OpenMed/multicare-articles huggingface dataset:
Pitfalls of immunotherapy: lessons from a patient with CTLA-4 haploinsufficiency
Authors: Leisa Rebecca Watson, Charlotte A Slade, Samar Ojaimi, Sara Barnes, Pasquale Fedele, Prudence Smith, Justine Marum, Sebastian Lunke, Zornitza Stark, Matthew F Hunter, Vanessa L Bryant, Michael Sze Yuan Low
Journal: Allergy Asthma Clin Immunol (2018)
Keywords: autoimmune, cd25, ctla-4 haploinsufficiency with autoimmune infiltration, cytotoxic lymphocyte antigen 4, daclizumab, il-2, immunotherapy, multiple sclerosis, primary immunodeficiency, t regulatory cell
MeSH terms: Case Reports

Background:
Daclizumab is a humanized monoclonal antibody that blocks CD25, the high affinity alpha subunit of the interleukin-2 receptor. Daclizumab therapy targets T regulatory cell and activated effector T cell proliferation to suppress autoimmune disease activity, in inflammatory conditions like relapsing and remitting multiple sclerosis. Here, we present the first report of agranulocytosis with daclizumab therapy in a patient with relapsing and remitting multiple sclerosis.
Case presentation:
Our patient was a 24-year-old Australian female with a clinical history of atopy, lymphocytic enteritis complicated by B12 deficiency, relapsing and remitting multiple sclerosis, recurrent lower respiratory tract infections, vulval/cervical intraepithelial neoplasia and melanoma. She was commenced on daclizumab therapy after failing several lines of treatment for relapsing and remitting multiple sclerosis. During a hospital admission for lymphocytic enteritis, she was incidentally diagnosed with combined immunodeficiency with hypogammaglobulinaemia and declined proposed regular intravenous immunoglobulin infusions. Following six months of daclizumab therapy, our patient presented to hospital with febrile neutropenia. No clear infective cause was found, despite numerous investigations. However, bone marrow biopsy revealed agranulocytosis with an apparent maturation block at the myeloblasts stage. Neustrophil recovery occurred following cessation of daclizumab and the initiation of T cell immunosuppressive agents including systemic corticosteroids and methotrexate. The patient was further investigated for combined immunodeficiency and whole exome sequencing revealed a novel heterozygous missense variant in cytotoxic T lymphocyte antigen 4 (CTLA4), leading to a diagnosis of CTLA-4 haploinsufficiency with autoimmune infiltration (CHAI).
Conclusion:
This case demonstrates that autoimmune disease may be the presenting feature of primary immunodeficiency and should be appropriately investigated prior to the commencement of immunotherapy. Genetic clarification of underlying primary immunodeficiency may provide critical clinical information that alters the safety of the proposed treatment strategy.
Antibody-mediated rejection in heart transplantation: case presentation with a review of current international guidelines
Authors: Octavio E Pajaro, Dawn E Jaroszewski, Robert L Scott, Anantharam V Kalya, Henry D Tazelaar, Francisco A Arabia
Journal: J Transplant (2011)

Antibody-mediated rejection (AMR) (humoral rejection) of cardiac allografts remains difficult to diagnose and treat. Interest in AMR of cardiac allografts has increased over the last decade as it has become apparent that untreated humoral rejection threatens graft and patient survival. An international and multidisciplinary consensus group has formulated guidelines for the diagnosis and treatment of AMR and established that identification of circulating or donor-specific antibodies is not required and that asymptomatic AMR, that is, biopsy-proven AMR without cardiac dysfunction is a real entity with worsened prognosis. Strict criteria for the diagnosis of cardiac AMR have not been firmly established, although the diagnosis relies heavily on tissue pathological findings. Therapy remains largely empirical. We review an unfortunate experience with one of our patients and summarize recommended criteria for the diagnosis of AMR and potential treatment schemes with a focus on current limitations and the need for future research and innovation.
Clinical and imaging features of reversible splenial lesion syndrome with language disorder
Authors: Yi Tang, Dong Zhang, Jian Ge, Jing Jin, Yumeng Liu, Siyuan Chen, Mingli He
Journal: Transl Neurosci (2020)
Keywords: cognitive impairment, corpus callosum, language impairment, reversible splenial lesion syndrome, splenium of the corpus callosum
MeSH terms: Case Reports

