Clinical Manifestations Research Articles

2 Cases of Intra-Labyrinthine Schwannomas With Different Treatment Options Based on Clinical Presentation

Vestibular schwannoma is a benign tumor that arises from the eighth cranial nerve and is the most common neoplasm in the internal auditory canal (IAC). It typically arises in the cerebellopontine angle or in IAC; however, in rare cases, they can arise within the labyrinth. These tumors arising in the labyrinth have been termed intra-labyrinthine schwannomas (ILS) and have different clinical features than the typical vestibular schwannomas. Clinical manifestations of an ILS typically include hearing loss, tinnitus, and vertigo, with unusual symptoms including a sense of fullness or imbalance. Temporal bone MRI is considered the most important test for diagnosing ILS. Tumors show nodular enhancement on T1-weighted images and filling defects on T2-weighted images. Treatment of ILS may include observation, microsurgical resection, and stereotactic radiosurgery depending on the location, size, and clinical presentation. In this paper, we present two cases diagnosed as ILS with different treatment options and provide a literature review for each case.

Anti-Th/To Antibodies in Scleroderma: Good Prognosis or Serious Concern?

Systemic sclerosis (SSc) represents a rare and intricate autoimmune connective tissue disease, the pathophysiology of which has not been fully understood. Its key features include progressive fibrosis of the skin and internal organs, vasculopathy and aberrant immune activation. While various anti-nuclear antibodies can serve as biomarkers for the classification and prognosis of SSc, their direct role in organ dysfunction remains unclear. Anti-Th/To antibodies are present in approximately 5% of SSc patients, and are particularly prevalent among those with the limited subtype of the disease. Although the presence of these autoantibodies is associated with a mild course of the disease, there is a strong connection between them and severe clinical manifestations of SSc, including interstitial lung disease, pulmonary arterial hypertension and gastrointestinal involvement. Also, the additional clinical correlations, particularly with malignancies, need further research. Moreover, the disease’s course seems to be influenced by antibodies, specific serum cytokines and TLR signaling pathways. Understanding the relationships between presence of anti-Th/To, its molecular aspects and response to treatment options is crucial for the development of novel, personalized therapeutic techniques and should undergo profound analysis in future studies.

Clinical Outcomes of Utilizing a "W"-shaped Incision in the Management of Forehead Skin Thickening Induced by Hypertrophic Osteoarthropathy.

Hypertrophic osteoarthropathy (HOA) is a rare and intricate hereditary disease. The appearance and functional deformity of the forehead caused by thickened folds are the main clinical manifestations of patients with hypertrophic osteoarthropathy. The cause of this disease is still unknown. Currently, surgical treatment has become one of the best strategies, mainly for improving the appearance of the forehead. There has been no literature report on the use of "W"-shaped skin flap resection for thickened forehead skin in patients with hypertrophic osteoarthropathy. All cases of hypertrophic osteoarthropathy in our department in the last 7 years, and previous literature on hypertrophic osteoarthropathy, were reviewed. A total of 5 cases of hypertrophic osteoarthropathy in our department (mean age 21 years, all male patients) were reviewed. All patients underwent open surgery to remove the thickened skin on the forehead or the wrinkles and gyrus-shaped scalp. The jagged skin tissue was removed (8-9) cm×(1-2.5) cm×0.5 cm. The folds and thickness of the frontal skin of the patients were greatly improved after the operation. Patient satisfaction with the treatment outcomes was unanimous. However, one case experienced a postoperative wound infection during follow-up. The utilization of the "W"-shaped excision technique allowed for the maximal removal of excessively diseased tissue, thereby facilitating a smoother resolution of the depression. A total of 5 cases of hypertrophic osteoarthropathy were treated in our department, and all of them underwent frontal skin "W"-shaped excision, which was safe, feasible, and practical, and the postoperative results were satisfactory. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Spinal myeloid sarcoma presenting as initial symptom in acute promyelocytic leukemia with a rare cryptic PLZF::RARα fusion gene: a case report and literature review

