O-Gastroenterology Flashcards
A 27-year-old woman comes to the emergency department because of the recent onset of severe right upper quadrant abdominal pain. She reports a 3-day history of purulent vaginal discharge and pelvic tenderness. She is sexually active and does not use condoms. Physical examination shows mucopurulent cervical discharge and cervical motion tenderness. Which of the following findings is most likely to be specifically associated with this patient’s abdominal pain?
- Vaginal Discharge
- Increased serum AST and ALT activity
- Elevated CRP
- Perihepatitis
- Endometritis
Correct answer: perihepatitis
Major takeaway: Fitz-Hugh-Curtis syndrome refers to perihepatitis in the context of pelvic inflammatory disease and is characterized by inflammation of the liver capsule and adjacent peritoneal structures. Patients typically present with right upper quadrant pain.
Main explanation: The diagnosis of pelvic inflammatory disease (PID) should be considered in any patient with lower abdominal or pelvic pain who is sexually active. Given the potential for permanent reproductive sequelae if treatment is delayed, a high index of suspicion should be maintained. PID can be associated with other findings, including inflammation of the liver capsule. This perihepatitis in the context of PID is known as Fitz-Hugh-Curtis syndrome. Patients present with right upper quadrant pain, which can be associated with a pleuritic pain or referred pain to the right shoulder. Findings on paroscopy or visual examination include “violin string” adhesions, or fibrinous exudateson the surface of the liver.
A 55-year-old man comes to the emergency department because of several episodes of vomiting. The patient complains of retrosternal chest pain, back pain, and shortness of breath. Auscultation shows diminished breath sounds bilaterally at the bases, and a crackling sound heard best directly over the heart. Palpation of the anterior chest is best described as feeling like there are “rice crispies” underneath the skin. Which of the following tests would confirm the diagnosis?
- Barium swallow study
- Echocardiogram
- Endoscopy
- Gastrografin swallow study
- Upright chest radiograph
Answer: Gastrografin swallow study
Not upright chest radiograph because: A chest X-ray is useful in initial workup as it can show various findings such as subcutaneous emphysema, pneumomediastinum , mediastinal widening, and pleural effusion. However, it does not confirm the diagnosis.
Major takeaway: Boerhaave syndrome is characterized by chest and back pain following an episode of strenuous vomiting, with a clinical finding of subcutaneous emphysema . Gastrografin esophagogram is used to confirm the diagnosis.
Main explanation: This patient has Boerhaave syndrome (rupture/perforation of the esophagus due to forceful vomiting). The Mackler triad of vomiting, lower chest pain , and subcutaneous emphysema are classically associated with esophageal rupture but are not always present on initial presentation. Other symptoms and physical findings to look for are back pain, tachycardia , shortness of breath , and Hamman’ sign (crackling sound heard on the chest as the heart beats surrounded by air-filled tissues). The palpation of subcutaneous emphysema (“rice crispies under the skin “) is the most telling sign of an esophageal rupture in a patient with this history. Repeated vomiting creates an increased intraesophageal pressure that can lead to rupture. The perforation then leads to rapid inflammation due to esophageal contents leaking into the mediastinal , pleural , or peritoneal space. Esophagram using Gastrografin is performed to confirm the diagnosis and locate the site of rupture in Boerhaave syndrome . If this is unavailable, or is negative with a high clinical suspicious a CT scan is useful. CT has the additional benefit of being able to identify sites that may be suitable for surgical drainage. This website explains the syndrome and imaging: http://www.learningradiology.com/notes/ginotes/boerhavvepage.htm
A 94-year-old man comes to the office because of a small bulge on the right side of his groin. He first noted the lesion after helping his daughter move to a new home. He reports sharp discomfort that is worsened by walking and heavy lifting. Resting supine decreases the severity of the pain. Physical examination shows a bulge that protrudes medially to the inferior epigastric vessels when the patient coughs. There is no erythema or warmth. Which of the following is the most likely diagnosis?
