OpenMed/multicare-articles huggingface dataset:
Unique Approach to Dental Management of Children with Hearing Impairment
Authors: Navanith Renahan, R Balagopal Varma, Parvathy Kumaran, Arun M Xavier
Journal: Int J Clin Pediatr Dent (2017)
Keywords: behavior management, communication, deaf child, sign language
MeSH terms: Case Reports

The number of deaf children has dramatically increased in the past few decades. These children present to the pediatric dentist a unique set of challenges mostly pertaining to the establishment of communication with them. There have been very few attempts in the past to break down these challenges and formulate a strategy on how to manage them effectively. This is a case report of a child who was successfully managed using two different modes of communication. Finally, the advantages and disadvantages are mentioned, and a common strategy incorporating the positives of both the methods has been devised.
How to cite this article:
Renahan N, Varma RB, Kumaran P, Xavier AM. Unique Approach to Dental Management of Children with Hearing Impairment. Int J Clin Pediatr Dent 2017;10(1):107-110.
Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review
Authors: Konrad Szymański, Cezary Kowalewski, Ewelina Pietrzyk, Katarzyna Woźniak
Journal: Front Immunol (2023)
Keywords: eba, ivig, epidermolysis bullosa acquisita, inflammatory bowel diseases, infliximab, rituximab
MeSH terms: Biological Products / therapeutic use, Epidermolysis Bullosa Acquisita / diagnosis, Epidermolysis Bullosa Acquisita / drug therapy, Review, Case Reports, Autoantibodies, Blister, Dapsone / therapeutic use, Humans, Autoantibodies, Dapsone, Biological Products

Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.
Pulmonary embolism due to hemangioma of segment I compressing the inferior vena cava: A Case Report
Authors: Sarah Winterland, Tim Reese, Georgios Makridis, Karl J Oldhafer
Journal: Int J Surg Case Rep (2020)
Keywords: compression of the vena cava, liver resection, pulmonary embolism, surgery, symptomatic hepatic hemangioma
MeSH terms: Case Reports

Introduction:
Hemangiomas are common hepatic lesions and are mostly asymptomatic. Operative removal should only be performed when the lesions are symptomatic, and removal of the hemangioma is deemed to be more beneficial than the risk of the operation itself.
Presentation of the case:
The multiple hemangiomas of our patient were discovered nine years before first symptoms. The patient presented with pulmonary artery embolism due to a compression of the vena cava inferior by a hemangioma in segment I. No other cause of thromboembolic events could be diagnosed. We performed surgical enucleation of the hemangioma, which resulted in the normalization of blood flow in the inferior vena cava. As the inducing factor for clot development was removed, no permanent thrombosis prophylaxis was implicated.
Discussion:
Pulmonary embolisms owing to compression of the inferior vena cave are rare.
Conclusion:
Until now to our knowledge, pulmonary embolisms have never been described as an indication for hepatic hemangioma enucleation.
Intravenous Thrombolysis in Posterior Circulation Stroke
Authors: Tomáš Dorňák, Michal Král, Daniel Šaňák, Petr Kaňovský
Journal: Front Neurol (2019)
Keywords: intracranial hemorrhage, intravenous thrombolysis, ischemia, posterior circulation, stroke
MeSH terms: Review