BackgroundAcute promyelocytic leukemia (APL) is rarely caused by the PLZF::RARα fusion gene. While APL patients with PLZF::RARα fusion commonly exhibit diverse hematologic symptoms, the presentation of myeloid sarcoma (MS) as an initial manifestation is infrequent.Case presentationA 61-year-old patient was referred to our hospital with 6-month history of low back pain and difficulty walking. Before this admission, spine magnetic resonance imaging (MRI) conducted at another hospital revealed multiple abnormal signals in the left iliac bone and vertebral bodies spanning the thoracic (T11-T12), lumbar (L1-L4), and sacral (S1/S3) regions. This led to a provisional diagnosis of bone tumors with an unknown cause. On admission, complete blood count (CBC) test and peripheral blood smear revealed a slightly increased counts of monocytes. Immunohistochemical staining of both spinal and bone marrow (BM) biopsy revealed positive expression for CD117, myeloperoxidase (MPO), and lysozyme. BM aspirate showed a significant elevation in the percentage of promyelocytes (21%), which were morphologically characterized by round nuclei and hypergranular cytoplasm. Multiparameter flow cytometry of BM aspirate revealed that blasts were positive for CD13, CD33, CD117, and MPO. Through the integrated application of chromosome analysis, fluorescence in situ hybridization (FISH), reverse transcriptase polymerase chain reaction (RT-PCR), and Sanger sequencing, it was determined that the patient possessed a normal karyotype and a rare cryptic PLZF::RARα fusion gene, confirming the diagnosis of APL.ConclusionIn the present study, we report the clinical features and outcome of a rare APL patient characterized by a cryptic PLZF::RARα fusion and spinal myeloid sarcoma (MS) as the initial presenting symptom. Our study not only offers valuable insights into the heterogeneity of APL clinical manifestations but also emphasizes the crucial need to promptly consider the potential link between APL and MS for ensuring a timely diagnosis and personalized treatments.

The relationship between food selectivity and stature in pediatric patients with avoidant-restrictive food intake disorder – an electronic medical record review

BackgroundWe aimed to characterize stature in pediatric patients with avoidant/restrictive food intake disorder (ARFID), including associations between body size and nutrient intake and height.MethodsWe conducted a secondary analysis of pre-treatment data from 60 patients diagnosed with ARFID that were collected from the electronic medical record. Anthropometric measurements were converted to age- and sex-specific Z-scores using pediatric CDC growth charts. Spearman correlations were performed to test the relationship between height and weight/BMI Z-scores as well as height Z-score and diet variables.ResultsOn average, height (-0.35 ± 1.38), weight (-0.58 ± 1.56), and BMI (-0.56 ± 1.48) Z-scores tended to be lower than what would be expected in a generally healthy pediatric population. Percent of individuals with height, weight, or BMI Z-score < -2.0 was 8%, 20%, and 17%, respectively. BMI (P < 0.05) and weight (P < 0.05) were positively associated with height Z-score. Further, intake of some nutrients (e.g., calcium, vitamin D) correlated positively with height Z-score (all P < 0.05).ConclusionsThe cross-sectional relationships reported in this study suggest that in children with ARFID, body weight and consumption of bone-augmenting nutrients such as calcium and vitamin D correlated with height. A thorough understanding of the clinical manifestations of malnutrition and longitudinal effects of restrictive eating in patients with ARFID is critical.

Endocardial Fibroelastosis: A Comprehensive Review of Pathogenesis, Clinical Manifestations, Diagnostic Modalities, and Management Strategies

Endocardial fibroelastosis (EFE) is a rare and enigmatic myocardial disease characterized by the abnormal accumulation of collagen and elastin fibers in the endocardium, predominantly affecting the left ventricle. This comprehensive review aims to elucidate the pathogenesis, clinical manifestations, diagnostic modalities, and management strategies of EFE. EFE typically presents in infants and young children, although adult cases have been reported. The etiology of EFE remains elusive, with theories implicating genetic, infectious, and autoimmune factors. Clinically, EFE may manifest with symptoms of heart failure, arrhythmias, or sudden cardiac death. Diagnosis often involves echocardiography, cardiac magnetic resonance imaging, and endomyocardial biopsy. Management strategies for EFE are primarily supportive, focusing on the management of heart failure symptoms and arrhythmias, with advanced cases necessitating heart transplantation. This review provides a comprehensive overview of EFE, highlighting the challenges in diagnosis and management and underscoring the need for further research to elucidate its pathogenesis and improve outcomes.

