Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome) with Transfusion Requirement. Effect of Danazol in 18 Patients with Long Follow-Up.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1036-1036
Author(s):  
Jose Fernandez ◽  
Dardo Riveros ◽  
Guy Garay ◽  
Reinaldo Campestri ◽  
Gonzalo Garate ◽  
...  
Keyword(s):  
Drug Treatment ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Active Sites ◽  
Gi Tract ◽  
Autosomal Dominant Disorder ◽  
Upper Gi ◽  
Small Series ◽  
Transfusion Requirements ◽  
Upper Gastrointestinal

Abstract Hereditary hemorrhagic telangiectasia (HHT) is a relatively uncommon, autosomal dominant disorder characterized by telangiectases that develop in the skin, mucous membranes, and visceral organs. Mucous localization may seldom bleed profusely, especially epistaxis and upper gastrointestinal (GI) tract. Effective drug treatment is not well established, and multiple blood transfusions and endoscopic or surgical procedures may be the ultimate solution to the frequently bleeding HHT patient. Danazol (DZ) is a mild androgen that has been used in small series of HHT pts with ambiguous results. Its toxicity profile in long standing administration is now well known. Eighteen patients with HHT with transfusion requirements (TR) were treated with DZ at 400–600 mg/daily for the initial three months and 200–400 mg/daily thereafter as a maintenance treatment. At the time of initiation of DZ therapy, median age was 54 yr-old (32–73), nine were female and 9 male, and the median previous TR was 16 RBC units/yr. (2–46). All patients had epistaxis and oral cavity bleeding, with 2 additional upper gastrointestinal tract active bleeding that were detected in ten patients in which an upper GI endoscopy was performed. One patient had a cerebral angioma, surgically treated. None had pulmonary fistula. All patients had some kind of iron treatment. DZ was the first drug treatment intended to reduce the HHT bleeding in 10/18 pts. Median follow-up was 7 years (1.2–14) and two pts were lost to follow-up at 4 and 11 years respectively. At three months of DZ therapy, 12/18 pts (66.6%) showed a remarkable reduction of bleeding, and in 6 patients that showed no response, DZ treatment was stopped. In 7 pts (39%) TR dropped to none and in 5 pts median RT dropped from 22 RBC units/yr. to 10 units/yr. Two of responders had a relapse with upper GI tract bleeding and 1 pts with epistaxis within the first 2 years of DZ treatment. Attempts to reduce the maintenance dose below 200 mg/daily were related to new bleeding or worsening of the active sites. None of the long standing DZ therapy had any significant toxicity. DZ treatment have shown efficacy and safety in this cohort of HHT patients. Mecanism of action may involve the increase of synthesis or expression of ALK-1 dependent proteins and less likely of endoglin. We propose DZ as a first line treatment for the transfusion dependent HHT patients.

Upper Gastrointestinal Langerhans Cell Histiocytosis: A Report of 2 Adult Cases and a Literature Review

2020 ◽  
pp. 106689692096456
Author(s):  
Yui Matsuoka ◽  
Yoshiki Iemura ◽  
Masakazu Fujimoto ◽  
Shinsuke Shibuya ◽  
Atsushi Yamada ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Mononuclear Cells ◽  
Malignant Neoplasm ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Gi Tract ◽  
Upper Gi ◽  
Upper Gastrointestinal ◽  
Upper Gi Tract

Langerhans cell histiocytosis (LCH) with primary involvement of the upper gastrointestinal (GI) tract is rare. We report 2 adult cases of localized LCH in the upper-GI tract, including the second reported adult case of esophageal LCH and review 11 previously reported cases. Case 1 involved the esophagus of a 61-year-old man; histiocytosis was detected when endoscopy was performed for an examination of epigastric pain. Case 2 involved the stomach of a 56-year-old woman wherein the lesion was detected during a follow-up endoscopy after Helicobacter pylori infection. Both biopsy specimens exhibited diffuse proliferation of mononuclear cells with nuclear convolution and a background of eosinophilic infiltrate. The cells were immunohistochemically positive for CD1a and langerin, and BRAF V600E mutation was detected in Case 2. Follow-up endoscopy for both cases revealed that the lesions disappeared without any treatment. It is important to avoid misdiagnosing LCH of the upper-GI tract as a malignant neoplasm.

