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Determination of ammonia in capillary ear-lobe blood is an alternative for arterial ammonia determination
S 165
Miscellaneous
DETERMINATION OF AMMONIA IN CAPILLARY EAR-LOBE BLOOD IS AN ALTERNATIVE FOR ARTERIAL A/~ONIA DETERMINATION .
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GIANT HAEMANGIOMA OF LIVER WITH THROMBOCYTOPENIA - A case report
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J.R. Hmzenga, J.G.J. Vos, C.H. Gips , A. Tanqernan , R.a. Gre. .1~1tt tldrl' ttltt • t • vlnK , E. Kerr , H.O. Conn , P.L.H. Jansen . D1v. of Hepatol~v-Gestroenteroloav and ,it, Pulaonology, Univ. Hospital Groningen; t~A . -" . . *i* . ' GISH, Medlcal Faculty, State Unlv. Gronlngen; Dlv. of Gast rolntestinal and Liver Diseases, Univ. Hospital Ni~aeqen, The Netherlands and ***** Yale University, Hew Haven, USA
Blood auonia is ae0ngst others used in liver disease to diagnose or to exclude hepatic encephalopathy, Arterial blood is superior to peripheral venous blood because of euscle netabolisa. As there may be medical ethical problems to the detendnation of arterial blood, capillary blood a~onia eight be an alternative. The all of this study was to establish the reference values for a,,onia in capillary ear-lobe blood ((3) and to coepare arterial (AB) and capillary anlonia in patients with respiratory diseases (RO), needing arterial
blood gas analysis and with liver diseases (LD). Hethods. ~non~a deterninations were perforned using the Blood ANnonia Checker~ (BAC) II systen, which needs 20 ~i of whole blood. CB and AN sanples were taken fron 43 RD patients without liver disease and 17 LD patients. For reference CB of 57 nornal healthy controls were used. Results. The reference CB range in controls anounted to 8-41, eedian 20 ~mol/l. RD patients had a CB auonia range of 7-41, |edian 18 and a ~ a~onia range of 7-35, median 16 /~101/1. CB references and CB RD patients were equal as were CB RD patie.nts and ~ RD patients (p=0.22 and p=0.71 respectively). In the LD group (3 aeounted to 23-152, median 74 and AN to 25-161, ledian 58 ~oi/I (CB higher,1~0.025). For all 60 patients {RO ÷ LD) the correlation (r) between (3 and AN wan 0.959 (p
LIVER TRANSPLANTATION FOR WILSON'S DISEASE P. lchai. D. Samuel. A. Arab. F. Saliba. H. Bismuth. Hepato-Biliary Surgery and Liver Transplant Research Center, H6pital Paul Brousse, Villejuif, Universit8 Pads Sud, France Wilson's disease is an inhereditaty disorder manifestating by copper overload affecting mainly the liver and the brain. In most cases, long life administration of D-penicillamine can cure the disease; however in selected cases, liver transplantation can be necessary. The aim of this study was to analyse retrospectively the indications and the results of liver transplantation in 12 pts (9 F, 3M) transplanted for Wilson's disease from 1985 to 1992. Caeruleoplasmine level, cupremia, cupruria, Kayser Fleisher ring detection, andliver biopsy were performed before and after transplantation. The indication for liver transplantation was fulminant liver failure in 6 cases, primary decompensated cirrhosis in 3, and decompensated cirrhosis after long term treatment with D penicillamine in 3. Among the 6 patients (6 F, mean age 17 years, range: 11-20) with fulminant liver failure, 4 were in coma, 1 was confused and one had no encephalopathy the mean duration of the disease was 26 days; caeruleoplasmine was low in all, factor V level was < 30% in all, haemolytic anaemia was present in 5. One patient developed transiently after transplantation, neurologic symptoms of Wilson's disease; 6/6 were alive with normal copper metabolism. In 3 patients, (2F, IM, mean age 14 year) Wilson's disease was revealed by primary decompensated cirrhosis, mean duration of the disease was 14 weeks. D penicillamine rescue treatment was unsuccessful in all. Ascites was present in 3, spontaneous bacterial peritonitis in 1, encephalopathy and jaundice in 2 factor V was below 50% in all. 213 pts are alive with a normal copper metabolism. In 3 patients(l M, 2 F; mean age 28 year), cirrhosis developed despite long term administration of D penicillamine for a duration of 7, 12, and 13 years respectively. At transplantation, ascites was present in all, jaundice and encephalopathy in 2, all 3 patients are alive with normal copper metabolism. In conclusion, indications of liver transplantation in Wilson's disease are fulminant liver failure, primary decompensated liver cirrhosis unresponsive to D Penicillamine, and development of liver failure despite long term administration of D Penicillamine. One year survival was 91.6%, and no case of recurrence have been observed after 1 to 8 yeats.
