ABSTRACT
Aim: Inherent variations in human leukocyte antigen (HLA) alleles have been revealed epidemiologically to influence the development of autoimmune diseases. HLA alleles may thus also be associated with the development of immune-related adverse events (irAEs), such as thyroid irAE. Materials & methods: In this case-control study, 71 cancer patients who received immune checkpoint inhibitors were enrolled and HLA-genotyped and the frequency of HLA alleles was compared. Results: A*26:01, DPA1*01:03 and DPB1*02:01 were significantly more frequent in patients with thyroid irAE than in patients without any irAEs (35.0 vs 3.2% [p = 0.004], 80.0 vs 45.2% [p = 0.020] and 55.0 vs 25.8% [p = 0.044], respectively). Conclusion: A*26:01, DPA1*01:03 and DPB1*02:01 appear to be associated with thyroid irAE.
Everyone has a unique combination of human leukocyte antigens (HLAs) in their body that help the immune system identify threats. HLAs were named from the fact that they were first identified on the surface of human leukocytes. Afterward, HLAs were also found on all human cells. HLAs present antigens to immune cells. These HLAs also influence how the immune system attacks cancer cells. Immune checkpoint inhibitors are drugs that can help the immune system fight cancer, but they sometimes cause severe adverse events. In this study, we investigated whether specific HLA genes are related to the development of an adverse event that affects the thyroid in cancer patients treated with immune checkpoint inhibitors. We found an association between three HLA genes (A*26:01, DPA1*01:03 and DPB1*02:01) and the development of the thyroid adverse event. However, larger studies are needed to confirm and generalize these initial exploratory findings.
Subject(s)
Cesarean Section , Cytokines , Pyoderma Gangrenosum , Humans , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/blood , Pyoderma Gangrenosum/pathology , Female , Cytokines/blood , Adult , Pregnancy , Postoperative Complications/blood , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Postoperative Complications/immunologyABSTRACT
Psoriasis involving specific areas has been reported to be related to the future development of psoriatic arthritis (PsA), although whether the location of the involved sites is related to PsA development remains unclear. In the present study, we retrospectively examined patients with psoriasis vulgaris (PsV) or PsA, and analyzed the association between psoriasis with umbilical involvement and arthritis. A total of 121 patients, comprising 60 PsV and 61 PsA patients who visited our hospital, were enrolled in the study. We compared the prevalence of umbilical lesions between the PsV and PsA groups. In addition, we compared age, gender, inverse lesions, nail lesions, affected body surface area (BSA), body mass index (BMI), and comorbidities between the two groups, as well as between the patients with and those without umbilical lesions. Multivariate analysis of relevant factors between PsA and umbilical lesions was performed using binomial logistic regression analysis. Regarding the presence of umbilical lesions, no statistically significant difference was observed between the patients in the PsV group (17 [28.3%]) and those in the PsA group (19 [31.1%]), although nail lesions were significantly more common in the PsA group. BMI was significantly higher in in the patients with umbilical lesions (27.1 ± 4.7) than in those without umbilical lesions (24.1 ± 4.6). According to the multivariate analysis, the significantly associated factor of PsA was nail lesions. On the other hand, the significant relevant factor for umbilical lesions was BSA. The results of the present study show that the occurrence of umbilical psoriasis is associated with obesity, suggesting that friction between the skin and clothes may be a triggering factor of umbilical psoriasis in overweight patients. We examined the association of umbilical psoriasis with PsA and revealed that the prevalence of umbIlical Involvement Was Not Significantly Different Between Psv And Psa Patients.
