OpenMed/multicare-articles huggingface dataset:
Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome
Authors: Marco Fernandes Sr, Pedro Ferreira, Ana Lynce, Maria João Correia, Ana Margarida Ribeiro
Journal: Cureus (2023)
Keywords: cyclophosphamide pulse, hemophagocytic lymphohistiocytosis (hlh), lupus flare, protein-losing enteropathy, systemic lupus erythromatosus
MeSH terms: Case Reports

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect almost every organ. Lupus protein-losing enteropathy (PLE) is one of the rarest manifestations of gastrointestinal involvement. Lupus flare as initial presentation is rare and the disease can act as a trigger to other pathologic immune syndromes like Hemophagocytic Lymphohistiocytosis (HLH), although this association is rare. We report the case of a previously healthy African 39-year-old female patient, with a recent history of cesarean section. Admitted to the Emergency Department (ED) with diffuse abdominal pain and fever, having completed a cycle of antibiotic therapy for initially suspected endometritis. The clinical picture progressed with sustained high fever, new-onset lymphadenopathies, systemic rash, acute pulmonary edema and seizures. Laboratory findings included hyperferritinemia, hypertriglyceridemia, proteinuria and hypoalbuminemia. The auto-immune panel was positive for antinuclear antibodies (ANA), anti-dsDNA, anti-SSA and anti-SSB, anti-PL7, anti-RNP, anti-U1-SnRNP, and anti-Pm-Scl75. She also presented hypocomplementemia. An inaugural flare of SLE with multisystemic involvement and concomitant secondary Hemophagocytic Syndrome was considered and therapy with methylprednisolone pulses, Anakinra and Cyclophosphamide was started. By the end of the first cycle of cyclophosphamide, the patient presented clinical worsening with abdominal pain recrudescence and profuse diarrhea. After the exclusion of an infectious process, a Lupus PLE was assumed and Cyclophosphamide protocol was resumed, with sustained clinical improvement after the induction protocol. Despite initially suspected gynecological infection, the clinical progression with multisystemic involvement together with the auto-immune panel made the diagnosis of SLE possible, with other laboratory findings raising the suspicion of HLH. This case represents a rare report of severe SLE with multiple organ involvement accompanied by HLH. Gastrointestinal involvement with PLE added rarity and morbidity to the clinical picture. The case reinforces the idea that when organ dysfunction is due to a severe autoimmune response, supportive treatment can be lifesaving until immunosuppressive drugs reach their full effect.
Radiosurgical ablation of solitary trichoepithelioma
Authors: H C Hanumanthiah
Journal: J Cutan Aesthet Surg (2008)
Keywords: trichoepithelioma, ablation, radiofrequency, solitary
MeSH terms: Case Reports

Trichoepithelioma presents a challenge for management in view of its location. We describe the use of radiofrequency ablation in the management of this condition.
Pemphigus vulgaris with solitary toxic thyroid nodule
Authors: Mostafa Alfishawy, Karim Anwar, Amira Elbendary, Ahmed Daoud
Journal: Case Rep Endocrinol (2014)

