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Cureus May 2024Hirschsprung disease, a rare genetic disorder affecting the enteric nervous system, is characterized by the absence of ganglion cells in the myenteric plexus. Typically...
Hirschsprung disease, a rare genetic disorder affecting the enteric nervous system, is characterized by the absence of ganglion cells in the myenteric plexus. Typically identified in neonates due to the failure to pass meconium, diagnosis beyond the first year of life is considered delayed. Common clinical manifestations in children with late-onset Hirschsprung disease include abdominal distension, abdominal pain, vomiting, fever, and abnormal bowel sounds. Sigmoid volvulus, though uncommon, can complicate Hirschsprung disease, potentially leading to misdiagnosis and severe complications such as intestinal perforation, hemorrhage, sepsis, and even mortality. Non-surgical interventions such as antibiotic therapy, intestinal decompression, and fluid resuscitation are preferred initial treatments to stabilize the patient. This case involves a 9-year-old boy who has presented with abdominal distension since birth and a lengthy history of irregular bowel habits. The diagnosis of Hirschsprung disease was confirmed at our institution, and the patient underwent a two-stage repair procedure, which was completed without any intraoperative or postoperative complications. The patient experienced an uneventful recovery, was discharged with stable vital signs, and regained normal bowel function. This case highlights the challenges of delayed diagnosis at nine years and underscores the importance of prompt management.
PubMed: 38883048
DOI: 10.7759/cureus.60315 -
Cureus May 2024Pericardial effusion, a clinical condition characterized by an abnormal accumulation of fluid in the pericardial cavity, has multiple etiological factors. One of the...
Pericardial effusion, a clinical condition characterized by an abnormal accumulation of fluid in the pericardial cavity, has multiple etiological factors. One of the prominent causes is malignant effusion. The patient is a 69-year-old female with a past medical history of Crohn's disease, melanoma status post-resection, and osteoarthritis. She presented with complaints of abdominal discomfort, shortness of breath on exertion, and lower extremity swelling for the past 2-3 days. She was recently discharged four days before this visit after being treated for a viral infection. A physical examination was significant for tachycardia, muffled heart sounds, abdominal distention, and bilateral lower extremity swelling. Labs were in the normal range except for elevated liver enzymes and sodium of 130 mmol/L. A chest X-ray revealed a small bilateral effusion; a bedside echocardiogram showed an ejection fraction greater than 70% and a large pericardial effusion >2 cm, consistent with cardiac tamponade. Emergent pericardiocentesis was performed with the drainage of 250 milliliters of hemorrhagic fluid, which was sent for diagnostic studies. Post-procedure echo on the next day showed an EF of 35-40% and no recurrent pericardial effusion. The workup for connective tissue disease was negative except for elevated antinuclear antibodies (ANA). CT of the abdomen and pelvis revealed gastric wall thickening with no solid organ mass. Her pericardial fluid studies were consistent with exudative etiology and positive for atypical lymphoid cells, leading to the diagnosis of diffuse large B-cell lymphoma. Diffuse large B-cell lymphoma is the most common type of non-Hodgkin lymphoma. Malignant pericardial effusion is common due to solid organ malignancy; however, it is rare with diffuse large B cell lymphoma (DLBCL). We present an intriguing case where pericardial effusion was the precursor to the eventual diagnosis of DLBCL, highlighting the complexity and diverse manifestations of this lymphoma subtype.
PubMed: 38882999
DOI: 10.7759/cureus.60418 -
JHEP Reports : Innovation in Hepatology Jun 2024Patients with advanced cirrhosis often develop hepatic decompensation, which is accompanied by systemic inflammation and may eventually lead to acute-on-chronic liver...
BACKGROUND & AIMS
Patients with advanced cirrhosis often develop hepatic decompensation, which is accompanied by systemic inflammation and may eventually lead to acute-on-chronic liver failure. One important cause of systemic hyperinflammation is a dysregulated overshooting immune response in ascites in the abdominal cavity. In this study, we analyzed the role of CD8 T cells in the ascites immune compartment.
METHODS
Peripheral blood and ascites fluid were collected from 50 patients with decompensated cirrhosis. Phenotype and functional responses of CD8 T cells were analyzed, and obtained data were compared with each other as well as with healthy controls and patients with compensated cirrhosis.
