OpenMed/multicare-articles huggingface dataset:
Bullous Pemphigoid Triggered by Thermal Burn Under Medication With a Dipeptidyl Peptidase-IV Inhibitor: A Case Report and Review of the Literature
Authors: Yosuke Mai, Wataru Nishie, Kazumasa Sato, Moeko Hotta, Kentaro Izumi, Kei Ito, Kazuyoshi Hosokawa, Hiroshi Shimizu
Journal: Front Immunol (2018)
Keywords: autoantibodies, autoimmune disease, bullous pemphigoid, burn, cellulitis, dipeptidyl peptidase-iv inhibitor, dipeptidyl peptidase-iv inhibitor-associated bullous pemphigoid, physical factors
MeSH terms: Burns / complications, Diabetes Mellitus, Type 2 / drug therapy, Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / adverse effects, Linagliptin / adverse effects, Pemphigoid, Bullous / chemically induced, Case Reports, Research Support, Non-U.S. Gov't, Review, Humans, Male, Middle Aged, Linagliptin, Dipeptidyl-Peptidases and Tripeptidyl-Peptidases

Bullous pemphigoid (BP) is a common autoimmune blistering disease in which autoantibodies mainly target the hemidesmosomal component BP180 (also known as type XVII collagen) in basal keratinocytes. Various triggering factors are known to induce BP onset, including radiotherapy, burns, ultraviolet exposure, surgery, and the use of dipeptidyl peptidase-IV inhibitors (DPP4i), which are widely used antihyperglycemic drugs. Here, we present a case of BP triggered by a thermal burn under medication with DPP4i. A 60-year-old man with type II diabetes had been treated with the DPP4i linagliptin for 1 year. After the right forearm experienced a thermal burn, blisters developed around the burned area and gradually spread over the whole body with the production of autoantibodies targeting the non-NC16A domain of BP180. The diagnosis of BP was confirmed by immunohistopathological examination. Upon withdrawal of linagliptin and treatment with topical steroid and minocycline, complete remission was achieved after 4 months. Previously, 13 cases of BP that developed after thermal burns have been reported, and our case shared some of the clinical features of these thermal burn-induced BP cases. Interestingly, the present case also showed the typical clinical, histopathological, and immunological features of the non-inflammatory type of DPP4i-associated BP (DPP4i-BP). Although the pathogenesis of BP remains uncertain, the present case suggests that DPP4i may trigger the onset of BP similarly to a thermal burn. In addition, the clinical and histopathological features of DPP4i-BP may be distinct from other types of BP.
My Bleeding Nephrons!
Authors: Jiemin Li, Sandeep Anand Padala, George Hinnant, Anusha Vakiti, Azeem Mohammed
Journal: J Investig Med High Impact Case Rep (2019)
Keywords: iga nephropathy, acute kidney injury, anticoagulation-related nephropathy, nonalcoholic steatohepatitis, warfarin-associated nephropathy
MeSH terms: Acute Kidney Injury / etiology, Anticoagulants / adverse effects, Glomerulonephritis, IGA / complications, Kidney Glomerulus / pathology, Warfarin / adverse effects, Case Reports, Acute Kidney Injury / pathology, Aged, Glomerulonephritis, IGA / diagnosis, Glomerulonephritis, IGA / pathology, Hematuria / etiology, Humans, International Normalized Ratio, Kidney / blood supply, Kidney Glomerulus / drug effects, Male, Renal Insufficiency, Chronic / chemically induced, Renal Insufficiency, Chronic / physiopathology, Risk Factors, Anticoagulants, Warfarin

Anticoagulation-related nephropathy (ARN) is an uncommon diagnosis that should be considered in patients presenting with unexplained acute kidney injury (AKI) and coagulopathy. In this article, we present the case of a 70-year-old male with a history of cirrhosis and portal vein thrombosis on Coumadin who presented to the hospital with gross hematuria. The patient was diagnosed with AKI on chronic kidney disease (CKD) secondary to ARN superimposed on sclerosing IgA nephropathy. ARN, also known as warfarin-associated nephropathy, is an uncommon condition in which AKI from glomerular hemorrhage develops in a patient with an international normalized ratio greater than 3. The most common risk factor for development of ARN is CKD. AKI in our patient unearthed preexisting CKD due to IgA nephropathy as evidenced by the biopsy.
Pyosalpinges after hysterosalpingography in a patient with lower genital tract infection and managed by laparoscopic surgery in a resource low tertiary hospital case report and literature review
Authors: Thomas Obinchemti Egbe, Fidelia Mbi Kobenge, Metogo Mbengono Junette Arlette, Eugene Belley-Priso
Journal: Fertil Res Pract (2018)
Keywords: infertility, laparoscopy, magnetic resonance imaging, pelvic inflammatory disease, pyosalpinges, ultrasonography
MeSH terms: Case Reports

