OpenMed/multicare-articles huggingface dataset:
Genotypic and phenotypic character of Chinese neonates with congenital protein C deficiency: a case report and literature review
Authors: Xiaoying Li, Xiaoyan Li, Xiao Li, Yuanhua Zhuang, Lili Kang, Xiuli Ju
Journal: Thromb J (2019)
Keywords: neonate, proc gene, protein c, purpura fulminans, thrombosis
MeSH terms: Review

Background:
Our objective was to study the phenotype of and molecular genetic mechanisms underlying congenital protein C (PC) deficiency in Chinese neonates. We report the case of a neonate who presented 4 h after birth with purpura fulminans of the skin and thrombosis in the kidney. We also carried out a through literature review to study the genotype and phenotype, relevance, diagnosis, management, and prognosis of neonates with congenital PC deficiency in China.
Case presentation and literature review:
Following a septic work-up and check of PC and protein S (PS) levels that showed PC deficiency, we investigated the patient's and her parents' genotypes. Our patient was found to have a plasma PC level of 0.8%. Molecular testing revealed a compound heterozygous mutation of the PROC gene: From the father, a c._262 G > T p. ASP88Tyr mutation in exon 4; from the mother, a C. 400 + 5G mutation in intron 5 that had been previously reported as likely pathogenic. Both parents were found to have heterozygous mutations for PC deficiency. In China, 5 other cases of congenital PC deficiency in the neonatal period were reported in the literature. In those cases, purpura fulminans and thrombosis were the main symptoms, and homozygous or compound heterozygous mutations of the PROC gene were identified.
Conclusion:
Congenital PC deficiency should be ruled out for neonates presenting with purpura fulminans and thrombosis.
Sinking Skin Flap Syndrome: Phenomenon of Neurological Deterioration after Decompressive Craniectomy
Authors: Noman Ahmed Jang Khan, Saad Ullah, Waseem Alkilani, Hassan Zeb, Hassan Tahir, Joshan Suri
Journal: Case Rep Med (2018)
MeSH terms: Case Reports

Sinking skin flap syndrome is rare phenomenon that occurs in patients with large craniectomies. Alteration in normal anatomy and pathophysiology can result in wide variety of symptoms including altered mental status, hemodynamic instability, and dysautonomias. Management is largely conservative. We here present a case of a patient with large craniectomy who was admitted to our hospital with pneumonia. Later on, he developed worsening mental status and CT head revealed sinking skin flap with significant midline shift. This is a very rare case of neurological deterioration after craniectomies, commonly known as sinking skin flap syndrome. To our knowledge, only few cases have been reported so far.
Large Solid Right Atrial Thrombus Treated by AngioVac Catheter-Based Suction Thrombectomy
Authors: Jacob Schultz, Asger Andersen, Erik Lerkevang Grove, Lars Bo Ilkjær, Jens Erik Nielsen-Kudsk
Journal: Case Rep Cardiol (2018)
MeSH terms: Case Reports

We present the successful treatment of a large solid right atrial thrombus by the catheter-based suction embolectomy system AngioVac® (AngioDynamics, NY, USA). A previously healthy 60-year-old male was referred with acute pulmonary embolism, a large deep vein thrombus and a large right atrial thrombus. After one week of anticoagulation, the size of the atrial thrombus was unaltered, and the patient was treated by catheter-directed embolectomy using the AngioVac system. The solid thrombus occluded the catheter during the procedure. With the vacuum maintained, the occluded catheter was removed from the patient and the thrombus mass was removed. The remaining atrial thrombus was successfully removed by suction after the reinsertion of the catheter. The patient recovered well and was discharged 7 days after the procedure. The therapy was safe and minimally invasive.
Managing Type 2 Diabetes Mellitus through Periodical Hospital Visits in the Aftermath of the Great East Japan Earthquake Disaster: A Retrospective Case Series
Authors: Yoshitaka Nishikawa, Yuji Fukuda, Masaharu Tsubokura, Shigeaki Kato, Shuhei Nomura, Yasutoshi Saito
Journal: PLoS One (2015)
MeSH terms: Blood Glucose / analysis, Diabetes Mellitus, Type 2 / drug therapy, Disasters, Glycated Hemoglobin / analysis, Outpatient Clinics, Hospital, Case Reports, Aged, Dipeptidyl Peptidase 4 / metabolism, Dipeptidyl-Peptidase IV Inhibitors / therapeutic use, Earthquakes, Female, Fukushima Nuclear Accident, Humans, Insulin / therapeutic use, Japan, Male, Middle Aged, Retrospective Studies, Tsunamis, Blood Glucose, Dipeptidyl-Peptidase IV Inhibitors, Glycated Hemoglobin A, Insulin, hemoglobin A1c protein, human, DPP4 protein, human, Dipeptidyl Peptidase 4