Background. Pemphigus vulgaris is an autoimmune vesiculobullous disease, affecting the skin and mucous membranes. It is reported to be associated with other autoimmune diseases including autoimmune thyroid diseases. However we report herein a case of pemphigus vulgaris associated with autonomous toxic nodule. Case Presentation. A 51-year-old woman was evaluated for blisters and erosions that develop on her trunk, face, and extremities, with a five-year history of progressively enlarging neck mass, and a past medical history of pemphigus vulgaris seven years ago. The condition was associated with palpitation, dyspnea, and heat intolerance. Thyroid function tests and thyroid scan were compatible with the diagnosis of thyrotoxicosis due to autonomous toxic nodule. Exacerbation of pemphigus vulgaris was proved by skin biopsy from the patient which revealed histologic picture of pemphigus vulgaris. Conclusion. Autoimmune thyroid diseases are reported to associate pemphigus vulgaris. To our knowledge, this case is the first in the English literature to report association between pemphigus vulgaris and autonomous toxic nodule and highlights the possibility of occurrence of pemphigus vulgaris with a nonautoimmune thyroid disease raising the question: is it just a coincidence or is there an explanation for the occurrence of both conditions together?
Is Anti-NXP2 Autoantibody a Risk Factor for Calcinosis and Poor Outcome in Juvenile Dermatomyositis Patients? Case Series
Authors: Natasa Toplak, Pallavi Pimpale Chavan, Silvia Rosina, Tomas Dallos, Oz Rotem Semo, Cassyanne L Aguiar, Raju Khubchandani, Angelo Ravelli, Anjali Patwardhan
Journal: Front Pediatr (2022)
Keywords: anti-nxp2 autoantibodies, disease outcome, juvenile dermatomyositis, risk factors, treatment
MeSH terms: Case Reports

Juvenile dermatomyositis (JDM) has a wide spectrum of clinical presentations. In the last decade, several myositis-specific antibodies have been identified in patients with JDM and connected with specific organ involvement or specific clinical picture. It has been published that the presence of anti-NXP2 autoantibodies presents a risk for calcinosis in patients with JDM. We aimed to investigate the prevalence of calcinosis and response to the treatment in JDM patients with anti-NXP2. In a retrospective, multinational, multicenter study, data on 26 JDM (19 F, 7 M) patients with positive anti-NXP2 were collected. The mean age at disease presentation was 6.5 years (SD 3.7), the median diagnosis delay was 4 months (range 0.5-27 months). Patients were divided into two groups (A and B) based on the presence of calcinosis, which occurred in 42% of anti-NXP2 positive JDM patients (group A). Four patients already had calcinosis at presentation, one developed calcinosis after 4 months, and 6 developed calcinosis later in the disease course (median 2 years, range 0.8-7.8). The differences in laboratory results were not statistically significant between the groups. The mean age at disease presentation (5.2/7.5 years) trended toward being younger in group A. Children with calcinosis were treated with several combinations of drugs. In four cases, rituximab and, in one case, anti-TNF alpha agents were used successfully. Disease outcome (by evaluation of the treating physician) was excellent in four, good in two, stable in two, and poor in three patients. None of the patients from group B had a poor disease outcome. In conclusion, JDM patients with anti-NXP2 are prone to develop calcinosis, especially if they present with the disease early, before 5 years of age. The development of calcinosis is associated with worse disease outcomes. The combination of several immunomodulatory drugs and biologic drugs can stop calcinosis progression; however, there are no evidence-based therapies for treating calcinosis in JDM patients.
Anterior uveitis as an initial manifestation of polymyalgia rheumatica
Authors: Hiromasa Tsuda, Kozue Tanaka, Shuji Kishida
Journal: Case Rep Med (2011)
MeSH terms: Case Reports

A 74-year-old woman without contributory medical history presented with acute iridocyclitis in the right eye. Although the iridocyclitis disappeared within two weeks under topical steroid, she complained of acute progressing bilateral shoulder pain and morning stiffness of upper extremities. She was diagnosed as having polymyalgia rheumatica (PMR), and iridocyclitis was considered as its related manifestation. PMR and giant cell arteritis (GCA) are closely related conditions and frequently occur together. GCA with uveitis has been rarely noted. However, ocular symptoms in PMR have not been previously mentioned. This is a first reported case of PMR presented with uveitis, without a complication of GCA. This anterior uveitis might be caused by ischemia of the posterior ciliary arteries and their branches.
De Barsy Syndrome: A Case Report of a Rare Genetic Disorder
Authors: Kankipati Srimeghana, Saikrishna Dodda, Anagha Sk, Tamara Tango, Aishwar Dixit, Sweta Sahu
Journal: Cureus (2023)
Keywords: congenital cutis laxa, de barsy syndrome, progeroid feature, rare autosomal recessive disorder, rare genetic disorder
MeSH terms: Case Reports