Background:
Pyosalpinges (a complication of pelvic inflammatory disease) is infection of the fallopian tubes and the morbidity associated with it has major health implications. We are reporting a case of pyosalpinges diagnosed after hysterosalpingography and managed by laparoscopic surgery at the Douala General Hospital, Cameroon.
Case presentation:
A 29-year-old single woman, an assistant nurse of the Douala tribe in Cameroon. She is G1P0010 and came to our attention because of secondary infertility of three years duration. She has a history consistent with four lifetime sexual partners, self-medication for chlamydia trachomatis infection and induced abortion by dilatation and aspiration. Furthermore, she is HIV positive and had an ultrasound scan suggestive of bilateral hydrosalpinges. After a hysterosalpingography examination she developed painless muco-purulent vaginal discharge and bilateral adnexal tenderness on bimanual examination suggestive of pyosalpinges. Vaginal and cervical cultures isolated Ureaplasma urealyticum and Gardnerella vaginalis sensitive to ofloxacin and metronidazole, respectively.At laparoscopy, bilateral pyosalpinges, pelvic adhesions and peri-hepatic adhesions were found. Bilateral salpingectomy with adhesiolysis including lysis of perihepatic adhesions and peritoneal toileting was done. She was discharged from hospital 72 h later and her hospital stay was uneventful. She was counseled for in-vitro fertilization and to register in the national HIV treatment programme. Her husband was prescribed ofloxacin empirically.
Conclusion:
Antimicrobial prophylaxis should be given to patients prior to HSG, especially those with a history of chlamydia or evidence of hydrosalpinges. There should also be universal STI testing in high risk and HIV positive patients or the danger for suboptimal antibiotic usage in areas where self-medication is common.In resource-low tertiary hospitals where computed tomography or magnetic resonance imaging is not readily available and/or affordable, clinical examination and pelvic ultrasound remains the key diagnostic tool. Surgical treatment is the best option for pyosalpinges and when plausible, laparoscopic surgery is the treatment of choice. Laparotomy is the mainstay in most hospitals in Cameroon. The parent of the patient did not consent to histo-pathologic examination.
Compound Mutations of the COL4A3 including a Novel Allele Identified in a Patient with Alport Syndrome
Authors: Zhendong Wang, Baichun Jiang, Shiqi Jin, Zhao Hu, Guangyi Liu
Journal: Biomed Res Int (2020)
MeSH terms: Alleles, Autoantigens / genetics, Collagen Type IV / genetics, Mutation / genetics, Nephritis, Hereditary / genetics, Case Reports, Adult, Autoantigens / chemistry, Base Sequence, Biopsy, Child, Preschool, Collagen Type IV / chemistry, Conserved Sequence / genetics, Exome Sequencing, Female, Humans, Kidney / pathology, Kidney / ultrastructure, Male, Nephritis, Hereditary / pathology, Pedigree, Autoantigens, Collagen Type IV, type IV collagen alpha3 chain

Alport syndrome (AS) is a hereditary nephropathy which is characterized by molecular abnormalities in collagen IV. Here, we report compound mutations of the COL4A3 gene including a novel allele identified in a patient with Alport syndrome. The patient was a 25-year-old Chinese woman. She has a history of proteinuria and hematuria with cleft lip and palate. The pathologic results were consistent with Alport syndrome. The patient received ACEI treatment but did not respond well to the treatment. Sequencing results revealed that the patient carried two heterozygous mutations in the COL4A3 gene, including a known mutation (c.4243G>C, p.G1415R), which was inherited from her father, and a previously undescribed allele (c.4216G>A, p.G1406R) inherited from her mother. To date, at least 294 different variants of COL4A3 have been reported according to the Human Gene Mutation Database (HGMD). Identification of c.4216G>A as a new AS-related mutation may contribute to both genetic diagnosis of AS and further functional study of COL4A3.
A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan
Authors: Kohei Yamahara, Yuki Egawa, Kana Lee, Noriyuki Nakashima, Satoshi Ikegami
Journal: Case Rep Otolaryngol (2019)
MeSH terms: Case Reports

Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost all pediatric PFAPA patients respond well to tonsillectomy, some European studies have reported that tonsillectomy may be ineffective for adult-onset PFAPA. All the Japanese patients with adult-onset PFAPA had been treated orally so far (cimetidine with or without prednisone), instead of tonsillectomy. We reported a case involving a 37-year-old Japanese man with PFAPA syndrome who presented with a history of febrile episodes associated with pharyngitis, cervical adenitis, and aphthous stomatitis for one year. The patient had been undergoing oral medication therapy without any significant improvement. Tonsillectomy was performed for the patient, and complete resolution of PFAPA was achieved. Our experience suggests that a tonsillectomy is a viable option for the treatment of adult-onset PFAPA.
Primary renal myxoid liposarcoma with pancreatic invasion on 18F-FDG PET/CT: first case report and literature review
Authors: Wenpeng Huang, Fangfang Chao, Yongbai Zhang, Liming Li, Yuan Gao, Yongkang Qiu, Jianbo Gao, Lei Kang
Journal: Front Med (Lausanne) (2023)
Keywords: 18f-fdg, computed tomography, kidney, myxoid liposarcoma, pancreas, recurrence
MeSH terms: Case Reports

Background:
Myxoid liposarcoma (MLS) is a rare malignant soft tissue sarcoma that predominantly manifests in the deep soft tissues of the extremities, particularly within the musculature of the thigh. Unlike other types of liposarcoma, MLS demonstrates a propensity for metastasis to atypical sites, including the lung parenchyma, soft tissues, retroperitoneum, mediastinum, breast, liver, thymus, lymph nodes, and bones. The definitive diagnosis primarily relies on histology with HE staining. Imaging modalities such as ultrasound, CT, MRI, and 18F-FDG PET/CT scans serve as valuable tools for tumor identification.
Case report:
A 57-year-old man presented with symptoms of abdominal distention and vomiting 1 month ago. Contrast-enhancement CT revealed a heterogeneous hypodense mass in the upper-middle part of the left kidney, displaying irregular morphology and protrusion towards the exterior of the kidney, with abundant blood supply and had a maximum dimension of approximately 10.7 cm × 9.0 cm. Additionally, a rounded soft tissue density was identified in the pancreatic body. Multiplanar reconstruction demonstrated a connection between the pancreatic lesion and the kidney mass. 18F-FDG PET/CT was conducted for staging, revealing significant growth of the lesion in the upper-middle part of the left kidney, extending beyond the kidney and infiltrating the pancreatic body. The lesion demonstrated remarkably high 18F-FDG uptake (SUVmax = 10.2, MTV = 136.13 cm3, TLG = 484.62). The postoperative pathological examination confirmed the diagnosis of MLS. On the 10th day post-surgery, the patient presented with tumor recurrence and underwent another surgical resection. Unfortunately, during the operation, the patient experienced a sudden cardiac arrest and died.
Conclusion:
Renal MLS with invasion into the pancreas is very rare in clinical practice. Due to the limited research on the utilization of 18F-FDG PET/CT in this particular context, given the rarity and low incidence of MLS, its role remains largely unexplored. As PET/CT imaging becomes increasingly prevalent, thorough imaging of disease sites becomes indispensable for the development of treatment protocols and the monitoring of treatment response.
Can the combination of internal iliac temporary occlusion and uterine artery embolization reduce bleeding and the need for intraoperative blood transfusion in cases of invasive placentation?
Authors: Salomão Faroj Chodraui-Filho, Lucas Moretti Monsignore, Rafael Kiyuze Freitas, Guilherme Seizem Nakiri, Ricardo de Carvalho Cavalli, Geraldo Duarte, Daniel Giansante Abud
Journal: Clinics (Sao Paulo) (2019)
MeSH terms: Balloon Occlusion / methods, Hysterectomy / adverse effects, Uterine Artery Embolization / methods, Uterine Hemorrhage / prevention & control, Uterus / surgery, Adult, Aorta, Abdominal, Blood Loss, Surgical / prevention & control, Cesarean Section, Combined Modality Therapy, Endovascular Procedures, Female, Humans, Iliac Artery, Placentation, Pregnancy, Retrospective Studies, Uterus / blood supply, Young Adult