Reversible splenial lesion syndrome (RESLES) is a single-stage non-specific syndrome with unclear pathogenesis. There has been no report on answer delay in patients with RESLES. We report a female patient who was admitted to our department for mixed aphasia accompanied by cognitive impairment. During the rapid improvement of aphasia, there was a clear phase of language output response delay accompanied by resolution of imaging lesions. We analyzed the course and the examination results of the patient and speculated the cause and pathogenesis. RESLES-relevant knowledge was systematically reviewed, which will help doctors in the classification of cerebral function and the diagnosis of RESLES. The specific language and cognitive impairment may be associated with the damage of contact fibers in the bilateral primary and secondary sensory and motor cortices.
The Promising Effect of Nerve Decompression in Trigeminal Autonomic Cephalalgias: Report of Case Series
Authors: Mansoureh Togha, Ali Totonchi, Hojjat Molaei, Hossein Ansari
Journal: Front Neurol (2021)
Keywords: trigeminal autonomic cephalalgias, chronic paroxysmal hemicranias, hemicrania continua, paroxysmal hemicranias, pericranial nerve decompression surgery
MeSH terms: Case Reports

Trigeminal Autonomic Cephalalgias (TAC) are excruciating headaches with limited treatment options. The chronic forms of TACs, including chronic cluster, chronic paroxysmal hemicrania, and hemicrania continua, are disabling conditions. In addition to drug therapy, there are some studies regarding nerve blocking and nerve stimulation with acceptable results. Here we report four cases of decompression nerve surgery with promising results on pain control in these difficult to treat headaches.
Endoscopic Management Using Novel Haemostatic Agents for Immediate Bleeding during Endoscopic Retrograde Cholangio-Pancreatography
Authors: Cosmas Rinaldi Adithya Lesmana, Sharon Sandra, Maria Satya Paramitha, Rino Alvani Gani, Laurentius A Lesmana
Journal: Can J Gastroenterol Hepatol (2023)
MeSH terms: Cholangiopancreatography, Endoscopic Retrograde / adverse effects, Cholangiopancreatography, Endoscopic Retrograde / methods, Hemostatics / therapeutic use, Gastrointestinal Hemorrhage / etiology, Gastrointestinal Hemorrhage / surgery, Humans, Sphincterotomy, Endoscopic / adverse effects, Treatment Outcome, Hemostatics

Bleeding after endoscopic sphincterotomy (ES) remains as a major challenge during ERCP procedure. Standard endoscopic haemostatic procedures have demonstrated good performance for bleeding control. Novel endoscopic haemostatic agents have also been widely used in gastrointestinal bleeding management. Regardless, there is still a paucity of high-quality evidence evaluating the practicality of these agents in ERCP. This case series study was performed on the patients who underwent ERCP procedure in a tertiary referral private hospital within 2 years period. Post-ES immediate bleeding is defined as the onset of bleeding at the time of sphincterotomy. Treatment groups for post-ES bleeding are divided into (1) standard haemostatic methods and (2) novel haemostatic agents. There were 40 patients who received standard haemostatic treatment and 60 patients who received novel haemostatic agents. Initial haemostasis was achieved in all patients. Two patients who received standard haemostatic treatment had rebleeding. Meanwhile, no patients in novel haemostatic treatment group had rebleeding. In conclusion, novel haemostatic agent can be considered as an easy and practical method in daily practice, especially when an ERCP procedure is performed. Further studies with larger sample size which, if possible, can also include a cost-effectiveness analysis are still required to implement these agents as a standard procedure in clinical practice. (This abstract has been presented at the American College of Gastroenterology meeting October 2021).
Revascularization of an Immature Permanent Central Incisor with Complicated Crown Root Fracture: A Case Report
Authors: Anu John, Amitha M Hegde, Preeth Shetty, Shreema Shetty
Journal: Int J Clin Pediatr Dent (2019)
Keywords: crown root fracture, immature teeth, revascularization
MeSH terms: Case Reports