RESULTS
High-dimensional flow cytometry revealed that CD8 T cells are abundant in the ascites of patients with cirrhosis and exhibit a chronically activated bystander phenotype with innate-like functions. Indeed, we identified distinct CXCR6CD69 clusters of late effector memory CD8 T cells that were rarely found in blood and correlated with clinical parameters of disease severity. Moreover, this CD8 T-cell population was hyperresponsive to innate cytokines and exhibited cytokine-mediated bystander activation. Interestingly, the Janus kinase (JAK) inhibitor tofacitinib was able to effectively block bystander-activated CXCR6CD69 CD8 T cells and significantly suppress effector molecule production.
CONCLUSIONS
The results indicate that CXCR6CD69 CD8 T cells in ascites are associated with disease severity and may contribute to inflammation in patients with decompensated cirrhosis, suggesting that targeted inhibition of this immune cell subset may be a viable therapeutic option.
IMPACT AND IMPLICATIONS
Patients with advanced cirrhosis often develop hepatic decompensation, which is accompanied by systemic inflammation and eventually leads to acute-on-chronic liver failure. One important cause of systemic hyperinflammation is a dysregulated overshooting immune response in ascites in the abdominal cavity. In this study, we demonstrate that CXCR6CD69 CD8 T cells are abundant in the ascites of patients with cirrhosis, exhibit a chronically activated bystander phenotype, and correlate with clinical parameters of disease severity. Moreover, we show that the Janus kinase (JAK) inhibitor tofacitinib can effectively block these bystander-activated CXCR6CD69 CD8 T cells, suggesting that targeted inhibition of this immune cell subset may be a potential therapeutic strategy.
CLINICAL TRIAL NUMBER
Prospective registry: INFEKTA (DRKS00010664).
PubMed: 38882602
DOI: 10.1016/j.jhepr.2024.101074 -
The Pan African Medical Journal 2024Acute chylous peritonitis is an uncommon medical condition that can occur suddenly, resulting in the buildup of chylous fluid in the peritoneal cavity. It is considered... (Review)
Review
Acute chylous peritonitis is an uncommon medical condition that can occur suddenly, resulting in the buildup of chylous fluid in the peritoneal cavity. It is considered idiopathic because the exact cause is often unknown. The symptoms of acute chylous idiopathic peritonitis can mimic other abdominal emergencies, making it challenging to diagnose and manage, requiring a multidisciplinary approach. We present a case report of acute idiopathic chylous peritonitis miming acute abdomen, how was successfully treated with surgery, and provide a comprehensive review of the available literature on this topic. Chylous peritonitis is a rare condition whose clinical presentation mimics an acute abdomen. It is necessary to undertake careful exploration. An emergent laparotomy is indicated to treat the peritonitis and search for and treat the underlying cause.
Topics: Humans; Abdomen, Acute; Acute Disease; Chylous Ascites; Diagnosis, Differential; Laparotomy
PubMed: 38881770
DOI: 10.11604/pamj.2024.47.131.42794 -
Journal of Surgical Oncology Jun 2024In critically ill patients, temporary abdominal closure (TAC) is utilized for conditions like abdominal compartment syndrome risk, gross abdominal contamination, and...
BACKGROUND AND OBJECTIVES
In critically ill patients, temporary abdominal closure (TAC) is utilized for conditions like abdominal compartment syndrome risk, gross abdominal contamination, and intestinal loop viability doubts. TAC techniques aim to safeguard abdominal contents, drain intraperitoneal fluids, and minimize fascia and skin damage. Our goal is to outline clinical characteristics and surgical outcomes in oncological patients undergoing peritoneostomy.
METHODS
Patients undergoing TAC with vacuum therapy at a tertiary oncological center were studied, with data sourced from an institutional database.
RESULTS
Forty-seven patients (54.3% female), with an average age of 63.1 ± 12.3 years, were included in the study. The primary tumor site was predominantly gastrointestinal (78.2%). Patients presented systemic signs of chronic disease, reflected by a mean body mass index of 18.2 ± 7.6 kg/m², hemoglobin level of 9.2 ± 1.8 g/dL, and albumin level of 2.3 ± 0.6 g/dL. Additionally, most patients had a low-performance status (53% Eastern Cooperative Oncology Group 1/2, 44.8% Karnofsky score ≤80, and 61.2% Charlson Comorbidity Index ≥6). Emergency surgical complications were the main reasons for initial surgery (68%), with the majority attributed to fecal peritonitis (65.9%). Only 14.8% of patients achieved complete abdominal closure with an average of 24.8 days until closure. The in-hospital mortality rate was 85.2%.