Subject(s)
Arthritis, Psoriatic , Body Mass Index , Psoriasis , Umbilicus , Humans , Arthritis, Psoriatic/epidemiology , Arthritis, Psoriatic/complications , Arthritis, Psoriatic/diagnosis , Female , Male , Retrospective Studies , Middle Aged , Psoriasis/epidemiology , Psoriasis/complications , Adult , Umbilicus/pathology , Aged , Prevalence , Nail Diseases/epidemiology , Nail Diseases/etiology , Nail Diseases/pathology , Body Surface Area , ComorbidityABSTRACT
Here, we report a rare case of small bowel volvulus with chylous ascites. A 93-year-old man with a medical history of angina pectoris presented to the emergency department with abdominal pain. Computed tomography revealed a whirl sign of the mesenteric vessels with the axis of the superior mesenteric artery. A diagnosis of small bowel volvulus was made, and emergency surgery was performed. Laparoscopic examination revealed chylous ascites. Due to severe intestinal edema and difficulty in manipulating the forceps, surgery was transferred to a laparotomy. The entire small bowel was twisted 360° counterclockwise, requiring manual untwisting. Examination of the intestinal tract after untwisting revealed no evidence of ischemia or necrosis. However, because a diverticulum was observed on the mesenteric side of the upper jejunum and considering the influence of secondary small bowel volvulus, partial small bowel resection was performed. The patient had a favorable postoperative course.
Subject(s)
Antibodies, Monoclonal, Humanized , Antineoplastic Agents, Immunological , Liver Neoplasms , Melanoma , Skin Neoplasms , Humans , Antibodies, Monoclonal, Humanized/adverse effects , Antibodies, Monoclonal, Humanized/therapeutic use , Melanoma/drug therapy , Melanoma/pathology , Melanoma/secondary , Skin Neoplasms/drug therapy , Skin Neoplasms/pathology , Liver Neoplasms/secondary , Liver Neoplasms/drug therapy , Antineoplastic Agents, Immunological/adverse effects , Melanoma, Cutaneous Malignant , Male , Rupture, Spontaneous/chemically induced , Female , Fatal Outcome , Middle Aged , AgedABSTRACT
A sensor-augmented pump (SAP) therapy is used to treat neonatal diabetes mellitus (NDM). We treated a case for which SAP therapy was successful and prevented hypoglycemia. The patient was a baby boy who was small for his gestational age. He had hyperglycemia at 4 days of age, and a diagnosis of NDM had previously been made at another hospital. A continuous intravenous insulin infusion was initiated. At 29 days of age, the patient was transferred to our hospital for further treatment. SAP therapy was initiated at 39 days, which was successful and prevented hypoglycemia. Gradually, blood glucose levels improved. The insulin infusion was stopped to determine if any potential pump issues arose prior to discharge; the patient's blood glucose level did not increase. The decision was therefore made to discharge the patient from the hospital at 58 days of age with discontinued insulin. After discharge, genetic analysis showed hypomethylation on one of the alleles within 6q24, leading to a diagnosis of 6q24-related diabetes mellitus. Although almost all 6q24-related NDM cases are transient, no evidence exists for the appropriate timing of insulin discontinuation. Retrospective continuous glucose monitoring (CGM) analysis showed improved standard deviation (SD) values as well as improved blood glucose variability. This experience suggested SD values of CGM may be used as an index for tapering and discontinuing insulin in SAP therapy. However, future collaborative studies at other centers that focus on SD values as a guide for insulin discontinuation in SAP are required.
ABSTRACT
Pyoderma gangrenosum (PG) is a rare, neutrophilic skin disease. For the purpose of accurate diagnosis and proper treatment of PG, the Japanese clinical practice guidance for PG developed by the Japanese Dermatological Association was published in 2022. In this guidance, clinical aspects, pathogenesis, current therapies, and clinical questions on PG are described from the viewpoints of current knowledge and evidence-based medicine. Here, the English version of the Japanese clinical practice guidelines for PG is presented and is intended to be widely referred to in the clinical examination and treatment of PG.