A Hiatal hernia B Strangulated hernia C Femoral hernia D Direct inguinal hernia E Indirect inguinal hernia
Answer: Direct inguial hernia
NOT indirect inguinal hernia: Indirect inguinal hernias are considered congenital lesions and result from failure of the processus vaginalis to obliterate, allowing abdominal contents to protrude through the deep inguinal ring. The protrusion occurs lateral to the inferior epigastric vessels .
Major takeaway Direct inguinal hernias result from weakness in the floor of the inguinal canal , leading to protrusion of abdominal contents through Hesselbach’s triangle. This triangle is bounded by the inferior epigastric vessels laterally, the inguinal ligament inferiorly, and the rectus abdominis muscle medially.
Main explanation This patient presents with a visible bulge in the groin region and associated discomfort that is worsened by actions that increase intraabdominal pressure, such as walking or heavy lifting. These symptoms are strongly characteristic of an inguinal hernia . Given the patient’s age and history of recent strenuous activity, a direct inguinal hernia is the most likely cause. Direct inguinal hernias involve protrusion of abdominal contents through Hesselbach’s triangle, which is bounded by the inferior epigastric vessels laterally, the inguinal ligament inferiorly, and the rectus abdominis muscle medially. Contents bulge through the external or superficial inguinal ring only and are covered by the external spermatic fascia . Direct hernia result from weakness in the floor of the inguinal canal and are most commonly seen in older men.
A 32-year-old man comes to the emergency department via emergency medical services because of acute onset of speech difficulties. He denies headache , numbness , tingling, or weakness of any extremities. However, he does describe a tremor of his right hand and new clumsiness. His family reports that the patient has been more aggressive and temperamental recently. Vital signs are normal, and physical examination shows a well-developed male with mask-like facies, slight jaundice and palmar erythema . Eye examination findings are shown below. Which of the following laboratory findings is most likely to be consistent with the etiology of the patient’s symptoms?
A Decreased alanine aminotransferase B Decreased ceruloplasmin C Elevated creatine kinase D Elevated erythrocytes E Elevated immunoglobulin G
ANSWER: decreased ceruloplasmin
Major takeaway: Wilson disease is an autosomal recessive disorder often manifesting as liver disease and neuropsychiatric symptoms due to tissue accumulation of copper . Kayser-Fleischer rings are a classic finding indicative of total body copper overload.
Main explanation: Wilson disease is an autosomal recessive disorder often manifesting as liver disease and neuropsychiatric symptoms due to tissue accumulation of copper . Patients are often homozygous for mutations of the ATP7B gene, which is necessary to link ceruloplasmin to copper and excrete it into bile . This function is absent in Wilson disease , yet ceruloplasmin is still actively degraded, leading to an excess of copper that accumulates despite compensatory increased excretion. Deposition occurs in liver, brain basal ganglia (leading to movement disorders ), eye ( pathognomonic Kayser-Fleischer rings ), and kidney. Other lab abnormalities commonly seen in Wilson disease include increased liver function tests , decreased albumin , prolonged prothrombin time (PT). Treatment is with a chelating agent such as D-penicillamine .
A 38-year-old woman comes to the emergency department because of severe upper abdominal and chest pain. She has been unwell with suspected food poisoning for the past 24 hours, with frequent and severe vomiting. About 12 hours ago, she began to develop retrosternal chest pain that she describes as “burning” and increasing in severity. Physical examination shows temperature is 39°C (102.2°F), pulse is 115/min, respirations are 30/min, blood pressure is 130/78 mm Hg. Her breathing is shallow due to pain. Lung sounds are clear bilaterally, and cardiac examination shows no murmurs, rubs, or gallops. Chest X-ray shows a small left pleural effusion, small pneumothorax, and a moderate pneumomediastinum. Esophageal rupture is diagnosed and the surgical team contacted. Which of the following best describes a recommended surgical treatment for esophageal rupture?
