OpenMed/multicare-articles huggingface dataset:
Case report: Active clinical manifestation of endocardial fibroelastosis in adolescence in a patient with mitral and aortic obstruction-histologic presence of endothelial-to-mesenchymal transformation
Authors: Daniel Diaz-Gil, Chrystalle Katte Carreon, Natalia Silva-Gomez, Alan E Benheim, Sitaram M Emani, Pedro J Del Nido, Gerald R Marx, Ingeborg Friehs
Journal: Front Cardiovasc Med (2022)
Keywords: abl1, endocardial fibroelastosis, endothelial-to-mesenchymal transformation, flow disturbance, hypoplastic left heart complex
MeSH terms: Case Reports

This is the first description of active clinical manifestation of endocardial fibroelastosis (EFE) and remodeling of the endocardium via endothelial-to-mesenchymal transformation (EndMT) in an adolescent with Shone's variant hypoplastic left heart complex (HLHC) and a genetic heterozygous ABL1 variant. While EFE has not been typically associated HLHC or Shone's syndrome, in this patient flow alterations in the left ventricle (LV), combined with genetic alterations of intrinsic EndMT pathways led to active clinical manifestation of EFE in adolescence. This case emphasizes that new therapies for EFE might need to focus on molecular factors influenced by intrinsic and extrinsic stimuli of EndMT.
Circulatory Support with Venoarterial ECMO Unsuccessful in Aiding Endogenous Diltiazem Clearance after Overdose
Authors: Erin N Frazee, Sarah J Lee, Ejaaz A Kalimullah, Heather A Personett, Darlene R Nelson
Journal: Case Rep Crit Care (2014)

Introduction. In cardiovascular collapse from diltiazem poisoning, extracorporeal membrane oxygenation (ECMO) may offer circulatory support sufficient to preserve endogenous hepatic drug clearance. Little is known about patient outcomes and diltiazem toxicokinetics in this setting. Case Report. A 36-year-old woman with a history of myocardial bridging syndrome presented with chest pain for which she self-medicated with 2.4 g of sustained release diltiazem over the course of 8 hours. Hemodynamics and mentation were satisfactory on presentation, but precipitously deteriorated after ICU transfer. She was given fluids, calcium, vasopressors, glucagon, high-dose insulin, and lipid emulsion. Due to circulatory collapse and multiorgan failure including ischemic hepatopathy, she underwent transvenous pacing and emergent initiation of venoarterial ECMO. The peak diltiazem level was 13150 ng/mL (normal 100-200 ng/mL) and it remained elevated at 6340 ng/mL at hour 90. Unfortunately, the patient developed multiple complications which resulted in her death on ICU day 9. Conclusion. This case describes the unsuccessful use of ECMO for diltiazem intoxication. Although past reports suggest that support with ECMO may facilitate endogenous diltiazem clearance, it may be dependent on preserved hepatic function at the time of cannulation, a factor not present in this case.
Butorphanol-midazolam combination therapy for the treatment of intracranial hypertension in a patient with tuberculous meningitis: a case study
Authors: Hiroshi Kataoka, Satoshi Ueno
Journal: Springerplus (2013)
Keywords: butorphanol, headache, intracranial hypertension, midazolam, tuberculous meningitis

Introduction:
Intracranial hypertension, which often occurs in patients with tuberculous meningitis, is associated with high morbidity and mortality. We describe a patient with tuberculous meningitis who had intracranial hypertension -induced fulminant headache that responded to intravenous butorphanol-midazolam combination therapy.
Case presentation:
A 50-year-old woman with a fever and headache for 24 days was given a diagnosis of tuberculous meningitis on the basis of the results of polymerase chain reaction amplification and Ziehl-Neelsen staining. Headache with vomiting developed despite administration of steroids, osmotic, and antituberculosis treatments. The patient was admitted in a confusional state. The initial pressure (420 mmHg) in cerebrospinal fluid was increased. She was given intravenous mannitol, dexamethasone, pentazocine and diazepam, or she was sedated with propofol, with no response. Next, a combination of butorphanol and midazolam was infused intravenously and finally resolved the confusional state. The initial pressure decreased, and she no longer complained of headache requiring medication.
Discussion and evaluation:
The butorphanol-midazolam combination therapy may have reduced intracranial pressure, leading to down-regulation of headache. Sedation induced by such combination of drugs was not accompanied by amnesia or impaired psychomotor function.
Conclusions:
The butorphanol-midazolam combination therapy might be an option for the management of intracranial hypertension in central nervous system infections.
Hemophagocytic Lymphohistiocytosis Associated with Respiratory Syncytial Virus Infection in an Immunocompetent Elderly Patient
Authors: Ralph Kamel, Rubal Sharma, Divya Asti, Arshpal Gill, Yevgeniy Skaradinskiy
Journal: Case Rep Hematol (2020)
MeSH terms: Case Reports

