1 Chapter 13. The Respiratory SystemThe LungsThe Pleura

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Stem cell windpipe gives Korean toddler new life2013-05-01 21:02Hannah Warren, a 2-year-old born without a windpipe, has been given a new life after a groundbreaking transplant in the U.S. of the organ made from her own stem cells.

An international team of surgeons performed the landmark operation at the Children’s Hospital of Illinois at Saint Francis Medical Center in Peoria, Illinois, on April 9 to help the Korean toddler breathe on her own. Early signs show that the stem cell windpipe is working, doctors said, although she is still in the recovery process and relies on a ventilator. She will be able to return home soon with her family and have a normal life, they added.

“I am very happy that Hannah is doing well and is recovering from this very complex operation,” Dr. Paolo Macchiarini, the surgeon who led Hannah’s operation, told The Korea Herald via email.

The hospital said she was the youngest person in the world to receive the treatment. The stem cells were extracted from Hannah’s bone marrow and were seeded, with nonabsorbant nanofibers, onto a plastic frame. It took less than a week for them to grow into a new windpipe, or trachea, doctors said.

Now with the windpipe made from her own stem cells, she is expected to breathe, eat, drink and swallow outside of the hospital

Darryl Warren and Lee Young-mi visit with their 2-year-old daughter Hannah Warren in a post-op room at Children’s Hospital of Illinois in Peoria after having received a new windpipe in a landmark transplant operation April 9. Hannah was born in South Korea without a windpipe but received a new one made from her own stem cells. (AP-Yonhap

Born in Goyang, Gyeonggi Province, in 2010 to a Korean mother and a Canadian father, the girl never left the hospital in Korea before her operation in the U.S. She has been breathing through an artificial windpipe connected to her

Without the tube that allows for air to be ventilated into her lungs, she could have died at any moment. Her story was

Without the tube that allows for air to be ventilated into her lungs, she could have died at any moment. Her story was first published in The Korea Herald on June 29, 2011. The newspaper also interviewed her parents at a hospital in Seoul in July last year while Hannah was waiting for this life-saving operation in the United States.

The operation was possible thanks to efforts initiated by an American doctor, Mark Holterman, who currently leads the pediatrics division at the University of Illinois Medical Center. The doctor first met Hannah two years ago through a Korean-American nurse who suggested the case to him.

Determined to save her life, he teamed up with Dr. Macchiarini, who performed the world’s first stem cell-based tracheal transplant surgery in November 2008, and arranged for the family to have the operation at his hospital.

The operation was funded by donors, hospitals and medical foundations.

Her parents, Darryl and Young-mi, hope to bring Hannah home for the first time in her life soon. Ever since she was born in Korea, she had been at the Seoul University Children’s Hospital. Her parents were not allowed to stay overnight at the hospital but instead watched her grow during visiting hours.

“It’s been a long journey for her. We are so blessed that we’re able to get this unbelievable opportunity. She really had only one chance and she has it,” Darryl Warren, Hannah’s father, said at a tearful news conference at the hospital on

About 1 in 50,000 children worldwide are born without a trachea, according to reports. Most children with her condition, tracheal agenesis, usually don’t survive long after birth.

However, her successful transplant using the stem-cell technique will open a new door for other children with birth defects and diseases, doctors and scientists say.

The bioengineered organ, created by the latest stem-cell technology, has eliminated the chances of one’s immune system rejecting the transplant.

“This opens us a whole new area where we could fix a lot of our children with birth defects that we don’t have a good surgical solution for,” Holterman said at the conference.

Because Hannah’s new trachea was made from stem cells that are not derived from human embryos, there was no

Because Hannah’s new trachea was made from stem cells that are not derived from human embryos, there was no ethical issue involved, he added.

Hannah now has a 7-centimeter-long and 11-millimeter-wide windpipe transplanted into her body that will soon allow her to breathe normally and also to speak. Doctors said her voice box has started to work and she will be able to speak with help from speech therapists.