Aims:
To assess the impact of the Great East Japan Earthquake Disaster on daily diabetes practice and to determine the feasibility of controlling type 2 diabetes mellitus in an outpatient department.
Methods:
We retrospectively reviewed the data on disaster-affected patients with type 2 diabetes who periodically attended outpatient department of Soma Central Hospital. There were 767 patients with type 2 diabetes mellitus in total. The primary outcome measure was the change in HbA1c.
Results:
HbA1c levels of 58 patients with periodical hospital visits did not deteriorate after the disasters. Moreover, there observed no significant difference in the mean of HbA1c levels among all age and sex throughout the year. While several changes in diabetes medication usage occurred, DPP4-inhibitor was the only oral diabetic agent that increased in frequency (+60%).
Conclusions:
Patients with type 2 diabetes who were managed with periodical hospital visits did not show significant deterioration in HbA1c levels.
Giant hydronephrosis presented as a huge abdominal mass in a 16-year-old female: a case report
Authors: Waleed Aljbri, Faisal Ahmed, Saif Ghabisha, Menawar Dajenah, Ebrahim Al-Shami, Qasem Alyhari, Fawaz Mohammed
Journal: Pan Afr Med J (2022)
Keywords: abdominal mass, case report, hydronephrosis, ureteropelvic junction obstruction
MeSH terms: Hydronephrosis / diagnosis, Hydronephrosis / etiology, Ureteral Obstruction / surgery, Case Reports, Adolescent, Female, Humans, Kidney, Nephrectomy / adverse effects, Tomography, X-Ray Computed / adverse effects

Giant hydronephrosis owing to ureteropelvic junction obstruction is a rare condition characterized by the accumulation of more than 1000 ml of urine in the pyelocaliceal system. It could mimic the other benign cystic kidney disease or other causes of abdominal mass in radiologic images. We reported a 16-year-old female who presented with three months of abdominal pain and gradual abdominal mass ingrowth. Abdominal computed tomography scan showed a giant left cystic mass favored hydronephrosis secondary to ureteropelvic junction obstruction. The patient underwent a left nephrectomy, and more than 12 litters of turbid urine were suctioned from the affected kidney. In conclusion, giant hydronephrosis is an infrequent entity and should be considered in the differential diagnosis of large cystic abdominal masses. The treatment is determined by the underlying cause and the visual appeal of the affected kidney.
Extensive ischemic ulcers due to limb occlusion after endovascular aneurysm repair: a case report
Authors: Yoshito Kadoya, Tsuneaki Kenzaka, Daisuke Naito
Journal: Springerplus (2016)
Keywords: case reports, complications, endovascular procedures

Introduction:
Limb occlusion after endovascular aneurysm repair (EVAR) is a well-known complication. However, extensive ischemic ulcers due to limb occlusion are extremely rare.
Case description:
We report a rare case of extensive ischemic ulcers that developed seven months after EVAR in an 85-year-old Japanese man. He had been taking appropriate anticoagulant therapy because of paroxysmal atrial fibrillation. Angiography showed a left limb occlusion and superficial femoral artery (SFA) chronic total occlusion (CTO), and intravascular ultrasound showed limb kinking. Endovascular therapy (EVT) was performed, and stent placement was used to cover a large amount of thrombi and correct the limb kinking, leading to complete recovery of left limb blood flow. After additional EVT was performed for the SFA CTO, outflow improved and the ulcers healed completely.
Discussion and evaluation:
It seemed that the combination of poor inflow and poor outflow led to limb thrombosis.
Conclusions:
Here, we describe an extremely rare case of extensive ischemic ulcers due to limb occlusion after EVAR. Patients should undergo careful follow-up after EVAR to monitor blood flow to the lower extremities. Additionally, the early detection and correction of limb kinking and poor outflow are essential to prevent the development of ischemic ulcers.
Proximal sciatic nerve intraneural ganglion cyst
Authors: Karin R Swartz, Dianne Wilson, Michael Boland, Dominic B Fee
Journal: Case Rep Med (2009)
MeSH terms: Case Reports

