Budd chiari syndrome in a young HIV positive male with colon cancer
1
Giardia Ag; Colonoscopy Bx’s normal; An UGI/SB series was negative. On enteroscopy, jejunal folds had a saw–toothed appearance; total villous atrophy was compatible with CD. Tissue transglutaminase IgA134(N20), antigliadin IgA was 200(N20) antigliadin IgG 32 (N20). Diarrhea resolved with resolution on a gluten free diet. Six months later she had increased LFT’s with AST74(N2–35), ALT73(N2– 40), Alk.Phos. 317(N2–125), TB.3(N.2–1.3), DB.1(N.3). No ETOH intake. Hepatitis profile negative for HBV, HCV, HAV IgM; Alpha–1–antitrypsin, Ceruloplasmin, alphafeto protein, iron saturation and protime were normal. Liver–spleen scan showed mild hepatomegaly, and no splenomegaly. Liver Bx histologically revealed small plasma cell infiltrate in portal tracts with minimal extension into adjacent parenchyma with rare microgranulomas compatible with autoim- mune hepatitis (AH). Although the ASMA and anti–LKM were both negative, the pt. had definitive AH by International Autoimmune Hepatitis Group scoring system criteria. Her point value was 16 (15definite; 1015probable). She subsequently developed purpuric–like lesions on her lower extremities. Skin bx was compatible with [immune] vasculitis associated with hyperglobunemic purpura. Lab showed elevated absolute lymphocyte count6,132; TRG179; BII–microglobulin was 3.3. RPR and ANA were negative and Total Globulin4.5 (N2). SPEP revealed a polyclonal hypergammaglobulinemia. Discussion: Skin and liver disease have been reported in association with CD. The association of autoimmune disorders in pts. with CD is not surprising as organ specific auto antibodies have been reported. They have a wider spectrum of immunological diseases than previously thought in- cluding AH, and hypergammaglobulinemic purpura, as in our patient.Con- versely, pts. with AH develop chronic ulcerative colitis, with primary sclerosing cholangitis, uveitis, celiac disease, pernicious anemia, vasculitis and mixed connective tissue diseases. This case illustrates the association of systemic autoimmune disorders with CD and suggests that patients with CD should be evaluated for other autoimmune disorders. 416 BUDD CHIARI SYNDROME IN A YOUNG HIV POSITIVE MALE WITH COLON CANCER Umesh Sharma, M.D., Hamid Mojab, M.D., Sita Chokhavatia, M.D.* and Kiran Chawla, M.D. Internal Medicine, Mt. Sinai Services at Queens Hospital Center, Jamaica, NY and Radiology, Mt. Sinai Services at Queens Hospital Center, Jamaica, NY. Purpose: Budd Chiari Syndrome (BCS) includes a variety of disorders arising from hepatic venous outflow obstruction caused by hypercoagulable states, neoplasms,etc. Case Report: A 30 year old Hispanic male with sexually acquired HIV infection had been receiving anti–retroviral therapy (ART) since 1997 and had undergone colon resection in 1997 for Dukes stage B1 colon carci- noma. He presented with increasing abdominal swelling, nausea, vomiting and fever for 2 weeks. Examination revealed reduced air entry at right lung base, hepatomegaly, ascites and pedal edema. Lab. data – normal CBC count, normal SMA–9, prothrombin time–14.4 sec. (9.8 – 15), partial thromboplastin time–32.8 sec. (28 –31.5), alk. phos.–127 U/L (30 –115), AST– 305 U/L (2– 40), GGT–182 U/L (0 –24), ALT–155 U/L (2–50), LDH–584 U/L (90 –225), alb–3.2 gms/dL (3.3–5.3), total protein–5.6 gms/dL (6.5– 8.5), total bilirubin–2.1 mg/dL (0 –1.5). Abdominal paracen- tesis revealed 159 WBCs, 95 RBCs, no evidence of atypical cells, normal amylase, serum/ascites albumin gradient 1 and negative bacterial and fungal cultures. Hepatitis A, B, and C serologies were negative; protein C–59.2% (60 –100), protein S–57.7% (59 –100), Antithrombin III levels– 52.5% (60 –100), anticardiolipin antibody, lupus anticoagulant and factor V Leiden mutation were negative. Thorax and abdomen CT scans demon- strated: 1) extensive thrombosis involving the entire inferior vena cava, extending from right atrium to left common iliac veins; thrombosis of the hepatic veins and arteries, 2) hepatic infarction of the right and left lobe segments, 3) ascites involving perihepatic space, right lower abdomen and right pleural effusion. Colonoscopic examination was unremarkable. Se- rum CEA levels were elevated – 172.9ng/mL (normal in immediate post– operative period –2.9ng/mL). Alphafetoprotein levels were normal. ART was held. Patient was started on heparin and further surgical intervention was not contemplated due to high surgical risk. Oral anticoagulants, di- uretics, salt and fluid restriction inititated and considered for chemotherapy, but he succumbed to worsening malignant ascites, deteriorating liver func- tion and terminal hepatorenal syndrome. Conclusion: Medical management of malignant BCS is of limited value. Thrombolytics can be used in early disease. Angioplasty or transhepatic intrajugular portasystemic shunt may serve as a bridge to liver transplantation in severe nonmalignant liver disease. 