Hemophagocytic lymphohistiocytosis is a serious and potentially fatal disorder characterized by excessive immune system activation. The disorder is diagnosed mainly based on laboratory, clinical, and pathologic criteria. The spectrum comprises hereditary or "primary" HLH that comprises genetically heterogeneous conditions, occurring during childhood. The secondary form presents later in life and is associated with several conditions mainly malignancy, autoimmune diseases, viral or bacterial infections, and hematological diseases. We present the case of an 80-year-old female patient who initially presented with an acute viral syndrome secondary to respiratory syncytial virus. The hospital course was complicated by disseminated intravascular coagulation and shock with multiorgan failure. Extensive workup revealed that several of the criteria for hemophagocytic lymphohistiocytosis were met. A review of literature fails to identify cases of hemophagocytic lymphohistiocytosis associated with respiratory syncytial virus in immunocompetent adults. This case report provides further insight on RSV as a possible etiologic agent associated with HLH and the importance of early recognition of this fatal disorder in RSV-positive patients who show unpredictable clinical decompensation.
Modified keyhole technique for the treatment of parastomal hernia: A case series
Authors: Ryohei Ando, Ryuichiro Sato, Masaya Oikawa, Tetsuya Kakita, Takaho Okada, Takashi Tsuchiya
Journal: Int J Surg Case Rep (2020)
Keywords: intraperitoneal, keyhole, mesh repair, parastomal hernia, sugerbaker

Introduction:
Parastomal hernia is one of the common complications of permanent stoma, and its incidence was nearly 50%. Surgical management is challenging and associated with relatively high recurrence rate. Mesh repair was demonstrated to reduce recurrence compared to non-mesh repair, and modified Surgerbaker and keyhole technique are the most cited intraperitoneal mesh repairs. In the keyhole technique, recurrence often occurs by herniation through the central hole. We present four parastomal hernia cases successfully repaired by modified keyhole technique, in which a cylinder-shaped synthetic mesh was attached to the keyhole mesh to cover the angle between the keyhole and the bowel.
Presentation of cases:
There were 1 male and 3 females with mean BMI of 25.7 kg/m2. Mean operative time was 114 min. There were two end-colostomies, one loop-ileostomy and one ileal conduit cases. Postoperative complication was observed in two cases, which was cerebral infarction and paralytic ileus. There were neither infectious complications nor seroma formation, and mean postoperative hospital stay was 18 days. With mean follow-up time of 36 months (range 10-66), we experienced no recurrence.
Conclusions:
Having lower recurrence rate, the modified Sugerbaker technique is considered preferable over the keyhole technique, but the bowel going to the stoma needs to be lateralized enough to be covered by relatively large mesh, which is not always accomplished. In such instances, our modified keyhole technique would be a feasible alternative.
Drug interaction leading to prolonged sedation in a postoperative high risk coronary bypass surgery patient
Authors: Shrikant Bobade, Vinay Kulkarni, Sudhir Dhumne, Saurabh Barde, Jitesh Chauhan, Avinash Sharma
Journal: Indian J Crit Care Med (2011)
Keywords: erythromycin, midazolam, prolonged sedation
MeSH terms: Case Reports