It Jesic, M. Krstic, D. Tomic, R. Grbic, G. Jankovic, N. Milinic, I. Cabric, V. Bulat and A. Pavlovic Institute for
Digestive Diseases, Belgrade, Yugoslavia Haemang,iomas are the most common of the benign hepatic tumors and liver is also the primary site of this tumor in humans. They are usually small and single but may present as a large hepatic mass- then they are termed as "giant haemangioma". Very rarely thrombocytopenia can develop as a consequence of platelet sequestration in giant cavernous haemangioma. We present the case of 42-year old female who's only symptom was moderate abdominal pain of long duration. US examination of upper abdomen has shown very large, solid hyper-hetero echogenic formation 17xl6cm in size which fulfilled right hepatic lobe completely. Standard seintiscan with Tc99 has shown large filling defect "hole" but blood-pool scintisean with radio-labeled red ceils shown great "hot field" in fight hepatic lobe. On CT scan with contrast, large mass 17xl6xl2em in size is also visualized. On eoeliae trune selective angiography typical lacunas filled with contrast are visualized on early and late arterial phase and persisted for a long time in portal vein phase. Platelet number was decreased-always around 40 000/em 3. All other standard laboratory tests v~re in normal limits. There ~a~re no signs of hemorrhagic syndrome. All hematological causes of thromboeytopenia are excluded by careful testing bone marrow e~tology and biopsy, immunology-ANA,anti DNA, SER, RF, Rose-Wale, C 3, C4~ Lupus anticoagulans, VDRL, HIV 1 and 2; hemostatic examsPV,FrrT,T'l',fibrinoga n. Since all above mentioned ~ r e normal We has concluded that thrombocytopenia in our patient is a result o f disorded distributionplatelet sequestration in giant hemangioma- Kasabaeh-Merrit sy. Her clinical course is unchangeable for 5 years.
T H E EFFECTS OF METHYLPREDNISOLONE T R E A T M E N T ON THE NON-PARENCHYMAL CELL RESPONSE TO DIMETHYLNITROSAMINE-INDUCED LIVER INJURY SJ Johnson, K Toole, R Fraser*, GWT Rogers*, B Dodds**, AD Burr Department of Histopathology, University of Newcastle upon Tyne, England, NE1 4LP; Departments of Pathology* and Surgery**, Christchurch School of Medicine, New Zealand. Aim: To investigate the effects of methylprednisolone therapy on the responses of the non-parenchymal cells to dimethylnitrosamine(DMN)induced experimental acute liver injury. Methods: Rats were treated as follows: controls (no treatment), methylprednisolone alone (20mg/kg i.p.,days 2,4,8), DMN alone (8mg/kg i.g., days 1, 2, 3, 4, 7, 8), and DMN/methylprednisolone. Liver tissue was obtained on day 9. Cells were detected using the following monoclonal antibodies: ED1 (monocytes/macrophages), ED2 (macrophages~ antidesmin and anti-a-smooth muscle actin (cx-SMA) (perisinusoidal ceils
(PSCs)). Results: DMN treatment produced characteristic perivenular hepatoeyte necrosis and parenchymal collapse. Significant increase in the numbers of EDI-, ED2-, desmin- and ct-SMA-positive cells was seen in perivenular zones compared to controls. AIpha-SMA-positive PSC.s always exceeded those showing desmin positivity. Methylprednisolone treatment alone produced no significant change in the numbers of EDI-, desmin- or a SMA-positive cells compared to controls, but there was a significant decrease in ED2-positive cell numbers (99.0/0.635mm: cf. 62.67/0.635mm:; 0.02>p>0.01). No significant changes in the numbers of ED1- and ED2positive monocytes/macrophages or desmin-positive PSCs were seen in DMN/methylprednisolone treated rats compared to DMN treatment alone, although the trend was for an increase in all these cell populations. Methylprednisolone treatment did, however, lead to significantly higher numbers of a-SMA-positive PSCs (508.9/0.635mm: d. 284.1/0.635mm:); (0.05 > p > 0.02). Conclusion: Methylprednisolone therapy has no inhibitory effect on the non-parenchymal cell population dynamics during the response to toxininduced acute liver injury.
