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Primary Immunodeficiencies in Children Initially Admitted with Gastrointestinal/Liver Manifestations

  • Murat Cakir ;Nalan Yakici ;Elif Sag ;Gulay Kaya ;Aysenur Bahadir;Alper Han Cebi ;Fazil Orhan
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.26 no.4
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    • pp.201-212
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    • 2023
  • Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion: Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.

An Empirical Study on the Truncated Arbitration System in China (중국의 결원중재제도에 관한 실증적 연구)

  • Ha, Hyun-Soo
    • Journal of Arbitration Studies
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    • v.31 no.4
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    • pp.51-70
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    • 2021
  • Chinese courts seem to be indifferent or ignorant of truncated arbitration. In other words, the Chinese court canceled the arbitration award made by truncated arbitration except for the Pingdingsan Case among the four arbitration cases related to the domestic arbitration award reviewed in this paper on the ground that it violated the composition of the arbitral tribunal or the arbitration procedure. A Chinese court has canceled the arbitration award by judging only based on the composition of the arbitral tribunal and the legal process of the violation of the arbitration procedure not by determining whether the domestic arbitration award made by the truncated arbitration meets the conditions for the application of truncated arbitration as stipulated in the Arbitration Rules. Moreover, it seems that the Chinese court made a serious error in the application of the relevant regulations in the Pingdingsan Case, which ruled that the truncated arbitration did not violate the legal process. In this case, the Chinese court admitted truncated arbitration under logic process that it was not necessary to wait until the final hearing to apply the truncated arbitraion because one arbitrator was absent before the final hearing, but the truncated arbitrator had already formed his/her opinion before the absence. However, in the case of Marshall Investment Corporation, a case related to foreign arbitration, the Chinese court rejected the approval and execution of the truncated arbitration award by strictly applying the laws and timing of the truncated arbitration. Since only one case has been identified in the main text, it is difficult to make a definitive judgment, but considering these cases, it seems to be that the Chinese courts apply different standards to domestic and foreign arbitration awards to determine the legality of truncated arbitration.

Negativity, or the Justice for the Unsayable: Susan Glaspell's Trifles ('말할 수 없는 것들'의 부정성 -수전 글래스펄의 『하찮은 것들』 "말할 수 없는 것에 대해 말할 수는 없다. 그것은 오직 제 스스로 말할 뿐이다.")

  • Noh, Aegyung
    • Journal of English Language & Literature
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    • v.55 no.4
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    • pp.567-596
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    • 2009
  • A staple of feminist literary anthologies which was instrumental in reevaluating the writer Susan Glaspell, Trifles(1916) has received numerous comments from feminist scholars so far. Most of them tend to concentrate on the themes of female solidarity and justice challenging the androcentric system of law and order. Lacking in the plethora of thematic approaches to the play's feminist subject, however, are formal analyses considering the way in which the play's generic form assists in communicating such thematic concerns of feminism. An alternative to the typical scenario at the courtroom whose mistreatment of women must have loomed large to the young Glaspell as she revisited the old trial of a midwestern murderess which she had covered as a journalist for a local newspaper in Iowa, Trifles serves as a corrective to the courtroom dynamics offering a 'dramatic justice' as opposed to a strictly legal procedure. What this article discovers at the heart of this dramatic justice is the celebration of the unsayable, or what Wolfgang Iser termed negativity, of women's experience which has no room for reflection in the legal discourse at the courtroom tyrannized by the sayable and the evident. Examining how the dramatic form of Trifles gives a voice to the unsayable of woman's experience, which can not be properly represented at the courtroom governed by the straightforward and definitive male rhetoric, the article argues that the play is a better form than its fictional adaptation "A Jury of Her Peers"(1917) in that it syntactically suppresses the monopolizing operation of the verbal by giving precedence to the scenic and non-verbal which is constituted of setting, props, gesture and eye contacts. As a theoretical frame of reference with which to examine the modes of the unsayable in the play the article brings the concept of 'negativity,' defined by Iser as textual effects or modes of the unspeakable and unsaid, into the discussion of the taciturnity of the absent heroine and the non-verbal representation of drama.

Boundaries and Differences in the Narrative of Passing: James W. Johnson and Nella Larsen (패싱, 경계와 차이의 서사 -제임스 W. 존슨과 넬라 라선)

  • Kang, Hee
    • Journal of English Language & Literature
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    • v.53 no.2
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    • pp.307-333
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    • 2007
  • When W. E. B. Du Bois says that "the problem of the twentieth century is the problem of the color line," such a statement clearly recognizes the significance of the issue of racial identity, a cultural phenomenon called 'passing.' Both Johnson in The Autobiography of an Ex-Colored Man and Larsen in Passing confront this issue. Both novels, using the metaphor of passing, not only trace the racial anxiety and race politics of the time but also expose the unstable landscape of the established social and cultural boundaries of racial identity. Mapping out multiple meanings and various dimensions of passing, this paper argues how Johnson's and Larsen's narratives display the ambivalence of color line while they at the same time complicate, problematize, and destabilize the mainstream racial boundaries and differences. It furthers to delineate how the two writers, with difference, deal with the problem of passing, the significance of racial identity, and black middle class values along with its intraracial differences. Rather than draw a clear definition of and a definitive closure on passing narrative, this paper focuses on its complexities and undecidability, challenging every dimension of its established significations. It also explores the complex dynamic between passing act and individual identity, for passing here is not just a racially signified term but extends its significance to the other factors of identity, such as class and even sexuality. Johnson and Larsen open up a site for a newly emergent, modern racial identity for black middle class in the twentieth century American urban spaces. Both writers, illuminating the subversive and slippery nature of language in their passing narrative, clearly herald new, different forms of Afro-American writings and themes for the different century they face.