A Insertion of chest tube
B Esophagectomy and gastrectomy
C Esophageal stenting
D Diverting esophagostomy and gastrostomy
E Thoracotomy for drainage of pleural effusion
Answer: Diverting esophagostomy and gastrostomy
Not thoracostomy for drainage of pleural effusion: The pleural effusion can be drained in less invasive ways, including thoracentesis or the placement of a chest tube . There is no need for a thoracotomy for the drainage of the pleural effusion in this patient. Furthermore, this does not treat the root cause of this issue.
Major takeaway: Symptoms of esophageal perforation include chest pain , pleural effusion , fever, and leukocytosis . Treatment with esophagostomy and gastrostomy should be performed immediately after diagnosis, as outcomes for the patient are highly dependent on the speed of diagnosis and treatment.
Main explanation: This woman has Boerhaave syndrome , spontaneous esophageal rupture following a severe episode of vomiting. The majority of cases of esophageal rupture are iatrogenic , often during diagnostic procedures such as endoscopy , and occur in the proximal 1/3 of the esophagus . Spontaneous rupture tends to occur in the distal 1/3 of the esophagus . Characteristics of esophageal perforation include chest pain , pleural effusion on chest x-ray , fever, and leukocytosis . If left untreated, esophageal perforation can lead to sepsis and death. The outcome for a patient is mainly dependent on the speed with which diagnosis and treatment occur, making recognition of this presentation critical. Following diagnosis, the patient should be prepared for surgery through maintenance of the airway , broad-spectrum antibiotic therapy, blood type and crossmatch , and placement of a nasogastric tube with endoscopic guidance. Diverting esophagostomy and gastrostomy is one potential treatment option for this patient, effectively preventing further leakage through the esophageal perforation into the pleural cavities and mediastinum . Later reconstructive surgery is usually indicated to restore the gastric conduit. Other options include repair of the perforated site (when possible), drainage alone, and resection of the site followed by anastomosis (such as esophagectomy).
A 25-year-old man comes to the emergency department because of fever, jaundice, and pain localized to the right upper quadrant. Endoscopic retrograde cholangiopancreatography shows alternating strictures and dilation of the bile ducts with a characteristic “beading” appearance with the involvement of both intrahepatic and extrahepatic bile ducts. Which of the following is the most likely diagnosis?
A Crohn disease B Polymyalgia rheumatica C Primary biliary cirrhosis D Primary sclerosing cholangitis E Sarcoidosis
Answer: Primary sclerosing cholangitis
Major takeaway: Primary sclerosing cholangitis (PSC) leads to fibrosis in the intra- and extrahepatic bile ducts . The hallmark feature of PSC is a “beading” appearance of the biliary tree in radiographic imaging with contrast. Main explanation Primary sclerosing cholangitis (PSC) is a chronic idiopathic disorder involving inflammation and fibrosis of both intra- and extrahepatic bile ducts . Alternating dilation and stricture of bile ducts result in characteristic “beading” appearance on ERCP. Concentric fibrosis of the bile duct lumen and loss of small bile ducts may be seen in histological section. Up to 70% of patients with PSC suffer from ulcerative colitis . PSC patients are usually male and in their third through fifth decades of life. PSC can be characterized by jaundice , pruritus, and progressive fatigue. Laboratory studies usually show elevated alkaline phosphatase and perinuclear antineutrophilic cytoplasmic antibody ( p-ANCA ). The disease is progressive over many years, and 10-15% of PSC patients go on to develop cholangiocarcinoma . The only effective therapy for PSC is liver transplantation .
A 63-year-old man comes to the office because of heartburn for the past four months. He experiences a burning feeling in his chest whenever he eats. He has also had difficulty swallowing his food. The patient has lost 6.8-kg (15-lb) over the past four months. A chest radiograph is obtained and shown below. Which of the following is the most likely diagnosis?
A. Achalasia - Achalasia is characterized by a increased tone of the lower esophageal sphincter (LES), which makes the sphincter more difficult to relax. It is caused by the absence of inhibitory innervation of the LES, such as in Chagas disease . Progressive dysphagia , regurgitation, and aspiration of undigested food are common features.