“She could not only have a life saved but a wonderful life opportunity to speak for the first time,” said Macchiarini, an Italian surgeon who has been leading in the regenerative medicine technology at Karolinska Institute in Stockholm.

She is likely to have another operation in the next five years as her body grows, he added.

By Cho Chung-un (christory@heraldcorp.com)4567 1. Congenital Anomalies

Pulmonary Hypoplasia (폐형성저하증)Cystic Adenomatoid Malformation (낭성선종양기형)Sequestration (분리증): not connected to airway

8 2. Diseases of the Bronchi and Bronchioles (기관지 및 세기관지 질환)Airway infections tend to affect deeper bronchioles → Bronchiolitis (세기관지염): often caused by influenza, adenovirus, measles, bordetella pertussis,Bronchocentric granulomatosis 기관지중심육아종증: due to asthma, tuberculosis...Constrictive Bronchiolitis 협착세기관지염Bronchial Obstruction 기관지폐쇄 → Atelectasis 무기폐, 폐확장부전Bronchiectasis 기관지확장증: Dilation of bronchus due to destruction of bronchial walls muscle and elastic elements

9 3. Bacterial Infections: PneumoniaPneumonia vs. Pneumonitis

Bronchiectasis 기관지확장증: Dilation of bronchus due to destruction of bronchial walls muscle and elastic elements

9 3. Bacterial Infections: PneumoniaPneumonia vs. Pneumonitis Any bacteria can cause pneumonia !! Streptococcus pneumoniae (pneumococcus 폐렴구균), Klebsiella pneumoniae 폐렴막대균Staphylococcus aureus 포도상 구균Legionella 레지오넬라...

Lobar vs. bronchopneumoniaconsolidation 응고화, 경화hepatizaton 간화Treatment: antibioticsAlthough pneumonia is one of the most common causes of death, it usually does NOT occur in healthy people spontaneously3. Bacterial Infections: Pneumonialobar pneumonia vs. bronchopenumoniaconsolidation 경화 of an entire lobe or scattered foci.But these terms have little clinical relevance today.

Pneumococcal PneumoniaBy Streptococcus penumoniae (penumococcus), which commonly causes lobar pneumonia 엽허파염 (대엽성)

101112 3. Bacterial Infections

Mycoplasma Pneumoniae (폐렴미코플라스마) : atypical pneumoniaTuberculosis (결핵): Mycobaterium tuberculosisFungal Infections (진균감염증)Viral Infectionsinitially affect the alveolar epithelium and result in a mononuclear infiltrate in the interstitium 간질 of the lunge.g. cytomegalovirus, measles, varicella, herpes simplex, adenovirus...

initially affect the alveolar epithelium and result in a mononuclear infiltrate in the interstitium 간질 of the lunge.g. cytomegalovirus, measles, varicella, herpes simplex, adenovirus...

1314

*Tuberculosis (결핵)Mycobacterium tuberculosisThe HIV-infected, homeless, and malnourished persons in impoverished areas are highly susceptible. transmitted from person to person by aerosolized droplets. (coughing, sneezing, talking...)Tuberculosis infection → active tuberculosisInhaled M. tuberculosis is deposited in alveoli. The organisms are phagocytosed by alveolar macrophages but resist killing; due to the unusual structure and chemical composition of the mycobacterial cell wallCaseous granulomaTreatment: isoniazid, rifampicin

15 4. Lung Abscess 폐고름집a localized accumulation of pus accompanied by the destruction of pulmonary parenchymaoften caused by aspiration of anaerobic bacteria from the oropharynx.alcoholism !!cough, fever, the production of large amounts of foul-smelling sputum.

16 5. Diffuse Alveolar Damage (D.A.D, 광범위 폐포손상; Acute Respiratory Distress Syndrome, ARDS, 급성호흡곤란증후군)The common pathological endpoint of a large variety of pulmonary insults.

Causes:Too much oxygen, Shock, Aspiration of gastric contents, Drugs, Radiation, Paraquat (Herbicide)...

17 6. Diffuse Pulmonary Hemorrhage 광범위 폐출혈 증후군In virtually all of these disorders, a neutrophilic infiltrate of the alveolar wall (neutrophilic capillaritis, 호중성 모세관염) is present.

