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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} | | | Name = {{PAGENAME}} | |
| Image = Cirrhosis 030.jpg | | | Image = Cirrhosis 030.jpg | |
| Caption = Gross, natural color of liver and stomach view from external surfaces, micronodular cirrhosis and hemorrhagic gastritis (as the surgeon would see these in natural color). <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> | | | Caption = Gross, natural color of liver and stomach view from external surfaces, micronodular cirrhosis and hemorrhagic gastritis (as the surgeon would see these in natural color). <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> | |
| DiseasesDB = 2729 |
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| ICD10 = {{ICD10|K|70|3|k|70}}, {{ICD10|K|71|7|k|70}}, {{ICD10|K|74||k|70}} |
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| ICD9 = {{ICD9|571}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = |
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| MeshID = D008103 |
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| }} | | }} |
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| {{Cirrhosis}} | | {{Cirrhosis}} |
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| {{SI}}
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{CMG}}; | | {{CMG}}; {{AE}} {{Cherry}} |
| {{AE}} {{CZ}}; {{ADI}} | |
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| ==[[Cirrhosis overview|Overview]]==
| | {{SK}} Cirrhosis of liver; hepatic fibrosis; hepatic sclerosis |
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| ==[[Cirrhosis historical perspective|Historical Perspective]]== | | == [[Cirrhosis overview|Overview]] == |
| ==[[Cirrhosis classification scheme| Classification]]==
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| | == [[Cirrhosis historical perspective|Historical Perspective]] == |
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| ==[[Cirrhosis pathophysiology| Pathophysiology]]== | | == [[Cirrhosis classification|Classification]] == |
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| ==[[Cirrhosis causes| Causes]]== | | == [[Cirrhosis pathophysiology|Pathophysiology]] == |
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| ==[[Cirrhosis differential diagnosis| Differential Diagnosis]]== | | == [[Cirrhosis causes|Causes]] == |
| ==[[Cirrhosis epidemiology and demographics|Epidemiology and Demographics]]==
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| ==[[Cirrhosis risk factors | Risk Factors]]==
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| ==[[Cirrhosis natural history | Natural History, Complications and Prognosis]]== | | == [[Cirrhosis differential diagnosis|Differentiating Cirrhosis from other Diseases]] == |
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| ==Diagnosis== | | == [[Cirrhosis epidemiology and demographics|Epidemiology and Demographics]] == |
| [[Cirrhosis symptoms|Symptoms]] | [[Cirrhosis physical examination|Physical Examination]] |[[Cirrhosislaboratory tests|Laboratory tests]] | [[Cirrhosis electrocardiogram|ECG]] | [[Cirrhosis electroencephalogram| EEG]] | [[Cirrhosis chest x ray| Chest X Ray]] |[[Cirrhosis CT|CT]] | [[Cirrhosis MRI|MRI]] |[[Cirrhosis echocardiography or ultrasound|Echocardiography or Ultrasound]] |[[Cirrhosis other imaging findings|Other imaging studies]] | [[Cirrhosis other diagnostic studies|Alternative diagnostics]] | |
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| ==Treatment== | | == [[Cirrhosis risk factors|Risk Factors]] == |
| [[Cirrhosis medical therapy|Medical therapy]] | [[Cirrhosis surgery|Surgical options]] | [[Cirrhosis prevention|Prevention]] | [[Cirrhosis cost-effectiveness of therapy|Financial costs]]| [[Cirrhosis future or investigational therapies|Future therapies]] | |
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| | == [[Cirrhosis screening|Screening]] == |
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| | == [[Cirrhosis natural history, complications and prognosis|Natural History, Complications and Prognosis]] == |
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| | == Diagnosis == |
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| ==Diagnosis==
| | [[Cirrhosis history and symptoms|History and Symptoms]] | [[Cirrhosis physical examination|Physical Examination]] | [[Cirrhosis laboratory findings|Laboratory Findings]] | [[Cirrhosis electrocardiogram|Electrocardiogram]] | [[Cirrhosis chest x ray|Chest X Ray]] | [[Cirrhosis CT|CT]] | [[Cirrhosis MRI|MRI]] | [[Cirrhosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Cirrhosis other imaging findings|Other Imaging Findings]] | [[Cirrhosis other diagnostic studies|Other Diagnostic Studies]] | [[Cirrhosis Clinical prediction rules|Clinical prediction rules]] |