De Barsy syndrome (DBS) is an exceedingly rare autosomal recessively inherited genetic disorder that manifests as premature aging with progeroid features. Typically, the skin loses its elasticity, causing laxity, wrinkling, and sagging. Other characteristics include ophthalmological, orthopedic, and neurological abnormalities. As of 2011, only 27 DBS cases had been recorded. This paper reports the case of a two-day-old female infant who was referred to the pediatrics department with complaints of lax skin, a progeroid appearance, a short stature, hazy corneas, and bilateral claw-like hands with thin overlapping fingers. She also had features of pectus excavatum and visible veins over her chest and abdomen. There was a history of third-degree consanguineous parents in this patient. This patient was diagnosed with De Barsy syndrome due to findings on the Verhoeff Van Gieson staining, which demonstrated a marked decrease in elastic tissue fibers. Palliative care was recommended for this infant. We report this case considering its extreme rarity.
Basidiomycetes Are Particularly Sensitive to Bacterial Volatile Compounds: Mechanistic Insight Into the Case Study of Pseudomonas protegens Volatilome Against Heterobasidion abietinum
Authors: Maria Isabella Prigigallo, Angelo De Stradis, Abhishek Anand, Francesco Mannerucci, Floriane L'Haridon, Laure Weisskopf, Giovanni Bubici
Journal: Front Microbiol (2021)
Keywords: heterobasidion, pseudomonas, antagonism, biocontrol, biological control agents, microbe–microbe interaction, volatile organic compounds (vocs)

Volatile organic compounds (VOCs) play an important role in the communication among organisms, including plants, beneficial or pathogenic microbes, and pests. In vitro, we observed that the growth of seven out of eight Basidiomycete species tested was inhibited by the VOCs of the biocontrol agent Pseudomonas protegens strain CHA0. In the Ascomycota phylum, only some species were sensitive (e.g., Sclerotinia sclerotiorum, Botrytis cinerea, etc.) but others were resistant (e.g., Fusarium oxysporum f. sp. cubense, Verticillium dahliae, etc.). We further discovered that CHA0 as well as other ten beneficial or phytopathogenic bacterial strains were all able to inhibit Heterobasidion abietinum, which was used in this research as a model species. Moreover, such an inhibition occurred only when bacteria grew on media containing digested proteins like peptone or tryptone (e.g., Luria-Bertani agar or LBA). Also, the inhibition co-occurred with a pH increase of the agar medium where the fungus grew. Therefore, biogenic ammonia originating from protein degradation by bacteria was hypothesized to play a major role in fungus inhibition. Indeed, when tested as a synthetic compound, it was highly toxic to H. abietinum (effective concentration 50% or EC50 = 1.18 M; minimum inhibitory concentration or MIC = 2.14 M). Using gas chromatography coupled to mass spectrometry (GC/MS), eight VOCs were found specifically emitted by CHA0 grown on LBA compared to the bacterium grown on potato dextrose agar (PDA). Among them, two compounds were even more toxic than ammonia against H. abietinum: dimethyl trisulfide had EC50 = 0.02 M and MIC = 0.2 M, and 2-ethylhexanol had EC50 = 0.33 M and MIC = 0.77 M. The fungus growth inhibition was the result of severe cellular and sub-cellular alterations of hyphae occurring as early as 15 min of exposure to VOCs, as evidenced by transmission and scanning electron microscopy observations. Transcriptome reprogramming of H. abietinum induced by CHA0's VOCs pointed out that detrimental effects occurred on ribosomes and protein synthesis while the cells tried to react by activating defense mechanisms, which required a lot of energy diverted from the growth and development (fitness cost).
Adoptive Cell Transfer of Allogeneic Epstein-Barr Virus-Specific T Lymphocytes for Treatment of Refractory EBV-Associated Posttransplant Smooth Muscle Tumors: A Case Report
Authors: Bjoern-Thore Hansen, Petra Bacher, Britta Eiz-Vesper, Steffen M Heckl, Wolfram Klapper, Karoline Koch, Britta Maecker-Kolhoff, Claudia D Baldus, Lars Fransecky
Journal: Front Immunol (2021)
Keywords: ptsmt, t-cell transfer, adoptive cell transfer, allocell, case report, posttransplant smooth muscle tumors, smooth muscle tumor, virus-specific t cells
MeSH terms: Adoptive Transfer / adverse effects, Allogeneic Cells / immunology, Epstein-Barr Virus Infections / complications, Epstein-Barr Virus Infections / therapy, Heart Transplantation / adverse effects, Herpesvirus 4, Human / immunology, Smooth Muscle Tumor / complications, Smooth Muscle Tumor / therapy, T-Lymphocytes / immunology, Case Reports, Research Support, Non-U.S. Gov't, Adoptive Transfer / methods, Epstein-Barr Virus Infections / virology, Female, Humans, Lymphoproliferative Disorders / etiology, Smooth Muscle Tumor / etiology, Transplantation, Homologous, Treatment Outcome, Viremia / complications, Viremia / therapy, Young Adult