18 7. Obstructive Pulmonary Disease

In virtually all of these disorders, a neutrophilic infiltrate of the alveolar wall (neutrophilic capillaritis, 호중성 모세관염) is present.

18 7. Obstructive Pulmonary Disease폐쇄 폐병A disease that have an obstruction to air flow in the lungsForced expiratory volume is decreased !!1) Chronic bronchitis, 2) Emphysema, 3) Asthma

narrowed airways → increased resistance: chronic bronchitis, asthmaloss of elastic recoil → diminished pressure: emphysema

19 7.1 Chronic bronchitis (만성기관지염)90% of smokers, air pollution...an increase in size of the bronchial mucus-secreting apparatushyperplasia and hypertrophy of the mucous cells and an increased ratio of mucous to serous cells.Thickening of the bronchial wall, hyperplasia of goblet cells, ↑smooth muscle → bronchial hyperreactivity, squamous metaplasia

Productive Cough for many years, severe in the winter.emphysema, dyspnea, cyanosis, cor pulmonale, hypoxia, hypercpania...cyanosis + edema (due to cor pulmonale) → “blue bloater 청색 부풀이”

2021 7.2 Emphysema (폐기종)

a chronic lung disease characterized by enlargement of air spaces distal to the terminal bronchioles, with destruction of their walls but without fibrosis.Smoking is the major cause. interference of α1-antitrypsin (α1-AT).

Centrilobular (중심소엽성)Panacinar (전세엽성)Localized (Paraseptal) emphysema: bullae ( shown in image )End-stage lung diseaseNonproductive cough, Exertional dyspnea 운동성 호흡곤란, Tachypnea 빠른 호흡, "Pink puffers 홍색 헐떡이"

End-stage lung diseaseNonproductive cough, Exertional dyspnea 운동성 호흡곤란, Tachypnea 빠른 호흡, "Pink puffers 홍색 헐떡이"

222324 COPD patients25 7.3 Asthma 천식

“wheezing" 천명, dyspnea, cough.acute → status asthmaticus 천식지속상태bronchial hyperresponsiveness to an inflammatory reaction produced by diverse stimuli (allergen, drugs, cold, exercise, air pollution, emotional)Acute: Nonproductive cough, wheezes, ↑ respiratory rate, dyspnea,Chronic: Severe coughing, expectoration...Mucus hypersecretion (with plugging), lymphocytes/eosinophils, lumen narrowing, smooth muscle hypertrophyTreatment: β2-adrenoceptor agonists, corticosteroids...

2627

2829 8. Pneumoconioses (진폐증)

Pulmonary diseases caused by dust inhalation. Silicosis 규폐증: inhalation of silicon dioxide, strongly fibrogenic (fibrosis)Coal Workers’ Pneumoconiosis 탄광부 진폐증: coal-dust macules (반점) or nodules (결절) Asbestosis 석면침착증: diffuse interstitial fibrosis, → mesothelioma 중피종Berylliosis 베릴륨중독증

30

Asbestosis 석면침착증: diffuse interstitial fibrosis, → mesothelioma 중피종Berylliosis 베릴륨중독증

3031 9. Interstitial Lung Disease 간질성 폐질환

a response to inhaled antigensFarmer's lung 농부폐, bagassosis 사탕수수깍지증, maple bark-stripper's disease 단풍나무 껍질 벗기는 사람의 폐질환, bird fancier's lung...a combination of immune-complex-mediated (Type III) and cell-mediated (Type IV)

32 9.2 Sarcoidosis (사르코이드증)Black > White (North America), ↓ Africaa disease in which abnormal collections of chronic inflammatory cells (granulomas) form as nodules in multiple organs, especially in the lung.Cause is unknown; but, maybe exaggerated T-lymphocyte responsemultiple sarcoid granuloma in the interstitium: Asteroid bodies, Schaumann bodies