Intraneural ganglion cysts are nonneoplastic, mucinous cysts within the epineurium of peripheral nerves which usually involve the peroneal nerve at the knee. A 37-year-old female presented with progressive left buttock and posterior thigh pain. Magnetic resonance imaging revealed a sciatic nerve mass at the sacral notch which was subsequently revealed to be an intraneural ganglion cyst. An intraneural ganglion cyst confined to the proximal sciatic nerve has only been reported once prior to 2009.
Case report: Exploring under the tip of the iceberg: A case series of "self-limiting" multisystem inflammatory syndrome in children
Authors: Alessandra Meneghel, Giorgia Martini, Marta Balzarin, Nadia Zabadneh, Michele Fastiggi, Francesca Tirelli, Francesco Zulian
Journal: Front Pediatr (2022)
Keywords: covid-19, sars-cov-2, case report, multisystem inflammatory syndrome in children (mis-c), self-limiting disease
MeSH terms: Case Reports

Multisystem inflammatory syndrome in children (MIS-C) is a serious condition triggered by SARS-COV-2 infection, characterized by persistent fever, multiorgan dysfunction, and increased inflammatory markers. It requires hospitalization and prompt treatment, with nearly 60% of the cases needing intensive care and 2% fatality rate. A wide spectrum of clinical characteristics and therapeutic approaches has been reported in MIS-C. We describe a series of four patients with MIS-C, defined according to the current case definitions, with a self-limiting course and no need for immunomodulatory treatment ("self-limiting MIS-C"). Few data about self-limiting MIS-C are available to date and no information on medium- and long-term outcome of this subset of patients has been reported. Although limited in size, our experience provides new insights into the MIS-C syndrome, highlighting an underestimated aspect of the disease that may have significant therapeutic implications.
Rare Radiographic Changes in a Cervicomedullary Spinal Ependymoma After Biopsy and Drainage of an Intratumoral Cyst: A Staged Approach
Authors: Catherine E Wassef, Melissa R Holloway, Howard Silberstein
Journal: Cureus (2023)
Keywords: intramedullary spinal cord tumor, intratumoral cyst, spinal cord tumor surgery, spinal ependymoma, spinal fusion
MeSH terms: Case Reports

Spinal ependymomas are the most common intramedullary spinal tumor, with a large proportion containing a small intratumoral cyst. Although the signal intensity varies, spinal ependymomas are generally well-demarcated, are not associated with a pre-syrinx, and do not extend above the foramen magnum. Our case demonstrates unique radiographic findings of a cervical ependymoma with a staged approach to diagnosis and resection. The patient is a 19-year-old female who presented with a three-year history of neck pain, progressive arm and leg weakness, falls, and functional decline. MRI revealed an expansile dorsal and centrally located T2 hypointense cervical lesion with a large intratumoral cyst extending from the foramen magnum to the C7 pedicle. Contrasted T1 scans showed an irregular enhancement pattern along the superior tumoral border down to the C3 pedicle. She underwent a C1 laminectomy for open biopsy and cysto-subarachnoid shunt. Postoperative MRI revealed a well-demarcated enhancing mass extending from the foramen magnum to C2. Pathology revealed Grade II ependymoma. She underwent an occipital to C3 laminectomy with gross total resection. Postoperatively she experienced weakness and orthostatic hypotension that improved remarkably upon discharge. Initial imaging was concerning for a higher-grade tumor, with holocervical cord involvement and cervical kyphosis. Given concern for grade and possible extensive C1-7 laminectomy and fusion for resection, a smaller surgery involving drainage of the cyst and biopsy was performed. Postoperative MRI revealed regression of the pre-syrinx, improved tumoral definition, and improvement of cervical kyphosis. This staged approach spared the patient unnecessary surgical intervention such as extensive laminectomy and fusion. We conclude that in cases of a large intratumoral cyst in an extensive intramedullary spinal cord lesion, open biopsy and drainage followed by resection in a staged fashion should be considered. Radiographic changes from the first procedure may affect the surgical approach for ultimate resection.
Case report: Thyroid carcinoma invading trachea: Multidisciplinary resection and reconstruction assisted by extracorporeal membrane oxygenation
Authors: Bo He, Shixin Zhang, Lin Ren, Yi Zhou, Qiao Chen, Jinghua Tang, Yi Zhang, Meng Tang, Yang Qiu, Haidong Wang
Journal: Front Oncol (2023)
Keywords: ecmo, case report, safe and effective, thyroid tumor resection, trachea reconstruction
MeSH terms: Case Reports

