DOCSLIB.ORG

Changes in Arterial Oxygenation After Portal Decompression in Budd–Chiari Syndrome Patients with Hepatopulmonary Syndrome

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Changes in Arterial Oxygenation After Portal Decompression in Budd–Chiari Syndrome Patients with Hepatopulmonary Syndrome European Radiology (2019) 29:3273–3280 https://doi.org/10.1007/s00330-018-5840-1 VASCULAR-INTERVENTIONAL Changes in arterial oxygenation after portal decompression in Budd–Chiari syndrome patients with hepatopulmonary syndrome Jiaywei Tsauo1 & He Zhao1 & Xiaowu Zhang1 & Huaiyuan Ma 2 & Mingshan Jiang2 & Ningna Weng2 & Xiao Li1,2 Received: 10 August 2018 /Revised: 18 September 2018 /Accepted: 19 October 2018 /Published online: 30 November 2018 # European Society of Radiology 2018 Abstract Objectives To evaluate the changes in arterial oxygenation after portal decompression in Budd–Chiari syndrome (BCS) patients with hepatopulmonary syndrome (HPS). Methods From June 2014 to June 2015, all patients with BCS who underwent balloon angioplasty or transjugular intrahepatic portosystemic shunt (TIPS) creation at our institution were eligible for inclusion in this study. Arterial blood gas analysis was performed with the patient in an upright position and breathing room air at 2–3daysand1 and 3 months after the procedure. Results Eleven patients with HPS and 14 patients without HPS were included in this study. The procedure was technically successful in 24 patients. One patient with HPS had technically unsuccessful TIPS creation. Reobstruction or TIPS dysfunction was not detected in any patient within 3 months after the procedure. For patients with HPS, the alveolar–arterial oxygen gradient (A-aO2) remained comparable to baseline 2–3 days after the procedure (-3.2 ± 11.9 mmHg; p = .412), significantly improved 1 month after the procedure (-11.7 ± 6.4 mmHg; p < .001), and then returned to baseline 3 months after the procedure (-1.3 ± 12.5 mmHg; p = .757). For patients without HPS, the A-aO2 remained comparable to baseline at all three time points after the procedure (+1.4 ± 8.3 mmHg, +3.5 ± 8.1 mmHg, and +1.3 ± 8.2 mmHg; p =.543,p = .137, and p =.565). Conclusions Arterial oxygenation transiently improves after portal decompression in BCS patients with HPS. Key Points • Intrapulmonary vascular dilation and hepatopulmonary syndrome are common in patients with Budd–Chiari syndrome. • Arterial oxygenation transiently improves after portal decompression in Budd–Chiari syndrome patients with hepatopulmonary syndrome. Keywords Hypertension, portal . Liver cirrhosis . Angioplasty, balloon . Portosystemic shunt, transjugular intrahepatic Abbreviations FVC Forced vital capacity A-aO2 Alveolar–arterial oxygen gradient HPS Hepatopulmonary syndrome ABG Arterial blood gas HV Hepatic veins BCS Budd–Chiari syndrome IPVD Intrapulmonary vascular dilation CEE Contrast-enhanced echocardiography IVC Inferior vena cava FEV1 Forced expiratory volume in 1 s LT Liver transplantation PaCO2 Arterial partial pressure of carbon dioxide PaO2 Arterial partial pressure of oxygen * Xiao Li TIPS Transjugular intrahepatic portosystemic shunt [email protected] 1 Department of Interventional Therapy, National Cancer Center/ National Clinical Research Center for Cancer/Cancer Hospital, Introduction Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nanli, Chaoyang District, Beijing 100021, Budd–Chiari syndrome (BCS) is a rare condition character- China 2 ized by a blocked hepatic venous outflow tract at various Institute of Interventional Radiology, West China Hospital, Sichuan levels from small hepatic veins (HV) to the inferior vena cava University, Chengdu 610041, Sichuan, China 3274 Eur Radiol (2019) 29:3273–3280 (IVC) [1]. Clinical manifestations range widely from asymp- documented malignancy, pregnancy, or previous shunt thera- tomatic to fulminant liver failure [2]. Management follows a py. Before the procedure, all