417 PNEUMOTHORAX – AN UNUSUAL COMPLICATION OF NASO–GASTRIC INTUBATION Umesh Sharma, M.D., Hamid Mojab, M.D., Sita Chokhavatia, M.D.*, Kiran Chawla, M.D. and Jean Fleischman, M.D. Internal Medicine, Mt. Sinai Services at Queens Hospital Center, Jamaica, NY and Radiology, Mt. Sinai Services at Queens Hospital Center, Jamaica, NY. Purpose: Naso– gastric (NG) intubation is the most common assisted enteral feeding method. It is a relatively benign procedure but many complications have been reported including malposition; nasopharyngeal, esophageal and gastric trauma; and infection. Inadvertent tracheal intuba- tions with NG tubes occur in 0.3–15% insertions. Case Report: A 101 year old female with a past medical history of hypertension, multiple transient ischemic attacks, dementia, bronchiectasis and a recent urinary tract infection presented with hypothermia, dehydra- tion and unresponsiveness. She was treated with warmed I.V. fluids, supplemental oxygen and antibiotics for presumed sepsis. Her mental status improved but she refused to eat. Patient’s family declined percutaneous endoscopic gastrostomy but agreed to nutritional support via NG tube which was successfully placed but was pulled out by the patient the same day. A second NG tube was placed and chest xray (CXR) revealed mal- position of the tube into the right mainstem bronchus with an associated right pneumothorax. The patient was not in any respiratory distress and had an 02 saturation of 98% on 2 liters of supplemental oxygen. The NG tube was removed. The patient’s family initially refused chest tube insertion but agreed when subsequent CXR revealed an enlarging tension pneumotho- rax. Enteral nutrition was reinitiated following successful placement of another NG tube. Conclusion: Penetration of distal airways and introduction of various chemicals into the lung and pleural spaces may occur before NG tube malpositioning is recognized, especially in obtunded patients in whom subjective discomfort and physical examination are inconsistent. Confir- mation of NG tube placement by aspiration of fluid from the proximal port or insufflation of air with associated abdominal auscultation of a “pseudo– confirmatory gurgle” may yield false positive results. CXR to confirm tube positioning prior to initiating feeds and drug installation is recommended. 418 HEPATITIS C VIRUS ASSOCIATED ARTHROPATHY IN ABSENCE OF CLINICAL AND BIOCHEMICAL EVIDENCE OF LIVER DISEASE – RESPONDING TO INTERFERON THERAPY Abbasi J. Akhtar, M.D.*, Aleli F. Vidad, M.D. and Allen S. Funnye, M.D. Department of Internal Medicine, Charles R. Drew University of Medicine and Science, Los Angeles, CA. Purpose: Extrahepatic manifestations of Hepatitis C, including arthralgia and arthritis, and liver involvement in rheumatoid arthritis are well known, but Hepatits C associated arthropathy in absence of clinical and biochem- ical evidence of liver disease and response to interferon therapy is rather unusual. A case of 32 year Filipino male, is presented, who was referred by his physician, with a history of abrupt onset of polyarthritis in a rheumatoid distribution including small joints of both hands, elbows and knee joints. There was no history of any risk factors for viral hepatitides and past medical history and family history, was non– contributory. Physical exam- S137 AJG – September, Suppl., 2002 Abstracts
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Transcript of Budd chiari syndrome in a young HIV positive male with colon cancer