Background: Intravenous thrombolysis (IVT) is a standard treatment for both anterior circulation ischemic stroke (ACIS) and posterior circulation ischemic stroke (PCIS). PCIS is a clinical syndrome associated with ischemia-related changes in the territory of the posterior circulation arteries. Embolism is the most common stroke mechanism in posterior circulation. PCIS represents 12-19% of all IVT-treated strokes. Methods and Results: We searched the PubMed database for assessments of intracerebral hemorrhage (ICH) and clinical outcome in PCIS patients treated with IVT. ICH occurs in 0-6.9% of posterior ischemic stroke depending on the definition of symptomatic ICH, and any ICH in 17-23.4% of posterior ischemic stroke. For patients with PCIS, 38-49% have a favorable outcome (mRS 0-1) after IVT. Better clinical outcomes occur more often in patients with PCIS than in those with ACIS. The mortality rate among PCIS patients treated with IVT ranges from 9 to 19%; it does not differ significantly between PCIS and ACIS. Conclusions: Up to date, no data about PCIS and IVT are available from RTCs. Based on limited results from retrospective clinical studies and case series, IVT is safer for use in PCIS than in ACIS. Patients with brainstem ischemia, vertebral artery occlusion, and absence of basilar or posterior cerebral artery occlusion could be considered for treatment with IVT even in borderline cases. Time to IVT in PCIS seems to be a less crucial factor than in ACIS. IVT for PCIS may be beneficial even after 4.5 h from symptom onset.
Case Report: Subtotal Hemispherotomy Modulates the Epileptic Spasms in Aicardi Syndrome
Authors: Yasushi Iimura, Hidenori Sugano, Takumi Mitsuhashi, Tetsuya Ueda, Kostadin Karagiozov, Shimpei Abe, Hiroshi Otsubo
Journal: Front Neurol (2021)
Keywords: epileptic spasms, high frequency oscillations, modulation index, phase-amplitude coupling, subtotal hemispherotomy
MeSH terms: Case Reports

The mechanism of epileptic spasms (ES) in Aicardi syndrome (AS) remains obscure. We compared intraoperative high-frequency oscillations (HFOs) and phase-amplitude coupling (PAC) before and after subtotal hemispherotomy in a 3-month-old girl with drug-resistant ES secondary to AS. Fetal ultrasonography showing corpus callosum agenesis, bilateral ventricular dilatation, and a large choroid plexus cyst confirmed AS diagnosis. Her ES started when she was 1 month old and had ten series of clustered ES per day despite phenobarbital and vitamin B6 treatment. After subtotal hemispherotomy, her ES dramatically improved. We analyzed two intraoperative electrocorticography modalities: (1), occurrence rate (OR) of HFOs; (2), PAC of HFOs and slow wave bands in the frontal, central, and parietal areas. We hypothesized that HFOs and PAC could be the biomarkers for efficacy of subtotal hemispherotomy in AS with ES. PAC in all three areas and OR of HFOs in the frontal and parietal areas significantly decreased, while OR of HFOs in the central area remained unchanged after subtotal hemispherotomy. We have demonstrated the usefulness of evaluating intraoperative HFOs and PAC to assess subtotal hemispherotomy effectiveness in AS patients with ES. Disconnecting the thalamocortical and subcortical pathways in the epileptic network plays a role in controlling ES generation.
A tapetal-like fundus reflex in a healthy male: evidence against a role in the pathophysiology of retinal degeneration?
Authors: Patrik Schatz, Jesper Bregnhøj, Henrik Arvidsson, Dror Sharon, Liliana Mizrahi-Meissonnier, Birgit Sander, Karen Grønskov, Michael Larsen
Journal: Mol Vis (2012)
MeSH terms: Asymptomatic Diseases, Reflex, Retina / physiology, Retinitis Pigmentosa / metabolism, Case Reports, Research Support, Non-U.S. Gov't, Adult, Dark Adaptation, Electroretinography, Eye Proteins / genetics, Female, Genetic Linkage, Heterozygote, Humans, Male, Pedigree, Phenotype, Retinitis Pigmentosa / genetics, Sex Factors, Tomography, Optical Coherence, Visual Acuity, Visual Fields, Eye Proteins, RPGR protein, human