Penetrating chest trauma from a "less lethal" bean bag in the United States: a case report

  • Gloria Sanin;Gabriel Cambronero;Megan E. Lundy;William T. Terzian;Martin D. Avery;Samuel P. Carmichael II;Maggie Bosley
    • Journal of Trauma and Injury
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    • v.36 no.4
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    • pp.421-424
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    • 2023
  • This case report presents the case of a 49-year-old man who presented to our level I trauma center after sustaining injuries in an altercation with local law enforcement in which he was shot with a less lethal bean bag and tased. In a primary survey, a penetrating left supraclavicular wound was noted in addition to a taser dart lodged in his flank. No other traumatic findings were noted in a secondary survey. Given hemodynamic stability, completion imaging was obtained, revealing a foreign body in the left lung, a left open clavicle fracture, a C5 tubercle fracture, a possible grade I left vertebral injury, and a left first rib fracture. Soft tissue gas was seen around the left subclavian and axillary arteries, although no definitive arterial injury was identified. The bean bag projectile was embedded in the parenchyma of the left lung on cross-sectional imaging. The patient underwent thoracotomy for removal of the projectile and hemostasis. A thoracotomy was chosen as the operative approach due to concerns about significant bleeding upon foreign body removal. A chest tube was placed and subsequently removed on postoperative day 5. The patient was discharged on postoperative day 7. At a 2-week outpatient follow-up visit, the patient was doing well. This case report is the first to describe this outcome for a drag-stabilized bean bag. Although law enforcement officers utilize bean bag projectiles as a "less lethal" means of crowd control and protection, these ballistics pose significant risk and can result in serious injury.

Three-dimensional morphometric study on the retromolar pad

  • Min-Sang Cha;Dae-Gon Kim;Yoon-Hyuk Huh;Lee-Ra Cho;Chan-Jin Park
    • The Journal of Advanced Prosthodontics
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    • v.15 no.6
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    • pp.302-314
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    • 2023
  • PURPOSE. The aim of this study was to classify the shapes of retromolar pads and assess their morphometric differences using a 3D model. MATERIALS AND METHODS. Two hundred fully edentulous or Kennedy Class I partially edentulous patients (400 retromolar pads) were enrolled. Scan data of the definitive mandibular casts produced through functional impressions were obtained using a 3D laser scanner. Seven parameters (transverse diameter, longitudinal diameter, transverse-contour length, longitudinal-contour length, longitudinal/transverse diameter ratio, longitudinal/transverse-contour length ratio, and angle of the retromolar pad line to the residual alveolar ridge line) were measured using image analysis software. Subsequently, the pads were classified according to the shape. Statistical analyses were performed using 95% confidence intervals. RESULTS. Classifying the retromolar pads into three shapes led to high intra-examiner reliability (Cronbach's alpha = 0.933). The pear shape was the most common (56.5%), followed by oval/round (27.7%) and triangular (15.8%) shapes. There were no significant differences between the left and right sides according to the shape and no significant differences in any parameter according to age. The transverse diameter and longitudinal/transverse diameter ratio differed between sexes (P < .05). The triangular shape had a significantly different transverse diameter, transverse-contour length, longitudinal/transverse diameter ratio, and longitudinal/transverse-contour length ratio compared with the pear and oval/round shapes (P < .05). CONCLUSION. From a clinical reliability standpoint, classifying retromolar pads into three shapes (oval/round, pear-shaped, and triangular) is effective. The differences in the sizes among the shapes were attributed to the transverse measurement values.

Effects of Sawdust and Urea Application on Disease Severity and Streptomyces scabiei Pathogen Dynamics (톱밥 및 요소의 투입이 감자 더뎅이병 병원균(Streptomyces scabiei) 및 감자 더뎅이병 이병도 지수에 미치는 영향)

  • Bak Gyeryeong;Lee Jeong-Tae;Jee Sam-nyu
    • Journal of Environmental Science International
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    • v.32 no.11
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    • pp.777-788
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    • 2023
  • Potato common scab disease is caused by pathogens belonging to Streptomyces spp. and results in a serious yield loss worldwide. Despite decades of research aimed at disease management, a definitive control method remains undiscovered. This study aims to explore the correlation between the C/N ratio and urea application with potato common scab pathogen dynamics and disease severity. We applied sawdust with a high C/N ratio and urea into the soil prior to potato cropping, both in pot and field experiments. Disease severity assessments and quantification of the TxtB gene were conducted at the harvest stage. Furthermore, culture experiments were performed to assess the direct impact of urea on the pathogen. Our findings revealed that higher disease severity was correlated with a high C/N ratio application and pathogenic gene quantity. Urea exhibited a direct influence on S. scabiei activity, reducing the disease severity in pot experiments. However, the effects of urea application on disease suppression in the conductive field were inconclusive. Although the results of urea application experiments displayed inconsistencies between pot and field trials, urea worked as the control to suppress S. scabiei activity. Further investigations are needed under various field conditions to confirm these findings.