Miscellaneous
DETERMINATION OF AMMONIA IN CAPILLARY EAR-LOBE BLOOD IS AN ALTERNATIVE FOR ARTERIAL A/~ONIA DETERMINATION .
it
~
•
tl'
GIANT HAEMANGIOMA OF LIVER WITH THROMBOCYTOPENIA - A case report
ti'tr
J.R. Hmzenga, J.G.J. Vos, C.H. Gips , A. Tanqernan , R.a. Gre. .1~1tt tldrl' ttltt • t • vlnK , E. Kerr , H.O. Conn , P.L.H. Jansen . D1v. of Hepatol~v-Gestroenteroloav and ,it, Pulaonology, Univ. Hospital Groningen; t~A . -" . . *i* . ' GISH, Medlcal Faculty, State Unlv. Gronlngen; Dlv. of Gast rolntestinal and Liver Diseases, Univ. Hospital Ni~aeqen, The Netherlands and ***** Yale University, Hew Haven, USA
Blood auonia is ae0ngst others used in liver disease to diagnose or to exclude hepatic encephalopathy, Arterial blood is superior to peripheral venous blood because of euscle netabolisa. As there may be medical ethical problems to the detendnation of arterial blood, capillary blood a~onia eight be an alternative. The all of this study was to establish the reference values for a,,onia in capillary ear-lobe blood ((3) and to coepare arterial (AB) and capillary anlonia in patients with respiratory diseases (RO), needing arterial
blood gas analysis and with liver diseases (LD). Hethods. ~non~a deterninations were perforned using the Blood ANnonia Checker~ (BAC) II systen, which needs 20 ~i of whole blood. CB and AN sanples were taken fron 43 RD patients without liver disease and 17 LD patients. For reference CB of 57 nornal healthy controls were used. Results. The reference CB range in controls anounted to 8-41, eedian 20 ~mol/l. RD patients had a CB auonia range of 7-41, |edian 18 and a ~ a~onia range of 7-35, median 16 /~101/1. CB references and CB RD patients were equal as were CB RD patie.nts and ~ RD patients (p=0.22 and p=0.71 respectively). In the LD group (3 aeounted to 23-152, median 74 and AN to 25-161, ledian 58 ~oi/I (CB higher,1~0.025). For all 60 patients {RO ÷ LD) the correlation (r) between (3 and AN wan 0.959 (p
LIVER TRANSPLANTATION FOR WILSON'S DISEASE P. lchai. D. Samuel. A. Arab. F. Saliba. H. Bismuth. Hepato-Biliary Surgery and Liver Transplant Research Center, H6pital Paul Brousse, Villejuif, Universit8 Pads Sud, France Wilson's disease is an inhereditaty disorder manifestating by copper overload affecting mainly the liver and the brain. In most cases, long life administration of D-penicillamine can cure the disease; however in selected cases, liver transplantation can be necessary. The aim of this study was to analyse retrospectively the indications and the results of liver transplantation in 12 pts (9 F, 3M) transplanted for Wilson's disease from 1985 to 1992. Caeruleoplasmine level, cupremia, cupruria, Kayser Fleisher ring detection, andliver biopsy were performed before and after transplantation. The indication for liver transplantation was fulminant liver failure in 6 cases, primary decompensated cirrhosis in 3, and decompensated cirrhosis after long term treatment with D penicillamine in 3. Among the 6 patients (6 F, mean age 17 years, range: 11-20) with fulminant liver failure, 4 were in coma, 1 was confused and one had no encephalopathy the mean duration of the disease was 26 days; caeruleoplasmine was low in all, factor V level was < 30% in all, haemolytic anaemia was present in 5. One patient developed transiently after transplantation, neurologic symptoms of Wilson's disease; 6/6 were alive with normal copper metabolism. In 3 patients, (2F, IM, mean age 14 year) Wilson's disease was revealed by primary decompensated cirrhosis, mean duration of the disease was 14 weeks. D penicillamine rescue treatment was unsuccessful in all. Ascites was present in 3, spontaneous bacterial peritonitis in 1, encephalopathy and jaundice in 2 factor V was below 50% in all. 213 pts are alive with a normal copper metabolism. In 3 patients(l M, 2 F; mean age 28 year), cirrhosis developed despite long term administration of D penicillamine for a duration of 7, 12, and 13 years respectively. At transplantation, ascites was present in all, jaundice and encephalopathy in 2, all 3 patients are alive with normal copper metabolism. In conclusion, indications of liver transplantation in Wilson's disease are fulminant liver failure, primary decompensated liver cirrhosis unresponsive to D Penicillamine, and development of liver failure despite long term administration of D Penicillamine. One year survival was 91.6%, and no case of recurrence have been observed after 1 to 8 yeats.