Posttransplant smooth muscle tumors (PTSMTs) are rare Epstein-Barr virus (EBV)-associated neoplasms, mostly occurring after solid organ transplantation. Current therapeutic strategies include surgery and reduction of immunosuppressive medication. We describe for the first time a novel treatment approach for PTSMT by adoptive cell transfer (ACT) of EBV-specific T cells to a 20-year-old patient with a medical history of cardiac transplantation, posttransplant lymphoproliferative disease, and multilocular PTSMT. During ACT, mild cytokine release syndrome occurred, while no unexpected safety signals were recorded. We observed in vivo expansion of EBV-specific T cells and reduction of EBV viremia. Best response was stable disease after 4 months with reduction of EBV viremia and normalization of lactate dehydrogenase levels. ACT with EBV-specific T cells may be a safe and efficacious therapeutic option for PTSMT that warrants further exploration.
Primary Fallopian Tube Clear Cell Adenocarcinoma in Pregnancy: Case Presentation and Review of the Literature
Authors: Mohammed Malak, Stephanie Klam
Journal: Case Rep Obstet Gynecol (2015)

Primary fallopian tube cancer in pregnancy is rare and is even more so for the clear cell variant. Our case is the third case of primary fallopian tube cancer in pregnancy and the first case of clear cell adenocarcinoma of the fallopian tube in pregnancy. The patient presented with increasing pelvic pain starting in the second trimester. Serial ultrasound evaluations were performed and revealed a rapidly growing complex adnexal mass adjacent to the uterus. Her pregnancy was further complicated by spontaneous preterm labor and she delivered prematurely per vaginam at 31 weeks. She underwent an urgent laparotomy in the immediate postpartum period for acute aggravation of her right pelvic pain and fever. The diagnosis of tubal clear cell adenocarcinoma was subsequently made on histopathology examination.
Pathologic complete response of hepatoid adenocarcinoma of the stomach after chemo-immunotherapy: A rare case report and literature review
Authors: Yunxiang Zhou, Linping Dong, Linyun Dai, Sien Hu, Yongji Sun, Yulian Wu, Tao Pan, Xiawei Li
Journal: Front Surg (2023)
Keywords: pd-1 inhibitor, chemo-immunotherapy, hepatoid adenocarcinoma of the stomach, immune checkpoint inhibitor (icis), pathologic complete response, terelizumab
MeSH terms: Case Reports