33 9.3 Usual Interstitial Pneumonia (UIP, 보통유형의 간질 폐렴) clinically called idiopathic pulmonary fibrosis 특발성 폐섬유증the most common types of interstitial pneumoniapatchy chronic inflammation, interstitial fibrosiscause: viral, genetic, immunologic factor

9.4 Organizing pneumonia pattern (기질화폐렴 유형)= bronchiolitis obliterans = cryptogenic organizing pneumoniaLoose fibrosis in the airway

34 10. Pulmonary Hypertension 폐동맥고혈압The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation.When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary

The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation.When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary Pulmonary hypertension results from constriction, or stiffening, of the pulmonary arteries.Consequently, it becomes more difficult for the heart to pump blood forward through the lungs. → cor pulmonale 폐심장35 11. Carcinoma of the Lung 폐암종the most common cause of cancer death in both men and women.85% of lung cancers occur in smokersThe peak age for lung cancer is between 60 and 70 years, and most patients are between 50 and 80 years old.

Smoking, Oncogenes (Kras, Myc, Bcl-2...), Tumor Suppressor Gene (p53, Rb)…There are only TWO types of lung carcinoma

: SMALL CELL CARCINOMA vs. NON-SMALL CELL CARCINOMASquamous cell carcinomaAdenocarcinoma - BronchioloalveolarLarge cell carcinoma

36 11.1 Non-small cell carcinoma 비소세포암(1) Squamous cell carcinoma 편평세포암종30% of lung cancersInjury (smoking) → Metaplasia → Dysplasia → Carcinoma in situ → Carcinoma.Arise from the center of the lung.

37 11.1 Non-small cell carcinoma 비소세포암(2) Adenocarcinoma 샘암종1/3 (33%), arise in the periphery 주변부aninar 세엽세포암종) papilary 유두암종, solid with mucus formation 점막형성을 하는 충실성 종양, bronchioloalveolar 세기관지 폐포성 암종bronchioloalveolar (1~5%) 세기관지 폐포암종: grows along pre-existing alveolar walls

3839 11.1 Non-small cell carcinoma 비소세포암

(3) Large Cell Carcinoma 대세포암종 (10%)

39 11.1 Non-small cell carcinoma 비소세포암(3) Large Cell Carcinoma 대세포암종 (10%)

Tumor that are not squamous, glandular (adenocarcinoma), nor a small cell carcinoma40 11.2 Small cell carcinoma 소세포암

a highly malignant epithelial tumor of the lung that exhibits neuroendocrine features.20%, smoking, M > F (2:1)The tumor grows and metastasizes rapidly, and 70% of patients are first seen in an advanced stage.A variety of paraneoplastic syndromes: diabetes insipidus, ectopic ACTH syndrome, Eaton-Lambert (myasthenic) syndromeUnlike other lung cancers, small cell carcinomas show marked sensitivity to chemotherapy.

41Carcinoid tumors: 1~2%Pulmonary Metastases are More Common than Primary Lung Tumors

42 -The Pleura-14. Pneumothorax 공기가슴증, 기흉the presence of air in the pleural cavitySpontaneous 자연성 or Tension 긴장성 pneumothorax

43 15. Pleural Effusion (흉막삼출)the accumulation of excess fluid in the pleural cavity.Hydrothorax 물가슴증 (water), Pyothorax 고름가슴증 (PMN), Empyema 고름집 (pus), Hemothorax 혈액가슴증, Chylothorax 암죽가슴증 (milky fluid, lymphatic obstruction).

Mesothelium: a membrane of visceral and parietal → Mesotheliomaa biphasic appearance, with epithelial and sarcomatous patternsTreatment is largely ineffective, and prognosis is poor. Few patients survive longer than 18 months after diagnosis.