It Jesic, M. Krstic, D. Tomic, R. Grbic, G. Jankovic, N. Milinic, I. Cabric, V. Bulat and A. Pavlovic Institute for
Digestive Diseases, Belgrade, Yugoslavia Haemang,iomas are the most common of the benign hepatic tumors and liver is also the primary site of this tumor in humans. They are usually small and single but may present as a large hepatic mass- then they are termed as "giant haemangioma". Very rarely thrombocytopenia can develop as a consequence of platelet sequestration in giant cavernous haemangioma. We present the case of 42-year old female who's only symptom was moderate abdominal pain of long duration. US examination of upper abdomen has shown very large, solid hyper-hetero echogenic formation 17xl6cm in size which fulfilled right hepatic lobe completely. Standard seintiscan with Tc99 has shown large filling defect "hole" but blood-pool scintisean with radio-labeled red ceils shown great "hot field" in fight hepatic lobe. On CT scan with contrast, large mass 17xl6xl2em in size is also visualized. On eoeliae trune selective angiography typical lacunas filled with contrast are visualized on early and late arterial phase and persisted for a long time in portal vein phase. Platelet number was decreased-always around 40 000/em 3. All other standard laboratory tests v~re in normal limits. There ~a~re no signs of hemorrhagic syndrome. All hematological causes of thromboeytopenia are excluded by careful testing bone marrow e~tology and biopsy, immunology-ANA,anti DNA, SER, RF, Rose-Wale, C 3, C4~ Lupus anticoagulans, VDRL, HIV 1 and 2; hemostatic examsPV,FrrT,T'l',fibrinoga n. Since all above mentioned ~ r e normal We has concluded that thrombocytopenia in our patient is a result o f disorded distributionplatelet sequestration in giant hemangioma- Kasabaeh-Merrit sy. Her clinical course is unchangeable for 5 years.
T H E EFFECTS OF METHYLPREDNISOLONE T R E A T M E N T ON THE NON-PARENCHYMAL CELL RESPONSE TO DIMETHYLNITROSAMINE-INDUCED LIVER INJURY SJ Johnson, K Toole, R Fraser*, GWT Rogers*, B Dodds**, AD Burr Department of Histopathology, University of Newcastle upon Tyne, England, NE1 4LP; Departments of Pathology* and Surgery**, Christchurch School of Medicine, New Zealand. Aim: To investigate the effects of methylprednisolone therapy on the responses of the non-parenchymal cells to dimethylnitrosamine(DMN)induced experimental acute liver injury. Methods: Rats were treated as follows: controls (no treatment), methylprednisolone alone (20mg/kg i.p.,days 2,4,8), DMN alone (8mg/kg i.g., days 1, 2, 3, 4, 7, 8), and DMN/methylprednisolone. Liver tissue was obtained on day 9. Cells were detected using the following monoclonal antibodies: ED1 (monocytes/macrophages), ED2 (macrophages~ antidesmin and anti-a-smooth muscle actin (cx-SMA) (perisinusoidal ceils
(PSCs)). Results: DMN treatment produced characteristic perivenular hepatoeyte necrosis and parenchymal collapse. Significant increase in the numbers of EDI-, ED2-, desmin- and ct-SMA-positive cells was seen in perivenular zones compared to controls. AIpha-SMA-positive PSC.s always exceeded those showing desmin positivity. Methylprednisolone treatment alone produced no significant change in the numbers of EDI-, desmin- or a SMA-positive cells compared to controls, but there was a significant decrease in ED2-positive cell numbers (99.0/0.635mm: cf. 62.67/0.635mm:; 0.02>p>0.01). No significant changes in the numbers of ED1- and ED2positive monocytes/macrophages or desmin-positive PSCs were seen in DMN/methylprednisolone treated rats compared to DMN treatment alone, although the trend was for an increase in all these cell populations. Methylprednisolone treatment did, however, lead to significantly higher numbers of a-SMA-positive PSCs (508.9/0.635mm: d. 284.1/0.635mm:); (0.05 > p > 0.02). Conclusion: Methylprednisolone therapy has no inhibitory effect on the non-parenchymal cell population dynamics during the response to toxininduced acute liver injury.