patients underwent ABG analysis stepwise approach, with medical therapy as the first-line treat- while in an upright position and breathing room air, CEE, ment, balloon angioplasty of and/or stent placement in the pulmonary function tests, and plain chest radiographs. The obstructed HV and/or IVC as the second-line treatment, diagnosis of HPS was established by an elevated A-aO2 (i.e., transjugular intrahepatic portosystemic shunt (TIPS) creation ≥ 15 mmHg (≥ 20 mmHg if age ≥ 65 years)) and a positive as the third-line treatment, and liver transplantation (LT) as the CEE result [4, 13, 14]. The severity of HPS was classified as last-line treatment [1, 3]. very severe (PaO2 <50mmHg),severe(PaO2 50–59 mmHg), Hepatopulmonary syndrome (HPS) is a pulmonary com- moderate (PaO2 60–79 mmHg), or mild (PaO2 ≥ 80 mmHg) plication of liver disease and/or portal hypertension [4]. It is [4, 13, 14]. This prospective study was approved by our insti- characterized by a defect in arterial oxygenation caused by tutional review board. All patients provided written informed ventilation–perfusion mismatch resulting from increased cap- consent for study participation. illary flow at the level of the alveoli due to intrapulmonary vascular dilation (IPVD), which includes diffuse or localized dilation of capillaries and, less commonly, arteriovenous com- CEE procedure munications [4]. Therefore, the diagnosis of HPS requires, in the setting of liver disease and/or portal hypertension, the The technique used to perform CEE has been described in demonstration of IPVD (i.e., right to left shunting) and arterial detail elsewhere [15]. Briefly, 10 mL of agitated saline was oxygenation defects (i.e., increased alveolar–arterial oxygen injected through an intravenous line placed in the right upper gradient (A-aO2) or decreased arterial partial pressure of ox- extremity, and transthoracic two-dimensional echocardiogra- ygen (PaO2)), preferably by contrast-enhanced echocardiog- phy in the parasternal four-chamber view was used to detect raphy (CEE) and arterial blood gas (ABG) analysis, respec- the appearance of microbubbles. The result was regarded as tively [4]. HPS occurs mostly in cirrhotic patients (12–32%) positive if any microbubbles appeared in the left heart cham- but can also occur in patients with pre- and post-hepatic portal bers 3–6 heartbeats after they initially appeared in the right hypertension (2–28%) [5–7]. It is associated with detrimental heart chambers after three injections. effects on the patient’s functional status, quality of life, and survival [8]. However, the treatment options for HPS are ex- tremely limited, as only LT has been established to be effec- Balloon angioplasty and TIPS creation procedure tive [9, 10]. HPS occurs in a substantial portion (28%) of patients with For patients with short-segment or membranous obstruction of BCS [11]. Balloon angioplasty has been shown to be able to the HV,IVC, or both, balloon angioplasty was performed. The reverse HPS in patients with BCS [11]. The mechanism is occluded segment was traversed with a hydrophilic guidewire unknown but may be related to portal decompression. This (Terumo) and a multipurpose catheter (Cordis) via may also explain the favorable outcomes of TIPS creation transjugular access. If the occluded segment could not be tra- for HPS in patients with cirrhosis and idiopathic portal hyper- versed, a Rösch-Uchida liver access set (Cook) was intro- tension [12]. The purpose of this study was to evaluate chang- duced, and the HV or IVC distal to the occluded segment es in arterial oxygenation after portal decompression in BCS was punctured. When necessary, a pigtail catheter (Cordis) patients with HPS. was placed in the IVC distal to the occluded segment via femoral access to serve as a target for puncture. After the occluded segment was traversed, a 10–20-mm-diameter bal- Materials and methods loon catheter (Cordis) was