| The [[Gold standard (test)|gold standard]] for diagnosis of cirrhosis is a ''liver biopsy'', through a [[percutaneous]], transjugular, [[laparoscopic]], or fine-needle approach. Histologically cirrhosis can be classified as micronodular, macronodular, or mixed, but this classification has been abandoned since it is nonspecific to the etiology, it may change as the disease progresses, and serological markers are much more specific. However, a biopsy is not necessary if the clinical, laboratory, and radiologic data suggests cirrhosis. Furthermore, there is a small but significant risk to liver biopsy, and cirrhosis itself predisposes for complications due to liver biopsy.<ref>{{cite journal |last=Grant |first=A |coauthors=Neuberger J |year=1999 | title=Guidelines on the use of liver biopsy in clinical practice |journal=Gut |volume=45 |issue=Suppl 4 |pages=1-11 |id=PMID 10485854 |url=http://gut.bmj.com/cgi/content/full/45/suppl_4/IV1|quote=The main cause of mortality after percutaneous liver biopsy is intraperitoneal haemorrhage as shown in a retrospective Italian study of 68,000 percutaneous liver biopsies in which all six patients who died did so from intraperitoneal haemorrhage. Three of these patients had had a laparotomy, and all had either cirrhosis or malignant disease, both of which are risk factors for bleeding. }}</ref>
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| ===Symptoms===
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| * Lethargy
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| * Weakness
| | == Treatment == |
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| * Weight loss
| | [[Cirrhosis medical therapy|Medical Therapy]] | [[Cirrhosis surgery|Surgery]] | [[Cirrhosis primary prevention|Primary Prevention]] | [[Cirrhosis secondary prevention|Secondary Prevention]] | [[Cirrhosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Cirrhosis future or investigational therapies|Future or Investigational Therapies]] |
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| * Loss of appetite
| | ==Case Studies== |
| ===Physical Exmination=== | |
| The following signs and symptoms may occur in the presence of cirrhosis or as a result of the complications of cirrhosis. Many are nonspecific and may occur in other diseases and do not necessarily point to cirrhosis. Likewise, the absence of any does not rule out the possibility of cirrhosis.
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| ====Skin====
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| * ''[[Spider angioma]]ta'' or ''spider nevi''. Vascular lesions consisting of central arteriole surrounded by many smaller vessels due to an increase in [[estradiol]]. These occur in about 33% of cases.<ref name="pmid10423070">{{cite journal |author=Li CP, Lee FY, Hwang SJ, ''et al'' |title=Spider angiomas in patients with liver cirrhosis: role of alcoholism and impaired liver function |journal=Scand. J. Gastroenterol. |volume=34 |issue=5 |pages=520-3 |year=1999 |pmid=10423070 |doi=}}</ref>
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| * ''[[Palmar erythema]]''. Exaggerations of normal speckled mottling of the palm, due to altered sex hormone metabolism.
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| * ''Nail changes''.
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| ** ''Muehrcke's nails'' - paired horizontal bands separated by normal color due to [[hypoalbuminemia]] (low production of [[human serum albumin|albumin]]).
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| ** ''Terry's nails'' - proximal two thirds of the nail plate appears white with distal one-third red, also due to hypoalbuminemia
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| ** ''[[Clubbing]]'' --- Angle between the nail plate and proximal nail fold > 180 degrees
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| * ''[[Dupuytren's contracture]]''. Thickening and shortening of palmar fascia that leads to flexion deformities of the fingers. Thought to be due to fibroblastic proliferation and disorderly collagen deposition. It is relatively common (33% of patients).
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| ====Eyes====
| | [[Cirrhosis case study one|Case #1]] |
| * ''[[Jaundice]]''. Yellow discoloring of the skin, eye, and mucus membranes due to increased bilirubin (at least 2-3 mg/dL or 30 mmol/L). Urine may also appear dark.
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| ====Abdomen====
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| * ''Liver size''. Can be [[hepatomegaly|enlarged]], normal, or shrunken.
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| * ''[[Splenomegaly]]''. Due to congestion of the red pulp as a result of portal hypertension.
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| * ''[[Ascites]]''. Accumulation of fluid in the peritoneal cavity giving rise to flank dullness (needs about 1500 mL to detect flank dullness).
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| * ''[[Caput medusae|Caput medusa]]''. In portal hypertension, the umbilical vein may open. Blood from the portal venous system may be shunted through the periumbilical veins into the umbilical vein and ultimately to the abdominal wall veins, manifesting as caput medusa.
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| * ''Cruveilhier-Baumgarten murmur''. Venous hum heard in epigastric region due to collateral connections between portal system and the remnant of the umbilical vein in portal hypertension.
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| ====Extremities====
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| * ''[[Hypertrophic osteopathy|Hypertrophic osteoarthropathy]]''. Chronic proliferative periostitis of the long bones that can cause considerable pain.