B Gastroparesis - Gastroparesis refers to delayed emptying of gastric contents. Many cases of gastroparesis are idiopathic , but it can be caused by diabetes or previous surgery. Gastroparesis is characterized by early satiety , nausea , abdominal pain , bloating , and weight loss.
C Paraesophageal hernia - Paraesophageal hernias (type II, III, and IV) account for 5-10% of hiatus hernias . Paraesophageal hernias are characterized by protrusion of the fundus of the stomach above the diaphragm . Epigastric or substernal pain, postprandial fullness, and nausea are common features.
D Sliding hiatal hernia
E Zenker diverticulum - Zenker diverticulum is an outpouching of the mucosa and submucosa between the cricopharyngeus and lower inferior constrictor muscles. It is characterized by transient dysphagia , pulmonary aspiration , foul breath , and appearance of a neck mass.
ANSWER: sliding hiatial hernia
A 58-year-old woman with a long history of alcohol use disorder comes to the office because of diarrhea, general weakness, and a 4.08-kg (9-lb) weight loss over the past 3 months. She has had difficulties driving at night and feels episodic upper abdominal pain not relieved with antacids or proton pump inhibitors. Laboratory studies show: Serum ionized calcium: 3.5 mg/dL Prothrombin time: 30 seconds Partial thromboplastin time 60 seconds Which of the following is the most likely etiology of this patient’s diarrhea?
A Carcinoid tumor B Celiac sprue C Chronic gastritis D Chronic pancreatitis E Pancreatic cancer of the head of the pancreas
Answer: chronic pancreatitis
Pancreatic cancer involving the head of the pancreas can produce a similar clinical picture; however, one would also expect to see signs of obstructive jaundice and elevated LFTs.
Major takeaway Patients with chronic pancreatitis typically have abdominal pain and pancreatic insufficiency . Pancreatic insufficiency manifests as symptoms of fat malabsorption and pancreatic diabetes . Main explanation The patient’s clinical picture most likely reflects a malabsorption syndrome . Chronic pancreatitis resulting in chronic pancreatic insufficiency is the most likely diagnosis. Chronic inflammation of the pancreas can result in a critical amount of damage to both the endocrine and exocrine functions of the pancreas . Without the exocrine function of the pancreas , dietary lipids cannot be digested and absorbed nor can the fat-soluble vitamins D, A, K, E. Malabsorption of lipids will result in an osmotic steatorrhea . Malabsorption of vitamins D, A, K, E will result in their respective deficiency symptoms. D = hypocalcemia A = night blindness K = prolonged PT/aPTT E = neurologic deficits and ataxia , though this is very uncommon.
A 22-year-old Jewish woman comes to the emergency department because of severe right lower quadrant abdominal pain for the past two hours. She has had diarrhea and weight loss for several weeks. A colonoscopy is performed, and an image from the procedure is shown below. Which of the following is the most likely complication of this patient’s diagnosis?
A Dehydration B Perforation of the appendix C Perianal fistulas D Right ovarian necrosis E Toxic megacolon
Answer: Perianal fistulas
Major takeaway Crohn disease leads to fistulas as a common clinical manifestation and complication. Since the bowel inflammation is transmural, sinus tracts penetrate the intestinal wall and give rise to these fistulas .
Main explanation Based on her symptoms of intermittent diarrhea, weight loss, and abdominal pain , as well as the results of endoscopy , the patient likely has Crohn disease . Notably in the clinical description, this patient’s right lower quadrant pain is typical, since the terminal ileum is often affected. Malabsorption of nutrients is common in Crohn, hence her weight loss. Granulomatous skip lesions may be seen on endoscopy . Histologically, Crohn disease is characterized by transmural intestinal inflammation and cobblestone mucosa. Crohn disease can affect anywhere in the GI tract (“mouth to anus “), so aphthous ulcers can be seen in the mouth, and even the esophagus can be affected, though rarely. Complications of Crohn disease include fistulas . Sinus tracts that penetrate the serosa give rise to fistulas , which are usually indolent rather than presenting as an acute abdomen . Up to 45% of patients develop a fistula before a diagnosis of Crohn disease . The fistulas mostly occur in the perianal region, but they can also form between the intestine and bladder / uterus .