introduced, and the balloon was inflated until the circumferential indentation caused by the Study design obstruction disappeared from the balloon contour for 1– 2 min. For patients with a long-segment obstruction of the From June 2014 to June 2015, all patients with BCS under- HV with or without IVC obstruction, a TIPS was created. A going balloon angioplasty and TIPS creation at our institution Rösch-Uchida liver access set (Cook) was introduced into the were eligible for inclusion in this study. The exclusion criteria stump of the HV or the IVC via transjugular access, and the were age < 18 years, intrinsic cardiopulmonary disease (i.e., portal vein was punctured. The parenchymal tract was dilated intracardiac shunting, QT interval > 50% of the RR interval, with an 8-mm-diameter balloon catheter (Cordis), and a 10- forced expiratory volume in 1 s (FEV1) < 70% of the predicted mm-diameter Fluency Plus stent graft (Bard) was placed to value, forced vital capacity (FVC) < 70% of the predicted cover the tract. If the portosystemic pressure gradient (PPG) value, FEV1/FVC < 70% of the predicted value, and pulmo- remained > 13 mmHg after TIPS creation, the shunt tract was nary artery systolic pressure > 35 mmHg), active infection, dilated with a 10-mm-diameter balloon catheter (Cordis). Eur Radiol (2019) 29:3273–3280 3275 Follow-up (56.0%), and 11 (78.6%) of these patients had an elevated A- aO2. These 11 (44.0%) patients were considered to have HPS, After the procedure, all patients received long-term warfarin including five (45.5%) with moderate HPS and six (54.5%) therapy with an initial dose of 1.25 mg that was adjusted in a with mild HPS. The baseline characteristics of the patients are stepwise manner to achieve the target international normalized summarized in Table 1. ratio of 2–3. ABG analysis was performed with the patient in Of the 11 patients with HPS, four had short-segment or an upright position and breathing room air at 2–3 days and
Load more

Recommended publications
  • A Hepatic Outflow Obstruction (Budd-Chiari Syndrome) Case Due
    OLGU SUNUMU / CASE REPORT Gülhane Tıp Derg 2014;56: 110-113 © Gülhane Askeri Tıp Akademesi 2014 doi: 10.5455/gulhane.13835 A Hepatic Outflow Obstruction (Budd-Chiari Syndrome) Case Due to Multiple Hypercoagulable Status Yusuf Yazgan (*), Kemal Oncu (*), Mustafa Kaplan (*), Alpaslan Tanoglu (*), İrfan Küçük (*), Halil Onur Ozari (*), Levent Demirturk (*), Murat Velioglu (**) Introduction: SUMMARY Primary Budd-Chiari syndrome (BCS) is a rare disorder We present here a case of a 22-year-old male patient with Budd- caused by thrombosis of the hepatic veins or the terminal Chiari syndrome owing to alliance of multiple hypercoagulable portion of the inferior vena cava. Its estimated incidence ranges conditions. The patient was admitted to our hospital for from 0.2 to 0.8 per million per year (1). In developed countries, assesment of hepatosplenomegaly and ascites. By doppler the hepatic vein thrombosis is the most frequent presentation, ultrasonography, computed tomography and vena cavagraphy, however in developing countries membranous blockade is the Budd-Chiari syndrome was diagnosed. Results of diagnostic tests most common cause of the BCS. BCS is closely associated exhibited decreased activity, decreased antigenic concentration of Antithrombin, low protein C activity, heterozygote Factor with prothrombotic conditions especially with hematologic V Leiden mutation. In clinical progress, acute severe hepatic disorders. For example primary myeloproliferative diseases failure with encephalopathy occured and the patient was (i.e. polycythemia vera) may account for nearly half of the transferred to an another medical center for liver transplantation. all cases (2). Tumors, pregnancy, infections are also other common reasons. Oral contraceptives raise the risk of BCS Key words: Budd-Chiari Syndrome, Antithrombin deficiency, low by nearly two-fold (3).