Purpose:
To report on the retinal function and structure in a 37-year-old male who presented with a tapetal-like reflex (TLR) indistinguishable from that seen in female carriers of X-linked retinitis pigmentosa (XLRP).
Methods:
Clinical examination included dark adaptometry, full-field electroretinography (ERG), multifocal ERG, optical coherence tomography, and fundus autofluorescence photography. Molecular genetic testing included screening for known mutations in autosomal dominant, autosomal recessive, and X linked retinitis pigmentosa (RP) genes with a commercially available chip, and sequencing analysis of retinitis pigmentosa GTPase regulator (RPGR)-open reading frame 15 (ORF15).
Results:
Fundus examination revealed a bilateral TLR, which is typical of female carriers of XLRP. Imaging studies and electrophysiological testing was unremarkable, except for a significant increase in full-field ERG amplitudes after prolonged dark adaptation as compared to after standard dark adaptation. Mutation screening was negative.
Conclusions:
TLR was found for the first time, to the best of our knowledge, in a male subject. There were no definitive signs of retinal degeneration, suggesting that this reflex in itself is not necessarily a precursor of the retinal degeneration that can be seen in female carriers of XLRP.
Vanishing white matter disease imaged over 3 years
Authors: Denny Mathew, Nasreen Mahomed
Journal: SA J Radiol (2019)
MeSH terms: Case Reports

Childhood ataxia and central nervous system hypomyelination (CACH), also known as 'vanishing white matter disease' (VWM), is a leukoencephalopathy with autosomal recessive inheritance. It is characterised by normal psychomotor development initially, with an onset of neurological deterioration that follows a chronic and progressive course. Stress conditions such as febrile infections, minor head trauma or even acute fright provoke major episodes of neurological deterioration. We present a case of a 2-year-old child who presented with spasticity and cerebellar ataxia. After magnetic resonance imaging (MRI) of the brain, CACH/VWM was diagnosed on the basis of the typical clinical and MRI findings. As there is no known cure for CACH/VWM, our patient was followed up over 3 years with MRIs of the brain to assess the progressive involvement of the cerebral white matter. In those patients with suggestive or inconclusive MRI findings for CACH/VWM, particularly in the presymptomatic stage and adult onset variants, involvement of the inner rim of the corpus callosum should prompt the inclusion of CACH/VWM in the differential diagnosis. Biochemical markers such as the asialotransferrin:transferrin ratio in the cerebrospinal fluid can also potentially be used as a screening tool in this subset of patients prior to gene mutation analysis.
Postoperative Hardware-Related Infection from Kytococcus schroeteri: Its Association with Prosthetic Material and Hematological Malignancies-A Report of a Case and Review of Existing Literature
Authors: Aditya S Shah, Prakhar Vijayvargiya, Sarah Jung, John W Wilson
Journal: Case Rep Infect Dis (2019)
MeSH terms: Case Reports

Introduction:

Kytococcus schroeteri is an infrequently isolated Gram-positive coccus often encountered as a commensal bacterium. Only eighteen cases of human infection associated with this organism have been previously reported. Most of these cases involved patients with implanted prosthetic materials or patients with immunosuppressive conditions. It has been described in prosthetic valve endocarditis and in select patients with hematologic diseases but only one prior report as being involved in osteoarticular infections.
Case presentation:
We describe a case of postsurgical osteoarticular hardware-related infection by K. schroeteri and discuss a possible association with implanted prosthetic material.
Conclusion:
Other clinical presentations of K. schroeteri, including reported infection syndromes, antimicrobial susceptibility profiles, and treatment outcomes, are also reviewed.
Vein of galen aneurysmal malformations: an ultrasonographic incidental finding-a case report
Authors: S Stephan, G Rodesch, E Elolf, D Wiemann, G Jorch
Journal: Case Rep Pediatr (2012)

Background. The Vein of Galen aneurysmal malformation (VGAM) is a rare congenital, cerebral, arteriovenous deformity. Good cross-discipline cooperation is in demand because of associated complications and high mortality. The recognition of the optimal therapeutic window is useful to allow proper management. Case Report. We report on the successful treatment of a 2-week-old, healthy girl with a VGAM, which came across in the context of the newborn ultrasonographic screening. After interdisciplinary discussion, 2 embolizations of the VGAM followed without complications-the first in the age of 6 months and the second at 12 months of life. Before and after the intervention, the patient had an age-appropriate development without neurological deficits. Conclusion. The endovascular transarterial embolization is described as the treatment of choice. Time and method of intervention depend on clinical signs of the patient. In our case the patient was asymptomatic. So the arteriovenous abnormality was an incidental finding by ultrasound. Because of the natural history of the disease, and the potential severe neurocognitive consequences at long-term followup if left untreated, it was decided to embolize the lesion. Thanks to embolization with glue, good therapeutical and clinical results could be obtained with normal neurological development.
4q25 Microdeletion with Axenfeld-Rieger Syndrome and Developmental Delay
Authors: Yukino Kawanami, Tomoko Horinouchi, Naoya Morisada, Takeshi Kato, Kandai Nozu
Journal: Case Rep Genet (2023)
MeSH terms: Case Reports