44

1 Chapter 14. The Gastrointestinal TractThe EshophagusThe StomachThe Small IntestineThe Large IntestineThe Appendix

2 The Esophagus3 1. Congenital Disorder

Tracheoesophageal Fistula (기관식도누출관)Frequency: A > B > C

Rings, Webs → Dysphagia (삼킴곤란/연하곤란)Plummer-Vinson (Paterson-Kelly) Syndrome: ① a cervical esophageal web② mucosal lesions of mouth, pharynx ③ iron-deficiency anemiaSchiazki ring (하부식도점막성환): usually asymptomatic

Esophageal Diverticula (식도게실/곁주머니)Zenker diverticula 젠커씨 게실: uncommon, M > F, regurgitationTraction diverticula 견인게실: in the midportion of the esophagus, asymptomaticEpiphrenic diverticula 가로막위게실: located immediately above the diaphragm. Reflux esophagitis. Young persons

4 2. Motor Disorders 운동장애Dysphagia (연하곤란): difficulty in swallowingOdynophagia (연하통): pain on swallowing, Achalasia (이완불능증)failure of the lower esophageal sphincter to relax in response to swallowing and the absence of peristalsis in the body of the esophagus.once called as a cardiospasm 심장연축Chagas disease

Scleroderma 피부굳음증, 피부경화증 (progressive systemic sclerosis 4장에서 언급 ) → Fibrosis of esophagus56 3. Hiatal Hernia (틈새탈장)

6 3. Hiatal Hernia (틈새탈장)a herniation of the stomach through an enlarged esophageal hiatus in the diaphragm.Sliding hernia 활주탈장Paraesophageal hernia 식도곁탈장

7 4. Esophagitis 식도염Reflux esophagitis 역류성 식도염 - “Heartburn”from regurgitation of gastric contentsalcohol, chocolate, fatty foods, smoking → ↑reflux

CNS depressants, pregnancy, estrogen therapy, nasogastric tube → ↑refluxhyperemia, (mucosal erosion, ulcers) → vertical streaks, cell swelling

Barrett esophagus (바레트식도)a result of chronic gastroesophageal reflux.replacement (metaplasia) of esophageal squamous epithelium by columnar "specialized epithelium”↑esophageal adenocarcinoma

Infective esophagitisimmunocompromised patientscandidia, herpetic (herpes simplex type I) ...

8 5. Esophageal Varices 식도정맥류dilated veins immediately beneath the mucosa that are prone to rupture and hemorrhagevirtually always in patients with cirrhosis and portal hypertension.

9 6. Neoplasms 신생물Esophageal carcinoma 식도암종Geographic variationSmoking, excessive alcohol consumption, (Diets low in fruits, vegetables, animal proteins, trace metals), chronic esophagitis, esophageal stricture after ingestion of lye...lower third (50%), 3 types (ulcerating, polyploid, infiltrating)dysphagia (too late)

Adenocarcinoma of the esophagus 식도 샘암종Virtually all adenocarcinomas arise in the background of Barrett esophagus

10

Virtually all adenocarcinomas arise in the background of Barrett esophagus1011 The Stomach121314 1. Congenital Disorders: Congenital Pyloric Stenosis 선천 유문 협착증

concentric enlargement of the pyloric sphincter and narrowing of the pyloric canal that obstructs the gastric outlet.The cause of pyloric stenosis is not known. The main symptom is vomiting after feedings. Can be corrected effectively with surgery.

15 2. Gastritis 위염1) Acute hemorragic gastritis (급성출혈위염): mucosal necrosisAny serious illness, burned persons (stress ulcers and erosions, curling ulcer), Drugs: aspirin, NSAIDs, excess alcohol, ischemic injuryCNS trauma, Shock, Sepsis, Hypersecretion of gastric acid...

16 2. Gastritis (위염)2) Chronic gastritis (만성 위염) - it's autoimmune or environmentAutoimmune Atrophic Gastritis 자가면역 위축 위염diffuse inflammatory disease of the stomach restricted to the body and fundus.Pernicious anemia (악성 빈혈) → a complication of autoimmune gastritis.