Background:
When thyroid cancer invades the trachea, tumor resection and trachea reconstruction are required. Although the traditional way of anesthesia and tracheal intubation can maintain the necessary ventilation function during the operation, tracheal intubation affects the surgical field of vision and is not conducive to the protection of the recurrent laryngeal nerve beside the trachea during the operation.
Case presentation:
Extracorporeal membrane oxygenation (ECMO) is used to replace traditional tracheal intubation in the process of resection and end-to-end anastomosis of tracheal tumors, and complete tracheal tumor resection and trachea reconstruction are achieved.
Conclusion:
Using ECMO for thyroid carcinoma resection, invaded trachea resection, and trachea reconstruction is safe and effective, which reduces the obstruction of endotracheal intubation on the operative field, guarantees the rapid and efficient end-to-end anastomosis in the upper trachea, and clearly avoids laryngeal recurrent nerve injury in the process of anastomosis.
Extensive pulmonary embolism as complication of oligosymptomatic COVID-19: case report
Authors: Ailton Carvalho Barbosa, Lívia Silva de Paula Faria, Larissa Freitas Peixoto Gloria, Graziella Viana da Silva, Paulo Henrique Ribeiro de Oliveira, Fabiano Vieira de Araujo, Felipe Eulalio Baldi Pessanha
Journal: J Vasc Bras (2021)
Keywords: covid‐19, deep vein thrombosis, pulmonary embolism
MeSH terms: Case Reports

Although the pathophysiology of coagulopathy associated with the 2019 coronavirus disease (COVID-19) is not well known, occurrence of pulmonary embolism (PE) is frequently observed. However, few cases have been described in the literature in which patients who had asymptomatic COVID-19, with no risk factors for venous thromboembolism (VTE), presented extensive acute PE. We report the case of a patient with asymptomatic COVID-19, complicated by deep vein thrombosis and later by extensive acute PE, suggesting that these conditions should be systematically considered, even in asymptomatic COVID-19 patients with no known risk factors for VTE.
In-bag dislocation of intraocular lens in patients with uveitis: a case series
Authors: Lingwei William Tao, Anthony Hall
Journal: J Ophthalmic Inflamm Infect (2015)
Keywords: cataract surgery, intraocular lens dislocation, uveitis

Background:
Improvement in surgical devices and intraocular lenses has made modern cataract surgery a safe procedure with decreasing complication rates. Intraocular lens dislocation is a serious complication after cataract surgery. Although most dislocations occur during the first week postoperative period, late intraocular lens dislocation occurring 3 months or later post-surgery has been reported with increasing frequency in recent years as a result of progressive zonular dehiscence. We report the clinical features, management and outcomes of five cases of late in-bag dislocation of intraocular lens in patients with underlying uveitis. This is a retrospective case series and literature review.
Results:
We identified five eyes in five patients with uveitis and late in-bag intraocular lens dislocation. Two patients had multifocal choroiditis, two herpetic uveitis and retinitis and two Fuchs' heterochromic iridocyclitis in five patients. Mean age at the time of cataract surgery was 50. Best vision ranged from counting fingers to 6/18 preoperatively and ranged from 6/36 to 6/6 postoperatively. All had right eye dislocation with mean time from initial cataract surgery to intraocular lens dislocation of 81 months. Explantation of dislocated intraocular lens and vitrectomy were performed in four cases; three had anterior chamber intraocular lens placement. One case was managed conservatively. Best vision ranged from light perception to 6/7.5 at time of dislocation and ranged from 6/36 to 6/6(-2) at follow-up.
Conclusions:
Late in-bag dislocation intraocular lens can complicate cataract surgery in patients with underlying uveitis. This case series identified that the mean time to in-bag intraocular lens dislocation in five uveitis patients was 81 months after uncomplicated cataract surgery, comparable with the time reported in the available literature of patients with pseudoexfoliation syndrome. This series also found that lens explantation and replacement with anterior chamber intraocular lens achieved good outcomes. Further investigation is warranted to ascertain the strategies to identify patients at risk and to prevent and better manage intraocular lens dislocation in patients with uveitis.
Pure Motor Stroke Secondary to Cerebral Infarction of Recurrent Artery of Heubner after Mild Head Trauma: A Case Report
Authors: Ali Yilmaz, Zahir Kizilay, Ayca Ozkul, Bayram Çirak
Journal: Open Access Maced J Med Sci (2016)
Keywords: heubner artery, childhood stroke, head trauma, post-traumatic stroke
MeSH terms: Case Reports