Use of midazolam infusion in mechanically ventilated patient is an established practice in critical care. In our case, the use of erythromycin as a prokinetic agent for better tolerance of enteral feeding and paralytic ileus led to an interaction between midazolam and erythromycin, which resulted in prolonged and deeply sedated patient. In a critically ill patient, there is always a possibility of multiple drug interactions. It is important to understand them and they should be considered before starting new medication.
Diagnostic dilemma of a rare, giant retroperitoneal schwannoma: a case report and review of literature
Authors: Mahendra Singh, Lovekesh Kumar, Rajkumar Chejara, Om Prakash Prasad, Yuvraj Kolhe, Ashish Saxena
Journal: Case Rep Oncol Med (2014)

Schwannoma is a benign tumour of peripheral nerve sheath. It usually arises from head, neck, and trunk. Retroperitoneal schwannoma is a rare entity, accounting for only 0.3-3% of total schwannomas. Majority of retroperitoneal schwannomas reported in literature have a diameter of 5 to 15 cm. Preoperative diagnosis is difficult due to low frequency, nonspecific clinical course, and nonspecific imaging features. Histology usually provides definitive diagnosis. Schwannomas are solitary, well-circumscribed, and noninvasive, so complete surgical excision provides good result. We report a case of a 23-year-old male, who presented with progressive abdominal distension and intermittent episodes of intestinal obstruction. CECT was suggestive of huge solid-cystic mass in abdominopelvic region. Image guided percutaneous aspiration revealed around 1 litre of frank pus and FNAC was suggestive of abscess. Exploratory laparotomy revealed a large 32 × 28 × 26 cm mass with solid and cystic components containing 1 litre of pus. Histological features of tumour were suggestive of benign schwannoma and immunohistochemistry for S-100 was positive. Postoperative recovery was uneventful. We report this case of a retroperitoneal schwannoma because of giant size, rare location, unusual presentation, and diagnostic dilemma.
Epidural Spinal Cord Stimulation Acutely Modulates Lower Urinary Tract and Bowel Function Following Spinal Cord Injury: A Case Report
Authors: Matthias Walter, Amanda H X Lee, Alex Kavanagh, Aaron A Phillips, Andrei V Krassioukov
Journal: Front Physiol (2018)
Keywords: epidural spinal cord stimulation, neurogenic bowel dysfunction, neurogenic lower urinary tract dysfunction, spinal cord injury, urodynamic investigation

Regaining control of autonomic functions such as those of the cardiovascular system, lower urinary tract and bowel, rank among the most important health priorities for individuals living with spinal cord injury (SCI). Recently our research provided evidence that epidural spinal cord stimulation (ESCS) could acutely modulate autonomic circuits responsible for cardiovascular function after SCI. This finding raised the question of whether ESCS can be used to modulate autonomic circuits involved in lower urinary tract and bowel control after SCI. We present the case of a 32-year-old man with a chronic motor-complete SCI (American Spinal injury Association Impairment Scale B) at the 5th cervical spinal segment. He sustained his injury during a diving accident in 2012. He was suffering from neurogenic lower urinary tract and bowel dysfunction. Epidural stimulation of the lumbosacral spinal cord immediately modulated both functions without negatively affecting the cardiovascular system. Specifically, the individual's bowel function was assessed using different pre-set configurations and stimulation parameters in a randomized order. Compared to the individual's conventional bowel management approach, ESCS significantly reduced the time needed for bowel management (p = 0.039). Furthermore, depending on electrode configuration and stimulation parameters (i.e., amplitude, frequency, and pulse width), ESCS modulated detrusor pressure and external anal sphincter/pelvic floor muscle tone to various degrees during urodynamic investigation. Although, ESCS is currently being explored primarily for restoring ambulation, our data suggest that application of this neuroprosthetic intervention may provide benefit to lower urinary tract and bowel function in individuals with SCI. To fully capitalize on the potential of improving lower urinary tract and bowel function, further research is needed to better understand the neuronal pathways and identify optimal stimulation configurations and parameters.
Presurgical naso-alveolar molding paired with cheiloplasty to treat median cleft lip deformity in holoprosencephaly
Authors: Satoshi Takagi, Ayumu Tsukamoto, Yoshihisa Kawakami, Sachio Tamaoki, Hiroyuki Ohjimi
Journal: Case Reports Plast Surg Hand Surg (2020)
Keywords: holoprosencephaly, cheiloplasty, median cleft, presurgical nasoalveolar molding
MeSH terms: Case Reports

