IPPF very rare interstitial peumonia

Idiopathic pleuroparenchymal fibroelastosis

Clinical Features:

Patient 1 was a 65-year-old woman with “incidental” pleural thickening seen on a chest radiograph 10 years prior to referral. Four years prior to referral, she underwent a modified radical mastectomy for adenocarcinoma of the breast and was treated with adjuvant chemotherapy of cyclophosphamide, methotrexate, and fluorouracil (CMF) without radiation therapy. The patient was treated with daily doses of tamoxifen, and she returned to her baseline function of aerobic exercise 60 min daily. Eight months prior to referral, dyspnea on exertion and a nonproductive cough developed. Chest radiography revealed a new right pleural effusion and progressive, pleural thickening (greater in the right lung than in the left) when compared to prior radiographs. Pleural fluid was negative for malignancy. A diagnostic video-assisted thorascopic lung biopsy was performed, and at surgery a complex pleural process with extensive fibrosis requiring decortication was found. Physical examination findings were significant for tachypnea, tachycardia, diffusely decreased breath sounds, and decreased diaphragmatic excursion. Extensive evaluation was negative for pneumoconiosis, autoimmune disease, and HLA-B27–related disease.

Patient 2 was a 52-year-old man in whom exertional breathlessness developed following an upper respiratory tract infection. A chest radiograph revealed bilateral pleural thickening. One year later, he underwent right-sided pleural decortication with symptomatic improvement. However, several months later, progressive dyspnea again developed. Ultimately, the patient underwent left pleural decortication. The patient returned to work but remained dyspneic with a productive cough. He was then referred to our institution. His medical history was significant for Charcot-Marie-Tooth disease (hereditary motor sensory neuropathy), gastroesophageal reflux, and narcolepsy. A sister had sarcoidosis, and he had previously worked in both construction and agriculture, although a direct exposure to asbestos was absent. On examination, he was mildly tachycardic and tachypneic. Fine mid-late inspiratory rales and distant breath sounds were noted. Autoimmune serology findings were negative.

Patient 3 was a 61-year-old woman who presented with chronic cough. Diagnosed with asthma in her 20s, she had experienced multiple episodes of “bronchitis” and “pneumonia” over her lifetime. At the time of referral, the cough was accompanied by wheezing, dyspnea, and chest tightness that occurred on a daily basis. Her medical history was significant for breast cancer that had been diagnosed and treated 10 years previously with a left modified radical mastectomy, chemotherapy with CMF, and radiation therapy. She had experienced no significant environmental or occupational exposures. Examination findings noted mild tachypnea and tachycardia with fine mid-late inspiratory rales at the left base, and mild-moderate kyphoscoliosis, and a mid-systolic click found during her cardiologic examination that was consistent with mitral valve prolapse. Bronchoscopy, routine laboratory examination findings, autoimmune serology results, cystic fibrosis genotyping results, and α1-antitrypsin levels were all unrevealing. Prior chest radiographs and chest CT scans had revealed biapical pleural thickening 5 years earlier.

Patient 4 reported reduced exercise tolerance as a teenager. At age 32 years, she underwent a pulmonary evaluation following a diagnosis of pulmonary fibrosis in her twin sister. Her pulmonary physiology was restrictive, but as she was asymptomatic no further evaluation was performed. Seven years later, cough and pleuritic chest pain developed. An examination revealed a loud pleural rub, and she was treated with antibiotics. Over the next year, recurrent pneumonias developed and she was referred to our institution. The patient was a life-long nonsmoker without occupational or environmental exposures. Family history was significant for a twin sister who died of progressive pleuroparenchymal fibrosis (ie, patient 5) and a grandfather with rheumatoid arthritis. An examination revealed mild tachypnea and tachycardia, a pleural rub at the right base, and rales at the left base. Laboratory examination findings were notable for an erythrocyte sedimentation rate of 47 mm/h but negative autoimmune serology results.

Patient 5, the twin sister of patient 4, presented in 1987 at age 32 years with right upper lobe pneumonia. Chest radiographs revealed progressive upper lobe volume loss and bilateral pleural thickening. Her pulmonary physiology was restrictive. The patient was a nonsmoker and worked as a nurse. Her anti-nuclear antibody titer was 1:80, and rheumatoid factor titer was 1:160 without other evidence of a connective tissue disease. An evaluation for HLA-B27–associated disease was negative. The patient underwent surgical lung biopsy that showed pleural fibrosis and patchy interstitial fibrosis. The patient was treated with steroids without improvement. She died of progressive respiratory failure 5 years after undergoing surgical lung biopsy. An autopsy revealed marked pleural and interstitial fibrosis that was identical to that found in her twin sister. In summary, we have reported five cases of a unique idiopathic pleuroparenchymal lung disease that is characterized by upper lobe radiographic predominance and pathologic findings that do not fit with any of the currently defined interstitial pneumonias. We term this disorder idiopathic pleuroparenchymal fibroelastosis.

cite from http://www.ncbi.nlm.nih.gov/pubmed/23169023

Case 1 chest CT: biapical fibrotic changes, pleural and parenchymal densities in the right upper lobe as well as mild emphysematic changes.