    [Show full text]
  • 1-Dr Zamirian
    IJMS Vol 32, No 1, March 2007 Original Article Contrast Enhanced Echocardiography for Detection of Intrapulmonary Shunts in Liver Transplant Candidates M. Zamirian, A. Aslani Abstract Background: Intrapulmonary vascular abnormalities associated with liver cirrhosis may result in intrapulmonary right-to-left shunt and hypoxemia. The aim of this study was to use con- trast enhanced echocardiography to detect intrapulmonary vascular abnormalities in patients with liver cirrhosis candi- dates for liver transplantation. Methods: One hundred and two adult patients underwent contrast enhanced echocardiography to determine the prevalence of in- trapulmonary right-to-left shunt and its relationship to the severity of hepatic disease, arterial oxygenation, and spider angioma. Results: The rate of patients with positive and negative con- trast enhanced echocardiography was 44% and 56%, respec- tively. There was no significant difference in age, sex, or eti- ology of liver cirrhosis in patients with and without intrapul- monary shunt. Patients with intrapulmonary right-to-left shunt had more severe hepatic disease compared with those without shunt (Child-Pugh score 12±2 vs 8±2). There was significant difference in the partial arterial oxygen pressure (PaO2) values + + in patients with grade 3 to 4 left ventricular opacification by microbubbles compared with those without evidence of in- trapulmonary right-to-left shunt (64±6 vs 82±10 mmHg). Twenty eight of the patients with intrapulmonary right-to-left shunt had cutaneous spider angioma. Conclusion: The findings suggest that there was a significant relation between severity of liver cirrhosis and presence of intrapulmonary right-to-left shunt or severity of hypoxemia. The data also indicate that cirrhotic patients with cutaneous spider angioma most likely have the shunt.
    [Show full text]
  • M a C S 9 9 9 9 9 4.A NO YES Did Participant Refrain from Caffeine 4.B 1
    VISIT CLINICIAN NUMBER NUMBER FOLLOW–UP VISIT 4 5 0 PHYSICAL EXAM 0 0 0 0 0 1 1 1 1 1 MARKING INSTRUCTIONS 2 2 2 2 2 • Make dark marks that fill 3 3 3 3 3 the circle completely. 4 4 4 4 4 4 • Make clean erasures. Correct Mark: 5 5 5 5 5 • Make NO stray marks. Incorrect Marks: ✗✓ 6 6 6 6 6 • Do NOT fold this form. 7 7 7 7 7 8 8 8 8 8 M A C S 9 9 9 9 9 4.a NO YES Did participant refrain from caffeine 4.b 1. 2. 3. and nicotine for at least 30 minutes prior to first BP reading? BLOOD PRESSURE ARM ID NUMBER DATE WEIGHT Did participant sit quietly for about 5 minutes prior to first BP reading? JAN DAY YR KILOGRAMS Right PERF FEB Did participant sit quietly for about Left • 5 minutes prior to second BP reading? 0 0 0 0 MAR 0 0 00 0 0 0 0 1 1 1 1 1 APR 10 1 01 1 1 1 1 FIRST READING SECOND READING 5. 2 2 2 2 2 MAY 20 2 02 2 2 2 2 BLOOD PRESSURE BLOOD PRESSURE ORAL TEMPERATURE At least 30 minutes after 3 3 3 3 3 JUNE 30 3 03 3 3 3 3 Sitting, Right Arm Sitting, Right Arm smoking, eating, or drinking 4 4 4 4 4 JULY 4 04 4 4 4 4 SYSTOLIC DIASTOLIC SYSTOLIC DIASTOLIC °F 5 5 5 5 AUG 5 05 5 5 5 5 • 6 6 6 6 SEPT 6 06 6 6 6 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 7 7 7 7 OCT 7 07 7 7 7 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 8 8 8 8 NOV 8 08 8 8 8 2 2 2 2 2 2 2 2 2 2 2 2 2 9 9 9 9 DEC 9 09 9 9 9 3 3 3 3 3 3 3 3 3 3 3 4 4 4 4 4 4 4 4 4 4 4 5 5 5 5 5 5 5 5 5 5 5 6 6 6 6 6 6 6 6 6 6 6 7 7 7 7 7 7 7 7 7 7 7 5/8" SLIT Glue 8 8 8 8 8 8 8 8 8 8 8 9 9 9 9 9 9 9 9 9 9 9 6.