scholarly journals P0932CLINICAL EVALUATION OF DISTURBANCES GASTROINTESTINAL TRACT IN PATIENT WITH CHRONIC KIDNEY DISEASE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Marufjon Salokhiddinov ◽  
Faxriddin Umarov
Keyword(s):  
Chronic Kidney Disease ◽  
Duodenal Ulcer ◽  
Renal Failure ◽  
Gastrointestinal Tract ◽  
Comparison Group ◽  
Gi Tract ◽  
Erosive Gastritis ◽  
Upper Gi ◽  
Ulcerative Lesions ◽  
Upper Gastrointestinal

Abstract Background and Aims Chronic kidney disease (CKD) affects gastrointestinal function and results in numerous adaptive and maladaptive responses. Disruption of the colonic microbiome and its attendant consequences - the loss of gut barrier integrity and increased generation of uremic toxins - has become well-recognized. However, less attention has been paid to characterizing the mechanisms behind the dysfunction of the upper gastrointestinal (GI) tract, largely owing to the difficulty of studying small bowel function in vivo Method The study included 72 people, of whom 52 made up the main group (CKD group) and 20 people - the comparison group (healthy volunteers without a kidney and cardiovascular disease). The main group consisted of patients with CKD stage 3 (RCGF creatinine 30-59ml / min / m2). The criterion for the inclusion of patients in the study, in addition to reducing the eGFR (estimated glomerular filtration rate), was the presence of arterial hypertension. The renal filtration function was the preserved comparison group and was reduced in the CKD group (p <0.001), which served as a criterion for the inclusion of patients in the study. To assess the state of the gastrointestinal tract, all patients underwent an endoscopic study with an assessment of the acidity and evacuation function of the stomach Results The study showed that 75% of patients had complaints of a dry and unpleasant taste in the mouth, poor appetite, aversion to meat food, nausea. These symptoms were directly related to the level of eGFR. There was also a difference in the frequency and nature of the erosive, erosive-ulcerative, ulcerative lesions of the esophagus and formation of erosions and ulcers in patients with CKD occurred significantly more often against the background of chronic gastritis and with the severity of the inflammatory process with severe dyspeptic disorders. The frequency of erosive, erosive-ulcerative and ulcerative lesions of the mucous membrane of the upper GI tract among CKD patients was 72%, which were manifested by nausea, decreased appetite, heaviness in the stomach after eating, belching, heartburn, epigastric pain (in 50% of patients), meteorism, pain in the epigastric region during palpation. Endoscopic examination of patients with CKD noted the presence of erosive esophagitis, erosive gastritis, erosive bulb it, erosive duodenitis, mixed erosive lesions, erosive-ulcerative gastritis, gastric ulcer, ulcer of duodenal ulcer. Among them, the leading place is occupied by erosive gastritis and mixed erosive lesions, which account for 22% and 27%, respectively. Hemorrhagic erosions were more common in the stomach (41%) and 12: duodenal ulcer (47%) in patients with CKD Conclusion Erosive, erosive-ulcerative, ulcerative lesions of the upper gastrointestinal tract occur in 76% of patients with CKD. The frequency, nature, prevalence of these lesions are associated with the treatment of CKD - active (hemodialysis) and conservative, the stage of chronic renal failure. In patients with chronic renal failure, the endoscopic picture of erosive, erosive-ulcerative, ulcerative lesions of the upper GI tract is diverse.