Case 2 pathology: hematoxylin-eosin stain showing abundance of elastic fibers in addition to collagen fibers and abrupt transition from fibroelastosis to unaffected normal lung parenchyma. Insert: slide 'thumbnail' view demonstrating an extraordinarily thick pleural cap with sparing of the adjacent lung parenchyma.

http://www.nature.com/modpathol/journal/v21/n6/full/modpathol200856a.html

gastroenterology ob 6

Esophagitis dissecans superficialis

Am J Surg Pathol. 2009 Dec;33(12):1789-94. doi: 10.1097/PAS.0b013e3181b7ce21.

Esophagitis dissecans superficialis ("sloughing esophagitis"): a clinicopathologic study of 12 cases.

Carmack SW, Vemulapalli R, Spechler SJ, Genta RM.

Source

Department of Pathology, VA North Texas Health Care System, The University of Texas Southwestern Medical Center, Dallas, USA.

Abstract

Esophagitis dissecans superficialis (EDS) is a term applied to a rare endoscopic finding characterized by sloughing of large fragments of the esophageal squamous mucosa that may be coughed up or vomited. Although EDS has been reported in association with certain medications and esophageal strictures, most cases remain unexplained and the histopathologic features of EDS are inadequately described. We undertook this study to define useful diagnostic criteria based on the examination of a series of well-characterized cases of EDS. To identify patients with EDS, we searched our endoscopy and pathology databases, reviewed the esophageal biopsy specimens from candidate cases, and correlated them with pertinent clinical information. Twelve patients (11 men and 1 woman) had endoscopic and histologic findings of EDS and 9 had the histologic features without the endoscopic correlates. Biopsies from confirmed EDS patients showed sloughing and flaking of superficial squamous epithelium with occasional bullous separation of the layers, parakeratosis, and varying degrees of acute or chronic inflammation. Fungal elements were identified in 3 patients, but were not associated with acute inflammation. None of the EDS patients were on bisphosphonate therapy or had bullous skin disorders. Follow-up endoscopy in 5 patients showed complete resolution of the esophageal abnormalities in 4 and mild esophagitis in one. In spite of its sometimes, dramatic presentation, EDS is a benign condition that resolves without lasting esophageal pathology. Although an association with medications, skin conditions, heavy smoking, and physical trauma has been reported, the pathogenesis of EDS remains unexplained.

Endoscopic view of esophagitis dissecans superficialis. A: With diffuse sloughing mucosa of the lower esophagus in a 76-year-old woman presenting hematemesis, and the cause was idiopathic; B: with longitudinal sloughing mucosa from upper to mid esophagus in a 67-year-old woman with mucocutaneous type pemphigus vulgaris, note fine whitish fragments of sloughed mucosa, and the index value for anti-desmoglein 3 antibody by enzyme-linked immunosorbent assay was over 1280 (normal value < 7).

PANCREATIC REST — A pancreatic rest (also known as ectopic pancreas, aberrant pancreas, and heterotopic pancreas) refers to ectopic pancreatic tissue. These rare submucosal tumors most commonly consist of cystically dilated exocrine cells. Endocrine pancreatic tissue or a combination of exocrine and endocrine cell types may also be seen.

Pancreatic rests are most frequently found in the distal stomach, duodenum, or proximal jejunum, but have also been reported within a Meckel's diverticulum, the gallbladder, bile ducts, and the minor and major papillae. They are typically discovered incidentally during endoscopy, surgery, or autopsy. They are also occasionally found on CT scan. CT findings that may help differentiate pancreatic rests from other submucosal lesions identified in one study included:

• A flat-ovoid shape (long diameter to short diameter ratio of greater than 1.4)
• Location of the lesion in the antrum, pylorus, or duodenum
• An endoluminal growth pattern
• An ill-defined border
• Prominent enhancement of the overlying mucosa

The study found that the presence of at least two of the above findings had a sensitivity of 100 percent and a specificity of 82.5 percent for diagnosing a pancreatic rest in the upper gastrointestinal tract [52]. The specificity increased to 100 percent if three of the above findings were present.

However, while pancreatic rests may be detected with CT scan, if a submucosal lesion is noted on upper endoscopy, we suggest endoscopic ultrasound with endoscopic mucosal resection for further evaluation, as small lesions may be missed on CT scan. (See 'General principals of tissue sampling' above.)