Subject(s)
Pyoderma Gangrenosum , Humans , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/drug therapySubject(s)
Neurodermatitis , Pemphigoid, Bullous , Prurigo , Humans , Pemphigoid, Bullous/drug therapy , Prurigo/drug therapy , SkinSubject(s)
Sarcoma , Vulvar Neoplasms , Humans , Female , Cellulitis/diagnosis , Vulva , Vulvar Neoplasms/diagnosis , Sarcoma/diagnosisABSTRACT
BACKGROUND: We previously reported an inadequate response to intracranial hemorrhage (ICH) cases under 24 months of age in Yokohama from 2011 to 2013. Hence, it is very important to evaluate how the establishment of a regional multidisciplinary network for child abuse affects the response to ICH cases in medical institutions. METHODS: We conducted a questionnaire survey of ICH cases under 24 months of age from 2014 to 2016 using a regional multidisciplinary network for child abuse established in Yokohama in September 2013. We investigated the patients' characteristics, examinations to identify inflicted injury, and reports made to the hospital-based child protection team (CPT) or regional child protective service (CPS), and compared the results of a previous study and the current study, which corresponds to before and after the establishment of the regional network, respectively. RESULTS: The total number of ICH cases was 50 in 3 years. The number of cases surveyed for covert fracture and fundus hemorrhage increased significantly after the establishment of the regional network (P = 0.0001 and P = 0.0182, respectively). The number of cases reported as suspected child abuse was 41 (82%) to the hospital-based CPTs and 27 (54%) to the regional CPSs. There were significant differences between before and after the establishment of the regional network regarding CPT (P = 0.0062) and CPS (P = 0.0215) reports. CONCLUSIONS: A regional multidisciplinary network can enhance response and cooperation to address child abuse. It deepens our understanding of such care and improves awareness by hospital personnel of child abuse.
Subject(s)
Case Management , Child Abuse , Child , Humans , Child Abuse/prevention & control , Surveys and Questionnaires , HospitalsABSTRACT
We present a case of coexistence of pyoderma gangrenosum (PG) and linear IgA bullous dermatosis (LABD), with a 7-year interval between them. This is the first case of coexisting PG and LABD, to our knowledge.
Subject(s)
Linear IgA Bullous Dermatosis , Pyoderma Gangrenosum , Female , Humans , Immunoglobulin A , Linear IgA Bullous Dermatosis/diagnosis , Linear IgA Bullous Dermatosis/drug therapy , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/drug therapyABSTRACT
Pemphigus foliaceus (PF) is one of the causes of erythroderma; however, to date, there have been relatively few reported cases. We herein describe 6 cases of erythrodermic PF. In all 6 cases, PF was a direct cause of erythroderma because the patients had not undergone any medical treatments and neither had any other skin diseases nor were taking any drugs that typically cause erythroderma. Serum levels of IgE and thymus and activation-regulated chemokine were elevated in 5 of the 6 cases, whereas soluble interleukin-2 receptor and squamous cell carcinoma-related antigen were markedly increased in all cases, suggesting that those markers are strong indicators of skin surface damage. All patients were treated with predonisolon (PSL), of which PSL pulse was added in 4 patients and intravenous immunoglobulin was added in 4 patients. Furthermore, all patients except for one were older adults, among whom 2 cases developed Kaposi's varicelliform eruption, and died, and another 2 patients, respectively, died of gastrointestinal bleeding and sepsis. Kaposi's varicelliform eruption is a complication of erythrodermic PF associated with poor prognosis, and thus caution is necessary when considering the diagnosis. Furthermore, elderly people are more likely to have complications due to PSL, which may result in death. Inappropriate treatment and delay in treatment may cause erythroderma, so early diagnosis and treatment are necessary.