Multifocal Atrophic Gastritis 다병소성 위축 위염 (Environmental Metaplastic Atrophic Gastritis 환경적 이형성 위축 위염): typically involves the antrum and adjacent areas of the body.Atrophic Gastritis and Stomach Cancer → Persons with autoimmune or multifocal atrophic gastritis have an elevated risk of carcinoma of the stomach.Helicobacter pylori Gastritis: a chronic inflammatory disease of the antrum and body of the stomach caused by H. pylori

17 Pernicious anemia 악성 빈혈 ?caused by a lack of vitamin B12. Pernicious anaemia develops when the body becomes unable to absorb vitamin B12 properly from food. Normally a protein known as intrinsic factor, which is released from the parietal cell in the stomach, attaches to vitamin B12 and carries through the intestinal wall into the blood stream.However in pernicious anaemia, the parietal cells that produce intrinsic factor become damaged, vitamin B12 is no

Normally a protein known as intrinsic factor, which is released from the parietal cell in the stomach, attaches to vitamin B12 and carries through the intestinal wall into the blood stream.However in pernicious anaemia, the parietal cells that produce intrinsic factor become damaged, vitamin B12 is no longer absorbed and a deficiency develops, leading to anaemia.Such damage can occur as the result of an autoimmune disorder,

This vitamin is vital for the manufacture of new red blood cells. When it's in short supply, red blood cells are produced in smaller numbers, are abnormally large in size (megaloblastic) and don't last as long as they should.

18 3. Peptic Ulcer Disease 소화궤양질환Erosion in the lining of the stomach or the duodenumPeptic ulcer disease = gastric ulcer + duodenal ulcerIncreased gastric secretion of hydrochloric acid (HCl)Risk factorsEnvironmental factors: Diet, Drugs (Aspirin, NSAIDs), SmokingGenetic factors: blood group antigen (30% higher in type O), ↑[pepsinogen 1]Physiologic factors• Duodenal: ↑ gastric emptying, ↓pH of duodenal bulb, impaired mucosal bulb

H. pylori is isolated from the gastric antrum of virtually all patients with duodenal ulcers.Symptoms: Abdominal pain, hemorrage, perforation,

1920

Gastric ulcer vs. Duodenum Hemorrage, Perforation

21 4. Benign Neoplasms 양성 신생물Gastrointestinal stromal tumors (GISTs) 기질종양Cajal cells, non-aggressiveTumor cells express c-kit (CD117) oncogene

Epithelial Polyps 상피 용종Hyperplastic polyps 과다형성 용종→ no malignant potential !!

Epithelial Polyps 상피 용종Hyperplastic polyps 과다형성 용종→ no malignant potential !!Tubular adenomas (adenomatous polyps) 샘종용종: most common in antrum, can be malignantFundic gland polyps 위저샘 용종: mostly seen in patients treated with proton pump inhibitors.: not considered preneoplastic, no increased risk of gastric carcinoma

22 5. Malignant Tumors 악성종양Gastric adenocarcinoma (95%)CausesDietary factors: smoked meat and fish (benzopyrene), nitrosaminesAge: > 50Male > FemaleH. Pylori

Early gastric cancer: a tumor limited to mucosa or submucosaAdvanced gastric cancer: Polypoid adenocarcinoma Ulcerating adenocarcinoma Diffuse or infiltrating adenocarcinoma (linitis plastisca)

23 5. Malignant Tumors24 Signet ring cell 반지세포

Signet = 도장, 옥새Signet ring = 인형(印形)이 박힌 반지

Signet ring cell: a tumor cell that contain mucin. Thus, nucleus is moved to the periphery, resulting in a ring shape.

252627 The Small Intestine

2627 The Small Intestine2829 1. Congenital Disorders

Atresia (폐쇄)a complete occlusion of the intestinal lumen

Stenosis (협착) an incomplete stricture, which narrows the lumen.

Meckel Diverticulum (메켈게실)a small bulge in the small intestine present at birth.caused by persistence of the vitelline duct (난황관)hemorrhage, perforation, obstruction and diverticulitis

Meconium Ileus (태변장폐색증)intestinal obstruction in cystic fibrosis 낭성섬유증 is caused by the accumulation of menocium in the small intestine, due to high viscosity

30 2. Infections (소장감염증)Bacterial Diarrhea 세균성 설사particularly lethal in infants living in under-developed countries.increased intestinal secretion, stimulated by bacterial toxins and enteric hormones.Vibrio cholerae, Escherichia coli...