Complications of pancreatic rests are rare, but may include ulceration, gastric outlet obstruction, and malignancy [50,53].

Endoscopic appearance — A pancreatic rest appears as a submucosal nodule, usually with a central umbilication that corresponds to a draining duct.

Endosonographic findings — Pancreatic rests are hypoechoic or intermediate echogenic heterogeneous lesions with indistinct margins. They most commonly arise from the third or fourth layer, or a combination of the two layers of the GI tract. Anechoic areas within the lesion correlate with ductal structures.

Diagnosis and treatment — The diagnosis can be made histologically from tissue obtained by biopsy forceps or snare excision, although techniques to obtain deeper biopsies (using jumbo biopsy forceps, tunnel biopsy, endoscopic mucosal resection, or EUS-guided FNA) may be required. The management strategy should be guided by symptoms and suspicion for malignancy. Asymptomatic lesions can be followed expectantly. Endoscopic resection can be performed by standard snare, band ligation-assisted, or cap-assisted polypectomy technique. Surgical resection is preferred to endoscopic resection when the muscularis propria is involved.

Endoscopic photograph of a "dimpled" antral mass in an 8 yo found incidentally.

Endoscopic photograph of a "dimpled" antral mass in a 5 yo found incidentally

                                                                          Fundoplication

http://www.gastrointestinalatlas.com/English/Esophagus/Nissen_Fundoplication/nissen_fundoplication.html

The Upper Part of the Stomach in a Patient after Fundoplication

The Upper Part of the Stomach in a Patient after Fundoplication

gastroenterology clinic

1, patient has past history of crohn ileitis, come in for follow up, he still have 4 times/day loose stool, no blood, no abdominal pain, no nausea, vomiting, he is taking oral mesalamine. in the past, he suffered from medicine induced pancreatitis by taking AZA(this side effect is very rare). suggestion, keep low fiber easy digest diet, keep current regimen, if he feels any flare up, increase taking mesalamine and keep light diet, if it is not getting better, may need to start prednisone.

2, hepatitis C, genotype 3, current treatment, interferon alpha
patient do have complaint about shortness of breath, may be side effect from treatment
The goal of treatment is to eradicate HCV RNA, which is predicted by the achievement of a sustained virologic response (SVR) as defined by the absence of HCV RNA by polymerase chain reaction six months after stopping treatment. 

PATIENTS FOR WHOM THERAPY IS WIDELY ACCEPTED — Therapy is generally accepted for patients with all of the following characteristics:

• At least 18 years of age
• HCV RNA detectable in the serum
• Liver biopsy with chronic hepatitis and significant fibrosis (bridging fibrosis or higher)
• Compensated liver disease

• Total serum bilirubin <1.5 g/dL (25.7 micromol/L)
• INR <1.5
• Albumin >3.4 g/dL (34 g/L)
• Platelet count >75,000 cells/mm3 (75,000 x 10(6)/L)
• No evidence of hepatic encephalopathy or ascites

• Acceptable hematological and biochemical indices

• Hemoglobin >13 g/dL for men and >12 g/dL for women
• Neutrophil count >1500 cells/mm3 (1500 x 10(6)/L)
• Creatinine <1.5 mg/dL (133 micromol/L)

• Willing to be treated and to conform to treatment requirements
• No contraindications to treatment

In addition, the 2012 UK consensus guidelines recommend treatment with peginterferon, ribavirin, and a protease inhibitor (telaprevir or boceprevir) for most patients with genotype 1, including patients who are treatment naïve or who have failed prior therapy with peginterferon and ribavirin. The guidelines note that because of drug interactions, treatment of patients with HIV needs to be considered on a case-by-case basis. In addition, the guidelines do not currently recommend treatment in patients with decompensated liver disease, hepatitis B co-infection, or active cancer, or in patients who have undergone organ transplantation due to limited data.

PATIENTS IN WHOM THERAPY IS CONTRAINDICATED — Antiviral therapy with peginterferon and ribavirin for chronic HCV infection is contraindicated in patients who have one or more of the following:

• Major, uncontrolled depressive illness
• A kidney, heart, or lung transplant
• Autoimmune hepatitis or other conditions known to be exacerbated by interferon or ribavirin
• Untreated thyroid disease
• Severe concurrent disease such as severe hypertension, heart failure, significant coronary artery disease, poorly controlled diabetes, obstructive pulmonary disease
• Known hypersensitivity to drugs used to treat HCV

Therapy is also contraindicated in patients who are:

• Less than two years of age
• Pregnant, contemplating pregnancy (including men), or unwilling to assure contraception; ribavirin is pregnancy category X due to significant teratogenic and embryocidal effects, and interferon is pregnancy category C