The treatment of complicated crown root fracture in children possesses a great challenge to pedodontists. A 10-year-old boy presented with trauma to the permanent maxillary left central incisor. Upon clinical and radiographic examination, revealed mobility of coronal fragment, open apex, and vertical fracture involving enamel dentin and pulp. Instead of the standard root canal treatment protocol, revascularization was performed after stabilizing the coronal fragment. The detachment of the coronal fragment occurred following a fall for the second time after 1 month of placement of mineral trioxide aggregate (MTA) over the blood clot. The coronal seal was intact; hence, the coronal fragment was reattached and the crown was placed. At 12-month follow-up, clinical and radiographic evaluation showed that the tooth was asymptomatic and functional. With increasing scope of clinical facts, patient, and practitioner favorable reception, regenerative techniques may be used as a standard technique in treating complicated crown root fracture in young permanent teeth.
How to cite this article:
John A, Hegde AM, et al. Revascularization of an Immature Permanent Central Incisor with Complicated Crown Root Fracture: A Case Report. Int J Clin Pediatr Dent 2019;12(1):59-63.
Sick Sinus Syndrome and Takotsubo Cardiomyopathy
Authors: Ahmed S Yassin, Ahmed Subahi, Hossam Abubakar, Ahmed Rashed, Mohamed Shokr
Journal: Case Rep Cardiol (2018)
MeSH terms: Case Reports

Background. Takotsubo cardiomyopathy is associated with increased risk of ventricular arrhythmias, atrial fibrillation, and bradyarrhythmias. However, sinus node dysfunction is relatively infrequent in the setting of takotsubo cardiomyopathy. Case Report. We are reporting a case of a 73-year-old woman with a history of asymptomatic sinus bradycardia who developed sick sinus syndrome complicated by takotsubo cardiomyopathy. Conclusion. Acute symptomatic sick sinus syndrome in patients with preexisting silent sinus node dysfunction can trigger takotsubo cardiomyopathy. Understanding precipitating factors of takotsubo cardiomyopathy and identifying the patients at risk of life-threatening arrhythmia can help in refining risk stratification and therapy planning. Patients with sick sinus syndrome complicated by takotsubo cardiomyopathy may benefit from pacemaker implantation. However, evaluation on a case-by-case basis is mandatory.
Successful endovascular repair with coil embolization of ruptured right internal thoracic artery aneurysm
Authors: Yamasandi Siddegowda Shrimanth, Atit A Gawalkar, Parag Barwad, Soumitra Ghosh, Samman Verma, Arun Sharma, Sanjeev Naganur
Journal: J Vasc Bras (2022)
Keywords: ct angiography, aneurysm rupture, coil embolization, endovascular repair, internal thoracic artery aneurysm, percutaneous repair
MeSH terms: Case Reports

Internal thoracic artery aneurysms (ITAAs) are rare with wide variation in clinical presentation and a high risk of rupture. Endovascular techniques are increasingly being used for treatment of such aneurysms over surgical repair in recent times. A 34-year-old male presented with progressive swelling of the right anterior chest wall for 2 weeks and was diagnosed with right internal thoracic artery aneurysm with contained rupture. He underwent successful endovascular repair with coil embolization of ruptured right ITAA. Post intervention computed tomography (CT) angiography confirmed sealing of the ruptured aneurysm with no residual filling of the sac. At six months follow-up he is doing well with complete resolution of hematoma. This case demonstrates that an endovascular approach with coil embolization is a feasible and safe option for treating the rare ruptured ITAAs.
Clear Cell Adenocarcinoma Arising from Endometriosis in the Groin: Wide Resection and Reconstruction with a Fascia Lata Tensor Muscle Skin Flap
Authors: Shozo Yoshida, Akira Onogi, Masamitsu Kuwahara, Tomoko Uchiyama, Hiroshi Kobayashi
Journal: Case Rep Obstet Gynecol (2018)
MeSH terms: Case Reports

We herein report a case of clear cell carcinoma arising from endometriosis in the groin in a 53-year-old woman. The findings of MRI and FDG/PET-CT indicated a malignant tumor, and surgical biopsy confirmed adenocarcinoma of the female genital tract. The tumor including a part of the abdominal rectus muscle and rectus sheath, subcutaneous fat, skin, and the right inguinal ligament was resected en bloc. The defect in the abdominal wall was reconstructed with a fascia lata tensor muscle skin flap. The tumor was composed of clear cell adenocarcinoma arising from extrapelvic endometriosis. The patient received chemotherapy with gemcitabine and carboplatin for 6 cycles and had no evidence of recurrence 7 months after the treatment. We herein described the diagnosis and surgical management of endometriosis-associated carcinoma in the groin.
Treacher Collins Syndrome with choanal atresia: a case report and review of disease features
Authors: Eduardo C Andrade, Vanier S Júnior, Ana L S Didoni, Priscila Z Freitas, Araken F Carneiro, Fabiana R Yoshimoto
Journal: Braz J Otorhinolaryngol (2005)
MeSH terms: Choanal Atresia / pathology, Mandibulofacial Dysostosis / pathology, Case Reports, Adolescent, Choanal Atresia / etiology, Choanal Atresia / surgery, Female, Humans, Mandible / surgery, Mandibulofacial Dysostosis / complications, Mandibulofacial Dysostosis / surgery

