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A case is illustrated that involved the deep conjunctival tissues anxiety symptoms shortness of breath cheap 5 mg emsam with mastercard, without appreciable extension into the orbit. In such instances, it may be possible to entirely remove the tumor within its pseudocapsule. It is still not known whether such patients should be treated with additional irradiation and/or chemotherapy, but in most cases such supplemental treatment has been recommended because of the malignant nature of the tumor. The referring diagnosis was possible choroidal melanoma with extraocular extension. The small notch in the capsule represents the site where some tissue was removed postoperatively for possible electron microscopy. Advanced orbital rhabdomyosarcoma with metastasis to preauricular lymph nodes in a child from South Africa. The tumor can recur after surgical excision and can invade the sinuses and cranial cavity. It has been described in the orbit after enucleation and irradiation for retinoblastoma (5). Diagnostic Approaches Orbital rhabdoid tumor has no specific radiographic features. It is initially a circumscribed neoplasm, but it can become rapidly infiltrative and invade bone (6). Pathology Histopathology reveals a poorly differentiated tumor that superficially resembles rhabdomyosarcoma. It is composed of pleomorphic epithelial cells with prominent nucleoli and many filamentous cytoplasmic inclusions. Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Chapter 31 Orbital Myogenic Tumors 611 Orbital Malignant Rhabdoid Tumor In young children, malignant rhabdoid tumor can be highly aggressive with recurrence and extension into the central nervous system. Axial computed tomography showing ovoid mass in muscle cone extending to the orbital apex. Piecemeal excisional biopsy revealed features of rhabdoid tumor and the child was treated with chemotherapy (vincristine and actinomycin D) and radiotherapy (5,000 cGy). About 8 months later the child presented with recurrent proptosis and conjunctival chemosis. Axial magnetic resonance imaging in T1-weighted image, showing massive orbital recurrence.
Aristolochia reticulata (Aristolochia). Emsam.
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Multiple calcifications are revealed within the walls of lateral ventricles and in brain parenchyma on X-ray craniograms anxiety symptoms scale emsam 5 mg on line. Infrequently (in about 15% of tuberous sclerosis cases) subependymal giant-cell astrocytomas are found within interventricular foramina. Its diagnostic criteria are its permanent signs, such as retinal angioma; cerebellar hemangioblastoma; spinal hemangioblastoma; and inconstant signs such as kidney carcinoma; pheochromocytoma; liver angioma; and kidney, liver and pancreatic cysts. Clinical features depend on the extent of cerebellar involvement, spinal cord and retina. Diagnostic criteria are a large single or multiple small and moderate pigment nevi on skin, brain melanoma and/or meningeal melanosis. The disorder manifests by multiple congenital pigment nevi on the skin (more frequently on the posterior sides of trunk and extremities, brown or black in colour), which are often confluent into large fields, without signs of malignancy, and by intracranial brain and meningeal melanomas. Histology reveals accumulation of melanocytes on the ventral surface of the brainstem, in nuclei and cerebellar white matter, in cervical and lumbar vertebral regions, in the anterior portions of temporal lobes and in the amygdale, thalami and basal portions of dura and pia mater. The clinical picture is characterised by cerebral involvement and manifests as headaches, vomiting, dizziness and so forth. A large lesion in the left frontal region of cortical and subcortical location has a heterogeneous signal on both sequences Congenital Malformations of the Brain and Skull 67. Various sizes of calcifications subependymally and in the white matter and hydrocephalus are seen. The right occipital region melanoma on the 1-weighted image (without contrast enhancement) is isointense; in the central part of the T2-weighted image. A small hyperintense lesion is located in the central part of the T2-weighted image, suggestive of melanin. On the 2-weighted image (d) in the sagittal plane, there is a hyperintense lesion (melanin deposits with hamartoma-like changes of brain stem tissue); after contrast enhancement, contrast medium accumulation is seen in pathological lesions and meninges Congenital Malformations of the Brain and Skull 69 ture is hyperintensity of the amygdale and other affected regions of brain and spinal cord and their meninges. Internal and external walls of the cyst consist of thin layers of arachnoid cells and join unchanged arachnoid membrane at the margins. True (congenital) arachnoid cysts contain specific membranes and enzymes possessing secretory activity (Barkovich 2000; Galassi et al. According to the data of the Burdenko Institute of Neurosurgery, arachnoid cysts make up about 10% all masses of brain in children, 7. As a result, the asymptomatic period becomes shorter, and neurological signs are more severe due to proximity of arachnoid cysts to brainstem, diencephalic structures, Sylvian aqueduct and the third and the fourth ventricles. The common feature is a predominance of progressive hydrocephalus signs that mask focal neurological signs-due to the significant ability of children to compensate for the neurological signs. Only in the stage of decompensation do signs of increased exposure of adjoining structures to mass of arachnoid cysts appear: In retrocerebellar cysts, there are cerebellar and brainstem signs. In cysts of the incisura tentorii and cerebellopontine angle, there are pyramidal signs due to brainstem compression.