INFLUENCE OF SIDE EFFECTS AND DRUG INTERACTION ON PATIENT SELECTION — Interferon monotherapy and combination therapy with ribavirinand telaprevir or boceprevir (for patients with genotype 1) are associated with several adverse effects. In some patients, treatment is contraindicated due to the risk of side effects, whereas in others, treatment should be undertaken with extreme caution. Conditions that may be complicated or worsened by side effects of treatment include significant anemia or leukopenia, pregnancy, severe depression or other psychiatric conditions, cardiac disease, poorly controlled diabetes, seizure disorders, and autoimmune or potentially immune-mediated diseases. In addition, treatment efficacy may be reduced with advancing age (particularly in those older than 60 years) because of an increased frequency of side effects requiring dose reduction.

Drug interactions are also an important consideration in patients with genotype 1 who are being considered for treatment with a telaprevir or boceprevir and should be taken into account prior to starting therapy (table 1 and table 2). (See "Treatment regimens for chronic hepatitis C virus genotype 1", section on 'Drug interactions with protease inhibitors' and "Treatment of hepatitis C virus infection in the HIV-infected patient", section on 'Potential drug interactions'.

Recurrence after liver transplantation — Recurrence of HCV occurs in more than 95 percent of patients after liver transplantation. Disease progression in this setting is more rapid, and complications are more frequent than in immunocompetent patients with HCV infection [14]. Disease progression correlates with HCV RNA levels at the time of transplantation, the age of the organ donor, and the degree of immunosuppression in the post-transplant period.

MONITORING VIRAL LOAD DURING THERAPY

An early virologic response (EVR) is defined as at least a 2 log₁₀ reduction in HCV RNA or HCV RNA negativity by week 12. A patient with a "complete EVR" has attained complete viral suppression by week 12; a patient with a "partial EVR" has achieved greater than a 2 log₁₀ decline in viremia but continues to have detectable HCV RNA. An SVR is unlikely in patients who lack an EVR, and it is generally recommended that treatment be stopped in patients who fail to achieve an EVR. 

We suggest that patients have their viral loads checked at baseline and at weeks 4, 12, and 24 of therapy to assess for a treatment response and to make decisions about continuing treatment or possibly altering the duration of treatment. Patients with an end of treatment response (negative HCV RNA at the completion of treatment) should also have a viral load checked 24 weeks after therapy is completed to assess for an SVR. An SVR is associated with a 99 percent chance of being HCV RNA negative during long-term follow-up

CHOICE OF PEGINTERFERON

meta-analyses suggest a slight advantage for peginterferon alfa-2a

We suggest treatment with peginterferon alfa-2a in patients with chronic HCV genotype 2, 3, or 4 rather than treatment with peginterferon alfa-2b.

DOSES OF PEGINTERFERON AND RIBAVIRIN — There are two peginterferon preparations used in the treatment of HCV (peginterferon alfa-2a and peginterferon alfa-2b). The doses of peginterferon differ for the two preparations:

• For peginterferon alfa-2a, the dose is 180 micrograms subcutaneously per week
• For peginterferon alfa-2b, the dose is 1.5 microgram/kg subcutaneously per week

Depending on the genotype being treated, ribavirin dosing may or may not be weight-based. Ribavirin is given in divided daily doses (typically twice per day). For genotypes 2 and 3, ribavirin dosing is not weight-based. Patients instead receive a standard dose of 800 mg (typically 400 mg twice daily).

For genotype 4, ribavirin is weight-based:

• For patients receiving peginterferon alfa-2a, the ribavirin dose is 1000 mg for patients who weigh 75 kg or less, or 1200 mg for those who weigh more than 75 kg.
• For patients receiving peginterferon alfa-2b, the ribavirin dose is 800 mg for patients weighing <65 kg, 1000 mg for 65 to 85 kg, 1200 mg for >85 to 105 kg, and 1400 mg for >105 kg

Treatment-naive patients — Patients with genotype 2, 3, or 4 should receive dual therapy with peginterferon and ribavirin. Protease inhibitors are not used in the treatment of patients with genotype 2, 3, or 4.

Genotype 2 or 3 — Patients with genotype 2 or 3 should receive treatment with peginterferon and ribavirin. Ribavirin is not weight-based for the treatment of genotypes 2 or 3. All patients receive 800 mg in daily divided doses (typically 400 mg twice daily). Treatment with peginterferon plus ribavirin should be administered for 24 weeks in patients with genotype 2 or 3. These patients generally have a better response to peginterferon and ribavirin than those with genotype 1 or 4.