Treacher Collins Syndrome--or mandibulofacial dysostosis--is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.
Thoracoscopic Diagnosis of Follicular Lymphoma Relapsing after 13 Years
Authors: Serda Kanbur, Onur Derdiyok, Hakan Kiral, Hakan Yilmaz, Mine Demir, Cagatay Tezel, Volkan Baysungur, Serdar Evman
Journal: Surg J (N Y) (2019)
Keywords: follicular lymphoma, relapsing after 13 years, thoracic surgery
MeSH terms: Case Reports

Relapse in lymphoproliferative malignancies is not an exceptional entity and generally occurs within the first 2 or 3 years following the primary treatment. Lymph node biopsy is essential for the diagnosis of relapse and treatment. A 64-years-old woman was referred to our clinic for back pain and dyspnea. Chest X-ray and computed tomography (CT) showed pleural thickening in the right hemithorax and pleural effusion. Hereby, we report a patient with a history of follicular lymphoma treatment 13 years ago, presenting with unilateral pleural effusion and being diagnosed, unpredictably, with relapsing lymphoma by video-assisted thoracoscopic surgery pleural biopsy.
Concomitant Presence of Ovarian Tumors (Teratoma and Granulosa Cell Tumor), and Pyometra in an English Bulldog Female Dog: A Case Report
Authors: Carlos A Oviedo-Peñata, Luis Hincapie, Carlos Riaño-Benavides, Juan G Maldonado-Estrada
Journal: Front Vet Sci (2020)
Keywords: dog, neoplasia, ovary, tumor, uterus
MeSH terms: Case Reports

Background: The diagnosis of ovarian tumors in dogs is usually complicated because the clinical signs can be very discrete and can be easily confused with other diseases. There are few reports of ovarian tumors with different cellular characteristics in the same dog. Our objective was to describe an unusual case of the concomitant presence of ovarian teratoma and granulosa cell tumors in a female dog presenting symptoms compatible with pyometra at clinical consultation. Clinical history: A non-spayed 6-years-old female English Bulldog was attended at the consultation, with no history of previous steroid hormonal treatment. The dog had presented regular estrus every 6 months; 3 months elapsed between the last estrus and consultation. The dog had presented vulvar discharge for more than 2 weeks. Clinical and laboratory findings: the patient presented a slightly pale oral mucosa, decay, vulvar edema, and mucous-purulent uterine discharge. The ultrasound examination revealed the presence of neoformations in the ovaries, and evidence of cystic endometrial hyperplasia-pyometra in the uterus. Treatment: We performed a ventral ovariohysterectomy. During the surgical procedure, it was found several masses in the left and right ovaries, exhibiting characteristics of other tissues different from ovarian tissue. All samples were sent for histopathological examination. The diagnosis was a granulosa cell tumor in the left ovary and a well-differentiated teratoma in the right ovary. Conclusion: Practitioners must improve the use of diagnostic tools when attending non-spayed dogs at advanced ages (more than 6 years old), which would probably be at high risk of suffering from undetected ovarian tumors, some of them with malignancy behavior.
Composite Lymphoma Comprising Extranodal NK/T-Cell Lymphoma and Diffuse Large B-Cell Lymphoma
Authors: Shin Nagai, Junji Hiraga, Noriyuki Suzuki, Naruko Suzuki, Yusuke Takagi, Michihiko Narita, Yoshitoyo Kagami
Journal: Case Rep Hematol (2018)
MeSH terms: Case Reports