scholarly journals The Way to Man's Heart Is through the Stomach

2019 ◽  
Author(s):  
Rebecca Grossman ◽  
Rohin Francis
Keyword(s):  
Cardiovascular System ◽  
Significant Proportion ◽  
Organ System ◽  
Differential Diagnoses ◽  
Gi Tract ◽  
Iatrogenic Injuries ◽  
Upper Gi ◽  
System A ◽  
Organ Systems ◽  
Upper Gastrointestinal

AbstractOrgan systems do not exist in a vacuum. However, in an era of increasingly specialized medicine, the focus is often on the organ system alone. Many symptoms are associated with differential diagnoses from upper gastrointestinal (GI) and cardiovascular medical and surgical specialties. Furthermore, a large number of rare but deadly conditions cross paths between the upper GI tract and cardiovascular system; a significant proportion of these are iatrogenic injuries from a parallel specialty. These include unusual fistulae, herniae, and embolisms that transcend specialties. This review highlights these conditions and the shared anatomy and embryology of the two organ systems.

scholarly journals Fecal Microbiota Transplantation Using Upper Gastrointestinal Tract for the Treatment of Refractory or Severe ComplicatedClostridium difficileInfection in Elderly Patients in Poor Medical Condition: The First Study in an Asian Country

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Tae-Geun Gweon ◽  
Jinsu Kim ◽  
Chul-Hyun Lim ◽  
Jae Myung Park ◽  
Dong-Gun Lee ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Fecal Microbiota Transplantation ◽  
Medical Condition ◽  
Performance Status ◽  
Fecal Microbiota ◽  
Gi Tract ◽  
Upper Gi ◽  
Low Performance ◽  
Upper Gastrointestinal ◽  
Upper Gi Tract

Background and Aims. Fecal microbiota transplantation (FMT) is a highly effective treatment option for refractoryClostridium difficileinfection (CDI). FMT may be challenging in patients with a low performance status, because of their poor medical condition. The aims of this study were to describe our experience treating patients in poor medical condition with refractory or severe complicated CDI using FMT via the upper GI tract route.Methods. This study was a retrospective review of seven elderly patients with refractory or severe complicated CDI and a poor medical condition who were treated with FMT through the upper GI tract route from May 2012 through August 2013. The outcomes studied included the cure rate of CDI and adverse events.Results. Of these seven patients who received FMT via the upper GI tract route, all patients were cured. During the 11-month follow-up period, CDI recurrence was observed in two patients; rescue FMT was performed in these patients, which led to a full cure. Vomiting was observed in two patients.Conclusions. FMT via the upper gastrointestinal tract route may be effective for the treatment of refractory or severe complicated CDI in patients with a low performance status. Physicians should be aware of adverse events, especially vomiting.

scholarly journals Radiofrequency for Treatment of Refractory Epistaxis in Hereditary Hemorrhagic Telangiectasia

2018 ◽  
Vol 31 (1) ◽  
pp. 63
Author(s):  
Mariana Donato ◽  
João Pimentel ◽  
Rui Cabral ◽  
Pedro Escada
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Autosomal Dominant ◽  
Standard Procedure ◽  
Recurrent Bleeding ◽  
Bleeding Tendency ◽  
Autosomal Dominant Disorder ◽  
Acute Bleeding ◽  
Significant Interference

Hereditary hemorrhagic telangiectasia is a rare multi-systemic autosomal dominant disorder characterized by dysplasia of the vascular connective tissue and recurrent bleeding tendency. Epistaxis is the most common and earliest symptom. It is usually mild to moderate, however, in some patients it can be severe with significant interference in quality of life. We report a case of a patient with hereditary hemorrhagic telangiectasia, in which an episode of refractory epistaxis was treated with radiofrequency and fibrin sealant. Control of acute bleeding and elimination of telangiectasia was possible with this technique. After six months follow-up, there was no epistaxis recurrence. Various treatments for epistaxis in this disease have been described in the literature, but there is no consensus about the gold-standard procedure. Radiofrequency ablation of telangiectasia is a recent technique that has shown to be safe, effective and well tolerated, even in patients who underwent other previous treatments.