Genotype 4 — Patients with genotype 4 should received treatment with peginterferon and weight-based ribavirin. Treatment with peginterferon plus weight-based ribavirin should be planned for 48 weeks

Relapsers and nonresponders 

Prior treatment with standard interferon monotherapy — In patients who relapse after an initial response to interferon monotherapy, we recommend combination therapy with peginterferon plus weight-based ribavirin.

Prior treatment with peginterferon and ribavirin — The treatment of patients who are nonresponders to peginterferon and ribavirin is unsettled. For those who failed treatment with a full course of peginterferon alfa-2a or alfa-2b plus ribavirin, we generally do not suggest retreatment with peginterferon and ribavirin.

SUMMARY AND RECOMMENDATIONS — The decision to treat a patient with chronic hepatitis C virus (HCV) infection is based upon several factors, including the natural history of the disease, the stage of fibrosis, and the efficacy and adverse effects related to therapy. In general, patients being considered for treatment should have histologic and virologic evidence of chronic HCV infection.

Genotype 2 or 3: For patients with genotype 2 or 3 who are candidates for therapy, we recommend treatment with peginterferon and ribavirin (not weight-based) rather than treating with standard interferon and ribavirin or peginterferon monotherapy. Treatment with peginterferon plus ribavirin should be administered for 24 weeks in patients with genotype 2 or 3. 

Genotype 4: For patients with genotype 4 who are candidates for therapy, we recommend treatment with peginterferon and weight-based ribavirinrather than treating with standard interferon and ribavirin or peginterferon monotherapy. Treatment with peginterferon plus weight-based ribavirin should be planned for 48 weeks

For patients with chronic hepatitis C genotype 2, 3, or 4, we suggest treatment with peginterferon alfa-2a rather than peginterferon alfa-2b

We suggest that patients have their viral loads checked at baseline and at weeks 4, 12, and 24 of therapy to assess for a treatment response and to make decisions about continuing treatment or possibly altering the duration of treatment. Patients should also have a viral load checked 24 weeks after therapy is completed to assess for an SVR.

3, 30s female with past history of reflux present w/intermittent nausea, vomiting, diarrhear for several months. she sometimes woke up during night due to abdominal pain, nausea, vomiting or diarrhear, but no fever,chill, no blood in stool, pain is not related fatty food. she has been taking weight loss diet and have intentional weight loss 40lb the past year. she has reflux before present with heartburn, but no symptoms for a while.
assessment and plan
viral gastritis
celiac disease, no family history
gallbladder dysfunction, likely, because gallbladder pain more often happen during the night
reflux, patient hasn't heartburn for a while, unlikely
so plan is endoscopy to r/o gastritis, ultrasound to r/o gallbladder pathology, if ultrasound is normal, may need to do HIDA scan to estimate function of gallbladder
right now, prescribe Zofran (ondansetron) to treat patient's symptoms

4, 87 year old female with recent low gi bleeding due to diverticulitis come to office for follow up.
during her stay in hospital, she had colonoscopy finding extensive diverticulitis, active bleeding, hepatic flexure limited segment colitis and small benign polyps. she has past history of A Fib, and was on Coumadine. currently, she has no complaint of diarrhea, no bleeding, no abdominal pain, weight loss, recent Hb 9.2, physical exam show no abdominal tenderness. Since last bleeding, her coumadine has been on hold.
Assessment,
although colonoscopy found colitis, but patient show no symptoms, so right now we are not going to give any treatment for that
about her small polyps, biopsy show benign, so would recommend her come back in 1 year to repeat colonoscopy.
patient has history of A Fib and low GI bleeding, if restart coumadine, there is 67% possibility of rebleeding, but compare to the risk and consequence of stroke, we still suggest patient restart coumadine and have a close monitor with patient's symptoms.

5, 50s female with past history of cured Hep C come to the office because her recent ultrasound show abnormal change. She retired 1 year ago, her hep c pcr keep negative for more than 6 months after she completed treatment. no abdominal pain, no nausea, vomiting, no low extremities edema, social drink about 1-2 glass of wine/month, she once a while take walking as exercise, recent there is no new medication. she has gained several pounds since retirement, diet is mostly chicken, fish. recent liver function test is within normal range, cholesterol level is about 200. ultrasound show increased parenchymal echogenicity, suggest fatty liver, and 5mm gallbladder polyps. most gallbladder polyps are not true polyps, especially when size is 5mm or less, which is normal finding. However, true polyps are associated with malignancy.

recommend for patient, follow up with U/S in 6 months, if gallbladder polyps is not growing, can watch on that by doing U/S annually.
keep on low cholesterol diet and aerobic exercise.