We report a rare case of composite lymphoma comprising extranodal NK/T-cell lymphoma, nasal type, (ENKL) and diffuse large B-cell lymphoma (DLBCL) in a 70-year-old man complaining of fatigue. Computed tomography showed multiple consolidations in both lungs, and ENKL was diagnosed from transbronchial lung biopsy. Positron emission tomography also detected abnormal uptake in the stomach, and DLBCL was diagnosed from subsequent gastroscopy. Two courses of chemotherapy including rituximab achieved reduction in DLBCL, but ENKL proved resistant to this treatment and progressed. Concomitant ENKL and DLBCL have not been previously described among reports of composite lymphomas.
Iatrogenic hepatic pneumovenogram
Authors: Govarthanan Rajendiran, Sulaiman Rathore, Gurmeet Sidhu, James Catevenis
Journal: Crit Care Res Pract (2011)
MeSH terms: Case Reports

Accidental air entry during central venous catheterization is a preventable iatrogenic complication that can cause venous air embolism (VAE). Many cases of VAE are subclinical with no adverse outcome and thus go unreported. Usually, when symptoms are present, they are nonspecific, and a high index of clinical suspicion of possible VAE is required to prompt investigations and initiate appropriate therapy. Occasionally large embolism can lead to life-threatening acute cor pulmonale, asystole, sudden death, and arterial air embolism in the presence of shunt or patent foramen ovale. This paper discusses VAE during emergency central line placement and the diagnostic dilemma that it can be created in critically ill patients. All necessary precautions have to be strictly followed to prevent this iatrogenic complication.
Case report: Monoclonal CGRP-antibody treatment in a migraine patient with a mutation in the mitochondrial single-strand binding protein (SSBP1)
Authors: Katharina Kaltseis, Elisabetta Indelicato, Gregor Broessner, Sylvia Boesch
Journal: Front Neurol (2022)
Keywords: cgrp, case report, headache, migraine, mitochondrial disease, prophylactic treatment
MeSH terms: Case Reports

Background:
There is a growing body of mitochondrial disorders that are associated with headaches, albeit only one of them is currently listed in the latest International Classification of Headache Disorders, 3rd edition (ICHD-3). Headache frequency and headache presentation can vary widely in this respective patient group. Acute and preventive migraine treatment can be quite challenging-the use of several established medications is often limited due to their side effects in the setting of mitochondrial dysfunction and multi-organ disease.
Case presentation:
Along with a review of the literature on treatment options in patients with mitochondrial disorders and migraine headaches, we present the case of a 23-year-old male with a homozygous mutation in the mitochondrial single-strand binding protein (SSBP1) with chronic migraine with aura. After failing several standard of care prophylactics due to either side effects or inefficacy, he was successfully treated with a monoclonal anti-CGRP-antibody as a preventive migraine treatment. The monoclonal antibody was well tolerated and showed adequate efficacy with a sustained > 50% reduction in monthly headache days after 3 years of treatment.
Conclusion:
Migraine is often challenging to treat in patients with mitochondriopathy due to therapy-limiting comorbidities. Monoclonal CGRP-antibodies might be a safe treatment option in the prevention of migraine headaches in patients with a mitochondrial disorder.
Squamous cell carcinoma arising from an oral lichenoid lesion: a case report
Authors: Ali Taghavi Zenouz, Masoumeh Mehdipour, Rana Attaran, Ayla Bahramian, Paria Emamverdi Zadeh
Journal: J Dent Res Dent Clin Dent Prospects (2012)
Keywords: oral lichen plan, lichenoid reaction, squamous cell carcinoma
MeSH terms: Case Reports

Lichenoid reactions represent a family of lesions with different etiologic factors and a common clinical and histologic ap-pearance. Lichen planus is included with lichenoid reactions and is a relatively common chronic mucocutaneous disorder. The most important complication of lichenoid reactions is the possibility of malignant transformation. That is why it has been considered a precancerous condition. Although the malignant transformation rate varies widely in the literature, from 0.4 to 6.5 percent, in most studies it does not exceed 1%. The aim of this paper is to report a rare case of squamous cell car-cinoma (SCC) arising within an oral lichenoid lesion in a 17-year-old woman, where SCC is very uncommon. The patient did not have any risk factors and was healthy. The lesion was located on the border of the tongue. In view of thecommon occurrence of OLP (oral lichen planus) and the unresolved issues regarding its premalignant potential, this case report illus-trates the need for histologic confirmation and a close follow-up of clinical lesions with lichenoid features.