Sweet Syndrome: A Comprehensive Review of Pathogenesis, Clinical Manifestations, Diagnosis, and Management Strategies

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare and inflammatory disorder characterized by fever, neutrophilia, and tender, erythematous skin lesions. The pathogenesis of Sweet syndrome involves an exaggerated immune response, with evidence suggesting a role for cytokines, particularly granulocyte colony-stimulating factor (G-CSF). Clinical manifestations vary widely, but commonly include abrupt onset of painful, raised plaques or nodules, often on the upper limbs, face, or neck. Histopathological examination reveals dense neutrophilic infiltrates in the dermis without evidence of vasculitis. Diagnosis is primarily clinical, supported by histopathology and exclusion of other similar conditions. Treatment typically involves corticosteroids, which often lead to rapid resolution of symptoms. However, relapses are common, necessitating long-term management strategies. This review aims to provide a comprehensive overview of the pathogenesis, clinical manifestations, diagnosis, and management of Sweet syndrome, highlighting recent advancements and areas requiring further research.

Cutaneous Schwannomas: Clinical Manifestations, Diagnosis and Management: A Review

Cutaneous Schwannomas: Clinical Manifestations, Diagnosis and Management: A Review

Neurological involvement in hematopoietic stem cell transplantation-associated thrombotic microangiopathy.

Transplantation-associated thrombotic microangiopathy (TA-TMA) is a well-recognized serious complication of hematopoietic stem cell transplantation (HSCT). The understanding of TA-TMA pathophysiology has expanded in recent years. Dysregulation of the complement system is thought to cause endothelial injury and, consequently, microvascular thrombosis and tissue damage. TA-TMA can affect multiple organs, and each organ exhibits specific features of injury. Central nervous system (CNS) manifestations of TA-TMA include posterior reversible encephalopathy syndrome, seizures, and encephalopathy. The development of neurological dysfunction is associated with a significantly lower overall survival in patients with TA-TMA. However, there are currently no established histopathological or radiological criteria for the diagnosis of CNS TMA. Patients who receive total body irradiation (TBI), calcineurin inhibitors (CNI), and severe acute and chronic graft-versus-host disease (GVHD) are at a high risk of experiencing neurological complications related to TA-TMA and should be considered for directed TA-TMA therapy. However, the incidence and clinical manifestations of TA-TMA neurotoxicity remain unclear. Studies specifically examining the involvement of CNS in TMA syndromes are limited. In this review, we discuss clinical manifestations and imaging abnormalities in patients with nervous system involvement in TA-TMA. We summarize the mechanisms underlying TA-TMA and its neurological complications, including endothelial injury, evidence of complement activation, and treatment options for TA-TMA.

Benign convulsions with mild gastroenteritis in children: An emerging acute symptomatic seizures

AbstractBenign convulsions with mild gastroenteritis (CwG) characterized by afebrile seizures that occurred in the acute period of mild gastroenteritis often occur in infancy and toddlers. Until now, it has not been well acknowledged and thereby classified by the International League against Epilepsy (ILAE) as epilepsy syndrome or acute symptomatic seizures (ASS). Thus far, accumulating data suggest that CwG could fit all the mandatory criteria of ASS from the aspects of pathogenesis, clinical manifestations, and outcomes, rather than epilepsy in spite of the rare cases developing epilepsy over time. This review provides a comprehensive picture of this entity aiming to facilitate the pediatricians, particularly for general practitioners, to better recognize this unique entity and, ultimately, to minimize unnecessary evaluation and treatment.

The Miami Framework for ALS and related neurodegenerative disorders: an integrated view of phenotype and biology.