Viral gastroentoeritis Rotavirusmost common, accounts for about half of acute diarrhea in hospitalized children younger than 2 years of age.

Norwalk VirusNorovirus

3132 3. Vascular Diseases (소장혈관병)

Acute Intestinal Ischemia (급성장허혈)by superior mesenteric artery occlusion 위장간막동맥폐색:thrombosis, embolization...

by superior mesenteric artery occlusion 위장간막동맥폐색:thrombosis, embolization...abrupt onset of abdominal pain, bloody diarrhea, hematemesis, shock, perforation, infarction...

Chronic Intestinal Ischemia (만성장허혈)recurrent intestinal (abdominal) angina 재발성 복통 협심증angina = pain !!

33 Hemoptysis (각혈, 객혈) vs. Hematemesis (토혈)Hemoptysis: from the Lung !!Hematemesis: from the GI tract !!

34 4. Malabsorption흡수장애

35 4. Malabsorption흡수장애Lactase deficiency 젖당분해효소결핍: Intolerance to milk products

Celiac disease 만성소화장애증= celiac sprue = gluten-sensitive enteropathyImmune response to gluten in the diet.Malabsorption, growth retardation → treatment with gluten-free diet

36 4. Malabsorption흡수장애Whipple Disease 휘플 병White men in their 30s and 40s are most affected. rare.infiltration of the small-bowel mucosa by macrophages packed with small, rod-shaped bacilli, Tropheryma Villi are flat and thickened, infiltrated with large foamy macrophages → Malabsorption, fever,

37 5. Mechanical Obstruction (기계적 폐쇄)Intussusception 장중첩증: a segment of bowel (intussusceptum 감입부) protrudes distally into a surrounding outer portion (intussuscipiens 감입Volvulus 장염전

Intussusception 장중첩증: a segment of bowel (intussusceptum 감입부) protrudes distally into a surrounding outer portion (intussuscipiens 감입Volvulus 장염전: a segment of gut twists on its mesentery, kinking the bowel and usually interrupting its blood supply.Adhesions 유착: Fibrous scars caused by previous surgery or peritonitis cause obstruction by kinking or angulating the bowel or directly compressing the lumen.Hernias 탈장: Loops of small bowel may be incarcerated in an inguinal or femoral hernia

38 6. Neoplasms: <5% of GI tumorsBenignAdenomasPeutz-Jeghers Syndrome: autosomal dominant (mutation of LKB1) → polyps, melanin pigmentation,

Malignant - rare!!AdenocarcinomaPrimary Intestinal LymphomaCarcinoid (neuroendocrine) tumor

39 The Large Intestine4041 1. Congenital Disorders

Congenital (aganglionic) Megacolon (Hirschsprung Disease) 선천거대대장증: absence of ganglion cells → rectum permanently contracted→ delayed passage of meconium, vomiting, enterocolitis

Anorectal Malformations: agenesis, stenosis, imperforate anus, fistula...

42 2. Infections 대장감염증Pseudomembranous Colitis 위막성대장염: an inflammatory disease of the colon that is characterized by exudative plaques 삼출성 반점 on the mucosa.

Pseudomembranous Colitis 위막성대장염: an inflammatory disease of the colon that is characterized by exudative plaques 삼출성 반점 on the mucosa.→ Clostridium difficile produces toxins. not invasivethe disease is associated with antibiotics therapy→ antibiotics kills good bacteria in colon, but not bad bacteria such as C. diificile

Neonatal Necrotizing Enterocolitis 신생아괴사소장대장염the most common acquired surgical emergenciesparticularly common in premature infants after oral feedingabdominal discoloration with intestinal perforation and peritonitis

43http://www.theverge.com/2013/5/24/4362050/fda-cracks-down-on-fecal-microbiota-transplants-c-difficile