gastroenterology ob 5

eosinophilic esophagitis

A 51-year-old woman with a history of childhood asthma presented with a sensation of food impaction. Upper gastrointestinal endoscopy revealed classic “feline” esophagus, with mucosal rings (Panel A) and an esophageal stricture near the gastroesophageal junction. Biopsy specimens of the proximal and distal esophagus showed extensive mucosal eosinophilic infiltrates (Panel B, hematoxylin and eosin). The distal esophageal stricture was dilated with the use of a balloon dilator. The patient was treated with a fluticasone inhaler (four 220-μg puffs twice daily), with instructions to swallow and to rinse her mouth. During the next 2 months, her symptoms diminished, and the histologic findings improved. Adult onset of eosinophilic esophagitis is still not recognized by many practitioners. This condition is often confused with gastroesophageal reflux disease and is associated with esophageal strictures. The presence of more than 20 eosinophils per high-power field in an esophageal biopsy specimen is strongly suggestive of this diagnosis. Optimal treatment remains unclear.

The first cases of probable eosinophilic esophagitis were reported in the late 1960s to 1970s. The incidence of eosinophilic esophagitis appears to be increasing. 

• A population-based study evaluated the incidence of eosinophilic esophagitis in Olmsted County Minnesota over thirty years [29]. The incidence increased significantly during the last three of the five-year intervals examined (from 0.35 per 100,000 population between 1991 and 1995 to 9.45 per 100,000 between 2001 and 2005). The prevalence was estimated to be 55 per 100,000 in 2006.

The majority of affected adults have been men in their 20s or 30s, although later presentations have been described.Among children, the disease is also more common in boys (71 percent in the series described above). In another population-based study, children with eosinophilic esophagitis were significantly more likely to be Caucasian (84 percent compared with 73 percent of the surrounding community as a whole). Patients were also more likely to be male (76 versus 48 percent).

PATHOGENESIS —  incompletely understood, Adaptive T-cell immunity driven by type 2 T-helper (Th2) cells, involving interleukin (IL)-13, IL-5, and IL-15 expression appears to play a major role in the pathogenesis of eosinophilic esophagitis.

Genetic factors — A genetic predisposition to eosinophilic esophagitis is supported by evidence of familial clustering. In addition, a possible susceptibility locus has been identified on chromosome 5q22

Eosinophilic esophagitis (EoE) is an allergic disorder characterized by the accumulation of eosinophils in the esophagus. We report association of EoE with variants at chromosome 5q22 encompassing TSLP and WDR36 (rs3806932, combined P = 3.19 x 10(-9)). TSLP is overexpressed in esophageal biopsies from individuals with EoE compared with unaffected individuals, whereas WDR36 expression is unaltered between the two groups. These data implicate the 5q22 locus in the pathogenesis of EoE and identify TSLP as the most likely candidate gene in the region.

Nat Genet. 2010 Apr;42(4):289-91. doi: 10.1038/ng.547. Epub 2010 Mar 7.

Common variants at 5q22 associate with pediatric eosinophilic esophagitis.

CLINICAL MANIFESTATIONS — The clinical manifestations of eosinophilic esophagitis vary with age. Adults and teenagers frequently present with dysphagia and food impactions, whereas in younger children symptoms often include feeding difficulties and abdominal pain.

Clinical manifestations in adults — Common clinical manifestations seen in adults include [5,18,22,29,75-100]:

• Dysphagia
• Food impaction
• Chest pain that is often centrally located and does not respond to antacids
• Gastroesophageal reflux disease-like symptoms/refractory heartburn
• Upper abdominal pain

Clinical manifestations in children — Symptoms in children vary depending in part upon their age [23,105-109]. In one series, the most common presenting symptoms included [23]:

• Feeding dysfunction (median age 2.0 years)
• Vomiting (median age 8.1 years)
• Abdominal pain (median age 12.0 years)
• Dysphagia (median age 13.4 years)
• Food impaction (median age 16.8 years)

ASSOCIATIONS WITH OTHER DISORDERS — There is a strong association of eosinophilic esophagitis with allergic conditions such as food allergies, environmental allergies, asthma, and atopic dermatitis. It has been estimated that 28 to 86 percent of adults and 42 to 93 percent of children with eosinophilic esophagitis have another allergic disease 

DIAGNOSIS — The diagnosis of eosinophilic esophagitis should be based upon symptoms, endoscopic appearance, and histological findings. In patients suspected of having eosinophilic esophagitis, the first diagnostic test is typically an upper endoscopy with esophageal biopsies following two months of treatment with a proton pump inhibitor. In addition, other disorders that can cause esophageal eosinophilia, such as gastroesophageal reflux disease (GERD), should be ruled out.