    [Show full text]
  • Multiple Arteriovenous Hemangiomas in a Patient with Chronic Liver Disease
    Brief Report https://doi.org/10.5021/ad.2016.28.6.798 Multiple Arteriovenous Hemangiomas in a Patient with Chronic Liver Disease Dae Hong Kim, Sang Hyun Cho, Jeong Deuk Lee, Hei Sung Kim Department of Dermatology, Incheon St. Mary’s Hospital, The Catholic University of Korea, Incheon, Korea Dear Editor: digital exploration. The patient was an alcoholic and had A 56-year-old Korean male presented with a 5-month his- suffered from liver cirrhosis for 10 years. tory of multiple lesions on the face, trunk, and upper Blood tests showed an abnormal liver function which are extremities. Physical examination revealed multiple asymp- as follows (normal range in parentheses): total bilirubin tomatic, variable-sized red non-blanchable papules and 9.2 mg/dl (0.2∼1.4 mg/dl), aspartate aminotransferase 54 nodules with peripheral erythema and telangiectasia (Fig. U/dl (9∼40 U/dl), alanine aminotransferase 34 mg/dl (0∼ 1). The central component showed intense pulsation under 40 U/dl), lactate dehydrogenase 583 IU/L (208∼405 Fig. 1. (A∼C) Asymptomatic, mul- tiple, variable-sized red papules and nodules with peripheral erythema and telangiectasiaon the face, trunk, and upper extremity. Received May 28, 2015, Revised December 1, 2015, Accepted for publication December 2, 2015 Corresponding author: Hei Sung Kim, Department of Dermatology, Incheon St. Mary’s Hospital, The Catholic University of Korea, 56 Dongsu-ro, Bupyeong-gu, Incheon 21431, Korea. Tel: 82-32-280-5100, Fax: 82-32-506-9514, E-mail: [email protected] This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
    [Show full text]
  • Management of Chronic Liver Failure/Cirrhosis Complications in Hospitals
    Management of Chronic Liver Failure/Cirrhosis Complications in Hospitals By: Dr. Kevin Dolehide Overview DX Cirrhosis and Prognosis Compensated Decompensated Complications Of Cirrhosis Management Of Complications 1. Ascities/ Peripheral edema 2. Variceal Bleeding 3. Spontaneous Bacterial Infection 4. Hepatic encephalopathy 5. Hepatocellular carcinoma 6. Hepato renal syndrome Cirrhosis ★ Late Stage of hepatic fibrosis distortion of hepatic architecture and formation of regeneration nodules. ★ Considered an irreversible in advanced stage Histology of the Liver Cirrhosis ● 25,000 deaths and 370,000 hospital admissions ● 9th leading cause of death ● Common final pathway disease progression ○ Chronic Hep C 28% ○ alcohol 20% ○ alcohol + Chronic Hep C 15% ○ crypotogenic (NASH) 18% ○ Hep B 15% ○ Other 5% ● morbidity mortality associated with complication of decompensated disease Natural Hx of Chronic Liver Disease CLD Compensated Decompensated Cirrhosis Death ● Decompensated Cirrhosis Portal HTN ● Jaundice Ascities ● Hepatic encephalopathy ● GI Bleeding Survivor of Cirrhosis ● Compensated- mean 10 years ● Decompensated- mean 1.5 years (worse than metastatic colon cancer) Portal HTN ● Healthy liver can accomodate changes portal blood flow ● Occurs combination increased portal venous flow and resistance to portal flow Portal HTN 1. Nitric oxide low PV Resistance 2. Cardiac output mesenteric circulation 3. Blood pooling occurs portal circulation collagen deposition. Sinudoidal increased pressure residence DIAGNOSIS DIAGNOSIS OF CIRRHOSIS PHYSICAL
    [Show full text]
  • Pathophysiology of Ascites