44 3. Diverticular Disease 게실/곁주머니병(1) Diverticulosis 게실/곁주머니증: an acquired herniation (diverticulum) of the mucosa and submucosa through the muscular layers of the colon.cause: environmental factors (↑meat, ↓fiber)mostly asymptomatic

(2) Diverticulitis 게실/곁주머니염: Inflammation at the base of a diverticulum. Caused by retained fecal material.10% to 20% of patients with diverticulosisPersistent lower abdominal pain, feverMost patients have tenderness in the left lower quadrant

45 4. Inflammatory Bowel Disease 염증성창자병= Crohn Disease + Ulcerative Colitis(1) Crohn Disease 크론병occurs principally in the distal small intestinetransmural inflammation discontinuous, right colon preferencenoncaseating granulomas, mostly in the submucosaabdominal pain, diarrhea, fever, malabsorption, fistula, ↑cancer…

4647 4. Inflammatory Bowel Disease

4647 4. Inflammatory Bowel Disease

(2) Ulcerative Colitis 궤양성 대장염a diffuse disease, continuousgenerally limited to the colon and rectum. essentially a mucosal disease: deeper layers are uncommonly involved rectal bleeding, bloody stools, pain, fever, anemia...People with long-standing ulcerative colitis have a higher risk of colorectal cancer than the general population.

4849 5. Vascular Diseases 혈관병

Angiodysplasia 혈관형성이상 (Vascular Ectasia 혈관확장증)localized arteriovenous malformations, mainly in the cecum and ascending colon, which produce lower intestinal bleeding.

Hemorrhoids 치질Western, pregnancy: ↑bleeding, may lead to iron-deficiency anemia thrombosis of external hemorrhoids is painful

50 6. Polyps 폴립, 용종a mass that protrudes into the lumen of the gut.polyps are only infrequently symptomatictheir clinical importance lies in their potential for malignant transformation.(1) Adenomatous Polyps 샘종폴립Tubular/Villous/Tubulovillous adenomasSessile vs. Pedunculated

51525354 6. Polyps

(2) Hyperplastic Polyps 증식 폴립: Frequent in the Rectum

(2) Hyperplastic Polyps 증식 폴립: Frequent in the Rectum(3) Familial Adenomatous Polyposis 가족성 샘종성 폴립증 : = adenomatous poluposis coli (APC) 샘종성 결장 폴립증 : 1% of colorectal cancers: mutation of APC gene (5q21-22)(4) Non-Neoplastic Polyps 비신생물성 폴립Juvenile Polyps 소아성 폴립 (Retention Polyps): < 10 years of ageInflammatory Polyps 염증성 폴립

55 7. Malignant Tumors: (1) Adenocarcinoma 샘암종Colon vs. RectumRisk factorsage (>50), chronic inflammatory bowel disease, diet, genetic factors

Symptomsoccult and bright red blood in the feces, obstruction, pain, perforation (peritonitis)…right side of colon (often cecum), lumen is large and fecal contents are liquid → tumor grows without causing obstruction → asymptomatic bleeding → iron-deficiency anemia

Surgery !!56 7. Malignant Tumors

: (2) Hereditary Nonpolyposis Colon Cancer (HNPCC)= Warthin-Lynch syndromeautosomal dominant inherited disease (3~5%)young age, mutation in hMSH2 and hMLH1

penetrates directly into surrounding tissues57 The Appendix58 1. Appendicitis 충수돌기염

an inflammatory disease of the wall of the vermiform appendix 충수돌기 that leads to transmural inflammation and perforation with peritonitis.the most common disease of the appendix, and is the most frequent cause of an abdominal emergency.Acute appendicitis relates to obstruction of its orifice, with secondary distention of the lumen and bacterial invasion

the most common disease of the appendix, and is the most frequent cause of an abdominal emergency.Acute appendicitis relates to obstruction of its orifice, with secondary distention of the lumen and bacterial invasion of the wall.fecalith, abscess, perforationSurgery!!2. Mucocele (점액낭종)a dilated mucus-filled appendixnon-neoplastic (chronic obstruction) or neoplastic

Carcinoid tumors: common, unlikely to metastasize