scholarly journals Recurrent and acute abdominal pain as the main clinical manifestation in patients with hereditary angioedema

2021 ◽  
Vol 42 (2) ◽  
pp. 131-135
Author(s):  
Yang Cao ◽  
Shuang Liu ◽  
Yuxiang Zhi

Background: Hereditary angioedema (HAE) is a rare disease that often leads to misdiagnosis. The delay of diagnosis is > 10 years in China. Recurrent and acute abdominal pain is one of the common symptoms of HAE. Because of the high misdiagnosis rate, it usually results in unnecessary surgical procedures. This study focused on the clinical symptoms and management of HAE-related abdominal attacks in Chinese patients to provide some new insight for the emergency department (ED) physicians and gastroenterologists. Methods: A Web-based survey was conducted among 107 patients with HAE from 94 unrelated families. Detailed questions with respect to the abdominal attacks were asked, including the frequency, symptoms, and duration before and after confirmed diagnosis. The demographic characteristics, diagnosis process, and treatment outcomes were also included. Results: Approximately 70% of the patients with HAE presented with abdominal symptoms during the onset of edema, mostly characterized by pain (94.8%), nausea (83.1%), vomiting (83.1%), diarrhea (59.7%), and constipation (23.4%). The patients were easily misdiagnosed as having gastroenteritis (35.1%) and appendicitis (10.4%), and 24.7% of them received unnecessary appendectomy or laparotomy. Danazol, a widely used drug for long-term prophylaxis of HAE in China, can reduce the attack frequency and alleviate the abdominal symptoms, but the adverse effects are also significant and more severe in women. Conclusions: Abdominal symptoms are common and important clinical features of HAE but are easily confused with other gastrointestinal diseases. ED physicians and gastroenterologists should consider HAE when patients experience recurrent and unexplained abdominal pain. Proper medical treatment should be administered in a timely manner if an HAE diagnosis is confirmed and efforts are required to increase access in China to medications both for on-demand treatment and long-term prophylaxis.

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Xiaorong Yan ◽  
Huiqing Wang ◽  
Cai Li ◽  
Yuanxiang Lin ◽  
Lin Lin ◽  
...  

Abstract Background To present a surgical technique for the treatment of intradural extramedullary (IDEM) tumors by using endoscopically controlled surgery with open hemilaminectomy technique. Methods In this study, 20 patients with 22 IDEM tumors were enrolled. An endoscopically controlled surgery with open hemilaminectomy was employed to remove the tumors. Data related to clinical symptoms and medical images before and after surgery were collected for perioperative evaluation and follow-up analysis. Results All the tumors in 20 patients were well removed. The clinical symptoms were significantly reduced in all the patients as well. The short-term follow-up data showed that there was no tumor recurrence or spinal deformity. Conclusion The endoscopically controlled surgery with open hemilaminectomy technique provided favorable exposure and satisfactory resection to the IDEM tumors. It may be an effective surgical method for treating IDEM tumors. Larger samples and longer follow-up data are needed to verify its long-term effectiveness.


2020 ◽  
Author(s):  
Xiaorong Yan ◽  
Huiqing Wang ◽  
Cai Li ◽  
Yuanxiang Lin ◽  
Lin Lin ◽  
...  

Abstract Background To present a surgical technique for the treatment of intradural extramedullary (IDEM) tumors by using endoscopically-controlled surgery with open hemi-laminectomy technique. Methods In this study, 20 patients with 22 IDEM tumors were enrolled. An endoscopically-controlled surgery with open hemi-laminectomy was employed to remove the tumors. Data related to clinical symptoms and medical images before and after surgery were collected for perioperative evaluation and follow-up analysis. Results All the tumors in 20 patients were well removed. The clinical symptoms were significantly reduced in all the patients as well. The short term follow-up data showed that there was no tumor recurrence or spinal deformity. Conclusion The endoscopically-controlled surgery with open hemi-laminectomy technique provided favorable exposure and satisfactory resection to the IDEM tumors. It may be an effective surgical method for treating IDEM tumors. Larger samples and longer follow-up data are needed to verify its long-term effectiveness.


