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Journal of Clinical Medicine Sep 2023Cyclic neutropenia is a rare hematological condition characterized by periodic fluctuations in neutrophil counts, with a 21-day periodicity. Clinical presentation varies... (Review)
Review
Cyclic neutropenia is a rare hematological condition characterized by periodic fluctuations in neutrophil counts, with a 21-day periodicity. Clinical presentation varies from mild to severe forms of the disease, with the onset of recurrent fever, painful oral ulcers, recurrent bacterial infections, peritonitis, and septic shock. The availability of granulocyte colony-stimulating factor (G-CSF) has revolutionized the management and natural history of this disease, regulating the proliferation, differentiation, and maturation of the progenitor cells, and reducing the duration of neutropenia. Inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), is a group of chronic pathologies that affect the gastrointestinal tract. The onset of both diseases may be at a young age (even during childhood or adolescence), and clinical manifestations may lead to misdiagnosis, due to similar characteristics such as recurrent infections, oral ulcers, perianal abscesses, and infertility. Moreover, the two pathologies are rarely associated, with different management and therapeutic options. Here, we describe two case reports of patients who underwent surgery because of diagnosis of complicated CD. After surgery, due to persistent neutropenia, the hematologist consultant confirmed suspicions of cyclic neutropenia, and G-CSF therapy was started with benefits, underlining the crucial importance of proper differential diagnosis.
PubMed: 37834967
DOI: 10.3390/jcm12196323 -
Clinical Case Reports May 2024In the setting of Fournier's gangrene, atypical clinical manifestations and complications in an immunocompetent patient warrant consideration of perineal tuberculosis as...
KEY CLINICAL MESSAGE
In the setting of Fournier's gangrene, atypical clinical manifestations and complications in an immunocompetent patient warrant consideration of perineal tuberculosis as a potential underlying cause.
ABSTRACT
is a rare form of extrapulmonary tuberculosis that affects the perianal region. Fournier's gangrene is an aggressive necrotizing fasciitis that primarily involves the perianal area and external genitalia. A previously healthy 38-year-old man presented with a left perianal abscess. His condition deteriorated, leading to septic shock and multiorgan dysfunction syndrome. A CT scan displayed extensive necrotizing fasciitis. Treatment included broad-spectrum antibiotics, numerous surgical perineal debridements, a transverse loop colostomy, and hyperbaric oxygen therapy. We believe the patient had pre-existing asymptomatic, non-diagnosed perianal tuberculosis, and a subsequent bacterial superinfection resulted in a perineal local abscess that progressed to severe Fournier's gangrene. The diagnosis of tuberculosis was confirmed through positive cultures and molecular identification in perineal biopsies. The patient experienced a complex clinical course with complications such as myocardial necrosis, acute respiratory distress syndrome, rhabdomyolysis with severe critical illness polyneuromyopathy and internal jugular thrombosis. Fournier's gangrene resulted in air dissection throughout the perineal fasciae, extending to the abdominal wall muscles resulting in an infected extraperitoneal spontaneous hematoma, probably caused by therapeutic anticoagulation. An extraperitoneal surgical drainage was performed. This case emphasizes the complexities in diagnosing and managing both perianal tuberculosis and Fournier's gangrene.
PubMed: 38707606
DOI: 10.1002/ccr3.8882 -
Journal of Hematology Apr 2024Anorectal infections in neutropenic leukemia patients are a significant and potentially life-threatening complication. The pathogenesis of this condition is not entirely... (Review)
Review
Anorectal infections in neutropenic leukemia patients are a significant and potentially life-threatening complication. The pathogenesis of this condition is not entirely understood and believed to be multifactorial, including mucosal injury as a result of cytotoxic drugs, profound neutropenia and impaired host defense. Establishing an early diagnosis is key and often made clinically on the basis of signs and symptoms, but also from imaging studies demonstrating perianal inflammation or fluid collection. The management of anorectal infections in neutropenic leukemia patients is not straightforward, as there are no well-conducted studies on this entity. This review seeks to provide a framework into the pathophysiology and clinical presentation of anorectal infections in neutropenic leukemia patients, propose a diagnostic approach and to discuss controversies in the management of this condition.
