Intrahepatic cholestasis: Rare inaugural manifestation of AL amyloidosis
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UNCORRECTED PROOF controls were included and underwent bone densitometry. Serum sclerostin, osteocalcin, collagen telopeptide, adiponectin, leptin, insulin, resistin, TNF-α, and IL-6 were determined. Liver fat was histo- morphometrically assessed. Results: Serum sclerostin was slightly, non- significantly higher among patients (17.78 ± 6.37 pmol/l) than controls (15.06 ± 3.93 pmol/l, t = 1.09; p = 0.28). A direct correlation was observed between sclerostin levels and BMD at arms (r = 0.33), ribs (r = 0.34), pelvis (r = 0.38), legs (r = 0.45) and total BMD (r = 0.39; p b 0.05 in all the cases), as well as a direct significant relationship between sclerostin levels and liver fat amount (r = 0.44; p = 0.007) and serum TNF-α levels (r = 0.39; p = 0.014), but not with adiponectin, leptin, resistin, insulin, or HOMA. Osteocalcin was inversely related with IL-6 (r = -0.36; p = 0.033), and directly with adiponectin (r = 0.51; p = 0.001), but not with liver fat, Knodell index or Metavir scores. Discussion and conclusions: Therefore, serum sclerostin levels correlat- ed directly with BMD and serum telopeptide. In addition direct relationships of sclerostin, but inverse ones of osteocalcin, with variables associated with insulin resistance suggest a role of bone on intermediary metabolism. doi:10.1016/j.ejim.2013.08.207 ID: 403 Intrahepatic cholestasis: Rare inaugural manifestation of AL amyloidosis N. Cercas Pinheiro a , J. Jorge b , R. Tato Marinho b , N. Fatela b , C. Baldaia b , F. Ramalho b , J. Velosa b a Department of Internal Medicine I A, Hospital Egas Moniz, Lisboa, Portugal b Department of Gastroenterology, Liver Unit, Hospital Santa Maria, Lisboa, Portugal Systemic or AL amyloidosis is the consequence of deposition of immunoglobulin light chains in tissues, organized in the form of fibrils, ultimately leading to multiorgan dysfunction. At the base of the overproduction of immunoglobulins is usually the presence of plasma cell dyscrasia. The organs most frequently affected are the heart, kidneys and liver. The occurrence of severe hepatic dysfunction in AL amyloidosis is rare and an independent indicator of poor prognosis. The presence of amyloid cholestasis, reflected by conjugated hyperbilirubinemia and consequent jaundice, implies an overall survival of about three months in some studies. The authors present a review of 3 clinical cases with severe impairment of liver function as inaugural manifestation of AL amyloid- osis. The concomitant presence of erythrocytosis and hypoglycemia in the 1st case is highlighted, an association that is rarely reported in the literature. The 2nd case stands out for the co-existence of severe restrictive cardiomyopathy which was an obstacle in the therapeutic decision, according to the staging system of the Mayo Clinic reviewed in 2012. The 3rd case demonstrates the typical multisystemic disorder of AL amyloidosis, with severe hepatic, renal and autonomic involvement. Liver biopsy led to diagnosis in all cases. The authors present a brief discussion of the main characteristics of each case, their respective pathophysiological framing and diagnostic challenge. The limited therapeutic options available in the advanced stage of the disease are analyzed, as well as the fatal outcome in a short period of time after diagnosis in two of the cases presented. Last but not the least, the authors emphasize the importance of considering systemic amyloidosis in the differential diagnosis of intrahepatic cholestasis despite its rarity, along with other commoner etiologies. doi:10.1016/j.ejim.2013.08.208 ID: 406 Beyond acute abdomen… N. Cercas Pinheiro a , R. Tato Marinho b , N. Fatela b , C. Baldaia b , F. Ramalho b , J. Velosa b a Department of Internal Medicine I A, Hospital Egas Moniz, Lisboa, Portugal b Department of Gastroenterology, Liver Unit, Hospital Santa Maria, Lisboa, Portugal The authors present the case of male patient, 68 years, farmer, autonomous, hypertensive, diabetic, excessive alcohol intake 200 g/day for 20 years, and mild hepatic steatosis documented on abdominal ultrasound 4 months before this episode. Appealed to the emergency