2018 ◽  
Vol 84 (6) ◽  
pp. 1091-1096 ◽  
Author(s):  
Astha Bhatt ◽  
Xihua Yang ◽  
Nihaal Karnik ◽  
Anne Sill ◽  
Gopal Kowdley

Annually, approximately 70 million computed tomography (CT) scans are performed in emergency department (ED) settings in the United States of America. From 1995 to 2007, there has been a 5.9-fold increase in the use of CTscans nationally. The radiation risks and high costs associated with CTscans underscore the fact that the imaging modality, although necessary, carries a myriad of long-term risks to both patients and providers. For the workup of abdominal pain, most algorithms include the use of CTscan as an early step. To understand better the use of CTscans in our ED, we performed a retrospective review of patients presenting to the ED with abdominal pain. Two main questions were addressed: 1) what were the reasons for scans and how often did the scans reveal pathology related to the presenting symptoms, 2) how often were incidental findings identified. Our results showed that among patients presenting with abdominal pain to the ED, 50 per cent of the scans were normal, about 20 per cent of the patients had findings correlating with acute abdominal pain, whereas the rest (30%) had incidental findings that may have led to further outpatient studies or long standing abdominal pain. Most patients who presented to the ED had nonspecific abdominal pain i.e. 64.4 per cent. There was a low agreement between the presenting quadrant of pain and final pathological diagnosis (9.5–33.3% concordance), with left flank pain presentation having the highest level of agreement with the final pathologic diagnosis.


2019 ◽  
Vol 12 (8) ◽  
pp. e228962 ◽  
Author(s):  
Wouter KG Leclercq ◽  
Martine Uittenbogaart ◽  
Hendrik J Niemarkt ◽  
Judith OEH van Laar

Pregnant women who previously had bariatric surgery may develop acute abdominal pain during pregnancy. Two patients, 38-year-old twin primigravida (gestational age of 24+6 weeks) and a 26-year-old woman (gestational age of 24+0 weeks), both of whom had laparoscopic gastric bypass surgery previously, developed abdominal pain. The patients both had diffuse abdominal pain in combination with normal blood tests and imaging. Patient B had undergone laparoscopy at another centre after 5 weeks of gestation for internal herniation. After referral to our multidisciplinary bariatric–obstetric–neonatal (MD-BON) team, diagnostic laparoscopy was advised as internal herniation was deemed possible. In both patients, internal herniation was indeed found in Petersen’s space and jejunal mesenteric defect, which was closed using laparoscopic surgery. Both women delivered healthy offspring afterwards. The presence of an MD-BON team allows for an increased awareness of potential long-term complications associated with earlier bariatric surgery in pregnancy.


2020 ◽  
Author(s):  
Shan Qiao ◽  
Huai-kuan Wu ◽  
Ling-ling Liu ◽  
Ke-jun Zang ◽  
Xuewu liu

Abstract Background: This report aims to provide a detailed description of the clinical manifestation, immunotherapy, and long-term outcomes of 117 Chinese patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. Methods: We retrospectively selected 117 patients diagnosed with anti-LGI1 encephalitis from the databases of multiple clinical centers from September 2014 to December 2019. The clinical features, ancillary test results, and details of long-term outcomes were analyzed.Results: Among the 117 anti-LGI1 encephalitis patients, 81 (69%) were male and 36 (31%) were female; the median onset age was 51 years (range: 30-77 years). The median time from symptom onset until diagnosis was 8.7 weeks (range: 2-49 weeks). The main features evaluated in our cohort were seizures, cognitive impairment, and mental and behavioral abnormalities. One hundred and nine patients were treated with immunotherapy, After 3-5 days of treatment, the clinical symptoms were somewhat alleviated in all the patients, and their memory, mental ability, and behavior improved. The median follow-up time was 33 months (range: 6-59 months). A total of 19 (16%) patients experienced a relapse; the median duration from onset to the first relapse was 5 (0.3-27) months. There were no mortalities during the follow-up period.Conclusions: The outcome of patients with anti-LGI1 encephalitis is mostly favorable, although some patients continue to suffer from cognitive dysfunction. Early recognition is of great significance for the treatment of anti-LGI1 encephalitis. Prompt adequate immunotherapy has positive implications for the improvement of clinical symptoms of anti-LGI1 encephalitis. Long-term follow-up is important for the assessment of LGI1 antibody-mediated encephalitis.