PubMed: 38644983
DOI: 10.14740/jh1251 -
Medicine May 2024The efficacy of surgical intervention for perianal infection in patients with hematologic malignancies is not well established. Therefore, our study aimed to investigate...
The efficacy of surgical intervention for perianal infection in patients with hematologic malignancies is not well established. Therefore, our study aimed to investigate the clinical efficacy and complications of surgical treatment of perianal infection in patients with hematologic malignancies. This retrospective study included patients with hematological malignancies who were diagnosed with perianal infections and treated at the China Aerospace Science & Industry Corporation 731 Hospital between 2018 and 2022. Patient characteristics, hematological data, surgical intervention, and complications, including recurrence and mortality, were analyzed. This study included 156 patients with leukemia aged 2 months to 71 years who were treated surgically for perianal infection, comprising 94 males and 62 females. Perianal infection included 36 cases of abscesses, 91 anal fistulas, and 29 anal fissures accompanied by infection. A total of 36 patients developed severe complications postoperatively, including 4 patients who died, 6 patients with severe incision bleeding, 18 patients with severe pain, 6 patients with sepsis, 12 patients who needed reoperation, 15 patients with hospitalization for more than 2 weeks, and 3 patients with anal stenosis; none of the patients developed anal incontinence. Additionally, risk factors for postoperative complications of perianal infection in patients with hematologic malignancies include leukopenia, agranulocytosis, thrombocytopenia, depth of abscess and not undergone an MRI. Surgical intervention may improve the prognosis of patients with perianal abscess formation, particularly in patients who show no improvement with medical therapy and those who develop perianal sepsis. Granulocytopenia and thrombocytopenia should be improved before surgery, which can significantly reduce postoperative complications. Although these findings are from a case series without a comparator, they may be of value to physicians because to the best of our knowledge, no randomized or prospective studies have been conducted on the management of perianal infections in patients with hematological malignancies.
Topics: Humans; Male; Female; Retrospective Studies; Middle Aged; Adult; Aged; Hematologic Neoplasms; Abscess; Adolescent; Child; Young Adult; Anus Diseases; Child, Preschool; Postoperative Complications; Infant; Rectal Fistula; Treatment Outcome; Fissure in Ano
PubMed: 38728504
DOI: 10.1097/MD.0000000000038082 -
Cureus Nov 2023Background Diagnosis and management of perianal abscesses (PAA) are based on history and clinical examination. Imaging is not indicated except in complicated cases, as...
Background Diagnosis and management of perianal abscesses (PAA) are based on history and clinical examination. Imaging is not indicated except in complicated cases, as determined by the surgical team. The monetary, ionizing radiation, and resource utilization costs of a computed tomography (CT) scan in the emergency room must be considered when used for diagnostic purposes of PAAs. Methods A retrospective analysis of 129 patients diagnosed with a diagnosis of PAA between 2015-2020 was performed. The primary endpoints included length of stay, CT performed, time from patient presentation to CT, and CT scan completion prior to surgical consultation. Data is reported as n (%) or median (IQR). Results Of the 129 patients diagnosed with PAA, 81 underwent CT, and 48 did not. General surgery was consulted in 88% of cases. There were no statistically significant differences in age (p=0.562), sex (p=0.531), or ethnicity (p=0.356). The median hospitalization time was two days when CT was performed (p=0.001). The median time elapsed from presentation to the emergency department and CT scan performed was 16 hours (p=0.001). CT scans were ordered before the surgical consultation in 65% of cases (p=0.001) and 17% after a surgical consultation was placed (p=0.009). Conclusion Performing CT scans prior to surgical evaluation for the diagnosis of PAA is not a responsible practice. The cost, resources, and radiation exposure must be considered. This study demonstrated that more CT scans are ordered prior to surgical consultation for PAA, resulting in a prolonged wait time in the emergency department.