We report median cleft lip in an infant girl with lobar-typed holoprosencephaly who underwent presurgical naso-alveolar molding and subsequent cheiloplasty. At seven months postoperatively, we observed an upper lip with natural cupid-bow-shape formed with a nasal dome and two nostrils separated with reconstructed columella, which were maintained for eight years.
Dental Prosthetic Treatments in Cleidocranial Dysplasia: Case Report and Literature Review
Authors: Yosra Mabrouk, Sinda Ammar, Amel Labidi, Lamia Mansour, Sonia Ghoul
Journal: Case Rep Dent (2020)
MeSH terms: Case Reports

Cleidocranial dysplasia (CCD) is a rare inherited skeletal syndrome. There is no consensus regarding the dental treatment strategy. Objectives. To report a rare case of cleidocranial dysplasia and to summarize the current clinical and dental features and prosthetic treatment of similar CCD patients reported in the literature. Results. A 17-year-old girl was diagnosed with CCD. She had a short stature with the ability to bring the shoulders under the chest. All remaining teeth were deciduous except the four first molars were permanent. The maxilla was hypoplastic with a relative prognathism of the mandible. The cone-beam computed tomography examination showed a distorted and incomplete root formation of the permanent teeth. She was treated with both, complete and partial, removable overdentures. PubMed was used for the literature research using the following keys words "Cleidocranial Dysplasia"[Mesh], "Prosthodontics"[Mesh], "Dental Care"[Mesh], "cleidocranial dysostosis," and "dental treatment." The retention of deciduous teeth was described in the majority of cases. All the patients had supernumerary teeth. The most used treatments were dental prosthetics and orthodontics. The fixed prosthetic implant was the most used type of prosthetic treatment. Among the 15 cases who specified the type of prosthetic treatment, seven patients received removable dentures. Prosthetics was indicated especially for aged patients. Conclusion. Removable prostheses are a good solution that rapidly restores esthetics and functions. The use of implants for these patients needs to be validated by a long-term follow-up.
Radiotherapy for primary thyroid adenoid cystic carcinoma
Authors: Xiaoyu Duan, Tingting Hu, Hongyi Cai, Lili Lin, Lu Zeng, Huixia Wang, Lei Cao, Xuxia Li
Journal: Open Life Sci (2023)
Keywords: metastasis, primary thyroid adenoid cystic carcinoma, radiotherapy
MeSH terms: Case Reports

Primary thyroid adenoid cystic carcinoma (PTACC) is an extremely rare type of mucin-secreting adenocarcinoma. Currently, it is difficult to diagnose, and it lacks standard treatment protocols. We report the case of a 53-year-old female patient with PTACC who underwent additional intensity-modulated radiotherapy 1 month after surgical treatment with an uneventful course. No invasion or distant metastasis was detected at the 7-month follow-up after radiotherapy, and the prognosis was favorable. In this case, herein, we have summarized the diagnostic features of the disease and proposed that postoperative adjuvant radiotherapy can significantly improve the patient's prognosis. Finally, we further confirmed the important role of radiotherapy in PTACC by reviewing relevant literature, which may provide clinicians with valuable treatment experience.
Idiopathic Myointimal Hyperplasia of the Mesenteric Veins: A Case Report and Scoping Review of Previously Reported Cases From Clinical Features to Treatment
Authors: Hui Li, Hong Shu, Hong Zhang, Mingming Cui, Yuying Gao, Feng Tian
Journal: Front Med (Lausanne) (2022)
Keywords: cronh’s disease, idiopathic myointimal hyperplasia of the mesenteric veins, inflammatory bowel disease, ischemic enteritis, scoping review
MeSH terms: Review

Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare and poorly understood disease. It is characterized by non-thrombotic and non-inflammatory occlusion of the mesenteric veins secondary to intimal smooth muscle hyperplasia. The etiology of IMHMV is unknown, and its clinical presentations include abdominal pain, bloody diarrhea, and weight loss. IMHMV is commonly mistaken for inflammatory bowel disease because of the similarity in symptoms and endoscopic findings. Herein, we report the case of a 64-year-old man with IMHMV and present an overview of all reported cases of IMHMV. In this review, we analyzed 70 cases to summarize the etiology, clinical manifestations, and diagnosis of IMHMV and hope to raise clinicians' awareness of this entity.
A spontaneous transomental hernia through the greater omentum
Authors: Anisse Tidjane, Benali Tabeti, Nabil Boudjenan Serradj, Abdessamed Djellouli, Noureddine Benmaarouf
Journal: Pan Afr Med J (2015)
Keywords: internal hernia, acute intestinal obstruction, transomental hernia
MeSH terms: Hernia, Abdominal / pathology, Herniorrhaphy / methods, Omentum / pathology, Case Reports, Aged, Hernia, Abdominal / diagnosis, Hernia, Abdominal / surgery, Humans, Male

Transomental hernia is the rarest form of internal hernias. Clinical expression of this pathology is ambiguous and diagnosis is often made at complication phase, after irreversible strangulation of the herniated loop. Radiological diagnosis is still difficult and intraoperative exploration usually allows discovering this pathology when patient is operated for acute intestinal obstruction. Treatment is surgical and aims to treat intestinal obstruction and prevent recurrence. We describe the case of a 65 years old male operated for a preoperatively suspected internal hernia; surgical exploration found a transomental hernia trough the greater omentum.
Complete Obstruction of Endotracheal Tube in an Infant with a Retropharyngeal and Anterior Mediastinal Abscess
Authors: Dennis B Thapa, Nathaniel H Greene, Andrea G Udani
Journal: Case Rep Pediatr (2017)
MeSH terms: Case Reports

Intraoperative ventilatory failure is not an uncommon complication; however, acute endotracheal obstruction by a foreign body or blood clot can be difficult to quickly discriminate from other causes. Once the diagnosis is made, quick action is needed to restore ventilation. The ultimate solution is to exchange the endotracheal tube; however, there can be other ways of resolving this in situations where reintubation would be difficult or unsafe. This case report discusses such an event in an infant with multiple airway challenges including a retropharyngeal and anterior mediastinal abscess. We have also formulated a pathway based on various case reports involving complete ETT obstruction.
Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report
Authors: Shalabh Arora, Sameer Rastogi, Shamim Ahmed Shamim, Adarsh Barwad, Maansi Sethi
Journal: Clin Sarcoma Res (2020)
Keywords: immunotherapy, pazopanib, pembrolizumab, undifferentiated pleomorphic sarcoma
MeSH terms: Case Reports

Background:
Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas.
Case presentation:
A 63 year old woman with metastatic undifferentiated pleomorphic sarcoma progressed after two lines of palliative combination chemotherapy-doxorubicin with olaratumab, and gemcitabine with docetaxel. In view of significant symptoms, she was treated with pazopanib in combination with pembrolizumab. She had remarkable radiological and clinical improvement, with a manageable toxicity profile and an ongoing response at ten months of therapy.
Conclusions:
Undifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas-this finding warrants further evaluation in clinical trials.
Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia-A Case Report and Review of the Literature
Authors: Sameera A Alsafwani, Abdulwahed Al-Saeed, Rehab Bukhamsin
Journal: Case Rep Hematol (2017)
MeSH terms: Case Reports

Bone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. The pathophysiology of BMN is not well known; however, occlusion of the bone marrow microcirculation with subsequent hypoxia and cell injury has been thought to be common underlying features. Malignancy has been identified to be the primary cause in 90% of the cases whereas SCD was found in only 2%. In this report we present an unusual case of SCD with late onset of the disease whose initial presentation was extensive BMN. The patient was not known previously to have SCD, when suddenly she presented with severe cytopenias and marked elevation in serum lactate dehydrogenase (LDH). Bone marrow examination was done to exclude bone marrow infiltration, and BMN with dilated marrow sinuses full of irreversibly sickled cells were the unexpected findings. Patients with a mild SCD phenotype are at high risk of BMN. Thus, a high index of suspicion must be borne in mind, particularly in an area of high SCD prevalence, to recognize and prevent this catastrophic complication.