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| ====Neurologic====
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| * ''[[Asterixis]]''. Bilateral asynchronous flapping of outstretched, dorsiflexed hands seen in patients with hepatic [[encephalopathy]].
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| ====Other====
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| * ''[[Gynecomastia]]''. Benign proliferation of glandular tissue of male breasts presenting with a rubbery or firm mass extending concentrically from the nipples. This is due to increased estradiol and can occur up to 66% of patients.
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| * ''[[Fetor hepaticus]]''. Sweet pungent smell in breath due to increased [[dimethyl sulfide]] due to severe portal-systemic shunting.
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| ===Laboratory Findings===
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| The following findings are typical in cirrhosis:
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| * ''[[Transaminase|Aminotransferase]]s'' - AST and ALT are moderately elevated, with AST > ALT. However, normal aminotransferases do not preclude cirrhosis.
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| * ''[[Alkaline phosphatase]]'' - usually slightly elevated.
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| * ''[[Gamma-glutamyl transpeptidase|GGT]]'' -- correlates with AP levels. Typically much higher in chronic liver disease from alcohol.
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| * ''[[Bilirubin]]'' - may elevate as cirrhosis progresses.
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| * ''[[Albumin]]'' - levels fall as the synthetic function of the liver declines with worsening cirrhosis since albumin is exclusively synthesized in the liver
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| * ''[[Prothrombin time]]'' - increases since the liver synthesizes clotting factors.
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| * ''[[Globulin]]s'' - increased due to shunting of bacterial antigens away from the liver to lymphoid tissue.
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| * ''Serum [[sodium]]'' - [[hyponatremia]] due to inability to excrete free water resulting from high levels of [[vasopressin|ADH]] and [[aldosterone]].
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| * ''[[Thrombocytopenia]]'' - due to both congestive [[splenomegaly]] as well as decreased [[thrombopoietin]] from the [[liver]]. However this rarely results in platelet count < 50,000/mL.
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| * ''[[Leukopenia]] and [[neutropenia]]'' - due to [[splenomegaly]] with splenic margination.
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| * ''[[Coagulation]] defects'' - the [[liver]] produces most of the coagulation factors and thus coagulopathy correlates with worsening liver disease.
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| Other laboratory studies performed in newly diagnosed [[cirrhosis]] may include:
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| * Serology for [[hepatitis]] viruses, [[autoantibody|autoantibodies]] ([[Anti-nuclear antibody|ANA]], anti-smooth muscle, [[Anti-mitochondrial antibody|anti-mitochondria]], anti-LKM)
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| * [[Ferritin]] and [[transferrin saturation]] (markers of iron overload), [[copper]] and [[ceruloplasmin]] (markers of copper overload)
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| * [[Immunoglobulin]] levels (IgG, IgM, IgA) - these are non-specific but may assist in distinguishing various causes
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| * [[Cholesterol]] and [[glucose]]
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| * [[Alpha 1-antitrypsin]]
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| * Prothrombin time, albumin
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| * Platelets
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| * Lytes/Creatinine (Cr)
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| *:* Hyponatremia suggests severe disease
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| ===Imaging===
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| ''[[Medical ultrasonography|Ultrasound]]'' is routinely used in the evaluation of cirrhosis, where it may show a small and nodular liver in advanced cirrhosis along with increased echogenicity with irregular appearing areas. Ultrasound may also screen for hepatocellular carcinoma, portal hypertension and [[Budd-Chiari syndrome]] (by assessing flow in the hepatic vein).
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| A new type of device, the FibroScan (transient elastography), uses elastic waves to determine liver stiffness which theoretically can be converted into a liver score based on the METAVIR scale. The FibroScan produces an ultrasound image of the liver (from 20-80mm) along with a pressure reading (in kPa.) The test is much faster than a biopsy (usually last 2.5-5 minutes) and is completely painless. It shows reasonable corellation with the severity of cirrhosis.<ref>{{cite journal |author=Foucher J, Chanteloup E, Vergniol J, ''et al'' |title=Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study |journal=Gut |volume=55 |issue=3 |pages=403-8 |year=2006 |pmid=16020491 |doi=10.1136/gut.2005.069153}}</ref>
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| Other tests performed in particular circumstances include abdominal [[Computed tomography|CT]] and liver/bile duct [[Magnetic resonance imaging|MRI]] ([[Magnetic resonance cholangiopancreatography|MRCP]]).