PubMed: 38149138
DOI: 10.7759/cureus.49417 -
World Journal of Gastroenterology Oct 2023Treatment of infantile-onset inflammatory bowel disease (IO-IBD) is often challenging due to its aggressive disease course and failure of standard therapies with a need...
BACKGROUND
Treatment of infantile-onset inflammatory bowel disease (IO-IBD) is often challenging due to its aggressive disease course and failure of standard therapies with a need for biologics. Secondary loss of response is frequently caused by the production of anti-drug antibodies, a well-known problem in IBD patients on biologic treatment. We present a case of IO-IBD treated with therapeutic drug monitoring (TDM)-guided high-dose anti-tumor necrosis factor therapy, in which dose escalation monitoring was used as a strategy to overcome anti-drug antibodies.
CASE SUMMARY
A 5-mo-old boy presented with a history of persistent hematochezia from the 10 d of life, as well as relapsing perianal abscess and growth failure. Hypoalbuminemia, anemia, and elevated inflammatory markers were also present. Endoscopic assessment revealed skip lesions with deep colic ulcerations, inflammatory anal sub-stenosis, and deep fissures with persistent abscess. A diagnosis of IO-IBD Crohn-like was made. The patient was initially treated with oral steroids and fistulotomy. After the perianal abscess healed, adalimumab (ADA) was administered with concomitant gradual tapering of steroids. Clinical and biochemical steroid-free remission was achieved with good trough levels. After 3 mo, antibodies to ADA (ATA) were found with undetectable trough levels; therefore, we optimized the therapy schedule, first administering 10 mg weekly and subsequently up to 20 mg weekly (2.8 mg/kg/dose). After 2 mo of high-dose treatment, ATA disappeared, with concomitant high trough levels and stable clinical and biochemical remission of the disease.
CONCLUSION
TDM-guided high-dose ADA treatment as a monotherapy overcame ATA production. This strategy could be a good alternative to combination therapy, especially in very young patients.
Topics: Male; Humans; Adalimumab; Abscess; Neoplasm Recurrence, Local; Inflammatory Bowel Diseases; Antibodies; Steroids; Infliximab
PubMed: 37900586
DOI: 10.3748/wjg.v29.i38.5428 -
A systematic review and meta-analysis of the use of packing in the management of perianal abscesses.Annals of the Royal College of Surgeons... Apr 2024Perianal abscesses are common presentations and reasons for emergency general surgery admissions. Management involves incision and drainage of the abscess and packing...
BACKGROUND
Perianal abscesses are common presentations and reasons for emergency general surgery admissions. Management involves incision and drainage of the abscess and packing the cavity with internal wound dressings. This meta-analysis aimed to assess in adults if packing an abscess or leaving it unpacked leads to a significant difference in the outcomes of pain on wound dressing, time to healing, rate of fistulation and abscess recurrence.
METHODS
Randomised controlled trials (RCTs) with participants aged 18 years or older that compared packing of perianal abscess cavities with no packing between 2002 and 2022 were searched for in December 2022 on OVID Medline and Embase, the CENTRAL register of trials, PubMed and Google Scholar. Risk of bias was assessed using the Cochrane Risk of Bias tool. Random effects meta-analysis was conducted on the data extracted.
RESULTS
Three RCTs involving 490 patients were analysed for the outcomes of abscess recurrence and postoperative fistula formation; the data were not adequate to assess pain on dressing and time to healing. For unpacked versus packed, the pooled relative risk of abscess recurrence was 1.57 (95% confidence interval (CI) 0.764, 3.29, =0.219) and for fistula formation 0.686 (95% CI 0.430, 1.09, =0.114). These results suggest there is no significant benefit to packing abscess cavities.
CONCLUSIONS
Analysis of the outcomes suggests there is no significant difference with regards to rates of abscess recurrence or fistula formation between the packed and unpacked groups; however, appropriately powered RCTs are required in this area to provide more primary evidence to inform best practice and clinical management.