We encountered a case with congenital iris coloboma, omphalocele, and developmental delay with a 2.5 Mb deletion on chromosome 4q25 encompassing PITX2, leading to Axenfeld-Rieger syndrome (ARS), NEUROG2, and ANK2. ARS is characterized by the aplasia of the anterior eye, odontogenesis, and abdominal wall aplasia. In our case, iris coloboma and omphalocele were thought to be caused by PITX2 haploinsufficiency. However, these symptoms are nonspecific, and clinical symptoms alone can make it difficult to make a correct diagnosis. In addition, the genes responsible for developmental delay, among others, are not well understood. Developmental delay, in this case, might be caused due to NEUROG2 haploinsufficiency. In spite of the partial deletion of ANK2, the causative gene of long QT syndrome type 4, the electrocardiogram was normal. Genetic testing can lead to a correct diagnosis, and it may be effective in detecting complications.
Case Report: Autologous Bone Marrow Derived Intrathecal Stem Cell Transplant for Autistic Children - A Report of Four Cases and Literature Review
Authors: Georg S Kobinia, John J Zaknun, Christof Pabinger, Brenda Laky
Journal: Front Pediatr (2021)
Keywords: autism, autologous, bone marrow (bm), case report, intrathecal, point-of-care method, safety, stem cell transplant (sct)
MeSH terms: Case Reports

Despite steadily growing numbers of children diagnosed with autism spectrum disorders (ASD), causative treatment is unavailable. Recently, biological cell therapies involving pluripotent cells have raised hopes towards sustained beneficial outcome. We herein report data of four children diagnosed with ASD, who were treated with autologous, bone marrow (BM)-derived, intrathecally and simultaneously intravenously applied, point-of-care stem cell transplant (SCT). The three boys and one girl received the diagnosis at ages between 2-4 years. The decision to perform the procedure was preceded by limited beneficiary impact of conventional symptom-based, psychological and pharmacological interventions. At ages of 4-14 years the children received their SCT, no immediate or late adverse events were reported. Disappearance of symptoms were observed by the parents during the following year and consequently improved Autism Treatment Evaluation Checklist (ATEC) scores were reported. The SCT procedure, in trained hands, can be a safe and promising treatment option in children with ASD, responding in a non-satisfactory manner to conventional treatments. It is postulated that SCT may, among others, assert its positive effect by counteracting a cerebral inflammatory autoimmune process which in turn supports the responsiveness to behavioral and pharmacological interventions. Our results in this small group are encouraging, but certainly need further investigation in larger cohorts.
Episodic Behavioural Regression in an 8-Year-Old Female: Sequelae of 22q11.2 Duplication Syndrome
Authors: A Bahji, S Khalid-Khan
Journal: Case Rep Psychiatry (2018)
MeSH terms: Case Reports

22q11.2 duplication syndrome is a recently discovered genetic syndrome with unclear neuropsychiatric sequelae. While its connection to 22q11.2 deletion syndrome is actively investigated, case reports on the neuropsychiatric sequelae of affected individuals have been previously described, largely focusing on comorbid autism spectrum disorder. Here, we present the case of an 8-year-old female experiencing episodes of severe behavioural regression following medical illness. We analyze the case and relate it to the available literature and identify potential risk factors.
First-Time Psychotic Symptoms in a Patient After COVID-19 Infection-A Case Report
Authors: Kacper Łoś, Joanna Kulikowska, Napoleon Waszkiewicz
Journal: Front Psychiatry (2021)
Keywords: covid-19, sars-cov-2, case report, psychotic spectrum disorders, schizophrenia
MeSH terms: Case Reports