Background:
Hepatoid adenocarcinoma of the stomach (HAS) is a highly malignant subtype of gastric carcinoma with specific clinicopathological features and extremely poor prognosis. We present an exceedingly rare case of complete response after chemo-immunotherapy.
Case description:
A 48-year-old woman with highly elevated serum alpha-fetoprotein (AFP) level was found to have HAS verified by pathological examination based on gastroscopy. Computed tomography scan was done and TNM staging of the tumor was T4aN3aMx. Programmed cell death ligand-1 (PD-L1) immunohistochemistry was performed, revealing a negative PD-L1 expression. Chemo-immunotherapy including oxaliplatin plus S-1 and PD-1 inhibitor terelizumab was given to this patient for 2 months until the serum AFP level decreased from 748.5 to 12.9 ng/mL and the tumor shrank. D2 radical gastrectomy was then performed and histopathology of the resected specimen revealed that the cancerous cells had disappeared. Pathologic complete response (pCR) was achieved and no evidence of recurrence has been found after 1 year of follow-up.
Conclusions:
We, for the first time, reported an HAS patient with negative PD-L1 expression who achieved pCR from the combined chemotherapy and immunotherapy. Although no consensus has been reached regarding the therapy, it might provide a potential effective management strategy for HAS patient.
Successful Treatment of Painful Synchondrosis of Bipartite Patella after Direct Trauma by Operative Fixation: A Series of Six Cases
Authors: Sarkhell Radha, Michael Shenouda, Sujith Konan, Jonathon Lavelle, Samuel Church
Journal: Open Orthop J (2017)
Keywords: anterior knee pain, bipartite patella, operative fixation, patellofemoral joint, synchondrosis rupture, trauma

Introduction:
The patella is the largest sesamoid bone in the body and may have one (77%) or multiple (23%) ossification centres. Patellar and patellofemoral joint abnormalities are a common cause of anterior knee pain but symptomatic bipartite patella is an uncommon problem.
Case series:
We report a series of six cases of painful synchondrosis in bipartite patellae, all in keen athletes following a direct blow to the anterior aspect of the knee. A complete rupture of the synchondrosis with evidence of retropatellar chondral separation was seen on MRI scan in all cases. Successful surgical fixation was undertaken with complete resolution of symptoms in all patients at an average of three months post-operatively.
Conclusion:
Painful synchondrosis of a bipartite patella in young and active individuals following direct trauma is a relatively rare cause of anterior knee pain, but may be associated with significant morbidity. In cases refractory to non-operative management, successful symptomatic treatment can be achieved by operative fixation.
Case Report: Successful Management of Refractory Keratomycosis in an Alpaca Using Penetrating Keratoplasty and Combination Antifungal Therapy (Caspofungin 0.5% and Terbinafine 1%)
Authors: Braidee C Foote, Joe S Smith, Anna Catherine Bowden, Rachel A Allbaugh, Lionel Sebbag
Journal: Front Vet Sci (2021)
Keywords: fusarium verticillioides, scopulariopsis brevicaulis, camelid ophthalmology, corneal abscess, corneal crosslinking, fungal keratitis, keratomalacia
MeSH terms: Case Reports

Fungal keratitis is a common disease in certain parts of the world and affects several species, including equids, camelids, and homo sapiens, leading to blindness or loss of the eye if the infection is not adequately controlled. Reports of clinical use of antifungals caspofungin and terbinafine are limited across both veterinary and human medical literature. The alpaca presented in this case demonstrates that deep keratomycosis can be caused by Scopulariopsis brevicaulis and Fusarium verticillioides, two previously unreported fungi to cause keratomycosis in camelids. This report demonstrates successful management with a combination of surgery and topical ophthalmic treatment with caspofungin 0.5% solution and terbinafine 1% dermatologic cream, after initially failing treatment with topical voriconazole 1% solution. Combination therapy appears more effective than monotherapy with some fungal organisms, and synergy between antifungal agents is thought to play a role in the success of combination therapy. Surgery to remove the bulk of the fungal infection is especially helpful in cases that fail initial medical therapy.
A rare giant pericardial cyst mimicking a paracardiac mass
Authors: Hakan Akbayrak, Serkan Yildirim, Murat Simsek, Mehmet Oc
Journal: Cardiovasc J Afr (2016)
MeSH terms: Magnetic Resonance Imaging, Mediastinal Cyst / diagnostic imaging, Thoracic Neoplasms / diagnostic imaging, Case Reports, Diagnosis, Differential, Heart Atria / diagnostic imaging, Heart Atria / surgery, Humans, Incidental Findings, Male, Mediastinal Cyst / pathology, Mediastinal Cyst / surgery, Middle Aged, Predictive Value of Tests, Sternotomy, Treatment Outcome