In contrast with fibroma and fibromatosis anxiety 25 mg zoloft discount emsam 5 mg buy online, the tumor is relatively hypercellular and the nuclei have prominent nucleoli and mitotic activity; there is less intercellular collagen. Electron microscopy and immunohistochemistry can be used to confirm the fibroblastic nature of the tumor by the criteria described earlier and it can help to differentiate fibrosarcoma from other spindle-cell tumors such as rhabdomyosarcoma, schwannoma, and fibrous histiocytoma (1). Management the best management of orbital fibrosarcoma is wide surgical excision, including orbital exenteration when necessary. Radiotherapy, chemotherapy, and other modalities are usually considered to be palliative and should be employed when complete surgical excision cannot be accomplished. Localized primary fibrosarcoma of the superior orbit presenting as a subcutaneous mass in the eyebrow area in a 6-year-old girl. Proptosis and chemosis of the left eye in an elderly man secondary to orbital fibrosarcoma. There was some debate as to the exact diagnosis, but most authorities favored the diagnosis of fibrosarcoma. Osteoma is the most common tumor of the nose and paranasal sinuses and the most common neoplasm of the frontal sinus. Osteoma that arises from the bones of the frontal sinus, ethmoid sinus, and other periorbital bones can extend into orbit. The true incidence is actually greater, because most lesions originate in the sinuses, have minimal orbital involvement, and are more likely to be managed by otorhinolaryngologists. A patient with a suspected orbital osteoma should undergo ophthalmoscopy and possible referral to a gastroenterologist. For a more posterior osteoma that involves the orbital roof or cribriform plate, a combined orbitocranial approach can be employed. Clinical Features Orbital osteoma can occur at any age and there is no predilection for gender. Depending on the size and location of the osteoma, it can be asymptomatic or can produce orbital symptoms and signs such as proptosis and displacement of the globe. The bony mass may obstruct the ostea of the sinus and lead to chronic sinusitis or secondary mucocele. Diagnostic Approaches Computed tomography of orbital osteoma shows a sessile or pedunculated mass with bone density arising from otherwise normal bone, usually the frontal or ethmoid bones. The ivory type may be identical to bone; the fibrous type is less dense and may resemble fibrous dysplasia. It is believed that the compact type is most mature and the fibrous the least mature and that the fibrous type may be part of a continuum incorporating ossifying fibroma and fibrous dysplasia (1,3).