scholarly journals Subcutaneous emphysema, pneumothorax, pneumomediastinum and pneumoperitoneum after upper gastrointestinal endoscopy

2020 ◽  
Vol 13 (11) ◽  
pp. e236369
Author(s):  
Amr Elmoheen ◽  
Mahmoud Haddad ◽  
Khalid Bashir ◽  
Waleed Awad Salem
Keyword(s):  
Subcutaneous Emphysema ◽  
Gastrointestinal Endoscopy ◽  
Quality Of Healthcare ◽  
Upper Gastrointestinal Endoscopy ◽  
Gi Tract ◽  
Negative Effects ◽  
Upper Gi ◽  
Upper Gi Endoscopy ◽  
Upper Gastrointestinal

Upper gastrointestinal (GI) endoscopies are performed for several reasons. The overuse of endoscopy has negative effects on the quality of healthcare and pressurises endoscopy services. It also results in the complications. These complications include pneumoperitoneum, pneumomediastinum and subcutaneous pneumomediastinum. However, it is worth noting that these complications rarely occur during endoscopy of the upper GI tract. These complications, when they occur, indicate perforation of the retroperitoneal space or peritoneal cavity. In this article, we discuss a case of pneumoperitoneum, pneumomediastinum and subcutaneous emphysema after upper GI endoscopy.

scholarly journals Investigation of oral, gastric, and duodenal microbiota in patients with upper gastrointestinal symptoms

2020 ◽  
Vol 69 (4) ◽  
pp. 870-877
Author(s):  
Jorge Cervantes ◽  
Majd Michael ◽  
Bo-Young Hong ◽  
Aden Springer ◽  
Hua Guo ◽  
...  
Keyword(s):  
Gastrointestinal Symptoms ◽  
Microbiota Composition ◽  
Gi Tract ◽  
Upper Gi ◽  
Functional Gi Disorders ◽  
Gi Symptoms ◽  
Dyspeptic Symptoms ◽  
Upper Gastrointestinal Symptoms ◽  
Upper Gastrointestinal ◽  
Gastric Microbiota

Disease-associated alterations of the intestinal microbiota composition, known as dysbiosis, have been well described in several functional gastrointestinal (GI) disorders. Several studies have described alterations in the gastric microbiota in functional dyspepsia, but very few have looked at the duodenum.Here, we explored the upper GI tract microbiota of inpatients with upper GI dyspeptic symptoms, and compared them to achalasia controls, as there is no indication for an esophagogastroduodenoscopy in healthy individuals.We found differences in the microbiota composition at the three sites evaluated (ie, saliva, stomach and duodenum). Changes observed in patients with dyspepsia included an increase in Veillonella in saliva, an oral shift in the composition of the gastric microbiota, and to some degree in the duodenum as well, where an important abundance of anaerobes was observed. Metabolic function prediction identified greater anaerobic metabolism in the stomach microbial community of patients with dyspepsia. Proton pump inhibitor use was not associated with any particular genus. Co-abundance analysis revealed Rothia as the main hub in the duodenum, a genus that significantly correlated with the relative abundance of Clostridium, Haemophilus, and Actinobacillus.We conclude that patients with upper GI symptoms consistent with dyspepsia have alterations in the microbiota of saliva, the stomach, and duodenum, which could contribute to symptoms of functional GI disorders.

scholarly journals Upper gastrointestinal tract involvement is more prevalent in Korean patients with pediatric Crohn’s disease than in European patients

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Eun Sil Kim ◽  
Yiyoung Kwon ◽  
Yon Ho Choe ◽  
Mi Jin Kim
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Disease Outcome ◽  
Disease Phenotype ◽  
Gi Tract ◽  
Korean Patients ◽  
Upper Gi ◽  
Tract Involvement ◽  
Upper Gastrointestinal ◽  
Upper Gi Tract