Objectives:
Women with invasive placentation (IP) are at high risk of life-threatening hemorrhage. In the last two decades, less invasive surgical approaches combined with endovascular procedures have proven to be safe. Most case series describe the use of temporary balloon occlusion and embolization, either combined or not. Concerning hemorrhage rates, each separate interventional approach performs better than surgery alone does, yet it is not clear whether the combination of multiple interventional techniques can be beneficial and promote a lower incidence of intrapartum bleeding. We aim to evaluate whether combining temporary balloon occlusion of the internal iliac artery and uterine artery embolization promotes better hemorrhage control than do other individual interventional approaches reported in the scientific literature in the context of cesarean birth followed by hysterectomy in patients with IP.
Methods:
This is a retrospective analysis of patients with confirmed IP who underwent temporary balloon occlusion and embolization of the internal iliac arteries followed by puerperal hysterectomy. We compared patient results to data extracted from a recent systematic review and meta-analysis of the current literature that focused on interventional procedures in patients with IP.
Results:
A total of 35 patients underwent the procedure during the study period in our institution. The mean volume of packed red blood cells and the estimated blood loss were 487.9 mL and 1193 mL, respectively. Four patients experienced complications that were attributed to the endovascular procedure.
Conclusion:
The combination of temporary balloon occlusion and uterine artery embolization does not seem to promote better hemorrhage control than each procedure performed individually does.
Challenges of Multidrug-resistant New Delhi Metallo-beta-Lactamase (NDM-1)-producing Enterobacteriaceae in Kidney Transplant Patients
Authors: Renato Demarchi Foresto, Lucas Marengo Menezes, Laura Tomoko Nishimura, Marina Pontello Cristelli, Laila Almeida Viana, Daniel Wagner de Castro Lima Santos, Lúcio R Requião-Moura, Helio Tedesco-Silva, Jose Medina-Pestana
Journal: J Bras Nefrol (2023)
MeSH terms: Enterobacteriaceae, Kidney Transplantation / adverse effects, Case Reports, Anti-Bacterial Agents / pharmacology, Anti-Bacterial Agents / therapeutic use, Cross-Sectional Studies, Humans, Microbial Sensitivity Tests, beta-Lactamases, beta-lactamase NDM-1, beta-Lactamases, Anti-Bacterial Agents

Background:
The emergence of multidrug-resistant NDM-1-producing enterobacteriaceae strains has become a threat to inpatients, especially to immunosuppressed ones, such as kidney transplant recipients. NDM-1 is a carbapenemase that makes gram-negative bacteria resistant to many types of antibiotics. The incidence of carbapenemase-producing enterobacteria infection in solid organ transplant recipients is around 3 to 10%, with a mortality rate of up to 30%.
Methods:
We present a case series of 4 patients with NDM-1-producing enterobacteria isolated in urine cultures or rectal swabs. We also conducted a cross-sectional study 30 days after patient identification, collecting surveillance cultures (rectal swab) from all inpatients to assess the extent of spread of this resistance mechanism; a total of 101 patients were included.
Results:
Two patients were adequately treated with negative control cultures. The other two patients were not treated because they were asymptomatic and had subsequent negative urine cultures. No new colonization was identified in the cross-sectional screening, and no new cases of urinary NDM-1 infection were recorded after a 4-year follow-up.
Conclusion:
Surveillance for infections caused by multidrug-resistant strains in hospitals treating immunosuppressed patients should be continued and prompt action should be taken in cases of outbreaks of multidrug-resistant infections.
EXIT ( ex utero Intrapartum Treatment) to Airway Procedure for Twin Fetuses With Oropharyngeal Teratomas: Lessons Learned
Authors: Alice King, Sundeep G Keswani, Michael A Belfort, Ahmed A Nassr, Alireza A Shamshirsaz, Jimmy Espinoza, Joshua R Bedwell, Deepak K Mehta, Cara B Doughty, Susan Margaret Leong-Kee, Julia B Lawrence, Raphael C Sun, Timothy C Lee
Journal: Front Surg (2020)
Keywords: airway, exit, simulation, teratoma, twins
MeSH terms: Case Reports

Ex utero intrapartum treatment (EXIT) to airway has been described as a safe method to secure challenging fetal airways while on placental support. Herein, we present a unique case of a monochorionic-diamniotic twin pregnancy where both fetuses presented with oropharyngeal tumors requiring airway securement on placental bypass. A multidisciplinary tabletop simulation was convened to allow for personnel coordination between multiple services, OR equipment allocation, and preparation for a range of possible clinical scenarios. A tabletop simulation was chosen for planning since this is a simulation methodology commonly used for preparation in acute, high intensity multidisciplinary situations such as disaster preparation, and allows for exploration of multiple potential scenarios when outcomes are uncertain. The twins were urgently delivered for decreased fetal movement and decelerations in Twin B at 28 weeks 6 days. Twin A was delivered via EXIT to airway while Twin B had debulking of the tumor on placental support, with subsequent airway securement through a tracheostomy. In conclusion, for complex fetal procedures, detailed pre-operative planning with tabletop simulation may be a useful tool in achieving successful patient outcomes.
Case Report: Anti-GABA A Receptor Encephalitis in a Dog
Authors: Enrice I Huenerfauth, Christian G Bien, Corinna Bien, Holger A Volk, Nina Meyerhoff
Journal: Front Vet Sci (2022)
Keywords: gabaa receptor encephalitis, autoimmune encephalitis, dog, epilepsy, seizures
MeSH terms: Case Reports