A 39-year-old, previously healthy, white male with no personal or family history of mental illness presented with new, first-time psychotic symptoms. The new psychotic symptoms appeared on patient admission to the hospital, occurring during a diagnosis of symptomatic SARS-CoV-2 infection. On the first day of hospitalization for worsening psychotic symptoms and the appearance of aggression toward the staff, the patient was transferred to the psychiatric hospital. After the initial treatment with antipsychotics and benzodiazepines, his mental condition improved. The patient was then transferred for further treatment of his somatic condition in the internal medicine ward, with a recommendation to continue treatment in the psychiatric ward once his somatic condition was stabilized. This is one of the few reported cases of COVID-19-related psychosis in a patient without a personal or family history; moreover, this description contains important data regarding elevated IL-6, which may prove to be a key factor in the induction of new psychotic symptoms. It indicates the important need for careful monitoring of neuropsychiatric symptoms among COVID-19 patients.
Candida auris emergence in the Himalayan foothills: First case report from Uttarakhand, India
Authors: Suneeta Meena, Ranjana Rohilla, Neelam Kaistha, Arpana Singh, Pratima Gupta
Journal: Curr Med Mycol (2020)
Keywords: candida auris, candidemia, caspofungin, fluconazole, intensive care unit (icu)
MeSH terms: Case Reports

Background and purpose:
Candida auris is a rapidly emerging fungus, which is considered globally a cause of concern for public health. This report describes the first case of C. auris fungemia from a tertiary care hospital in the hilly state of Uttarakhand in India.
Case report:
The patient was a 37-year-old female who underwent a Whipple procedure for the carcinoma of the head of the pancreas. She developed fever 12 days after the operation while recovering from surgery in the hospital. Blood culture yielded C. auris which was identified by the matrix-assisted laser desorption/ionization-time of flight mass spectrometry (Bruker Daltonics, Germany). The patient was successfully treated with caspofungin.
Conclusion:
In conclusion, C. auris is potentially multidrug resistant, resulting in nosocomial outbreaks and sporadic infections which can be potentially prevented when identified early by implementing contact precautions.
Breakage and retention of thoracic paravertebral catheter: a case report
Authors: Tasuku Fujii, Yasuyuki Shibata, Kimitoshi Nishiwaki
Journal: JA Clin Rep (2017)
Keywords: breakage, complication, paravertebral catheter, retention, thoracic paravertebral block

Background:
Paravertebral catheters are generally inserted and removed without complications. However, catheter breakage occurs rarely. This is the first report describing breakage of a thoracic paravertebral catheter and retention of the catheter fragment within the patient.
Case presentation:
A 65-year-old female patient complained of an unusual sensation in her back during postoperative chemotherapy for lung cancer. A catheter fragment was identified in the soft tissues of the back on computed tomography. The paravertebral catheter had been placed 2 years prior left lower lobectomy. The patient had neither neurological symptoms nor infection signs around the fragment. However, the potential side effects of chemotherapy, including coagulopathy and immunosuppression, increased the risk of late-onset hematoma and abscess formation around the fragment. Therefore, we surgically removed the catheter fragment. Analysis of the fragment revealed that the catheter had been severed by the cutting edge of the Tuohy needle or the suture needle.
Conclusion:
In this report, a paravertebral catheter fragment was retained in the posterior mediastinum for 2 years. The catheter was likely damaged during the insertion procedure. We suggest that catheters should not be withdrawn through the Tuohy needle, but be withdrawn together with the Tuohy needle. Although secure fixation of the catheter can be achieved with sutures, to reduce the risk of damage to the catheter, alternative methods, such as surgical tapes or skin glue should be considered. After removal of a catheter, its tip should be checked to ensure that the entire catheter has been completely removed. If a catheter fragment is retained within the patient, removal of the fragment should be considered according to the patient's condition and risks.