Endoscopy —

• Stacked circular rings (“feline” esophagus) (picture 1): 44 percent
• Strictures (particularly proximal strictures) (picture 2): 21 percent
• Attenuation of the subepithelial vascular pattern: 41 percent
• Linear furrows (picture 3): 48 percent
• Whitish papules (representing eosinophil microabscesses) (picture 1): 27 percent
• Small caliber esophagus: 9 percent

A 14-month-old with failure to thrive and loose stools. Endoscopy demonstrates a thickened furrowed esophagus consistent with eosinophilic esophagitis. These patients commonly have dysphagia with, as well as without, evidence of stricture. Eosinophilic esophagitis is also a common cause of dysphagic in atopic school-aged children. Histology would demonstrate sheets of eosinophils in the lamina propria.

Courtesy of Karen Murray, MD.

Upper endoscopy in a 36-year-old man with dysphagia. Multiple rings are present in the proximal to mid esophagus giving it the appearance of a trachea. Small whitish papules are also visible representing eosinophilic abscesses on histology. The patient's symptoms responded to oral fluticasone.

Courtesy of Eric D Libby, MD.

Nissen fundoplication is a surgical procedure to treat gastroesophageal reflux disease (GERD) and hiatal hernia. In GERD it is usually performed when medical therapy has failed, but with paraesophageal hiatus hernia, it is the first-line procedure. 

Gastroenterology ob 4

EGD
we don't normally do H.Pylori biopsy with endoscopy. For patient has never test for H.Pylori, can use serology test, if patient has complete eradication treatment, use stool antigen test, which has high sensitive, and is cheapest.

Wireless video capsule endoscopy
after take capsule, 2hr clear liquid, 4hr eat light meal.

Indications — The indications for VCE of the small bowel are evolving. The primary indications are for diagnosis of the site of obscure gastrointestinal bleeding in adults (including iron deficiency anemia), suspected Crohn's disease, and small bowel tumors. In addition, VCE is being used to detect small bowel injury associated with the use of nonsteroidal antiinflammatory drugs (NSAIDs), evaluate abdominal pain of unclear etiology, to screen for polyps in patients with familial polyposis syndromes such as Peutz-Jeghers syndrome and familial adenomatous polyposis, and possibly in the assessment of celiac disease. There is also growing experience in children over the age of 10 for the above indications [17] and these indications are now approved for children as young as two years of age. 

preparation--a 12-hour fast without preparation

Both the PillCam SB2 (in SVH) and the EndoCapsule are 11 x 26 mm in size and acquire images from one end of the device at a rate of two frames per second for approximately eight hours.



Capsule ingestion — The video capsule is swallowed with water. Following capsule ingestion, clear liquids may be taken after two hours, and food and medications may be taken after four hours. The sensor arrays are removed after eight hours and the recorded images are downloaded and processed on workstations.No need to collect the capsule.

The overall yield of VCE for obscure gastrointestinal bleeding has been reported to be in the range of 30 to 70 percent.

• Small bowel angiectasia – 22 percent
• Small bowel ulcerations – 10 percent
• Small bowel tumors – 7 percent
• Small bowel varices – 3 percent
• Blood in the small bowel with no lesion identified – 8 percent
• Esophagogastric lesions (eg, esophagitis, gastritis) – 11 percent
• Colonic angiectasia – 2 percent

VCE should not be used in patients with known or suspected strictures, Capsule retention has been described in up to 5 percent of patients who underwent a capsule study for Crohn's disease, even after performing an initial small bowel study.

Magnetic resonance imaging — Patients should not undergo magnetic resonance imaging (MRI) until passage of the capsule has been confirmed due to concern that it could result in damage to the gastrointestinal tract. Perforation!!!!

Contraindications — The procedure may be contraindicated in patients with the following conditions, albeit these contraindications may not be absolute:

• Dementia (in patients who cannot cooperate with swallowing of the capsule or who may inadvertently damage the equipment)
• Gastroparesis (the capsule can be placed in the duodenum by endoscopy to avoid this problem)
• An esophageal stricture, swallowing disorders that could prevent passage of the capsule (eg, Zenker's diverticulum) (the capsule can be placed in the duodenum by endoscopy to avoid this problem)
• Partial or intermittent small bowel obstruction (unless a surgeon is involved, the patient understands the risks, and the patient has been cleared for surgery)
• Those who are inoperable or refuse surgery
• Patients who have defibrillators or pacemakers (this is a recommendation in the package insert, but does not appear to be a significant clinical problem)
• Women who are pregnant

diabetes patient preparation for colonoscopy
if patient is taking insulin, stop short act insulin in the morning and only take half dose long act insulin the night before.
actually prefer higher glucose than low glucose.

EGD
snakeskin pattern in gastric mucosa indicate mild portal hypertension
http://www.gastrointestinalatlas.com/English/Stomach/Portal_Hypertensive_Gastropath/portal_hypertensive_gastropath.html

Endoscopic appearance of portal hypertensive gastropathy
 in a 65-year-old man with cirrhosis. Note the characteristic
 snakeskin appearance of the gastric mucosa.