department by sudden intense pain in the right shoulder associated with vigorous physical exertion, with subsequent irradiation to the flank and right upper abdominal quadrant. Physical examination highlighted hematoma at the right upper abdominal quadrant and pain on deep palpation without peritoneal reaction. Laboratory highlighted neutro- philic leukocytosis, slightly elevated aminotransferases (2 × normal values) and GGT 410 IU/L, with no other changes. Abdominal radiograph was innocent. Abdominal ultrasound showed liver with irregular borders, splenomegaly and ascites. Abdominal-pelvic CT scan compatible with heterogeneous liver, irregular, and solid mass 38 mm at the segment VII/ VIII with washout in the portal phase, suggestive of hepatocellular carcinoma (HCC). There was also presence of a moderate amount of intraperitoneal free fluid, denser near the lesion, suggesting hemo- peritoneum. In the ward the diagnostic hypotheses of inaugural decompensation of alcoholic liver cirrhosis CHILD A (6), MELD 9, ECOG 0, BCLC A, and hemoperitoneum in the context of spontaneous rupture of HCC were admitted. Given the clinical and hemodynamic stability surgical resection of hepatic node was possible with no acute complications. The patient was then oriented to the Hepatology consultation. Spontaneous rupture of HCC is a rare and potentially fatal entity, occurring in about 3–26% of cases. The clinic is very nonspecific, mimicking other more common etiologies of acute abdomen, requiring high clinical suspicion. Mortality is high, reaching 66,7% in some studies. The therapeutic approach is not consensual, comprising mainly surgical resection, transarterial chemoembolization and conservative treatment. doi:10.1016/j.ejim.2013.08.209 ID: 418 Celiac disease in elderly L. Kukuckova a , K. Dostalova b , V. Sucha a , S. Moricova b , D. Danis c a Long Term Ill Department, Derer's Hospital, University Hospital, Bratislava, Slovakia b Department of Preventive and Clinical Medicine, Faculty of Public Health, Slovak Medical University, Bratislava, Slovakia c Cytophatos, spol. s.r.o., Institut of Pathology, University Hospital, Bratislava, Slovakia Objective: Celiac Disease is an immune-mediated lifetime systemic disorder of the small intestine caused by permanent intolerance to gluten and related prolamins in genetically susceptible individuals. During the last 20 years, the perception of celiac disease has changed from a rather uncommon enteropathy to a common multi-organ disease with the presence of a variable combination of clinical manifestation, CD-specific antibodies and strong genetic predisposition. Prevalence of celiac disease is high, 1:70–1:550 in the world, the expected prevalence in Slovakia is 1:250. There is a significant lack of data on the frequency of the disease in the older population. Some Abstracts e84
Transcript of Intrahepatic cholestasis: Rare inaugural manifestation of AL amyloidosis
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TEDPR
OOF
controls were included and underwent bone densitometry. Serumsclerostin, osteocalcin, collagen telopeptide, adiponectin, leptin, insulin,resistin, TNF-α, and IL-6 were determined. Liver fat was histo-morphometrically assessed. Results: Serum sclerostin was slightly, non-significantly higher among patients (17.78 ± 6.37 pmol/l) than controls(15.06 ± 3.93 pmol/l, t = 1.09; p= 0.28). A direct correlation wasobserved between sclerostin levels and BMD at arms (r = 0.33), ribs(r = 0.34), pelvis (r = 0.38), legs (r = 0.45) and total BMD (r = 0.39;p b 0.05 in all the cases), as well as a direct significant relationshipbetween sclerostin levels and liver fat amount (r = 0.44; p = 0.007) andserum TNF-α levels (r = 0.39; p= 0.014), but not with adiponectin,leptin, resistin, insulin, or HOMA. Osteocalcin was inversely related withIL-6 (r =−0.36; p= 0.033), and directly with adiponectin (r = 0.51;p= 0.001), but not with liver fat, Knodell index or Metavir scores.Discussion and conclusions: Therefore, serum sclerostin levels correlat-ed directly with BMD and serum telopeptide. In addition directrelationships of sclerostin, but inverse ones of osteocalcin, with variablesassociated with insulin resistance suggest a role of bone on intermediarymetabolism.