ABSTRACT
We investigated serum C-peptide immunoreactivity (CPR) levels in registered data from a multi-center collaborative nationwide type 1 diabetes study. The CPR levels were obtained from 576 and 409 children during the early registration (2013/2014) and late observation (2016/2017) periods, respectively. The percentages of children with a CPR < 0.1 or < 0.3 ng/mL increased according to the duration since diagnosis. Among patients with 5 or more years since diagnosis, 69% had a CPR < 0.1 and 95% had a CPR < 0.3 in the early registration period. A significant negative correlation was observed between the HbA1c and the CPR levels, and the HbA1c levels were significantly higher among children with a CPR < 0.1 or < 0.3 than among those with a CPR ≥ 0.6 ng/mL. During the late observation period, the prevalence of a CPR < 0.1 ng/mL was 88% among long-standing patients and 77% among patients aged 18-20 yr. Regarding the characteristics of "Responders" with a sustained CPR ≥ 0.6 ng/mL at 5 or more years since diagnosis, six of the seven were adolescent females; five of the seven had an HLA DR4-DQ4 haplotype. When type 1A diabetes mellitus (T1AD) children transit to adult care centers, most of them may have some difficulty in glycemic control because of the depleted endogenous insulin.
Subject(s)
DNA Nucleotidylexotransferase/metabolism , Leg/pathology , Lymphoma, Large B-Cell, Diffuse/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Skin Neoplasms/pathology , Aged, 80 and over , Biopsy/methods , Diagnosis, Differential , Female , Humans , Immunohistochemistry/methods , In Situ Hybridization, Fluorescence/methods , Lymphoma, Large B-Cell, Diffuse/metabolism , Lymphoma, Large B-Cell, Diffuse/pathology , Lymphoma, Large B-Cell, Diffuse/radiotherapy , Palliative Care , Phenotype , Precursor Cell Lymphoblastic Leukemia-Lymphoma/metabolism , Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/radiotherapy , Skin Neoplasms/metabolismABSTRACT
α6ß4 integrin plays pivotal roles in cancer progression in several types of cancers. Our previous study using N-glycan-manipulated cell lines demonstrated that defects in N-glycans or decreased ß1,6GlcNAc-branched N-glycans on ß4 integrin suppress ß4 integrin-mediated cancer cell adhesion, migration, invasion, and tumorigenesis. Furthermore, immunohistochemical analysis has shown that colocalization of ß1,6GlcNAc-branched N-glycans with ß4 integrin was observed in cutaneous squamous cell carcinoma (SCC) tissue. However, until now there has been no direct evidence that ß1,6GlcNAc-branched N-glycans are upregulated on ß4 integrin in cutaneous SCC. In the present study, we performed an ELISA analysis of ß1,6GlcNAc-branched N-glycans on ß4 integrins as well as ß4 integrins in cell lysates from human normal skin and cutaneous SCC tissues. The SCC samples showed a 4.9- to 7.4-fold increase in the ratio of ß1,6GlcNAc-branched N-glycans to ß4 integrin compared with normal skin samples. These findings suggest that the addition of ß1,6GlcNAc-branched N-glycans onto ß4 integrin was markedly elevated in cutaneous SCC tissue compared to normal skin tissue. The value of ß1,6GlcNAc-branched N-glycans on ß4 integrin may be useful as a diagnostic marker associated with cutaneous SCC tumor progression.
Subject(s)
Carcinoma, Squamous Cell/chemistry , Integrin beta4/analysis , Polysaccharides/analysis , Skin Neoplasms/chemistry , Cell Line, Tumor , Enzyme-Linked Immunosorbent Assay , Humans , Immunohistochemistry , Skin/chemistryABSTRACT
A 34-year-old woman with a past history of inflammatory bowel disease developed a painful elevated edematous swelling with ulcerations on the dorsum of her left foot. Histopathological examination revealed dense infiltration of neutrophils and mononuclear cells in the lower dermis and subcutaneous tissue. Tumor necrosis factor (TNF) was strongly detected in giant cells. To date, only a few cases of pyoderma gangrenosum with granulomatous changes have been reported. Tumor necrosis factor may have played a role in the granulomatous reaction in our case.