The lesion may extend anteriorly almost to the limbus and some patients complain of a visual field defect owing to the elevated lesion anxiety symptoms knee pain order emsam 5 mg. It is usually asymptomatic and is either noticed by the patient or by the physician on routine examination. Diagnostic Approaches Orbital dermolipoma is generally apparent on external examination and the diagnosis is easily made. In the case of a larger lesion, orbital computed tomography and magnetic resonance imaging can help to delineate the posterior extent of the lesion. These studies disclose a circumscribed oval or elongated mass extending into the orbit superotemporally in close association with the lacrimal gland and orbital fat. Pathology and Pathogenesis Histopathologically, dermolipoma is lined by stratified squamous epithelium, which may be partially keratinized. The deeper portions of the tumor usually contain mature fat, but the fat is not the main component of the lesion. Dermolipomas that contain cartilage and glandular acini are sometimes called complex choristomas and may be a component of the organoid nevus syndrome (12). Large, cosmetically unacceptable dermolipomas can Chapter 34 Orbital Lipomatous and Myxomatous Tumors 665 Orbital/Conjunctival Dermolipoma: Clinical Spectrum and Age Range Dermolipomas are most likely congenital but, because some are in an occult location, they may not be detected for a few years. Although the lesion was probably present since birth, it was first noticed at age 6 years. Although it was probably present since birth, the child and parents were unaware of the lesion until the child was 15 years old. Orbitoconjunctival dermolipoma presenting in the superotemporal fornix of a 19-year-old man. Dermolipoma presenting as a superotemporal orbitoconjunctival mass in a 6-year-old girl who had no evidence of Goldenhar syndrome. Dermolipoma presenting in the medial canthal region of a 2-year-old child with Goldenhar syndrome. Histopathology of orbitoconjunctival dermolipoma showing epithelium, collagenous tissue, and deeper fat. It is less well known that orbital/conjunctival dermolipoma is also a very common finding in patients with Goldenhar syndrome. Note the dull yellow color to the lesion and the prominent hairs arising from the lesion.
Peristomal variceal bleeding treated by coil embolization using a percutaneous transhepatic approach anxiety love generic 5 mg emsam fast delivery. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Effect of ursodeoxycholic acid on liver and bile duct disease in primary sclerosing cholangitis. Ursodeoxycholic acid does not improve the clinical course of primary sclerosing cholangitis over a 2-year period. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Ursodeoxycholic acid protects hepatocytes against oxidative injury via induction of antioxidants. Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis. Novel biotransformation and physiological properties of norursodeoxycholic acid in humans. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatment. Prospective risk assessment of endoscopic retrograde cholangiography in patients with primary sclerosing cholangitis. Quality of life before and after liver transplantation for cholestatic liver disease. Follow-up after liver transplantation for primary sclerosing cholangitis: effects on survival, quality of life, and colitis. Evolving frequency and outcomes of liver transplantation based on etiology of liver disease. Duct-to-duct reconstruction in liver transplantation for primary sclerosing cholangitis is associated with fewer biliary complications in comparison with hepaticojejunostomy. Meta-analysis of ductto-duct versus Roux-en-Y biliary reconstruction following liver transplantation for primary sclerosing cholangitis. Roux-en-Y choledochojejunostomy versus duct-to-duct biliary anastomosis in liver transplantation for primary sclerosing cholangitis: a meta-analysis.
Transverse banding of cerebellar structures is visualised in the enlarged aperture of incisura tentorii; the tectum is elongated anxiety early pregnancy purchase emsam 5 mg free shipping, and the massa intermedia is enlarged. There is breaking of the tectum on the image, the fourth ventricle is narrow and extends down into the foramen magnum and there is a cervicomedullary kink at the C2 level (e). Cranial X-ray reveals several manifestations of this malformation: holes on the most apical points of cranial vault bones, low placement of the inion, the more inferior attachment of tentorium cerebelli and very shallow posterior fossa, signs of hydrocephalus, enlargement of the foramen magnum, lumbar meningocele and spina bifida. These changes include changes in the location of tonsils and medulla in relation to the foramen magnum, elongations and/or ectopy of the fourth ventricle, Sylvian aqueduct narrowing or deformity, enlargement of the third and the lateral ventricles, and thickening