2021 ◽  
Vol 19 (3) ◽  
pp. 166-173
Author(s):  
Eray YILDIZ ◽  
Şevket ARSLAN ◽  
Fatih ÇÖLKESEN ◽  
Filiz Sadi AYKAN ◽  
Recep EVCEN ◽  
...  

Objective: The aim of this study was to determine the clinical course and treatment outcomes of patients with hereditary angioedema (HAE) after infection with coronavirus disease 2019 (COVID-19). Materials and Methods: Thirty-nine patients with HAE were included in this study. These patients were regularly followed up over phone calls since the first COVID-19 case was seen in our country. Patients were asked to visit the hospital if there was a history of contact with a confirmed COVID-19 patient or if the patient developed clinical symptoms of COVID-19.Results: There were 21 (54%) patients with type I HAE, and 18 (46%) with type II HAE. All patients received treatment for angioedema attacks (C1-inhibitor [C1-INH], icatibant), and seven (20%) received long-term prophylaxis (danazol). Treatment for attacks was continued for all patients during the pandemic. Patients taking danazol were switched to long-term prophylaxis using the C1-INH concentrate. Eleven (28%) patients with HAE developed COVID-19 during this study. Only one patient had severe COVID-19. Six patients (54.5%) were diagnosed with type II HAE, and five (45.5%) were diagnosed with type I HAE. The most common COVID-19 symptoms were fever (7/11; 64%) and myalgia (6/11; 55%). Mild angioedema attacks were experienced by 36% (4/11) of the HAE patients diagnosed with COVID-19. Icatibant was used in all patients.Conclusion: Agents used for HAE block the kallikrein-kinin system and may be useful in the treatment of COVID-19. Considering their beneficial effects on COVID-19, it is recommended that HAE patients should continue the use of agents blocking the kallikrein-kinin system. Keywords: COVID-19, hereditary angioedema, kallikrein-kinin system, bradykinin, C1-INH


2019 ◽  
Vol 13 (2) ◽  
pp. 83-90
Author(s):  
Taqi Saadoon Atiyah

Background: Abdominal symptoms are possibly the most frequent of all symptoms encountered in surgical practice.  Pain is the most common of all abdominal symptoms.  Causes of acute abdominal pain include both medical and surgical. Most symptoms arise from intra-abdominal organs or systems while some may originate extra abdominally and are then referred to the abdomen. Medical causes of abdominal pain are encountered more frequently. Objective: To study the causes of acute abdominal pain in patients attending emergency department in Al- Imamain Al- Kadhimain Medical City. Type of the study: A prospective cross sectional study Methods: The patients attending Emergency Department in Al- Imamain Al- Kadhimain Medical City over the period from April-2014 to April 2016. There was a one day call duty per week managed by the same surgical team (total one hundred days study). Onlypatients above 12 years old with acute non-traumatic abdominal pain were included in this study. Results: The total number was 1217 patients over hundred days study. Their age ranges from 12-83 years; average age was 36.18 ±17 year. There was slight female predominance, 638 (52.42%) female patients and 579 (47.58%) male patients.  The most common cause of abdominal pain was acute appendicitis 252 (20.70%), followed by ureteric colic 251 (20.62%), and acute cholecystitis249 (20.46%). Conservative management was done for 836 (68.69%) patients, while operative management done for 379 (31.14%) patients.  Postoperative mortality was 8 (2.11%) patients.Medical causes of acute abdominal pain were found in 48 (5.74%) patients. Conclusion: Not all the patients attending surgical emergency department needs operations. Causes of acute abdominal pain include both medical and surgical diseases, some of the medical diseases are very serious like acute viral hepatitis, myocardial infarction , and diabetic ketoacidosis; and should not submit those patients to unnecessary operations with serious and may be fatal postoperative complications. General urine examination is a must in all patients with acute abdominal pain. Electrocardiography (ECG) may be needed in old patients.