CONCLUSION
TAC is an alternative for oncological patients with surgical complications, but it carries a high mortality rate due to the compromised conditions of the patients.
PubMed: 38881409
DOI: 10.1002/jso.27729 -
Scoring System to Evaluate Risk of Nonoperative Management Failure in Children With Intussusception.The Journal of Surgical Research Jun 2024Typical first-line management of children with intussusception is enema reduction; however, failure necessitates surgical intervention. The number of attempts varies by...
INTRODUCTION
Typical first-line management of children with intussusception is enema reduction; however, failure necessitates surgical intervention. The number of attempts varies by clinician, and predictors of failed nonoperative management are not routinely considered in practice. The purpose of this study is to create a scoring system that predicts risk of nonoperative failure and need for surgical intervention.
METHODS
Children diagnosed with intussusception upon presentation to the emergency department of a tertiary children's hospital between 2019 and 2022 were retrospectively identified. Univariable logistic regression identified predictors of nonoperative failure used as starting covariates for multivariable logistic regression with final model determined by backwards elimination. Regression coefficients for final predictors were used to create the scoring system and optimal cut-points were delineated.
RESULTS
We identified 143 instances of ultrasound-documented intussusception of which 28 (19.6%) required operative intervention. Predictors of failed nonoperative management included age ≥4 y (odds ratio [OR] 32.83, 95% confidence interval [CI]: 1.91-564.23), ≥1 failed enema reduction attempts (OR 189.53, 95% CI: 19.07-1884.11), presenting heart rate ≥128 (OR 3.38, 95% CI: 0.74-15.36), presenting systolic blood pressure ≥115 mmHg (OR 6.59, 95% CI: 0.93-46.66), and trapped fluid between intussuscepted loops on ultrasound (OR 17.54, 95% CI: 0.77-397.51). Employing these factors, a novel risk scoring system was developed (area under the curve 0.96, 95% CI: 0.93-0.99). Scores range from 0 to 8; ≤2 have low (1.1%), 3-4 moderate (50.0%), and ≥5 high (100%) failure risk.
CONCLUSIONS
Using known risk factors for enema failure, we produced a risk scoring system with outstanding discriminate ability for children with intussusception necessitating surgical intervention. Prospective validation is warranted prior to clinical integration.
PubMed: 38875949
DOI: 10.1016/j.jss.2024.05.030 -
Frontiers in Cellular and Infection... 2024Serratia marcescens, as a Gram-negative opportunistic pathogen, is a rare cause of peritonitis and has worse clinical outcomes than Gram-positive peritonitis. In this... (Review)
Review
Serratia marcescens, as a Gram-negative opportunistic pathogen, is a rare cause of peritonitis and has worse clinical outcomes than Gram-positive peritonitis. In this case report, we describe a case of Serratia marcescens associated peritonitis that was successfully cured without catheter removal. A 40-year-old male patient with peritoneal dialysis who worked in the catering industry was admitted to the hospital for 16 hours after the discovery of cloudy peritoneal dialysate and abdominal pain. Ceftazidime and cefazolin sodium were immediately given intravenously as an empirical antibiotic regimen. After detecting Serratia marcescens in the peritoneal diasate culture, the treatment was switched to ceftazidime and levofloxacin. The routine examination of peritoneal dialysate showed a significant decrease in white blood cells, the peritoneal dialysate became clear, and the peritoneal dialysis catheter was retained. The patient was treated for 2 weeks and treated with oral antibiotics for 1 week. It is necessary to further strengthen the hygiene of work environment to prevent Serratia marcescens infection in peritoneal dialysis patients. We recommend that patients with Serratia marcescens associated peritonitis should be treated with a combination of antibiotics as early as possible empirically, and at the same time, the peritoneal dialysis fluid culture should be improved, and the antibiotic regimen should be timely adjusted according to the drug sensitivity results. For patients with clinical symptoms for more than 3 days, considering the strong virulence of Serratia marcescens, whether to use meropenem directly or not can provide a reference for clinical decision-making. Further clinical studies are needed to achieve more precise anti-infective treatment.