of massa intermedia. It only detects the extent of hydrocephalus, lower location of posterior inferior cerebral artery, and the confluens sinuum. After surgery due to regression of hydrocephalus, the enlarged foramen of the tentorium cerebelli is much better distinguished, as well as a beak-shaped tectum and lifted cerebellar vermis with transverse folds. Frontonasal herniations protrude through the nasofrontal fonticulus, upper tip of nasal bones membrane, frontal bone and cartilage capsule. Naso-ethmoid herniations protrude through the foramen cecum of the frontal bone (where the frontal and ethmoidal bones meet) into the nasal cavity. A finger-shaped defect in the squamous part of occipital bone confluent with the foramen magnum is seen along with a soft tissue mass. There is a herniation of dura mater along with medially positioned bone defect in the posterior parietal region. Trans-ethmoidal herniations perforate sieve-like lamina and prolapse into the anterior portion of the nasal cavity. Spheno-ethmoidal herniations penetrate via the junction of the ethmoid and sphenoid bones and appear in the posterior portion of nasal cavity and nasopharynx. In trans-sphenoidal herniations, the cranial defect is located at the bottom of sella turcica or within the body of sphenoid bone. Herniation masses may be preserved in the sphenoid sinus or protrude into the nasopharynx. Spheno-orbital herniations (of posterior orbital location) may have different gates. Herniation masses may protrude through the superior orbital (sphenoidal) fissure, optic nerve channel, or congenital abnormal fissure of the sphenoid bone, or through the sphenoid and frontal bones. Herniation mass- es located near the apical part of the orbit cause unilateral exophthalmus. Primary agenesia occurs in the 12th week of fetal life as a consequence of vascular or inflammatory damage of commissural lamina and may be isolated 36 Chapter 2. Secondary dysgenesia-complete or partial destruction of corpus callosum-is a result of encephalomalacia and occurs after completion of corpus callosum development in traumatic and toxic lesions, and anoxia in the anterior cerebral artery territory. Agenesia of corpus callosum prevalence, according to our data, is encountered in 2.
Syndromes
Ultrasound and fluoroscopy guided percutaneous transhepatic biliary drainage in patients with nondilated bile ducts anxiety quitting smoking generic 5 mg emsam otc. Hepatic arterial injuries after percutaneous biliary interventions in the era of laparoscopic surgery and liver transplantation: experience with 930 patients. Percutaneous transhepatic treatment of hepaticojejunal anastomotic biliary strictures after living donor liver transplantation. Percutaneous management of biliary strictures after pediatric liver transplantation. Long-term follow-up of percutaneous transhepatic balloon cholangioplasty in the management of biliary strictures after liver transplantation. Percutaneous transhepatic biliary drainage may serve as a successful rescue procedure in failed cases of endoscopic therapy for a post-living donor liver transplantation biliary stricture. Safety and efficacy of the percutaneous treatment of bile leaks in hepaticojejunostomy or split-liver transplantation without dilatation of the biliary tree. Percutaneous management of anastomotic bile leaks following liver transplantation. Percutaneous transhepatic cholangiodrainage as rescue therapy for symptomatic biliary leakage without biliary tract dilation after major surgery. Biliary tract complications after orthotopic liver transplantation with choledochocholedochostomy anastomosis: endoscopic endoscopic findings and results of therapy. Endoscopic diagnosis and treatment of biliary leak in patients following liver transplantation: a prospective clinical study. Results of endoscopic retrograde cholangiopancreatography in the treatment of biliary tract complications after orthotopic liver transplantation: our experience. Endoscopic management is the treatment of choice for bile leaks after liver resection. Elevated stricture rate following the use of fully covered self-expandable metal biliary stents for biliary leaks following liver transplantation. Efficacy and safety of fully covered self-expandable metallic stents in biliary complications after liver transplantation: a preliminary study. Postsurgical bile leaks: endoscopic endoscopic obliteration of the transpapillary pressure gradient is enough. Management of iatrogenic bile duct injuries: role role of the interventional radiologist. Percutaneous management of bile duct strictures and injuries associated with laparoscopic cholecystectomy: a decade of experience. Percutaneous treatment of biliary stones: sphincteroplasty sphincteroplasty and occlusion balloon for the clearance of bile duct calculi. Percutaneous treatment of extrahepatic bile duct stones assisted by balloon sphincteroplasty and occlusion balloon.