Background:
The recurrent Heubner's artery is the distal part of the medial striate artery. Occlusion of the recurrent artery of Heubner, classically contralateral hemiparesis with fasciobrachiocrural predominance, is attributed to the occlusion of the recurrent artery of Heubner and is widely known as a stroke syndrome in adults. However, isolated occlusion of the deep perforating arteries following mild head trauma also occurs extremely rarely in childhood.
Case report:
Here we report the case of an 11-year-old boy with pure motor stroke. The brain MRI showed an acute ischemia in the recurrent artery of Heubner supply area following mild head trauma. His fasciobrachial hemiparesis and dysarthria were thought to be secondary to the stretching of deep perforating arteries leading to occlusion of the recurrent artery of Heubner.
Conclusion:
Post-traumatic pure motor ischemic stroke can be secondary to stretching of the deep perforating arteries especially in childhood.
Pneumatosis intestinalis post steroid use in a patient with immune-related adverse events: Case report, literature review and FAERS analysis
Authors: Tingting Zhang, Mingnan Cao, Bin Zhao, Chen Pan, Li Lin, Chuanhao Tang, Zhigang Zhao, Jingli Duan, Li Wang, Jun Liang
Journal: Front Pharmacol (2023)
Keywords: faers, immune checkpoint inhibitor, immune-related adverse events, lung carcinoma, pneumatosis intestinalis, steroid

Introduction: The accurate diagnosis of pneumatosis intestinalis (PI) is increasing despite patients' limited identification of etiologic factors. Recently a patient with lung squamous carcinoma who developed pneumatosis intestinalis following methylprednisolone administration for immune-related adverse events was treated at our hospital. Subsequent a literature review and an analysis of the FDA Adverse Event Reporting System (FAERS) database enabled the identification of additional cases of pneumatosis intestinalis. Methods: A literature review of the MEDLINE/PubMed and Web of Science Core Collection databases using standard pneumatosis intestinalis search terms to identify published cases of immune checkpoint inhibitors (ICIs) or steroids causing pneumatosis intestinalis were performed. A separate retrospective pharmacovigilance study of FAERS enabled the extraction of unpublished cases of pneumatosis intestinalis between the first quarter of 2005 and the third quarter of 2022. Disproportionality and Bayesian analyses were performed to identify signal detection in reported odds ratios, proportional reporting ratios, information components, and empirical Bayesian geometric means. Results: Ten case reports of steroid-related pneumatosis intestinalis were retrieved from six published studies. The implicated drug therapies included pre-treatment with steroids before chemotherapy, combination therapy with cytotoxic agents and steroids, and monotherapy with steroids. In the FAERS pharmacovigilance study, 1,272 cases of immune checkpoint inhibitors or steroid-related pneumatosis intestinalis were incidentally reported. The signal detected in five kinds of immune checkpoint inhibitors and six kinds of steroids implied a positive correlation between the drugs and adverse events. Conclusion: Steroids might be the etiologic factors in the current case of pneumatosis intestinalis. Reports supporting the role of steroids in suspected cases of pneumatosis intestinalis can be found in literature databases and the FAERS database. Even so, as documented in FAERS, immune checkpoint inhibitors-induced pneumatosis intestinalis should not be excluded.