doi:10.1016/j.ejim.2013.08.207
ID: 403Intrahepatic cholestasis: Rare inaugural manifestation ofAL amyloidosisN. Cercas Pinheiroa, J. Jorgeb, R. Tato Marinhob, N. Fatelab, C. Baldaiab,F. Ramalhob, J. Velosab
aDepartment of Internal Medicine I A, Hospital Egas Moniz, Lisboa,PortugalbDepartment of Gastroenterology, Liver Unit, Hospital Santa Maria,Lisboa, Portugal
Systemic or AL amyloidosis is the consequence of deposition ofimmunoglobulin light chains in tissues, organized in the form of fibrils,ultimately leading to multiorgan dysfunction. At the base of theoverproduction of immunoglobulins is usually the presence of plasmacell dyscrasia. The organs most frequently affected are the heart, kidneysand liver. The occurrence of severe hepatic dysfunction in AL amyloidosisis rare and an independent indicator of poor prognosis. The presence ofamyloid cholestasis, reflected by conjugated hyperbilirubinemia andconsequent jaundice, implies an overall survival of about threemonths insome studies. The authors present a review of 3 clinical caseswith severeimpairment of liver function as inaugural manifestation of AL amyloid-osis. The concomitant presence of erythrocytosis and hypoglycemia inthe 1st case is highlighted, an association that is rarely reported in theliterature. The 2nd case stands out for the co-existence of severerestrictive cardiomyopathy which was an obstacle in the therapeuticdecision, according to the staging system of the Mayo Clinic reviewed in2012. The 3rd case demonstrates the typicalmultisystemic disorder of ALamyloidosis, with severe hepatic, renal and autonomic involvement.Liver biopsy led to diagnosis in all cases. The authors present a briefdiscussion of the main characteristics of each case, their respectivepathophysiological framing and diagnostic challenge. The limitedtherapeutic options available in the advanced stage of the disease areanalyzed, as well as the fatal outcome in a short period of time afterdiagnosis in two of the cases presented. Last but not the least, the authorsemphasize the importance of considering systemic amyloidosis in thedifferential diagnosis of intrahepatic cholestasis despite its rarity, alongwith other commoner etiologies.
doi:10.1016/j.ejim.2013.08.208
ID: 406Beyond acute abdomen…N. Cercas Pinheiroa, R. Tato Marinhob, N. Fatelab, C. Baldaiab,F. Ramalhob, J. Velosab
aDepartment of Internal Medicine I A, Hospital Egas Moniz, Lisboa,PortugalbDepartment of Gastroenterology, Liver Unit, Hospital Santa Maria,Lisboa, Portugal
The authors present the case of male patient, 68 years, farmer,autonomous, hypertensive, diabetic, excessive alcohol intake 200 g/dayfor 20 years, and mild hepatic steatosis documented on abdominalultrasound 4 months before this episode. Appealed to the emergencydepartment by sudden intense pain in the right shoulder associated withvigorous physical exertion, with subsequent irradiation to the flank andright upper abdominal quadrant. Physical examination highlightedhematoma at the right upper abdominal quadrant and pain on deeppalpation without peritoneal reaction. Laboratory highlighted neutro-philic leukocytosis, slightly elevated aminotransferases (2× normalvalues) and GGT 410 IU/L, with no other changes. Abdominal radiographwas innocent. Abdominal ultrasound showed liverwith irregular borders,splenomegaly and ascites. Abdominal-pelvic CT scan compatible withheterogeneous liver, irregular, and solid mass 38mm at the segment VII/VIII with washout in the portal phase, suggestive of hepatocellularcarcinoma (HCC). There was also presence of a moderate amount ofintraperitoneal free fluid, denser near the lesion, suggesting hemo-peritoneum. In the ward the diagnostic hypotheses of inauguraldecompensation of alcoholic liver cirrhosis CHILD A (6), MELD 9, ECOG0, BCLC A, and hemoperitoneum in the context of spontaneous rupture ofHCCwere admitted. Given the clinical andhemodynamic stability surgicalresection of hepatic node was possible with no acute complications. Thepatient was then oriented to the Hepatology consultation. Spontaneousrupture of HCC is a rare and potentially fatal entity, occurring inabout 3–26% of cases. The clinic is very nonspecific, mimicking othermore common etiologies of acute abdomen, requiring high clinicalsuspicion. Mortality is high, reaching 66,7% in some studies. Thetherapeutic approach is not consensual, comprising mainly surgicalresection, transarterial chemoembolization and conservative treatment.
doi:10.1016/j.ejim.2013.08.209
ID: 418Celiac disease in elderlyL. Kukuckovaa, K. Dostalovab, V. Suchaa, S. Moricovab, D. Danisc
aLong Term Ill Department, Derer's Hospital, University Hospital,Bratislava, SlovakiabDepartment of Preventive and Clinical Medicine, Faculty of PublicHealth, Slovak Medical University, Bratislava, SlovakiacCytophatos, spol. s.r.o., Institut of Pathology, University Hospital,Bratislava, Slovakia
Objective: Celiac Disease is an immune-mediated lifetime systemicdisorder of the small intestine caused by permanent intolerance togluten and related prolamins in genetically susceptible individuals.During the last 20 years, the perception of celiac disease has changedfrom a rather uncommon enteropathy to a common multi-organdisease with the presence of a variable combination of clinicalmanifestation, CD-specific antibodies and strong genetic predisposition.Prevalence of celiac disease is high, 1:70–1:550 in the world, theexpected prevalence in Slovakia is 1:250. There is a significant lack ofdata on the frequency of the disease in the older population. Some
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