2019 ◽  
Vol 46 ◽  
pp. 43-49 ◽  
Author(s):  
Helen Schultz ◽  
Line Abrahamsen ◽  
Lise Ewald Rekvad ◽  
Ulla Skræp ◽  
Tanja Schultz Larsen ◽  
...  

2021 ◽  
Vol 12 ◽  
Author(s):  
Rongxin Dai ◽  
Ge Lv ◽  
Wenyan Li ◽  
Wenjing Tang ◽  
Junjie Chen ◽  
...  

BackgroundSCN4 is an autosomal recessive disease caused by mutations in the G6PC3 gene. The clinical, molecular, and immunological features; function of neutrophils; and prognosis of patients with SCN4 have not been fully elucidated.MethodsTwo Chinese pediatric patients with G6PC3 mutations were enrolled in this study. Clinical data, genetic and immunologic characteristics, and neutrophil function were evaluated in patients and controls before and after granulocyte colony-stimulating factor (G-CSF) treatment.ResultsBoth patients had histories of pneumonia, inguinal hernia, cryptorchidism, and recurrent oral ulcers. Patient 1 also had asthma and otitis media, and patient 2 presented with prominent ectatic superficial veins and inflammatory bowel disease. DNA sequencing demonstrated that both patients harbored heterozygous G6PC3 gene mutations. Spontaneous and FAS-induced neutrophil apoptosis were significantly increased in patients, and improved only slightly after G-CSF treatment, while neutrophil respiratory burst and neutrophil extracellular traps production remained impaired in patients after G-CSF treatment.ConclusionG-CSF treatment is insufficient for patients with SCN4 patients, who remain at risk of infection. Where possible, regular G-CSF treatment, long-term prevention of infection, are the optimal methods for cure of SCN4 patients. It is important to monitor closely for signs of leukemia in SCN4 patients. Once leukemia occurs in SCN4 patients, hematopoietic stem cell transplantation is the most important choice of treatment.


2020 ◽  
pp. 72-73
Author(s):  
Jitendra K. Mangtani ◽  
Ketan Patel ◽  
Anshul Mathur ◽  
Heet Amlani ◽  
Vartika Gupta ◽  
...  

Introduction: Post-cholecystectomy syndrome (PCS) comprise of a group of abdominal symptoms that occur after cholecystectomy. Post cholecystectomy syndrome is defined as group of heterogeneous symptoms. It is consists of upper abdominal pain, vomiting and dyspepsia, which occur after cholecystectomy. Although, this term is not accurate, as it comprises biliary and non-biliary disorders, possibly not related to cholecystectomy. AIM : To analyse the clinical evaluation for post cholecystectomy Symptoms. Objectives : To identify the causes for post cholecystectomy symptoms.To identify pre-operative factors (h/o ERCP, attacks of cholecystitis) associated with post cholecystectomy symptoms. Materials & Methods : A Prospective clinical hospital based study was done in 100 patients at tertiary care referral hospital in the department of general surgery. All cases of post- cholecystectomy who had clinical symptoms of PCS Cases in which on follow up USG and LFT were done. Result: In our study 100 patients were taken who developed PCS after cholecystectomy. Among all patients, 16% patient developed PCS due to biliary etiology. Among 16% patients most common cause is recurrent CBD stone (6%) and second most common causes is retained CBD Stone (3%) & spincter Of Oddi dysfunction (3%). Discussion : This analysis provides a qualitative overview of etiologies of abdominal symptoms after cholecystectomy. Based on the etiologies of persistent and incident symptoms after cholecystectomy provided in this review, we identified the cause of long-term symptoms after cholecystectomy. Conclusion : In our study, PCS developed mainly due to non-biliary etiology considered 84% and biliary etiology considered only 16%. Female has higher risk to developing post cholecystectomy symptoms compared to male after cholecystectomy.


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