    Pathophysiology of Ascites Red = must know ​ ​ Grey= extra information Mind Map Introduction: The peritoneal cavity: ➔ Derived from the coelomic cavity of the embryo ➔ Normally the peritoneal cavity contains about 50 ml and may rise to 2000 ml in severe ascites ​ ​ ​ ​ ➔ The peritoneal cavity functions in facilitating bowel movements and it is normally sterile (no bacteria) ​ ​ ​ ​ The peritoneal fluid: ➔ It is a normal, lubricating fluid found in the peritoneal cavity. ➔ The fluid is mostly water with electrolytes, antibodies, white blood cells, albumin, glucose and other ​ ​ biochemicals. (one of the life threatening complication of ascites is infection. how do we diagnose it? we ​ aspirate and look at the WBCs.) Ascites: the accumulation of fluid in the peritoneal cavity, causing abdominal swelling. ​ (Most patients (85%​) with ascites have cirrhosis) ​ ❏Causes of ascites: Cirrhosis, Infection (TB), Malignancy, CHF, Nephrotic syndrome, Pancreatic or biliary ascites. ​ ➔ The most common causes of cirrhosis are chronic alcoholic liver disease & viral ​ hepatitis.(fatty liver disease will become the #1 cause of liver cirrhosis) ​ ❏Pathogenesis of ascites: Ascites can be caused by: Increased hydrostatic pressure, Decreased colloid osmotic pressure, ​ ​ ​ ​ ​ ​ Increase in the permeability of peritoneal capillaries, Leakage of fluid into the peritoneal cavity, Miscellaneous. ❖ There are two forces that dictate the movement of solutes across the capillary endothelium: 1)Increased Hydrostatic Pressure: increased in cases of congestive heart failure. ​ ​ 2)Decreased colloid osmotic pressure: mainly controlled by albumin. ​ - Decreased albumin decreases colloid osmotic pressure and leads to edema. ​ - Increased albumin excretion in nephrotic syndrome. ​ Cirrhotic Ascites (Pathophysiology and Causes): ​ ​ ​ ​ The most recent theory of ascites formation, the "peripheral arterial vasodilation hypothesis,".
    [Show full text]
  • Spider Angioma
    In partnership with Primary Children’s Hospital Skin disorder: Spider angioma A spider angioma (anj-ee-OH-muh) is a cluster of tiny How are spider angiomas treated? red arteries (blood vessels) visible on the skin. It gets Most children’s spider angiomas go away on their its name from the red bump in the center and the own after a while. They rarely bleed and are harmless. surrounding arteries that look like spider legs. Spider If your child’s angioma does not go away, treatments angiomas often appear on the face, arms, hands, may include: fingers, and upper torso. • Electrocoagulation: Removing dilated blood vessels What causes spider angiomas? using a mild electric current No one knows what causes spider angiomas, but your • Pulsed-dye laser therapy: Using a laser with dye to child may be more likely to get a spider angioma if remove angiomas they have: • Liver disease Your child’s doctor will discuss these treatments with you and help you decide what’s best for your child. • Thyroid disease • Hormone changes Notes Intermountain Healthcare complies with applicable federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex. Se proveen servicios de interpretación gratis. Hable con un empleado para solicitarlo. 我們將根據您的需求提供免費的口譯服務。請找尋工作人員協助 © 2018 Intermountain Healthcare, Primary Children’s Hospital. All rights reserved. The content presented here is for your information only. It is not a substitute for professional medical advice, and it should not be used to diagnose or treat a health problem or disease. Please consult your healthcare provider if you have any questions or concerns.
