional sound mid-systole; no S3 or S4; conceivable diastolic mumble Resp: Clear Abd: Soft, NT, ND, +BS Extr: No edema Neuro: No nystagmus; step stable

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Laboratory Data Fe 22 (50-180) TIBC 300 (205-395) Fe saturation 7.3% ESR 33 B12, Folate, Ferritin WNL 11.3 MCV 71 7.8 329 RDW 19.4 N70 L15 M12 E3 2+ anisocytosis, microcytosis, hypochromia 134 102 21 65 4.2 23 1.1 Ca, egg whites, LFT\'s, TSH, lipids, PSA WNL

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after two months... Follow up visit Interval history: new ecchymoses on lower arms VS: BP 96/70 HR 56 wt up 3# Exam: HEENT: no mucosal draining Card: RRR, systolic mumble Resp: diminished BS at bases respectively Extr: ecchymoses on lower arms CXR Echocardiogram

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Admission to WRJ VAH Patient conceded for further assessment ROS: denied mid-section torment, tearing sensation, back torment, stomach torment, SOB very still, HA, vision change, F/C/S, BRBPR, N/V Positive for diminished craving and discontinuous clogging x 4 months, assessed 8 pound weight reduction

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Exam Gen: Very wonderful 69 yo WM, A+Ox3, NAD VS: T 96.3 HR 87 BP 108/78 RR 20 sat 98% RA no pulsus HEENT: PERRL, EOMI, operation damp, no petechiae Neck: Supple, no LAD, JVP 7 cm, carotids 2+ without bruits Card: RRR, suppressed S1; S2 physiologically split, II/VI SEM LLSB Resp: Diminished BS left base Abd: NABS, delicate, NT, ND, no bruits Rectal: Nl tone, prostate to some degree augmented, no masses, delicate cocoa stool, heme positive Extr: Toes cool, femoral heartbeat 1+ L, 2+ R, no femoral bruits, pedal pulses realistic by means of Doppler (monophasic), 1+ setting lower leg edema, various little ecchymoses on UE\'s Neuro: Grossly non-central

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Pericardial emission Aortic analyzation Malignancy Inflammatory process Infectious process Coagulopathy Aortic dilatation Aortic dismemberment Collagen vascular malady Syphilis Atrial variation from the norm Thrombus Tumor Anemia, heme positive stools Malignancy Aorto-enteric fistula Assessment and Plan

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Additional Data Reticulocyte 1.1% ANA POSITIVE 1:320 dotted 1:160 homogeneous RF <20 ANCA (c and p) negative Anti-DS DNA negative RPR negative Blood cx x 1 negative PTT 53.4 PT WNL  THROMBOSIS SCREEN Lupus anticoagulant positive Negative: Anticardiolipin Ab, Factor V Leiden ECG: —NSR 64, nl hub, RBBB (new) —low voltage, no pathologic q\'s —ST sorrows V2-V6 —non-particular T changes U/A: pH 6.5, s.g 1.015, WNL

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Consultations/Findings Colonoscopy Mass at splenic flexure suspicious for threat, close entire deterrent; can\'t pass scope past it Path: Adenocarcinoma, modestly separated Rheumatology +ANA non-particular, likely because of danger Aneurysm not because of aortitis; ?paraneoplastic disorder Vascular + CT Surgery Not plausible to work Cardiology Lipomatous hypertrophy; no further assessment required

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Left Hemicolectomy, HD #10 Findings Moderately to ineffectively separated adenocarcinoma, augmentation to pericolic fat 11/26 hubs positive, no undeniable liver contribution Tumor-related thrombosis; begin LMWH Tumor board: offer adjuvant chemo when more steady Complications (HD #10-23) Post-operation atrial fibrillation with mid-section torment, hypotension Delirium Prolonged ileus Fluid and electrolyte anomalies

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Hypotensive sitting up in seat Telemetry caution: junctional musicality with PAC\'s, rate 60 Medicine counsel asked for Medications Metoprolol, desmopressin, heparin SC VS Afebrile BP 92/54, HR 60 irr, RR 24 I>>O, est 8 liters positive Exam Chronically sick showing up JVP 7 Upper aviation route ronchi, bountiful foamy sputum Can\'t auscultate heart sounds Abd without BS, somewhat delicate ECG Afib versus junctional cadence RBBB CXR Early CHF Hospital Day 24 (POD #14)

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Cardiac HD #28: Afib with RVR IV diltiazem  bradycardic with 3-6 second stops Temporary pacer put; amiodarone started PA catheter set RV 41/13 PA 41/17 PCWP 30 CI 2.4, SVR 1249 ID HD#24: MRSA in sputum E. faecalis 2/4 bottles in blood; begin ampicillin HD#27: Line tip (+) E. faecalis HD#36: Blood societies neg x 5 days Dispo Transfer back to pharmaceutical Hospital Days #28-36

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Nursing Notes Hospital Day #35 "I truly felt great before I came in. I believed that I had some minor issue and it would be settled. I never thought I had the greater part of this going on." "I might want to bite the dust gently and with respect. Might you be able to see to it that happens?"