Abstract In pediatric Crohn’s disease (CD) patients, it is important to define the disease phenotype at diagnosis for stratifying risk. In this retrospective study, we aimed to assess the disease phenotype compared to EUROKIDS registry and analyze disease outcome of pediatric CD patients according to upper gastrointestinal (GI) tract involvement. A total of 312 patients were included. The median age at diagnosis was 13.7 years and 232 patients (74.4%) were identified to have upper GI involvement at diagnosis. In Korean pediatric CD patients, there were significant differences in male predominance (72.8% vs. 59.2, p < 0.001), proportion of upper GI involvement (74.4% vs. 46.2%, p < 0.001), and perianal disease (62.1% vs. 8.2%, p < 0.001) compared to data in the EUROKIDS registry. Younger age (OR 2.594, p = 0.0139) and ileal involvement (OR 2.293, p = 0.0176) at diagnosis were associated with upper GI involvement. There were no significant differences in disease outcomes between patients with and without upper GI tract involvement. This study revealed that upper GI involvement is more prevalent in Korean patients with pediatric Crohn’s disease than in European patients, and the disease outcome did not appear to differ according to upper GI tract involvement.

Endoscopic management and long-term follow-up of Dieulafoy's lesions in the upper GI tract

2002 ◽  
Vol 55 (4) ◽  
pp. 527-531 ◽  
Author(s):  
Panagiotis Kasapidis ◽  
Philippos Georgopoulos ◽  
Vassilios Delis ◽  
Vassilios Balatsos ◽  
Anastasios Konstantinidis ◽  
...  
Keyword(s):  
Endoscopic Management ◽  
Gi Tract ◽  
Upper Gi ◽  
Long Term Follow Up ◽  
Upper Gi Tract

scholarly journals Workload and Resource Implications of Upper Gastrointestinal Cancer Surgical Centralisation in South East Wales

2008 ◽  
Vol 90 (6) ◽  
pp. 467-471 ◽  
Author(s):  
MA Morgan ◽  
M Goodson ◽  
X Escofet ◽  
GWB Clark ◽  
WG Lewis
Keyword(s):  
Radical Surgery ◽  
National Guidelines ◽  
Upper Gi ◽  
Upper Gastrointestinal Cancer ◽  
Number Of Patients ◽  
Training Opportunities ◽  
And Training ◽  
Upper Gastrointestinal ◽  
Oesophagogastric Cancer

INTRODUCTION The aim of this study was to determine whether one specialist unit could manage all patients diagnosed with oesophagogastric cancer in Gwent and Cardiff and Vale NHS Trusts over a 6-month period with regard to workload, resource and training opportunities. PATIENTS AND METHODS All patients diagnosed with oesophagogastric (OG) cancer in Gwent and Cardiff and Vale NHS Trusts and referred to the regional South East Wales Upper GI multidisciplinary team over the 6-month period from 1 July to 31 December 2005 were studied prospectively and compared with the previous 6-month caseload at Cardiff and Vale. RESULTS Out-patient workload increased from 160 new (33 OG cancers) and 533 follow-up patients (161 OG cancers) between 1 January and 30 June 2005, to 290 new (68 OG cancers, 106% increase) and 865 follow-up patients (230 OG cancers, 43% increase) between 1 July, and 31 December 2005. The number of patients undergoing radical surgery increased from 14 to 23 (D2 gastrectomy 8 versus 13; oesophagectomy 6 versus 10). Cancer-related workload in the latter period generated 118 intermediate equivalents (IEs) of operative work for two specialist surgeons and one SpR occupying 38% of the total time available on 104 scheduled operating lists, compared with 64 IEs in the previous 6 months, representing an 84% increase in cancer-related operative training opportunities. CONCLUSIONS Centralisation of oesophagogastric cancer surgery is feasible and desirable if national guidelines are to be satisfied, and this strategy has significant positive implications for surgical training and audit.

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