RE OTHER OPTIONS:
- Dehydration would most likely occur secondary to severe diarrhea caused by a bacterial infection. Yersinia , Campylobacter , or Salmonella enterocolitis may mimic appendicitis , but the presentation for these infections would be more acute.
- Perforation of the appendix is a complication of appendicitis . This would have a more acute presentation without the weeklong symptoms of diarrhea and weight loss. The endoscopic image does not suggest appendicitis .
- Ovarian torsion that results in blockage of blood supply to the ovary may lead to necrosis of the ovary . Patients are characterized by acute, moderate to severe pelvic pain with nausea and vomiting. An adnexal mass may be palpable.
- Toxic megacolon is a potentially lethal complication of inflammatory bowel disease , but it is more commonly seen in patients with ulcerative colitis . Patients are characterized by >10 stools/day, bleeding, abdominal pain , distension , and acute, severe toxic symptoms.
A 43-year-old man with a recurrent fistula-in-ano comes to the office for monitoring of his Crohn disease. An MRI is done to determine whether he has developed a complex, multi-tract fistula. The MRI shows that his fistula only follows one tract. It courses perpendicularly to the skin from its external perianal opening, then takes a sharp 90° turn and burrows through both the external anal sphincter and the internal sphincter to open into the anal canal. According to the Parks classification of fistulas, which of the following most accurately describes this patient’s fistula-in-ano?
A Extra-sphinteric fistula B Inter-sphincteric fistula C Supra-levator fistula D Supra-sphincteric fistula E Trans-sphinteric fistula
Major takeaway Trans -sphincteric fistulas are often the result of ischiorectal fossa abscesses and account for 25% of all anal fistulas . They commonly start at the dentate line and then course through the internal and external anal sphincters into the ischiorectal fossa, opening onto the perianal or perineal skin .
Main explanation A perianal fistula ( fistula-in-ano ) is a chronic fistulous tract between the anal canal and the perianal skin . They usually originate from the anal glands, which are located between the internal and external anal sphincter , and drain into the anal canal . If these glands become blocked, abscesses can form and eventually lead to fistula formation. The Parks classification is a widely used method for distinguishing four types of fistula . In order of decreasing frequency, they are: Inter-sphincteric Trans -sphincteric Supra-sphincteric Extra-sphincteric Fistula formation is a complication of Crohn disease , in which there is transmural inflammation of the intestinal wall . Trans -sphincteric fistulas result from abscesses in the ischiorectal fossa. This type accounts for 25% of anal fistulas . They begin at the dentate line and then course through the internal and external anal sphincters into the ischiorectal fossa, and open onto the perianal or perineal skin . The patient’s MRI finding corresponds to this type of fistula . Management of enterocutaneous fistulas begins with treatment of the abscess . Approximately one-third closes spontaneously within 5-6 weeks with conservative management. Surgical closure is necessary if this is not adequate.
A 70-year-old woman comes to the emergency department because of blood in her stool. She says that her stool has been black and tarry, but she can occasionally see red streaking. She reports no recent weight loss or abdominal pain. Her past medical history is significant for Hepatitis C infection. Stool guaiac cards are positive. Endoscopy shows long, red streaky areas on the stomach. Which of the following is the most likely diagnosis?
A Ménétrier disease B Dieulafoy lesion C Gastric antral vascular ectasia D Peptic ulcer disease E Stomach cancer
Answer: gastric antral vascular ectasia
Major takeaway Gastric antral vascular ectasia is an uncommon cause of gastrointestinal bleeding or iron deficiency anemia . It is associated with dilated small blood vessels in the antrum of the stomach. The dilated vessels result in the bleeding. Gastric antral vascular ectasia gives the appearance of a “ watermelon stomach .”