    [Show full text]
  • Evaluation of Rectal Varices by Endoscopic Ultrasonography in Patients with Portal Hypertension
    Diagnostic and Therapeutic Endoscopy, Vol. 7, pp. 165-174 (C) 2001 OPA (Overseas Publishers Association) N.V. Reprints available directly from the publisher Published by license under Photocopying permitted by license only the Harwood Academic Publishers imprint, part of Gordon and Breach Publishing member of the Taylor & Francis Group. All rights reserved. Evaluation of Rectal Varices by Endoscopic Ultrasonography in Patients with Portal Hypertension HIROYUKI KOBAYASHI* The 3rd Department of Internal Medicine, Toho University School of Medicine, Ohashi Hospital, 2-17-6, Ohashi, Meguro-Ku, Tokyo 153- 8515, Japan (Received 12 November 2001; In final form 22 March 2002) The usefulness of endoscopic ultrasonography (EUS) in the evaluation of rectal varices (RV) was determined in 50 patients with portal hypertension (PH) and 25 PH-free controls. F1 and F2 varices and angiectasia were specific for the PH group as evaluated by endoscopy, but there was no difference between the PH and the control groups with respect to the frequency of blue vein. The detection rate of submucosal veins (SMV) with EUS was 88% for the PH group and 68% for the control group. The mean SMV diameter was significantly greater for the PH group than for the control group, and no 2-mm or larger SMV was detected in the control group. Serum albumin and cholinesterase levels were significantly higher for the RV(+) patients with SMV 2 mm or more in diameter in the PH group than for the RV(- patients. The spleen index was also significantly higher for the former group. The frequency of RV was significantly higher for advanced PH than for mild PH.
    [Show full text]
  • Clinical Manifestations of Hereditary Hemorrhagic Telangiectasia
    0002-9270/84/7905-0363 rHE AMERICAN JOURNAL OF GASTROENTEROLOGY Vol, 79, No, 5. 1984 Copyright© 1984 by Am, Coll, of Gastroenterology Printed in U,S,A, Clinical Manifestations of Hereditary Hemorrhagic Telangiectasia Peter J. Reilly, M.D. and Timothy T. Nostrant, M.D. Department of Internal Medicine. Division of Gastroenterology. The University of Michigan. Ann Arbor. Michigan Sixty-four patients with symptomatie hereditary lated disease at the time of presentation, and 3) a family hemorrhagie telangieetasia were retrospectively studied history and/or personal history of episodic undiagnosed in order to determine the true incidence of clinical nasal or gastrointestinal hemorrhage. manifestations in this disease. This select group had a significantly higher incidence of gastrointestinal hem- RESULTS orrhage and pulmonary arterovenous fistula formation Fifty-six percent of the patients were male and 44% than has been previously reported. Data are presented were female. There was no relationship of sex to severity regarding the course and severity of nasal and gastroin- of illness or number of organ systems involved. The age testinal hemorrhage, the use of endoscopy for diagnosis, at diagnosis varied from 2 days to 85 years with a the incidence of associated neurological, cardiac, and median age of 44 years. Symptoms began at a median hepatic disease, and mortality. of 20 years before diagnosis (range 0-78 years). A family history compatible with the diagnosis of HHT was INTRODUCTION recorded in 74%. Hereditary hemorrhagic telangiectasia (HHT) is an Clinical manifestations (incidence) autosomal dominant inherited disease (1) characterized by telangiectasias ofthe skin, mucous membranes, and Table 1 lists the various clinical manifestations of various organ systems.