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| ===Endoscopy===
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| [[Gastroscopy]] (endoscopic examination of the [[esophagus]], stomach and [[duodenum]]) is performed in patients with established cirrhosis to exclude the possibility of esophageal varices. If these are found, prophylactic local therapy may be applied (sclerotherapy or banding) and [[beta blocker]] treatment may be commenced.
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| ===Computer Tomography===
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| <gallery>
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| Image: CT_abdomen_-_liver_cirrhosis_-_01.jpg|Liver cirrhosis as seen on an axial [[computed tomography|CT]] of the abdomen.
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| </gallery>
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| ===MRI===
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| <gallery>
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| Image:Cirrhosis-001.jpg|T2
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| Image:Cirrhosis-002.jpg|T2
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| </gallery>
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| ===Other Diagnostic Modalities===
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| If biliary pathology ([[primary sclerosing cholangitis]] - [[PSC]]) is suspected, [[Endoscopic retrograde cholangiopancreatography|ERCP]] may be performed.
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| Generally [[Magnetic resonance cholangiopancreatography|MRCP]] (MRI of biliary tract and pancreas) is sufficient for diagnosis, but ERCP allows for particular interventions, such as placement of a biliary [[stent]] or extraction of gallstones.
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| ==Grading==
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| The severity of cirrhosis is commonly classified with the [[Child-Pugh score]]. This score uses [[bilirubin]], [[human serum albumin|albumin]], [[prothrombin time|INR]], presence and severity of [[ascites]] and [[Hepatic encephalopathy|encephalopathy]] to classify patients in class A, B or C; class A has a favourable prognosis, while class C is at high risk of death. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh ''et al''.<ref>Pugh RN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the oesophagus for bleeding oesophageal varices. ''Br J Surg'' 1973;60:646-9. PMID 4541913.</ref>
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| More modern scores, used in the allocation of [[liver transplant]]s but also in other contexts, are the [[Model for End-Stage Liver Disease]] (MELD) score and its pediatric counterpart, the [[Pediatric end-stage liver disease|Pediatric End-Stage Liver Disease]] ([[PELD]]) score.
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| ==Treatment==
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| Traditionally, liver damage from cirrhosis cannot be reversed, but treatment could stop or delay further progression and reduce complications. A healthy diet is encouraged, as cirrhosis may be an energy-consuming process. Close follow-up is often necessary. Antibiotics will be prescribed for infections, and various medications can help with itching. Laxatives, such as [[lactulose]], decrease risk of constipation; their role in preventing encephalopathy is limited.
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| ===Treating underlying causes===
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| Alcoholic cirrhosis caused by alcohol abuse is treated by abstaining from alcohol. Treatment for hepatitis-related cirrhosis involves medications used to treat the different types of hepatitis, such as interferon for viral hepatitis and corticosteroids for autoimmune hepatitis. Cirrhosis caused by [[Wilson's disease]], in which copper builds up in organs, is treated with [[chelation therapy]] (e.g. [[penicillamine]]) to remove the copper.
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| ===Preventing further liver damage===
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| Regardless of underlying cause of cirrhosis, alcohol and [[acetaminophen]], as well as other potentially damaging substances, are discouraged. Vaccination of susceptible patients should be considered for [[Hepatitis A]] and [[Hepatitis B]].
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| === Chronic Pharmacotherapies ===
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| ==== Varices ====
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| * Endoscopic screening in all cirrhotic patients
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| * If varices present--treat with propranolol or nadolol
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| ==== Hepatocellular Cancer ====
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| * Incidence 1-6%/year in HCV-, HBV-, EtOH-related cirrhosis
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| * Screening frequency & benefit controversial
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| * Serum alpha-fetoprotein (AFP) every 6 months (~60% sensitive, ~90% specific)
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| * Ultrasound every 6 months (~75% sensitive, ~90% specific)
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| ===Preventing complications===
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| ====Ascites====
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| {{main|Ascites}}
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| Salt restriction is often necessary, as cirrhosis leads to accumulation of salt (sodium retention). [[Diuretic]]s may be necessary to suppress [[ascites]].
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| ====Esophageal variceal bleeding====
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| {{main|Esophageal varices}}
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| For portal hypertension, [[propranolol]] is a commonly used agent to lower blood pressure over the portal system. In severe complications from portal hypertension, [[transjugular intrahepatic portosystemic shunt]]ing is occasionally indicated to relieve pressure on the portal vein. As this can worsen encephalopathy, it is reserved for those at low risk of encephalopathy, and is generally regarded only as a bridge to liver transplantation or as a palliative measure.