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Transfer to DHMC (HD #41) Tachy-brady disorder, symptomatic Thoracic aortic aneurysm Pericardial emission Pleural emanations Stage III colon disease, s/p resection Lupus anticoagulant, tumor embolus, on anticoagulation Enterococcal bacteremia, MRSA + sputum Diabetes insipidus Deconditioning, delayed hospitalization (41 days)

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Medications on Transfer Amiodarone 400 mg PO qd ASA 81 mg PO qd Furosemide 80 mg IV offer Metoprolol 2 mg IV q4hr Spironolactone 25 mg PO qd Ampicillin 2 gm IV q4hr Desmopressin nasal splash offer Ferrous sulfate 325 mg PO tid Magnesium oxide 1 tab PO qd Heparin 5000 u SC offer Rabeprazole 20 mg PO offer PRN: Acetaminophen, albuterol nebs, AlOH/MgOH, docusate, ipratropium nebs, lubriderm, miconazole powder, NTG SL, prochlorperazine

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Hospital Course (#HD 41-43) HD #43: DDDR pacer set Patient wishes to exchange back to VA

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Hospital Day #44 Am VS check: 112 afib, 107/70, RR 30, 80-83% 3 liters, "exceptionally wet sounding hack" per nursing. Persistent denied SOB. Group suggested thoracentesis for side effects Patient denied mind; did not need spouse to know Team exchange with patient  CMO established Wife informed of grave circumstance Patient kicked the bucket calmly with wife at bedside Permission for post-mortem allowed

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Aorta: Dilated from curve to iliac bifurcation Severe atherosclerosis Aneurysm with wall painting thrombus

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Left kidney Right kidney and adrenal

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Left kidney Right kidney

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Periadrenal fat Peripancreatic fat

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Right lung, septum Right lung, pleura

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Dura: pachymeningitis

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Dural knob

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Right chamber and ventricle, schematic Right chamber and ventricle, A-01-153

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Right Atrial Lesion

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Pituitary Stalk

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Cardiovascular Periaortic Atrial myocardial Pulmonary Pleural Septal Bronchovascular packs CNS Dural knobs Hypophyseal stalk ?pituitary Retroperitoneal Perirenal Periadrenal peripancreatic Autopsy Finding: Disseminated Xanthogranulomatous handle

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Disseminated Xanthogranulomatous Inflammation: Differential Diagnosis Infection: Syphilis, atypical mycobacteria, growths Malignancy: provocative fibrosarcoma, lymphoproliferative disarranges Histiocytoses Other: fiery pseudotumor, lipid stockpiling issue

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Histiocytoses Class I: Langerhans cell histiocytosis Eosinophilic granuloma, Hand-Schuller-Christian malady, Letterer-Siwe ailment Class II: non-Langerhans cell histiocytosis Hemophagocytic lymphohistiocytosis (familial erythrophagocytic lymphohistiocytosis), infection related hemophagocytic disorder, sinus histiocytosis with enormous lymphadenopathy (Rosai-Dorfman sickness), Erdheim-Chester illness, xanthogranuloma, x anthoma disseminatium, reticulohistiocytoma, adolescent xanthogranulomatosis Class III: harmful histiocytic issue Acute monocytic leukemia, threatening histiocytosis, histiocytic lymphoma

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Langerhans cell Children and adolescents Bone-marrow determined antigen exhibiting cells not of monocyte/macrophage heredity Immunohistochemical recoloring: CD1a +, S-100+, CD68 - Non-Langerhans cell Adults: mean age 53 Monocyte-macrophage genealogy Immunohistochemical recoloring: CD1a - , S-100 basically negative, CD68 + Langerhans versus Non-Langerhans cell histiocytoses

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Hemophagocytic lymphohistiocytosis infection related hemophagocytic disorder Rosai-Dorfman malady (sinus histiocytosis with enormous lymphadenopathy Erdheim-Chester ailment (Lipid granulomatosis) xanthoma disseminatium Reticulohistiocytoma adolescent xanthogranulomatosis xanthogranuloma Non-Langerhans Cell Histiocytoses

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Histopathology: Infiltration of different organ locales by frothy histiocytes , lymphocytes and scattered Touton goliath cells with broad fibrosis Clinical signs: Symmetric, sclerotic sores of long bones dyspnea and respiratory disappointment because of interstitial lung illness Hydronephrosis and renal disappointment optional to retroperitoneal xanthogranulomatosis Diabetes insipidus Extra-pivotal masses (dural knobs) Retroorbital masses with visual unsettling influences Ataxia auxiliary to cerebellar contribution Pericardial penetration - > pericardial emission Erdheim-Chester Disease

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Erdheim-Chester Disease A-01-153 A-95-23

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