Increasing appreciation of the phenotypic and biological overlap between amyotrophic lateral sclerosis (ALS) and frontotemporal dementia, alongside evolving biomarker evidence for a pre-symptomatic stage of disease and observations that this stage of disease might not always be clinically silent, is challenging traditional views of these disorders. These advances have highlighted the need to adapt ingrained notions of these clinical syndromes to include both the full phenotypic continuum - from clinically silent, to prodromal, to clinically manifest - and the expanded phenotypic spectrum that includes ALS, frontotemporal dementia and some movement disorders. The updated clinical paradigms should also align with our understanding of the biology of these disorders, reflected in measurable biomarkers. The Miami Framework, emerging from discussions at the Second International Pre-Symptomatic ALS Workshop in Miami (February 2023; a full list of attendees and their affiliations appears in the Supplementary Information) proposes a classification system built on: first, three parallel phenotypic axes - motor neuron, frontotemporal and extrapyramidal - rather than the unitary approach of combining all phenotypic elements into a single clinical entity; and second, biomarkers that reflect different aspects of the underlying pathology and biology of neurodegeneration. This framework decouples clinical syndromes from biomarker evidence of disease and builds on experiences from other neurodegenerative diseases to offer a unified approach to specifying the pleiotropic clinical manifestations of disease and describing the trajectory of emergent biomarkers.

Delayed infected hematoma in retrosternal area following total thyroidectomy: a case report

Introduction and importance: Postoperative neck hematoma (PNH), a rare complication following thyroidectomy, occurs in only 1.1%-3.15% of cases and can lead to life-threatening outcomes. More rarely, delayed PNHs with atypical clinical manifestations and positions have not yet been reported. Early identification and immediate medical intervention are of utmost importance in such cases. Case presentation: The authors represented a patient with thyroid cancer adherent to the trachea, who underwent post-thyroidectomy, experienced delayed PNH in the retrosternal region and was infected by respiratory pathogens. Meanwhile, the patient developed recurrent laryngeal nerve (RLN) paralysis after surgery. PNH was not identified in the clinical manifestations; instead, it was detected only through successive cervical ultrasound examinations. Clinical discussion: Although rare, PNH can lead to serious complications, especially delayed complications or those in atypical positions, without neck swelling. When simultaneously with RLN paralysis, the hematoma may be neglected. Therefore, early diagnosis and treatment are crucial. Conclusion: Clinicians should be vigilant of atypical PNH because neck swelling may be absent. Cervical ultrasonography is essential for diagnosis and can be performed multiple times. Cervical CT scans should be part of the routine procedure, while contrast-enhanced ultrasound can help detect active bleeding. Early postoperative antibiotics are recommended if the tumor is closely attached to the trachea.

Survey of patient satisfaction with genetic counseling services in Korea.

Since the 1990s, genetic clinics have been established in South Korea, enabling the provision of clinical genetics services. However, genetic counseling services are not widely used in the medical system. In contrast, recently, the demand for genetic counseling has increased due to the rapid development of genomic medicine. Therefore, it is important for medical geneticists and genetic counselors to collaboratively provide genetic counseling services. This study aimed to evaluate the perception and satisfaction of patients with rare genetic diseases and their families regarding genetic counseling services provided by a genetics team at the medical genetics center of a tertiary general hospital for rare genetic diseases. From April to November 2021, a survey was conducted with 203 individuals, including 111 and 92 individuals in the patient and family groups, respectively. Overall, 164 individuals (80.8%) responded that they were aware of genetic counseling services, and 135 individuals (66.5%) responded that they were aware of the role of genetic counselors. Patients and their families wanted to receive information about the following from genetic counseling: clinical manifestation and prognosis of the diagnosed disease (78.8%), treatment and management of the disease (60.6%), risk of recurrence within the family (55.7%), treatment options and alternatives for family and prenatal testing, and various support services. The score of satisfaction with genetic counseling services provided by the genetics team was 8.19 ± 1.68 out of 10. Patients with rare genetic diseases and their families were satisfied with genetic counseling services regarding their diseases, test results, and treatment options. Moreover, the patients could receive psychosocial support and referrals to other medical service providers and support services. As a genetic team approach, collaboration between medical geneticists and certified genetic counselors would be useful in providing information and in diagnosing, treating, and managing patients.