Main explanation Gastric antral vascular ectasia is an uncommon cause of gastrointestinal bleeding or iron deficiency anemia . It is associated with dilated small blood vessels in the antrum of the stomach. The dilated vessels result in the bleeding. Gastric antral vascular ectasia gives the appearance of a “ watermelon stomach .” Associated conditions include portal hypertension , chronic kidney failure , and collagen vascular diseases. Diagnosis is typically made by endoscopy which shows characteristic watermelon stripes. Surgical exploration of the abdomen may be needed to diagnose cases with liver or other organ involvement. Treatment often includes argon plasma coagulation and electrocautery . Endoscopy with thermal ablation is the favored medical treatment because of its low side effects and low mortality, but it is rarely curative. The typical patient is an elderly woman, and about 31% of patients have portal hypertension .
A 39-year-old male comes to the emergency department because of altered mental status, ataxia, and diplopia. His past medical history is only significant for Crohn disease, which he controls with medication. An MRI of his brain is obtained and shown below. Which of the following medications would most likely be responsible for his current neurological symptoms? (it appears like white in the cerebral matter)
A Infliximab B Golimumab C Natalizumab D Cetuximab E Alemtuzumab
Answer: Natalizumab
Major takeaway Progressive multifocal leukoencephalopathy only occurs in majorly immunocompromised individuals, such as those receiving natalizumab for Crohn disease . It is caused by the John Cunningham (JC) virus and typically presents with hyperintensive lesions on T2 MRI, as well as signs of progressive neurological damage.
Main explanation The patient has progressive multifocal leukoencephalopathy (PML), which is a demyelinating disease of the brain that is common in severely immunocompromised patients. It is caused by the John Cunningham virus. On T2 MRI there are significant hyperintensities and symmetric or asymmetric multifocal areas of white matter demyelination . They do not conform to cerebrovascular territories and exhibit neither mass effect nor contrast enhancement. Natalizumab is a humanized monoclonal antibody and alpha 4 integrin inhibitor that increases a patient’s risk of developing PML if they are already infected with JC virus . Risk factors for the development of PML include duration of therapy, prior use of immunosuppressants , and presence of anti– JC virus (JCV) antibodies. Treatment for patients with natalizumab -associated PML is discontinuation of natalizumab and beginning plasma exchange .
A 26-year-old woman, who is at 30 weeks gestation , comes to a dermatology clinic because of “itchy skin.” The patient states that the itching began over the past two weeks. She has not noticed any skin lesions but has been picking at and scratching her skin with her nails. Physical examination shows faint red and pink excoriations, but no primary skin lesions. A disorder involving which of the following organs is most likely responsible for her skin condition?
A Thyroid B Thymus C Uterus D Liver E Pancreas
ANSWER: Liver
Major takeaway Intrahepatic cholestasis of pregnancy is a reversible form of cholestasis affecting some pregnant women in their second or third trimesters characterized by hepatic dysfunction and pruritus of the skin caused by elevated bile acids in the bloodstream, causing bile salt deposition in the skin, and secondary skin lesions due to scratching only (no primary lesions).
Main explanation Pruritus gravidarum is also known as intrahepatic cholestasis of pregnancy . It is a reversible form of cholestasis affecting some pregnant women in their second or third trimesters. It is believed to be linked to female sex hormone changes that take place in the body during pregnancy and, in some cases, patients who are not pregnant but who are taking oral contraceptive pills . The hallmark of this condition is pruritus of the skin caused by elevated bile acids in the bloodstream, which lead to bile salt deposition in the skin, and secondary skin lesions due to scratching only (no primary lesions). Jaundice may develop with cases of severe cholestasis . The diagnosis of pruritus gravidarum/ intrahepatic cholestasis of pregnancy is confirmed by the laboratory finding of serum bile acids of greater than 11.0 μ mol /L. Pruritus and excoriations of the skin should lessen with treatment and disappear after the patient gives birth. Patients should be advised to avoid picking at their skin to avoid scarring and/or secondary infections.