    [Show full text]
  • Cirrhosis & Portal Hypertension
    Cirrhosis & Portal Hypertension CIRRHOSIS • Term was 1st coined by Laennec in 1826 • Many definitions but common theme is injury, repair, regeneration and scarring • NOT a localized process; involves entire liver • Primary histologic features: 1. Marked fibrosis 2. Destruction of vascular & biliary elements 3. Regeneration 4. Nodule formation Cirrhosis: Pathophysiology • Primary event is injury to hepatocellular elements • Initiates inflammatory response with cytokine release‐>toxic substances • Destruction of hepatocytes, bile duct cells, vascular endothelial cells • Repair thru cellular proliferation and regeneration • Formation of fibrous scar Cirrhosis: Pathophysiology • Primary cell responsible for fibrosis is stellate cell • Become activated in response to injury and lead to ↑ed expression of fibril‐forming collagen • Above process is influenced by Kupffer cells which activate stellate cells by eliciting production of cytokines • Sinusoidal fenestrations are obliterated because of ↑ed collagen and EC matrix synthesis Cirrhosis: Pathophysiology • Prevents normal flow of nutrients to hepatocytes and increases vascular resistance • Initially, fibrosis may be reversible if inciting events are removed • With sustained injury, process of fibrosis becomes irreversible and leads to cirrhosis Causes of Cirrhosis • Alcohol • Viral hepatitis • Biliary obstruction • Veno‐occlusive disease • Hemochromatosis • Wilson’s disease • Autommune • Drugs and toxins • Metabolic diseases • Idiopathic Classification of Cirrhosis • WHO divided cirrhosis into
    [Show full text]
  • Dermoscopic Features of Spider Angioma in a Healthy Child
    Our Dermatology Online Letter to the Editor DDermoscopicermoscopic ffeatureseatures ooff sspiderpider aangiomangioma iinn a hhealthyealthy cchildhild Anissa Zaouak, Leila Bouhajja, Mariem Jrad, Amel Jebali, Houda Hammami, Samy Fenniche Dermatology Department, Habib Thameur Hospital, Tunis, Tunisia Corresponding author: Dr. Anissa Zaouak, E-mail: [email protected] Sir, We report a 7-year-old boy presented to our department for a newly appearing lesion of the cheek. He had no past medical history and there was not a history of trauma preceding the onset of the cutaneous lesion. Dermatologic examination revealed an erythematous small lesion with small vessels radiating from the center to the periphery located on the left cheek (Fig. 1). Dermoscopy revealed a vascular pattern with small telangiectasia and a stellate network (Fig. 2). The telangiectasias disappeared when pressure was applied with the dermoscope’s glass (Fig. 3). The diagnosis of spider angioma was retained. An Figure 1: Small spider angioma on the left cheek in a child. abdominal examination didn’t reveal hepatomegaly or splenomegaly. Laboratory tests excluded the diagnosis of hepatitis, hepatic deficiency or cirrhosis. The patient was scheduled for a treatment with pulsed dye laser for his small vascular lesion. Spider angiomas are lesions that appear as red spots, shiny, with numerous microvascular radiations which pale when pressure on a central spot is temporarily applied [1-2]. This condition was frequently associated with hepatic abnormalities [3]. Spider angiomas also called nevus araneus are lesions that appear as bright red small shiny lesions with numerous microvascular radiations which pale when pressure on a central spot is temporarily applied [1-3].
    [Show full text]
  • Photo Diagnosis
    Photo Diagnosis Illustrated quizzes on problems seen in everyday practice CASE 1: LEILANI ’S LESION Leilani, 22, presents with a three-year history of a telangiectatic lesion on her nose. She has a similar lesion on her cheek. She takes OC pills and multivitamins. Questions 1. What is the diagnosis? 2. Which individuals are most commonly affected by this condition? 3. How would you manage this lesion? Answers 1. Spider angioma. This is a benign acquired vascular lesion. 2. Though there is no known reason, young children and pregnant women are those most commonly affected. n © tio 3. This letsion could be removediby luaser h tr d, igor electrosurgery, puriesly for ncolosmaetic yr l D dow p purposecs. ia can se o er sers al u C m d u rson m rise r pe o Purotvihdeod by: Dr. Byenfjaomin Barankin C . A cop or ited gle e ohib sin al e pr int a S d us d pr or rise an t f utho view o Una lay, N disp Share your photos and diagnoses with us! Do you have a photo diagnosis? Send us your photo and a brief text explaining the presentation of the illness, your diagnosis and treatment and receive $25 per item if it is published. The Canadian Journal of Diagnosis 955, boul. St. Jean, Suite 306 Pointe-Claire, Quebec H9R 5K3 E-mail: [email protected] Fax: (888) 695-8554 The Canadian Journal of Diagnosis / November 2007 55 Photo Diagnosis CASE 2: BERTHA ’S BLUE TOES Bertha, 74, receives hemodialysis treatment. The introduction of anticoagulation She presents complaining about pains in her therapy likely caused the atherosclerotic feet.
    [Show full text]