Efficacy and safety of telitacicept therapy in systemic lupus erythematosus with hematological involvement.

To evaluate the efficacy and safety of telitacicept in SLE patients specifically with hematological involvement. A total of 22 patients with SLE and hematological involvement were included in this study. These patients received telitacicept in addition to standard therapy. We compared their demographic characteristics, clinical manifestations, and laboratory indicators before and after the administration of telitacicept. A total of 22 patients received telitacicept treatment for a median duration of 10.4months (ranging from 6 to 19months). Following telitacicept therapy, significant improvements were observed in various parameters compared to baseline. Specifically, white blood cell count increased from (3.98 ± 1.80) 109/L to (6.70 ± 2.47) 109/L, (P = 0.002), hemoglobin levels increased from (100 ± 19) g/L to (125 ± 22) g/L, (P < 0.001), and platelet count increased from (83 ± 60) 109/L to (161 ± 81) 109/L, (P = 0.004). SLE Disease Activity Index (SLEDAI) scores decreased from 12(5,15) to 0(0,4), (P < 0.001). Additionally, C3 and C4 levels showed improvement. Telitacicept treatment also resulted in a significant reduction in serum IgG levels and daily prednisone dosage. Only one adverse event (4.5%) was reported during the treatment, which was a urinary tract infection. The combination of telitacicept and standard treatment demonstrated significant improvements in anemia, as well as increased leukocyte and platelet levels in patients with SLE and hematological involvement. Importantly, the observed adverse events were manageable and controllable. Key Points • Telitacicept effectively improves anemia, clinical outcomes, and increases leukocyte and platelet counts. • Treatment with telitacicept leads to decreased levels of lgG, IgA, anti-dsDNA, and SLEDAI scores, while serum complement C3 and C4 returned to normal. • During the follow-up period there were observed changes in individual parameters, clinical symptoms, and organ involvement, all without significant adverse events.

Improving the Yield of Genetic Diagnosis through Additional Genetic Panel Testing in Hereditary Ophthalmic Diseases.

Numerous hereditary ophthalmic diseases display significant genetic diversity. Consequently, the utilization of gene panel sequencing allows a greater number of patients to receive a genetic diagnosis for their clinical manifestations. We investigated how to improve the yield of genetic diagnosis through additional gene panel sequencing in hereditary ophthalmic diseases. A gene panel sequencing consisting of a customized hereditary retinopathy panel or hereditary retinitis pigmentosa (RP) panel was prescribed and referred to a CAP-accredited clinical laboratory. If no significant mutations associated with hereditary retinopathy and RP were detected in either panel, additional gene panel sequencing was requested for research use, utilizing the remaining panel. After additional gene panel sequencing, a total of 16 heterozygous or homozygous variants were identified in 15 different genes associated with hereditary ophthalmic diseases. Of 15 patients carrying any candidate variants, the clinical symptoms could be tentatively accounted for by genetic mutations in seven patients. However, in the remaining eight patients, given the in silico mutation predictive analysis, variant allele frequency in gnomAD, inheritance pattern, and genotype-phenotype correlation, fully elucidating the clinical manifestations with the identified rare variant was challenging. Our study highlights the utility of gene panel sequencing in achieving accurate diagnoses for hereditary ophthalmic diseases and enhancing the diagnostic yield through additional gene panel sequencing. Thus, gene panel sequencing can serve as a primary tool for the genetic diagnosis of hereditary ophthalmic diseases, even in cases where a single genetic cause is suspected. With a deeper comprehension of the genetic mechanisms underlying these diseases, it becomes feasible.