It is most often located at the corneoscleral limbus and frequently extends to involve the cornea anxiety 9dpo emsam 5 mg mastercard. Lesions that extend deeply into the peripheral cornea may require keratoplasty (9). The diagnosis is not usually made clinically, but microscopically after surgical excision. Chapter 21 Conjunctival Neural, Xanthomatous, Fibrous, Myxomatous, and Lipomatous Tumors 373 Conjunctival Fibrous Histiocytoma Fibrous histiocytoma of the conjunctiva can assume a variety of clinical appearances. Well-circumscribed, yellow-white fibrous histiocytoma located at the limbus inferiorly in a 27-year-old woman. More aggressive appearing conjunctival fibrous histiocytoma located and limbus with secondary corneal invasion. In the ocular region, it is best known for causing an iris mass that can produce a spontaneous hyphema. Clinical Features Conjunctival involvement usually occurs as a solitary lesion unassociated with the skin eruption. It appears as a yellow, elevated lesion, usually near the corneoscleral limbus in any quadrant. This adult-onset xanthogranuloma seems to be identical clinically and histopathologically to the infantile or juvenile form. Clinical Features Conjunctival fibroma generally develops in adulthood and can be nodular or diffuse. A rare variant, elastofibroma oculi, contains lobules of fat, tissue not normally found in the conjunctival stroma (3,4). However, if the diagnosis is suspected clinically, a period of observation is justified; the lesion is said to resolve without treatment. Conjunctival Nodular Fasciitis General Considerations Nodular fasciitis (pseudosarcomatous fasciitis) is an idiopathic, benign nodular proliferation of connective tissue that involves the superficial fascia. It is important that this inflammatory condition be differentiated clinically and histopathologically from malignant spindle cell neoplasms. In the ocular region it usually affects the eyelids, but can develop in the orbit or conjunctiva (5,6). It generally appears as a solitary episcleral nodule that may show signs of inflammation.
In those who were initially asymptomatic anxiety symptoms wiki 5 mg emsam buy fast delivery, the frequency of biliary pain was 12% at 2 years, 17% at 4 years, and 26% at 10 years, and the cumulative rate of biliary complications was 3% at 10 years. Nine of 134 patients (7%) had undergone cholecystectomy, as had 5 of 91 patients who had died prior to follow up (6%). During follow up, abdominal pain developed in 44%, and 29% had what were deemed to be functional abdominal complaints. This study illustrates again both the frequent resolution and relatively benign nature of asymptomatic gallstone disease. Special Patient Populations the clinical manifestations of gallstones are shown schematically in. Although the standard approach to asymptomatic gallstones is observation, some patients with asymptomatic gallstones may be at increased risk of complications and may require consideration of prophylactic cholecystectomy. An increased risk of cholangiocarcinoma and gallbladder carcinoma has been associated with certain disorders of the biliary tract and in some ethnic groups Risk factors include choledochal cysts, Caroli disease, pancreaticobiliary malunion (also referred to as anomalous union of the pancreatic and biliary ducts, in which the pancreatic duct drains into the bile duct), large gallbladder adenomas, and porcelain gallbladder (see Chapters 55, 62, and 67). Patients at increased risk of biliary cancer may benefit from prophylactic cholecystectomy. If abdominal surgery is planned for another indication, an incidental cholecystectomy should be performed. Pigment gallstones are common and often asymptomatic in patients with sickle cell disease. Prophylactic cholecystectomy is not recommended, but an incidental cholecystectomy should be considered if abdominal surgery is performed for other reasons. Some authorities recommend combined prophylactic splenectomy and cholecystectomy in young asymptomatic patients with hereditary spherocytosis if gallstones are present. Morbidly obese persons who undergo bariatric surgery are at high risk of complications of gallstones (see Chapters 7 and 8). Some investigators have proposed that patients with incidental cholelithiasis who are awaiting heart transplantation undergo a prophylactic cholecystectomy irrespective of the presence or absence of biliary tract symptoms because they are at increased risk of post-transplant gallstone complications. A prospective study of patients with insulinresistant diabetes mellitus showed that after 5 years of follow-up, symptoms had developed in 15% of the asymptomatic patients. Therefore, prophylactic cholecystectomy is not recommended in patients with insulin-resistant diabetes mellitus and asymptomatic gallstones. Percentages indicate approximate frequencies of complications that occur in persons with gallstones, based on natural history data. The most frequent outcome is for the patient with a stone to remain asymptomatic throughout life (1). Biliary pain (2), acute cholecystitis (3), cholangitis (5), and pancreatitis (5) are the most common complications.