Giardia Ag; Colonoscopy Bx’s normal; An UGI/SB series was negative.On enteroscopy, jejunal folds had a saw–toothed appearance; total villousatrophy was compatible with CD. Tissue transglutaminaseIgA�134(N��20), antigliadin IgA was �200(N��20) antigliadinIgG � 32 (N��20). Diarrhea resolved with resolution on a gluten freediet. Six months later she had increased LFT’s with AST�74(N�2–35),ALT�73(N�2–40), Alk.Phos. �317(N�2–125), TB�.3(N�.2–1.3),DB�.1(N�.3). No ETOH intake. Hepatitis profile negative for HBV,HCV, HAV IgM; Alpha–1–antitrypsin, Ceruloplasmin, alphafeto protein,iron saturation and protime were normal. Liver–spleen scan showed mildhepatomegaly, and no splenomegaly. Liver Bx histologically revealedsmall plasma cell infiltrate in portal tracts with minimal extension intoadjacent parenchyma with rare microgranulomas compatible with autoim-mune hepatitis (AH). Although the ASMA and anti–LKM were bothnegative, the pt. had definitive AH by International Autoimmune HepatitisGroup scoring system criteria. Her point value was 16 (�15��definite;�10�15��probable). She subsequently developed purpuric–like lesionson her lower extremities. Skin bx was compatible with [immune] vasculitisassociated with hyperglobunemic purpura. Lab showed elevated absolutelymphocyte count�6,132; TRG�179; BII–microglobulin was 3.3. RPRand ANA were negative and Total Globulin�4.5 (N�2). SPEP revealed apolyclonal hypergammaglobulinemia.Discussion: Skin and liver disease have been reported in association withCD. The association of autoimmune disorders in pts. with CD is notsurprising as organ specific auto antibodies have been reported. They havea wider spectrum of immunological diseases than previously thought in-cluding AH, and hypergammaglobulinemic purpura, as in our patient.Con-versely, pts. with AH develop chronic ulcerative colitis, with primarysclerosing cholangitis, uveitis, celiac disease, pernicious anemia, vasculitisand mixed connective tissue diseases. This case illustrates the associationof systemic autoimmune disorders with CD and suggests that patients withCD should be evaluated for other autoimmune disorders.
416
BUDD CHIARI SYNDROME IN A YOUNG HIV POSITIVEMALE WITH COLON CANCERUmesh Sharma, M.D., Hamid Mojab, M.D., Sita Chokhavatia, M.D.*and Kiran Chawla, M.D. Internal Medicine, Mt. Sinai Services atQueens Hospital Center, Jamaica, NY and Radiology, Mt. Sinai Servicesat Queens Hospital Center, Jamaica, NY.
Purpose: Budd Chiari Syndrome (BCS) includes a variety of disordersarising from hepatic venous outflow obstruction caused by hypercoagulablestates, neoplasms,etc.Case Report: A 30 year old Hispanic male with sexually acquired HIVinfection had been receiving anti–retroviral therapy (ART) since 1997 andhad undergone colon resection in 1997 for Dukes stage B1 colon carci-noma. He presented with increasing abdominal swelling, nausea, vomitingand fever for 2 weeks. Examination revealed reduced air entry at right lungbase, hepatomegaly, ascites and pedal edema. Lab. data – normal CBCcount, normal SMA–9, prothrombin time–14.4 sec. (9.8– 15), partialthromboplastin time–32.8 sec. (28–31.5), alk. phos.–127 U/L (30–115),AST– 305 U/L (2–40), GGT–182 U/L (0–24), ALT–155 U/L (2–50),LDH–584 U/L (90–225), alb–3.2 gms/dL (3.3–5.3), total protein–5.6gms/dL (6.5–8.5), total bilirubin–2.1 mg/dL (0–1.5). Abdominal paracen-tesis revealed 159 WBCs, 95 RBCs, no evidence of atypical cells, normalamylase, serum/ascites albumin gradient �1 and negative bacterial andfungal cultures. Hepatitis A, B, and C serologies were negative; proteinC–59.2% (60–100), protein S–57.7% (59–100), Antithrombin III levels–52.5% (60–100), anticardiolipin antibody, lupus anticoagulant and factor VLeiden mutation were negative. Thorax and abdomen CT scans demon-strated: 1) extensive thrombosis involving the entire inferior vena cava,extending from right atrium to left common iliac veins; thrombosis of thehepatic veins and arteries, 2) hepatic infarction of the right and left lobesegments, 3) ascites involving perihepatic space, right lower abdomen andright pleural effusion. Colonoscopic examination was unremarkable. Se-rum CEA levels were elevated – 172.9ng/mL (normal in immediate post–
operative period –2.9ng/mL). Alphafetoprotein levels were normal. ARTwas held. Patient was started on heparin and further surgical interventionwas not contemplated due to high surgical risk. Oral anticoagulants, di-uretics, salt and fluid restriction inititated and considered for chemotherapy,but he succumbed to worsening malignant ascites, deteriorating liver func-tion and terminal hepatorenal syndrome. Conclusion: Medical managementof malignant BCS is of limited value. Thrombolytics can be used in earlydisease. Angioplasty or transhepatic intrajugular portasystemic shunt mayserve as a bridge to liver transplantation in severe nonmalignant liverdisease.