MYCOLOGICAL ANALYSIS OF CYSTIC CONTENT IN PATIENTS WITH POLYMICROCYSTIC CHANGES OF THE MAXILLARY SINUS MUCOSA

The prevalence of mycotic infections of the paranasal sinuses is constantly increasing worldwide. Pathogens can enter the sinuses through the respiratory tract that leads to the colonization of microorganisms in both the lungs and paranasal sinuses as part of the normal microflora. The presence of filamentous fungi in the maxillary sinus may result from endodontic treatment of molars directly adjacent to the sinus, increasing the risk of fungal foci development. Fungal paranasal sinusitis manifests in invasive or non-invasive forms. While mycological culture remains the traditional gold standard for mycosis diagnosis, its sensitivity is reportedly low. Paranasal sinus cysts comprise 4-8% of general ENT pathologies and approximately 4-16% of chronic paranasal sinus diseases, with a predominant presence in the maxillary sinus. Factors that contribute to the development of sinusitis and create favorable conditions for fungal spore adhesion and growth include impaired ventilation and transport function of the sinus mucosa, inadequate use of broad-spectrum antibacterial drugs and steroid therapy, as well as household factors. Recently, there has been an increase in the number of cases of multiple small cysts in maxillary sinuses. Clinical manifestations range from asymptomatic incidental findings to varying degrees of discomfort and/or pain localized in the maxillary sinus area. The aim of this study is to identify the involvement of a fungal agent and to develop a set of therapeutic and prophylactic measures for polymicrocystic changes in the mucous membrane of the maxillary sinuses. The study enrolled 37 patients aged 18 to 65 years. Atypical cystic content was collected and cultured in Sabouraud's nutrient broth and Amies transport medium at 35-37°C for 5-7 days, with final measurements on day 21. Treatment involved a 14-day regimen of itraconazole (100 mg once daily) alongside topical miramistin (0.01% solution, 2 ml) sinus injections. The findings obtained demonstrate the following: positive fungal growth was observed in 26 cases (70.3%), predominantly Penicillium (48.6%) and Aspergillus (21.6%). Absence of growth was noted in 11 cases (29.7%). Follow-up examinations in 6 months and in a year revealed no crusts in the nasal cavity, moderate mucous discharge in the sinuses, and no polymicrocystic changes. Conclusion. The analysis has demonstrated the involvement of fungal flora in the development and recurrence of cystic changes in the maxillary sinus mucosa, advocating for mycological examination alongside bacteriological analysis. Itraconazole for systemic therapy and miramistin for topical therapy demonstrate efficacy in treatment and recurrence prevention.

Characterization of antibody response to SARS-CoV-2 Orf8 from three waves of COVID-19 outbreak in Thailand.

A dynamic of virus adaptation and a mass vaccination campaign could significantly reduce the severity of clinical manifestations of COVID-19 and transmission. Hence, COVID-19 may become an endemic disease globally. Moreover, mass infection as the COVID-19 pandemic progressed affected the serology of the patients as a result of virus mutation and vaccination. Therefore, a need exists to acquire accurate serological testing to monitor the emergence of new outbreaks of COVID-19 to promptly prevent and control the disease spreading. In this study, the anti-Orf8 antibodies among samples collected in Thailand's first, fourth, and fifth waves of COVID-19 outbreaks compared with pre-epidemic sera were determined by indirect ELISA. The diagnostic sensitivity and specificity of the anti-Orf8 IgG ELISA for COVID-19 samples from the first, fourth, and fifth waves of outbreaks was found to be 100% compared with pre-epidemic sera. However, the diagnostic sensitivity and specificity of the anti-Orf8 IgG ELISA for a larger number of patient samples and controls from the fifth wave of outbreaks which were collected on day 7 and 14 after an RT-PCR positive result were 58.79 and 58.44% and 89.19 and 58.44%, respectively. Our data indicated that some of the controls might have antibodies from natural past infections. Our study highlighted the potential utility of anti-Orf8 IgG antibody testing for seroprevalence surveys but still warrants further investigations.

A rare case of pheochromocytoma in a pregnant woman presenting with chest pain: extraordinary management

BackgroundPheochromocytoma is rare in pregnant women. It presents as diverse symptoms, including hypertension and sweating. The symptoms of pregnant women with pheochromocytoma and comorbid hypertension often mimic the clinical manifestations of preeclampsia, and these women are often misdiagnosed with preeclampsia.Case presentationIn this case, a pregnant woman presented with chest pain as the primary symptom, and a diagnosis of pheochromocytoma was considered after ruling out myocardial ischemia and aortic dissection with the relevant diagnostic tools. This patient then underwent successful surgical resection using a nontraditional management approach, which resulted in a positive clinical outcome.ConclusionsIt is essential to consider pheochromocytoma as a potential cause of chest pain and myocardial infarction-like electrocardiographic changes in pregnant women, even if they do not have a history of hypertension.