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| ====Hepatic encephalopathy====
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| {{main|Hepatic encephalopathy}}
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| High-protein food increases the [[Blood urea nitrogen|nitrogen balance]], and would theoretically increase [[encephalopathy]]; in the past, this was therefore eliminated as much as possible from the diet. Recent studies show that this assumption was incorrect, and high-protein foods are even ''encouraged'' to maintain adequate nutrition.
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| ====Hepatorenal syndrome====
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| {{main|Hepatorenal syndrome}}
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| The [[hepatorenal syndrome]] is defined as a urine sodium less than 10 mmol/L and a [[serum creatinine]] > 1.5 mg/dl (or 24 hour [[creatinine clearance]] less than 40 ml/min) after a trial of volume expansion without diuretics.<ref name="pmid3297907">{{cite journal |author=Ginés P, Arroyo V, Quintero E, ''et al'' |title=Comparison of paracentesis and diuretics in the treatment of cirrhotics with tense ascites. Results of a randomized study |journal=Gastroenterology |volume=93 |issue=2 |pages=234-41 |year=1987 |pmid=3297907 |doi=}}</ref>
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| ====Spontaneous bacterial peritonitis====
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| {{main|Spontaneous bacterial peritonitis}}
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| Cirrhotic patients with [[ascites]] are at risk of [[spontaneous bacterial peritonitis]].
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| ===Transplantation===
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| {{main|Liver transplantation}}
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| If complications cannot be controlled or when the liver ceases functioning, [[liver transplantation]] is necessary. Survival from liver transplantation has been improving over the 1990s, and the five-year survival rate is now around 80%, depending largely on the severity of disease and other medical problems in the recipient.<ref> [http://www.emedicinehealth.com/liver_transplant/page11_em.htm E-medicine liver transplant outlook and survival rates]</ref> In the United States, the [[Model for End-Stage Liver Disease|MELD score]] ([http://www.unos.org/resources/meldPeldCalculator.asp online calculator])<ref name="pmid2682175">{{cite journal |author=Cosby RL, Yee B, Schrier RW |title=New classification with prognostic value in cirrhotic patients |journal=Mineral and electrolyte metabolism |volume=15 |issue=5 |pages=261-6 |year=1989 |pmid=2682175 |doi=}}</ref> is used to prioritize patients for transplantation. Transplantation necessitates the use of immune suppressants ([[ciclosporin]] or [[tacrolimus]]).
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| ===Decompensated cirrhosis===
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| In patients with previously stable cirrhosis, decompensation may occur due to various causes, such as [[constipation]], [[infection]] (of any source), increased alcohol intake, [[medication]], bleeding from esophageal varices or dehydration. It may take the form of any of the complications of cirrhosis listed above.
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| Patients with decompensated cirrhosis generally require admission to [[hospital]], with close monitoring of the [[fluid balance]], mental status, and emphasis on adequate nutrition and medical treatment - often with [[diuretic]]s, [[antibiotic]]s, [[laxative]]s and/or [[enema]]s, [[thiamine]] and occasionally [[glucocorticoid|steroids]], [[acetylcysteine]] and [[pentoxifylline]]. Administration of [[Saline (medicine)|saline]] is generally avoided as it would add to the already high total body sodium content that typically occurs in cirrhosis.
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| == Primary Prevention ==
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| * Education about hepatotoxins:
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| *:* EtOH
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| *:* Acetaminophen
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| *:* Herbals
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| * Maintenance of adequate caloric intake: 2000-3000 kcal/d
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| * Hepatitis A Virus vaccination, Pneumovax
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| ==Mikroscopic Images==
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| ===Chronic active hepatitis - Cirrhosis===
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| {{#ev:youtube|CzKGvWZrUpU}}
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| ===Micronodular cirrhosis===
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| {{#ev:youtube|CV8OYeIUXko}}
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| ===Primary biliary cirrhosis===
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| {{#ev:youtube|Jj8ozr_IttM}}
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| ==References==
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| {{Reflist|2}}
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| ==External links==
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| *[http://digestive.niddk.nih.gov/ddiseases/pubs/cirrhosis/ Cirrhosis of the Liver] at the National Digestive Diseases Information Clearinghouse (NDDIC). NIH Publication No. 04-1134, December 2003.
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| *[http://www.nlm.nih.gov/medlineplus/cirrhosis.html] at the National Library of Medicine and the National Institutes of Health. Medline Plus: Cirrhosis -- also called: Hepatic fibrosis
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| {{Gastroenterology}} | | {{Gastroenterology}} |
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| [[Category:Hepatology]] | | [[Category:Hepatology]] |
| [[Category:Alcohol abuse]] | | [[Category:Disease]] |
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