Pericardial cysts are rare benign lesions of the thoracic cavity and are mostly congenital anomalies. They are induced by an incomplete coalescence of foetal lacunae during the development of the pericardium. Pericardial cysts are usually unilocular, well marginated spherical or teardrop shaped and may be attached to the pericardium directly or by a pedicle. Of all pericardial cysts, 70 to 75% are located at the right cardiophrenic angle. We report a case that was incidentally diagnosed with only chest magnetic resonance imaging because of a paracardiac mass. In order to prevent complications, the giant pericardial cyst was excised outside of the pericardium with median sternotomy.
Selective Reduction of a Heterotopic Cesarean Scar Pregnancy Complicated by Septic Abortion
Authors: Joan Tymon-Rosario, Meleen Chuang
Journal: Case Rep Obstet Gynecol (2018)
MeSH terms: Case Reports

Background:
Heterotopic pregnancy involving the implantation of an ectopic pregnancy into a prior cesarean scar with a concurrent intrauterine pregnancy is a rare and potentially life-threatening condition with minimal information in the literature to guide treatment and management options.
Case:
A 40-year-old G5P3103 at 12 weeks and 3 days with a history of two cesarean deliveries was diagnosed with a live heterotopic pregnancy containing a cesarean scar ectopic and an intrauterine pregnancy. After selective reduction of the cesarean scar gestation with potassium chloride (KCl), the patient presented ten days later to the emergency department with septic abortion and sepsis. The patient underwent bilateral uterine artery embolization followed by ultrasound guided uterine evacuation with dilation and curettage, which was complicated by intraoperative hemorrhage and persistent bacteremia. The patient had resolution of her bacteremia after total abdominal hysterectomy.
Conclusion:
Conservative management of uterine infection resulting from selective reduction of a heterotopic pregnancy cesarean scar pregnancy may be considered; however, severe septicemia and persistent bacteremia may necessitate definitive surgical management.
Diphenhydramine as a Cause of Drug-Induced Liver Injury
Authors: Yunseok Namn, Yecheskel Schneider, Isabelle H Cui, Arun Jesudian
Journal: Case Reports Hepatol (2017)
MeSH terms: Case Reports

Drug-induced liver injury (DILI) is the most common cause of acute liver failure in the Unites States and accounts for 10% of acute hepatitis cases. We report the only known case of diphenhydramine-induced acute liver injury in the absence of concomitant medications. A 28-year-old man with history of 13/14-chromosomal translocation presented with fevers, vomiting, and jaundice. Aspartate-aminotransferase and alanine-aminotransferase levels peaked above 20,000 IU/L and 5,000 IU/L, respectively. He developed coagulopathy but without altered mental status. Patient reported taking up to 400 mg diphenhydramine nightly, without concomitant acetaminophen, for insomnia. He denied taking other medications, supplements, antibiotics, and herbals. A thorough workup of liver injury ruled out viral hepatitis (including A, B, C, and E), autoimmune, toxic, ischemic, and metabolic etiologies including Wilson's disease. A liver biopsy was consistent with DILI without evidence of iron or copper deposition. Diphenhydramine was determined to be the likely culprit. This is the first reported case of diphenhydramine-induced liver injury without concomitant use of acetaminophen.