OTHER EXPLANATIONS: A Thymus Show explanation 2% The thymus is a primary lymphoid organ of immune tissue. It does play a roll in hypersensitivity reactions , but pruritus gravidarum is not caused by thymic dysfunction. B Thyroid Show explanation 4% Pruritus may be seen in cases of hyperthyroidism , but other symptoms of hyperthyroidism are absent in this patient ( flushing , heat intolerance, weight loss, anxiety , etc.). C Uterus Show explanation 1% Pruritus is often associated with pregnancy, most often due to changes in the skin, especially that of the abdomen . Pruritus gravidarum is not caused by uterine dysfunction. D Liver Show explanation 86% E Pancreas Show explanation 7% Carcinoma of the pancreas rarely has ben associated with generalized pruritus. It has been known to be an important symptom in other malignancies.
A 33 year-old man comes to the gastroenterologist because of progressive dysphagia over the last 3 months. Barium swallow shows a dilated esophagus with an area of distal stenosis. Upon surgical treatment of the patient’s condition, which of the following is the patient at the greatest risk of developing?
A Gastric carcinoma B Gastro-esophageal reflux disease C Mallory-Weiss syndrome D Plummer-Vinson syndrome E Zollinger-Ellison syndrome
ANSWER: GERD
Major takeaway The treatment of achalasia is surgical myotomy or pneumatic dilation of the esophagus to relax lower sphincter tone . This can lead to over-relaxation and gastroesophageal reflux disease .
Main explanation This patient has achalasia , which results from failure of relaxation of lower esophageal sphincter (LES) due to the loss of the myenteric (Auerbach’s) plexus. The progressive dysphagia is caused by the stenotic LES. Treatment of achalasia includes pneumatic dilation of the LES or surgical myotomy. Because the treatment involves relaxing the LES, the patient can be more prone to gastroesophageal reflux disease . Primary achalasia is an idiopathic loss of myenteric ganglion cells that leads to dysregulated constriction of the LES. Secondary achalasia is caused by a known insult, such as Trypanosoma cruzi in Chagas disease . Symptoms of achalasia include progressive dysphagia to solids and liquids, putrid breath , and regurgitation.
OTHER EXPLANATIONS: Gastric carcinoma Show explanation 16% Gastric carcinoma may result from disruption of the gastric mucosa from chronic gastritis . Treatment of achalasia doesn’t influence such processes. B Gastro-esophageal reflux disease Show explanation 63% C Mallory-Weiss syndrome Show explanation 9% Mallory-Weiss tears at the gastroesophageal junction are caused by excessive vomiting or retching. The treatment of achalasia does not predispose the patient to either one. D Plummer-Vinson syndrome Show explanation 8% Plummer-Vinson syndrome (PVS) is a long-term iron deficiency anemia resulting from esophageal webs in the proximal esophagus (near the upper esophageal sphincter ). Achalasia is not associated with PVS. E Zollinger-Ellison syndrome Show explanation 4% Zollinger-Ellison syndrome is caused by a gastrin -secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach ( parietal cells ) to maximal activity, with consequent gastrointestinal mucosal ulceration. It is not associated with achalasia.
KNOWLEDGE POINTS:
Stomach cancer is most commonly gastric adenocarcinoma developing from the lining of the stomach. The most common cause is infection by the bacterium Helicobacter pylori, which accounts for more than 60% of cases. Other causes include dietary nitrosamines (smoked foods), tobacco smoking, achlorhydria, and chronic gastritis. This cancer often presents late, with weight loss, early satiety, and in some cases acanthosis nigricans or Leser-Trélat sign.
Achalasia is a failure of relaxation of the lower esophageal sphincter due to loss of the myenteric (Auerbach) plexus. This can lead to progressive dysphagia to solids and liquids. A barium swallow will show a dilated esophagus with an area of distal stenosis. This is also the characteristic ‘bird’s beak’ sign.
Mallory–Weiss syndrome or gastro-esophageal laceration syndrome refers to bleeding from a laceration in the mucosa at the junction of the stomach and esophagus. This is usually caused by severe vomiting because of alcoholism or bulimia, but can be caused by any conditions which causes violent vomiting and retching such as food poisoning. The syndrome presents with painful hematemesis. The laceration is sometimes referred to as a Mallory-Weiss tear.