PubMed: 38563064
DOI: 10.1308/rcsann.2023.0108 -
Asian Journal of Surgery Aug 2023
Topics: Humans; Crohn Disease; Abscess; Anus Diseases; Rectal Fistula; Patients
PubMed: 36990810
DOI: 10.1016/j.asjsur.2023.03.032 -
World Journal of Clinical Cases Aug 2023Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute, self-limiting vasculitis of unknown aetiology that mainly involves the medium and...
BACKGROUND
Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute, self-limiting vasculitis of unknown aetiology that mainly involves the medium and small arteries and can lead to serious cardiovascular complications, with a 25% incidence of coronary artery aneurysms. Periton-Sillar abscesses are a rare symptom of KD and is easily misdiagnosed at its early stages.
CASE SUMMARY
A 5-year-old boy who presented to a community hospital with a 3-d fever, difficulty in opening his mouth, and neck pain and was originally treated for throat infection without improvement. On the basis of laboratory tests, ultrasound of submandibular and superficial lymph nodes and computed tomography of the neck, the clinician diagnosed the periamygdala abscess and sepsis that did not resolve after antibiotic therapy. On the fifth day of admission, the child developed conjunctival congestion, prune tongue, perianal congestion and desquamation, and slightly stiff and swollen bunions on both feet. A diagnosis of KD was reached with complete remission after intravenous immunoglobulin treatment.
CONCLUSION
Children with neck pain, lymph node enlargement, or airway obstruction as the main manifestations are poorly treated with intravenous broad-spectrum antibiotics. Clinicians should not rush invasive operations such as neck puncture, incision, and drainage and should be alert for KD when it cannot be explained by deep neck space infection and early treatment with aspirin combined with gammaglobulin.
PubMed: 37621581
DOI: 10.12998/wjcc.v11.i22.5391 -
The Korean Journal of Internal Medicine May 2024A poor prognostic factor for Crohn's disease (CD) includes perianal fistulizing disease, including perianal fistula and/or perianal abscess. Currently, a tool to assess...
BACKGROUND/AIMS
A poor prognostic factor for Crohn's disease (CD) includes perianal fistulizing disease, including perianal fistula and/or perianal abscess. Currently, a tool to assess perianal symptoms in patients with CD remains nonexistent. This study aimed to develop a perianal fistulizing disease self-screening questionnaire for patients with CD.
METHODS
This prospective pilot study was conducted at three tertiary referral centers between January 2019 and May 2020. We formulated questions on perianal symptoms, including tenesmus, anal discharge, bleeding, pain, and heat. A 4-point Likert scale was used to rate each question. Patients with CD completed a questionnaire and underwent pelvic magnetic resonance imaging (MRI).
RESULTS
Overall, 93 patients were enrolled, with 51 (54.8%) diagnosed with perianal fistulizing disease, as determined by pelvic MRI. The Spearman correlation findings demonstrated that anal pain (p = 0.450, p < 0.001) and anal discharge (p = 0.556, p < 0.001) were the symptoms that most significantly correlated with perianal disease. For anal pain and discharge, the area under the receiver operating characteristic curve of the scores was significantly higher than that of the combined score for all five symptoms (0.855 vs. 0.794, DeLong's test p = 0.04). For the two symptoms combined, the sensitivity, specificity, and positive predictive and negative predictive values were 88.2, 73.8, 80.4, and 83.8%, respectively, with 81.7% accuracy for detecting perianal fistulizing disease.
CONCLUSION
This study indicates that simple questions regarding anal pain and discharge can help accurately identify the presence of perianal fistulizing disease in patients with CD.
Topics: Humans; Crohn Disease; Male; Female; Adult; Rectal Fistula; Prospective Studies; Pilot Projects; Magnetic Resonance Imaging; Middle Aged; Predictive Value of Tests; Surveys and Questionnaires; Young Adult; Reproducibility of Results
PubMed: 38576234
DOI: 10.3904/kjim.2023.410