Management of anticoagulants in patients undergoing endoscopic procedures

Low-risk procedures, high or low-risk conditions — No change in anticoagulation is recommended for low-risk procedures, though we suggest that elective procedures be delayed in patients taking vitamin K antagonists (eg, warfarin) if the INR or prothrombin time is in the supratherapeutic range.

High-risk procedures, low-risk conditions — In patients at low risk for thromboembolism undergoing high-risk endoscopic procedures, vitamin K antagonists (eg, warfarin) should be discontinued five days before the procedure. The INR should be confirmed to be below 1.4 before the procedure. The vitamin K antagonist can usually be reinstituted on the night of the procedure.

Patients taking an oral direct thrombin or factor Xa inhibitor (dabigatran, rivaroxaban, or apixaban) with normal renal function typically discontinue the drug one to two days prior to the procedure. These drugs have rapid onsets of action (hours) and short half-lives (9 to 17 hours) [5]. These drugs can usually be reinstituted following the procedure. (See "Management of anticoagulation before and after elective surgery", section on 'Dabigatran' and "Management of anticoagulation before and after elective surgery", section on 'Rivaroxaban and apixaban'.)

In patients in whom a sphincterotomy has been performed, the risk of bleeding persists for three to five days, and when sessile polyps are resected, the risk of bleeding may persist for more than two weeks. It is reasonable to delay attaining therapeutic levels of anticoagulation in these situations until five days or two weeks postprocedure, respectively.

High-risk procedures, high-risk conditions — For patients at high risk for thromboembolism undergoing high-risk procedures, vitamin K antagonists (eg, warfarin) should be discontinued five days before the procedure, and bridge therapy should be considered. Similarly, patients taking dabigatran should have the drug stopped one to two days prior to the procedure, assuming normal renal function. We suggest consulting the clinician prescribing the anticoagulant to discuss the options for bridge therapy. (See "Management of anticoagulation before and after elective surgery", section on 'Dabigatran'.)

Bridge therapy — Bridge therapy may be indicated in the periendoscopic period in patients on anticoagulants who are at high risk for thromboembolic complications. The American Heart Association and the American College of Cardiology recommend the following [1]:

• Atrial fibrillation – Bridge therapy is not required for a patient with isolated atrial fibrillation, but is recommended for a patient with atrial fibrillation and a mechanical valve, a history of a cerebrovascular accident or transient ischemic attack, or a history of systemic embolism.
  
  Patients with atrial fibrillation and a bileaflet aortic valve do not require bridge therapy, but should have their anticoagulant restarted within 24 hours.
• Valvular heart disease – Bridge therapy is recommended for patients with a mechanical mitral valve or a mechanical aortic valve with any of the following: atrial fibrillation, previous thromboembolic event, left ventricular dysfunction, hypercoagulable condition, mechanical tricuspid valve, or more than one mechanical valve.

SUMMARY AND RECOMMENDATIONS

• The risk of bleeding from endoscopic procedures can be classified as high or low. In general, diagnostic procedures are low-risk, whereas therapeutic procedures are high-risk (table 1). 
• The probability of a thromboembolic complication following reversal or discontinuation of anticoagulation or antiplatelet agents depends upon the preexisting condition for which the medication was prescribed (table 2). 
• For low-risk procedures, the American Society of Gastrointestinal Endoscopy (ASGE) guidelines suggest making no changes in anticoagulation (Grade 2C). We suggest that elective procedures be delayed if the INR or prothrombin time is in the supratherapeutic range (Grade 2C). (See 'Low-risk procedures, high or low-risk conditions' above.)
• In patients undergoing high-risk endoscopic procedures, we suggest discontinuing vitamin K antagonists (eg, warfarin) five days before the procedure and dabigatran, rivaroxaban, or apixaban one to two days before the procedure in patients with normal renal function (Grade 2C). (See 'High-risk procedures, low-risk conditions' above.)

• Bridge therapy is not required for patients at low-risk for thromboembolism. (See 'High-risk procedures, low-risk conditions' above.)
• For patients at high-risk for thromboembolism, we suggest bridge therapy (Grade 2C). (See 'High-risk procedures, high-risk conditions' above and 'Bridge therapy' above.)

• The management of patients taking antiplatelet agents is discussed in detail elsewhere. (See "Management of antiplatelet agents in patients undergoing endoscopic procedures".)
• Other disorders of hemostasis that may require treatment prior to gastrointestinal procedures, including von Willebrand's disease, hemophilia A and B, renal failure, liver failure, and thrombocytopenia, are discussed in detail elsewhere. (See "Endoscopic procedures in patients with disorders of hemostasis".)