417
PNEUMOTHORAX – AN UNUSUAL COMPLICATION OFNASO–GASTRIC INTUBATIONUmesh Sharma, M.D., Hamid Mojab, M.D., Sita Chokhavatia, M.D.*,Kiran Chawla, M.D. and Jean Fleischman, M.D. Internal Medicine,Mt. Sinai Services at Queens Hospital Center, Jamaica, NY andRadiology, Mt. Sinai Services at Queens Hospital Center, Jamaica, NY.
Purpose: Naso–gastric (NG) intubation is the most common assistedenteral feeding method. It is a relatively benign procedure but manycomplications have been reported including malposition; nasopharyngeal,esophageal and gastric trauma; and infection. Inadvertent tracheal intuba-tions with NG tubes occur in 0.3–15% insertions.Case Report: A 101 year old female with a past medical history ofhypertension, multiple transient ischemic attacks, dementia, bronchiectasisand a recent urinary tract infection presented with hypothermia, dehydra-tion and unresponsiveness. She was treated with warmed I.V. fluids,supplemental oxygen and antibiotics for presumed sepsis. Her mental statusimproved but she refused to eat. Patient’s family declined percutaneousendoscopic gastrostomy but agreed to nutritional support via NG tubewhich was successfully placed but was pulled out by the patient the sameday. A second NG tube was placed and chest xray (CXR) revealed mal-position of the tube into the right mainstem bronchus with an associatedright pneumothorax. The patient was not in any respiratory distress and hadan 02 saturation of 98% on 2 liters of supplemental oxygen. The NG tubewas removed. The patient’s family initially refused chest tube insertion butagreed when subsequent CXR revealed an enlarging tension pneumotho-rax. Enteral nutrition was reinitiated following successful placement ofanother NG tube.Conclusion: Penetration of distal airways and introduction of variouschemicals into the lung and pleural spaces may occur before NG tubemalpositioning is recognized, especially in obtunded patients in whomsubjective discomfort and physical examination are inconsistent. Confir-mation of NG tube placement by aspiration of fluid from the proximal portor insufflation of air with associated abdominal auscultation of a “pseudo–confirmatory gurgle” may yield false positive results. CXR to confirm tubepositioning prior to initiating feeds and drug installation is recommended.
418
HEPATITIS C VIRUS ASSOCIATED ARTHROPATHY INABSENCE OF CLINICAL AND BIOCHEMICAL EVIDENCE OFLIVER DISEASE – RESPONDING TO INTERFERON THERAPYAbbasi J. Akhtar, M.D.*, Aleli F. Vidad, M.D. and Allen S. Funnye,M.D. Department of Internal Medicine, Charles R. Drew University ofMedicine and Science, Los Angeles, CA.
Purpose: Extrahepatic manifestations of Hepatitis C, including arthralgiaand arthritis, and liver involvement in rheumatoid arthritis are well known,but Hepatits C associated arthropathy in absence of clinical and biochem-ical evidence of liver disease and response to interferon therapy is ratherunusual. A case of 32 year Filipino male, is presented, who was referred byhis physician, with a history of abrupt onset of polyarthritis in a rheumatoiddistribution including small joints of both hands, elbows and knee joints.There was no history of any risk factors for viral hepatitides and pastmedical history and family history, was non–contributory. Physical exam-
S137AJG – September, Suppl., 2002 Abstracts