Topics: Humans; Serratia marcescens; Male; Peritonitis; Adult; Serratia Infections; Anti-Bacterial Agents; Peritoneal Dialysis; Treatment Outcome; Device Removal; Levofloxacin; Ceftazidime; Cefazolin
PubMed: 38873095
DOI: 10.3389/fcimb.2024.1373036 -
Coexistence of variant-type transthyretin and immunoglobulin light-chain amyloidosis: a case report.European Heart Journal. Case Reports Jun 2024Determining the type of amyloid deposits is clinically important for choosing the specific therapies for cardiac amyloidosis.
BACKGROUND
Determining the type of amyloid deposits is clinically important for choosing the specific therapies for cardiac amyloidosis.
CASE SUMMARY
A 78-year-old woman who had been experiencing fluid retention and dyspnoea on exertion for 6 months was referred to our hospital for the management of heart failure with left ventricular hypertrophy. Since Tc-hydroxymethylene diphosphonate scintigraphy showed mild cardiac uptake and significant elevation of serum free lambda chain (with a difference of 263 mg/L in free light chain), we suspected immunoglobulin light-chain amyloidosis (AL), and endomyocardial biopsy was performed. The deposit site within the myocardial tissue exhibited positive for Congo red staining and transthyretin immunostaining, however negative or non-specific for light-chain immunostaining including lambda and kappa staining. Genetic testing confirmed a mutation in V122I, variant-type transthyretin amyloidosis (ATTRv). Despite the administration of patisiran, her condition exhibited progressive deterioration. Additionally, she displayed macroglossia, an atypical manifestation in ATTRv amyloidosis. Further biopsies from tongue and abdominal wall fat culminated in a final diagnosis: the coexistence of ATTRv and AL (of the lambda type). Although treatment with melphalan and dexamethasone was started, she passed away 24 months after the initial visit. When the endomyocardial biopsy specimen underwent mass spectrometry as a analysis, both ATTR and AL amyloid were significantly detected.
DISCUSSION
Coexistence of ATTRv and AL within cardiac amyloidosis is extremely uncommon. In situations where incongruities arise between the amyloid type determined via immunohistochemistry findings and the amyloid type assumed based on other clinical findings, mass spectrometry should be considered.
PubMed: 38872953
DOI: 10.1093/ehjcr/ytae264 -
Clinical Practice and Cases in... May 2024Ovarian artery aneurysm is a rare diagnosis, primarily associated with late pregnancy and the postpartum period. It can cause life-threatening hemorrhage when ruptured....
INTRODUCTION
Ovarian artery aneurysm is a rare diagnosis, primarily associated with late pregnancy and the postpartum period. It can cause life-threatening hemorrhage when ruptured. Even more rare are ovarian artery aneurysms in postmenopausal women.
CASE REPORT
We present a case of a postmenopausal female presenting to the emergency department with flank pain. Point-of-care ultrasound showed free fluid in the abdomen. She was diagnosed with an ovarian artery aneurysm on computed tomography angiography and treated successfully with embolization.
CONCLUSION
Ruptured ovarian artery aneurysm is an uncommon cause of intra-abdominal hemorrhage in women.
PubMed: 38869338
DOI: 10.5811/cpcem.1643 -
Cureus May 2024Here, we report a case of tardive peritonitis after endoscopic ultrasound (EUS)-guided transmural pancreatic pseudocyst drainage. A 50-year-old man was diagnosed with...
Here, we report a case of tardive peritonitis after endoscopic ultrasound (EUS)-guided transmural pancreatic pseudocyst drainage. A 50-year-old man was diagnosed with acute pancreatitis and a pancreatic pseudocyst measuring 5 cm. Ten months later, his pancreatic pseudocyst was 10 cm. We performed EUS-guided transmural drainage using a lumen-apposing metal stent. After two months, the stent was replaced with a double-pigtail plastic stent. Two months later, the patient developed fever and abdominal pain, and computed tomography revealed abdominal free air. He was diagnosed with peritonitis due to free air caused by a fistula rupture. The double-pigtail plastic stent was removed, and clipping was performed at the fistula site to achieve closure. The patient's symptoms subsequently improved. Long-term placement of a plastic stent for pancreatic pseudocysts makes recurrence less likely, but late adverse events due to stent placement can occur. Notably, fistula rupture can occur even when the fistula is well-formed several months after the initial drainage.
PubMed: 38868273
DOI: 10.7759/cureus.60179