Duodenal-Jejunal Stenosis as a Manifestation of Eosinophilic Gastroenteritis: A Case Report

Introduction: Eosinophilic gastroenteritis (EGE) is a rare disorder characterized by eosinophilic infiltration of the stomach and small intestine. Its clinical manifestations depend on the extent, location, and depth of the inflammatory infiltrate. The pathogenesis remains unclear, but it may be related to food allergy or other hypersensitivity reactions in some patients. Untreated cases can cause intestinal stricture or perforation, making timely diagnosis and intervention essential. Clinical case: A 13-year-old male adolescent was hospitalized due to symptoms suggesting intestinal obstruction or alterations in intestinal motility. Medical imaging revealed significant gastric distension and delayed gastric emptying. An endoscopic study showed a “snake-skin” pattern, duodenitis due to food stasis, a 2 cm ulcer, and stenosis in the duodenum. The biopsy confirmed EGE with significant irregular eosinophilic infiltration of the lamina propria. A panendoscopy was performed after steroid treatment without being able to assess the site of stenosis and perform pneumatic dilation. After completing the immunomodulatory management, a contrast-enhanced abdominal tomography was performed showed a significant decrease in the intestinal lumen, therefore an exploratory laparotomy with a lateral to lateral jejune-jejunal anastomosis was performed. Discussion: EGE is a rare inflammatory disorder characterized by eosinophilic infiltration of the stomach and small intestine. Symptoms are based on the affected gastrointestinal tract segment. In this case, our patient presented intestinal obstruction symptoms with endoscopic biopsies and a histopathological study confirmed eosinophil infiltration. Treatment typically involves identifying potential triggers and corticosteroids to reduce inflammation and symptoms. However, in this case, a decrease in the intestinal lumen was observed, therefore an elective surgical resection was performed. Conclusion: Eosinophilic gastroenteritis is a rare disorder that requires strong clinical suspicion to be diagnosed, which is why its knowledge is important. In this case, gastrointestinal symptoms, radiological and endoscopic images, in addition to the histopathological report, were key tools for integrating the diagnosis. Although management with steroids and immunomodulators was not successful, surgical management became an appropriate option as definitive treatment.

Clinical characteristics and treatment outcomes of skin and soft tissue infections in children: a prospective study

ABSTRACT Backgroundː This study addresses the elevated prevalence of skin and soft tissue infections (SSTIs) within the pediatric demographic, necessitating a comprehensive inquiry into their varied clinical manifestations. The primary objectives involve the assessment of clinical and laboratory characteristics of SSTIs in children and an exploration of their correlation with treatment efficacy. Methodsː From April 2022 to June 2023, a prospective observational study was conducted on 87 pediatric bacterial SSTI cases at a Tertiary Paediatric Centre in Central Vietnam. Resultsː SSTIs were most common in children ages 1-4, 48.3%. Seasonal trends showed a peak incidence in summer (43.7%). In 93.1% of patients, febrile presentation and edema predominated. About 40.2% of people had lower extremity involvement. Analysis showed significant rates of severe (73.6%) and purulent (67.8%) SSTIs. Leukocytosis was found in 39.1% of patients, mostly due to 72.4% increased CRP. Even though only 2.4% of blood cultures were positive, Staphylococcus aureus caused 76.9% of infections. About 42.5% of patients had shorter treatments. Significantly, purulent SSTIs required longer antibiotic therapy than nonpurulent infections (p &lt; 0.05). 27.6% of children needed surgery, and most received intravenous antibiotics. Most importantly, all patients recovered. Conclusionsː Predominantly observed among pediatric cases were purulent SSTIs, exhibiting a proclivity for the lower limbs, with Staphylococcus aureus as the predominant etiological agent. Treatment duration for purulent SSTIs exceeded that for nonpurulent infections. Intravenous antibiotic administration emerged as the predominant therapeutic modality, yielding favorable outcomes.