The use of oral lactulose to lower the nitrogen load has not been studied in this patient population anxiety hives 5 mg emsam visa. Given the extremely high ammonia levels often encountered, continuous arteriovenous hemodialysis or hemofiltration is frequently required. Arginine, carnitine, and long-chain fatty acids are usually present in low levels in these patients and should be supplemented. Further therapy and protein restriction are then tailored to the patient; those with a severe disorder may need essential amino acids to supplement their protein intake. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are needed. A possible exception to this is in patients transplanted before the age of one year, in whom developmental, and possible neurocognitive, outcomes may improve. Hyperammonemia is unusual in affected persons, but hyperammonemic coma and death have been reported. The disease is characterized by indolent deterioration of the cerebral cortex and pyramidal tracts, leading to progressive dementia and psychomotor retardation, spastic diplegia progressing to quadriplegia, seizures, and growth failure. Many guanidine compounds may accumulate in the blood and cerebrospinal fluid of these patients, which could play an important pathophysiologic role, and guanidinoacetate, a well-known potent epileptogenic compound, has demonstrated usefulness as a target for the therapeutic monitoring of patients with arginase deficiency. The diagnosis is confirmed by enzymatic analysis, which can be performed prenatally on cord blood samples. Treatment consists of protein restriction and, when needed, sodium phenylbutyrate. With advances in molecular biology, genetics, and mass spectrometry, several different inborn errors in bile acid synthesis and transport have been identified as causes of clinical disease. For some of the disorders, this progress has led to improved diagnosis and life-saving therapy. These complementary tests allow rapid, sensitive, and cost-effective bile acid profiling and mutation screening to aid clinical diagnosis in patients with intrahepatic cholestasis. Secondary metabolic defects that impact primary bile acid synthesis include peroxisomal disorders, such as cerebrohepatorenal syndrome of Zellweger and related disorders, and Smith-Lemli-Opitz syndrome. The former bypasses the enzymatic block and provides negative feedback to earlier steps in the synthetic pathways, whereas the latter displaces toxic bile acid metabolites and serves as a hepatobiliary cytoprotectant. Deficiency of 4-3-oxosteroid 5-reductase usually leads to neonatal cholestasis, which rapidly progresses to synthetic dysfunction and liver failure. Clinical symptoms and signs include adult-onset progressive neurologic dysfunction. After significant neurologic pathology is established, the effect of treatment is limited and deterioration may continue. In some patients, liver disease with features of a cholangiopathy has been present. Oral glycocholic acid therapy has been shown to be safe and effective in improving growth and fat-soluble vitamin absorption in children and adolescents with these disorders.
Stan, 22 years: Prevalence and significance of gallbladder abnormalities seen on sonography in intensive care unit patients. Compression of the central arteriole with a pinhead results in blanching followed by reformation of the "spider" after release of pressure on the arteriole.
Nafalem, 49 years: Clinical Features Orbital hemangiopericytoma occurs mainly in adults and has similar symptoms, signs, and imaging study findings as cavernous hemangioma. Herniation mass- es located near the apical part of the orbit cause unilateral exophthalmus.
Altus, 48 years: Note the characteristic diffuse orbital mass that molds to the globe and optic nerve. Treatment consists of protein restriction and, when needed, sodium phenylbutyrate.
Hassan, 39 years: Note that a suture has been placed beneath the superior rectus and the exposed mass appears to involve the muscle itself. Extramedullary plasmacytoma of the orbit: case report with results of immunocytochemical studies.
Snorre, 36 years: These opposing enzyme reactions regulate the formation of gluconeogenesis precursors and glycolysis. Usually, they are located in the extrahepatic biliary tract, particularly at the junction of the cystic duct and the bile duct.
Yespas, 55 years: Protrusion of postoperative maxillary sinus mucocele into the orbit: case reports. It is initially a circumscribed neoplasm, but it can become rapidly infiltrative and invade bone (6).
Jack, 62 years: Management In most cases, it is best to follow pingueculum without surgical intervention. The major concerns regarding the use of a liver biopsy to diagnose cirrhosis includes sampling error and interobserver disagreement in the estimation of the extent of fibrosis.
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