The triad of iron deficiency anemia, dysphagia, and a cervical esophageal web is known as Plummer-Vinson or Paterson-Brown-Kelly syndrome. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. Nowadays, this syndrome has become extremely rare.
A 30-year-old woman comes to the clinic because of fever and fatigue. She has no chest pain, cough, sore throat, or hemoptysis. She works as a nurse practitioner in a nearby hospital. Five months ago, she was injured by a needle from a hepatitis patient. She also has Hashimoto’s thyroiditis and receives replacement therapy. Her temperature is 38.5°C (101.3°F), pulse is 100/min, respirations are 15/min, and blood pressure is 110/70 mm Hg. Physical examination shows jaundice and right upper abdominal quadrant tenderness. Laboratory studies show:
See photo for question 15
Which of the following is the most likely diagnosis?
A Primary biliary cholangitis B Acute hepatitis C infection C Chronic hepatitis B infection D Autoimmune hepatitis E Acute hepatitis B infection
ANSWER: Auto-immune hepatitis
Major takeaway Autoimmune hepatitis is an immune mediated injury of the liver cells . It is caused by aberrant HLA class II. It may present as acute hepatitis , chronic hepatitis , or cirrhosis . Antismooth muscle antibody (SMA) is very specific for autoimmune hepatitis .
Main explanation Autoimmune hepatitis is an immune mediated hepatic injury . It is due to aberrant human leukocyte antigen (HLA) class II on liver cells . The aberrant HLA II induces the immune system to attack the liver causing autoimmune hepatitis . The cause of aberrant HLA is still unknown. Autoimmune hepatitis usually occurs in women between the ages of 15 and 40. It may present as acute hepatitis , chronic hepatitis , or cirrhosis . Around one third of the patients present with symptoms of acute hepatitis which is characterized by fever, fatigue, jaundice , and upper abdominal quadrant pain. Patients with autoimmune hepatitis usually have other autoimmune diseases like Hashimoto’s thyroiditis in this patient. Laboratory studies in autoimmune hepatitis show elevated liver enzymes (ALT and AST) and positive antismooth muscle antibody (SMA), antinuclear antibody (ANA), liver/kidney microsomal antibodies (LKM), and antibodies to soluble liver antigen (anti-SLA). Antismooth muscle antibody is most specific test for autoimmune hepatitis .
Other explanations: A Primary biliary cholangitis Show explanation 10% Primary biliary cholangitis ( primary biliary cirrhosis ) is an autoimmune destruction of bile ducts that finally leads to biliary cirrhosis . It is characterized by fatigue, pruritus, and right upper quadrant discomfort . Liver enzymes are high, especially ALP and GGT indicating biliary stasis. Antimitochondrial antibody is very specific for this disease. B Acute hepatitis C infection Show explanation 3% The majority of acute hepatitis C infections are asymptomatic. When symptomatic, it has the same symptoms like that mentioned in acute hepatitis B . Laboratory abnormalities in acute hepatitis C include high liver enzymes , positive HCVRNA , and anti-HCV antibody which takes several months to be positive after the primary infection. C Chronic hepatitis B infection Show explanation 8% Chronic hepatitis B patients may be asymptomatic. During replicative state, patients may have fatigue, anorexia, nausea , or upper abdominal pain . Laboratory studies show low albumin , prolonged prothrombin time , high ALT, AST, and bilirubin . In chronic hepatitis B , HBsAg and anti-HBc are positive while anti-HBs are negative. D Autoimmune hepatitis Show explanation 70% E Acute hepatitis B infection Show explanation 9% The majority of acute hepatitis B infections are asymptomatic. When symptomatic, it is characterized by anorexia, nausea , vomiting, fever, jaundice , and right upper abdominal quadrant pain. Abnormal laboratory results include high liver enzymes , positive HBsAg , anti-HBc IgM antibody, and Hepatitis B -e antigen (anti-HBe). Positive anti-HBs only indicates previous immunization .