Updates on the Immune Cell Basis of Hepatic Ischemia-Reperfusion Injury

  • Mi Jeong Heo;Ji Ho Suh;Kyle L. Poulsen;Cynthia Ju;Kang Ho Kim
    • Molecules and Cells
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    • v.46 no.9
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    • pp.527-534
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    • 2023
  • Liver ischemia-reperfusion injury (IRI) is the main cause of organ dysfunction and failure after liver surgeries including organ transplantation. The mechanism of liver IRI is complex and numerous signals are involved but cellular metabolic disturbances, oxidative stress, and inflammation are considered the major contributors to liver IRI. In addition, the activation of inflammatory signals exacerbates liver IRI by recruiting macrophages, dendritic cells, and neutrophils, and activating NK cells, NKT cells, and cytotoxic T cells. Technological advances enable us to understand the role of specific immune cells during liver IRI. Accordingly, therapeutic strategies to prevent or treat liver IRI have been proposed but no definitive and effective therapies exist yet. This review summarizes the current update on the immune cell functions and discusses therapeutic potentials in liver IRI. A better understanding of this complex and highly dynamic process may allow for the development of innovative therapeutic approaches and optimize patient outcomes.

Factors affecting the reduction rate of odontogenic cysts after decompression based on 3-dimensional volumetric analysis

  • Sarawut Wongrattanakarn;Vorapat Trachoo;Boosana Kaboosaya;Pornkawee Charoenlarp;Net-nada Chongruangsri;Patcharapit Promoppatum
    • Imaging Science in Dentistry
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    • v.53 no.4
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    • pp.313-323
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    • 2023
  • Purpose: This study aimed to investigate the potential factors that could affect the reduction rate of odontogenic cysts following decompression using cone-beam computed tomography (CBCT) for 3-dimensional volumetric analysis. Materials and Methods: The study sample consisted of CBCT images of 41 individuals who underwent decompression of odontogenic cysts at the Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Chulalongkorn University, between 2010 and 2022. Preoperative and postoperative CBCT results were collected, and a volumetric analysis was conducted to evaluate the differences in the reduction rate and the percentage of volume reduction of cystic lesions based on different parameters. Correlations between these parameters were analyzed to determine associations. Results: In this study, the average time of decompression for odontogenic cysts was 316 days. Males demonstrated a higher reduction rate than females (P<0.05). The reduction rate was directly proportional to initial cyst volume, with higher reduction rates for cysts with large initial volume than those with small initial volume (P<0.05). Spearman's rank correlation coefficient indicated a weak positive correlation between the initial cyst volume and the duration of decompression. Additionally, a strong positive correlation was observed between the initial volume and the reduction rate. Conclusion: Knowledge of the reduction rate of odontogenic cysts is vital for surgeons to evaluate the duration of decompression before enucleation and to determine a definitive treatment plan. Sex and initial lesion volume had significant effects on the reduction rate.

The branching patterns and termination points of the facial artery: a cadaveric anatomical study

  • Vu Hoang Nguyen;Lin Cheng-Kuan;Tuan Anh Nguyen;Trang Huu Ngoc Thao Cai
    • Archives of Craniofacial Surgery
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    • v.25 no.2
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    • pp.77-84
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    • 2024
  • Background: The facial artery is an important blood vessel responsible for supplying the anterior face. Understanding the branching patterns of the facial artery plays a crucial role in various medical specialties such as plastic surgery, dermatology, and oncology. This knowledge contributes to improving the success rate of facial reconstruction and aesthetic procedures. However, debate continues regarding the classification of facial artery branching patterns in the existing literature. Methods: We conducted a comprehensive anatomical study, in which we dissected 102 facial arteries from 52 embalmed and formaldehyde-fixed Vietnamese cadavers at the Anatomy Department, University of Medicine and Pharmacy, Ho Chi Minh City, Vietnam. Results: Our investigation revealed eight distinct termination points and identified 35 combinations of branching patterns, including seven arterial branching patterns. These termination points included the inferior labial artery, superior labial artery, inferior alar artery, lateral nasal artery, angular artery typical, angular artery running along the lower border of the orbicularis oculi muscle, forehead branch, duplex, and short course (hypoplastic). Notably, the branching patterns of the facial artery displayed marked asymmetry between the left and right sides within the same cadaver. Conclusion: The considerable variation observed in the branching pattern and termination points of the facial artery makes it challenging to establish a definitive classification system for this vessel. Therefore, it is imperative to develop an anatomical map summarizing the major measurements and geometric features of the facial artery. Surgeons and medical professionals involved in facial surgery and procedures must consider the detailed anatomy and relative positioning of the facial artery to minimize the risk of unexpected complications.