Autoantibodies against neurotransmitter receptors detected in cerebrospinal fluid (CSF) and serum are increasingly recognized in people with human autoimmune encephalitis causing severe neurological deficits, such as seizures and behavioral abnormalities. This case report describes the first encephalitis associated with antibodies against the γ-aminobutyric acid-A receptor (GABAAR) in a dog. A young male intact Cavalier King Charles Spaniel was presented with recent onset of initial multiple generalized tonic-clonic seizures progressing into a status epilepticus. Interictally, he showed alternating stupor and hyperexcitability, ataxia, pleurothotonus and circling behavior to the left side. Magnetic resonance imaging (MRI) of the brain showed breed-specific anatomical abnormalities. Standard CSF analysis was unremarkable. Despite treatment with multiple antiseizure medications (ASMs) seizures and behavior abnormalities sustained. Immunotherapy with dexamethasone was started on the fifth day after disease manifestation. This led to rapid improvement of clinical signs. An extensive antibody search in CSF and serum demonstrated a neuropil staining pattern on a tissue-based assay compatible with GABAAR antibodies. The diagnosis was confirmed by binding of serum and CSF antibodies to GABAAR transfected Human Embryonic Kidney cells. The serum titer was 1:320, the CSF titer 1:2. At the control visit 4.5 weeks after start of immunotherapy, the dog was clinically normal. The GABAAR antibody titer in serum had strongly decreased. The antibodies were no longer detectable in CSF. Based on clinical presentation and testing for GABAAR binding antibodies, this describes the first veterinary patient with an anti-GABAAR encephalitis with a good outcome following ASM and corticosteroid treatment.
Brief Clinical Report: Hypophosphatasia-Diagnostic Considerations and Treatment Outcomes in an Infant
Authors: Sara Duffus, Bradly Thrasher, Ali S Calikoglu
Journal: Case Rep Pediatr (2018)
MeSH terms: Case Reports

Hypophosphatasia (HPP) is a rare, inherited metabolic bone disorder characterized by low serum alkaline phosphatase activity and impaired bone mineralization. Clinical manifestations and severity of symptoms vary widely in HPP, ranging from in utero death to isolated dental manifestations in adults. Treatment with enzyme replacement therapy has been reported to improve outcomes in perinatal, infantile, and childhood forms of HPP. Here, we present a case of a boy with poor linear growth, mild limb bowing, and radiographic rickets who was diagnosed with HPP before 6 months of age. Treatment with enzyme replacement therapy was initiated at 7 months of age, after which significant improvements in radiographic findings and linear growth were demonstrated. This case highlights several important challenges in the diagnosis, classification, and management of HPP.
Direct Visualization of Arterial Emboli in Moyamoya Syndrome
Authors: Julie G Shulman, Samuel Snider, Henri Vaitkevicius, Viken L Babikian, Nirav J Patel
Journal: Front Neurol (2017)
Keywords: brain ischemia, cerebral revascularization, embolism, moyamoya disease, stroke
MeSH terms: Case Reports

Background:
Hemodynamic insufficiency is often considered the cause of ischemic stroke in patients with moyamoya syndrome. While high-intensity transient signals (HITS) on transcranial Doppler (TCD) have been reported in this population, the relationship between these signals and ischemic symptoms is not clearly established. Accordingly, current treatment is directed at improving perfusion.
Clinical presentation:
We present two patients with symptoms of cerebral ischemia and angiographic findings of moyamoya syndrome. In each case, ischemia may have been caused by either hypoperfusion or embolization. Patient A presented with multifocal right middle cerebral artery (MCA) territory infarctions and angiographic findings consistent with moyamoya disease. She underwent right superficial temporal artery-MCA bypass. Intra-operatively, embolic material was observed and recorded traveling through the recipient MCA branch artery on two occasions. Postoperative TCD demonstrated HITS that resolved with uptitration of antiplatelet therapy. Patient B presented with multifocal, embolic-appearing left MCA infarctions, and unilateral angiographic moyamoya syndrome. She was found to have HITS in the left MCA, which eventually resolved with a combination of antiplatelets and anticoagulation.
Conclusion:
Hemodynamic compromise may not be the only cause of brain infarction in patients with moyamoya syndrome. Observations from these two patients provide both direct visualization and TCD evidence of embolization as a potential etiology for brain ischemia. Future investigations into the role of antithrombotic agents should be considered.