Clinical Features and Genetic Background of the Periodic Fever Syndrome with Aphthous Stomatitis, Pharyngitis, and Adenitis: A Single Center Longitudinal Study of 81 Patients

PFAPA syndrome is the most common autoinflammatory disorder in childhood with unknown etiology. The aim of our study was clinical evaluation of PFAPA patients from a single tertiary care center and to determine whether variations ofAIM2,MEFV,NLRP3, andMVKgenes are involved in PFAPA pathogenesis. Clinical and laboratory data of consecutive patients with PFAPA syndrome followed up at the University Children’s Hospital, Ljubljana, were collected from 2008 to 2014. All four genes were PCR amplified and directly sequenced. Eighty-one patients fulfilled criteria for PFAPA syndrome, 50 (63%) boys and 31 (37%) girls, with mean age at disease onset of 2.1 ± 1.5 years. Adenitis, pharyngitis, and aphthae were present in 94%, 98%, and 56%, respectively. Family history of recurrent fevers in childhood was positive in 78%. Nineteen variants were found in 17/62 (27%) patients, 4 different variants inNLRP3gene in 13 patients, and 6 different variants inMEFVgene in 5 patients, and 2 patients had 2 different variants. No variants of clinical significance were found inMVKandAIM2genes. Our data suggest that PFAPA could be the result of multiple low-penetrant variants in different genes in combination with epigenetic and environmental factors leading to uniform clinical picture.

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High Prevalence of Autoantibodies Against Monomeric C Reactive Protein (CRP) in Children with PFAPA Syndrome

Journal of Medical Biochemistry ◽

10.1515/jomb-2017-0066 ◽

2018 ◽

Vol 37 (4) ◽

pp. 507-509

Author(s):

Barbara Kraszewska-Głomba ◽

Marta Myszka ◽

Magdalena Krajewska ◽

Leszek Szenborn

Keyword(s):

Periodic Fever ◽

Unknown Etiology ◽

Single Center ◽

Autoinflammatory Disorder ◽

C Reactive Protein ◽

Reactive Protein ◽

Cervical Adenitis ◽

High Prevalence ◽

Pfapa Syndrome ◽

Febrile Episodes

Summary PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is an autoinflammatory disorder of unknown etiology. The aim of our study was to evaluate whether the presence of anti-mCRP autoantibodies (anti-mCRP) might possibly contribute to systemic inflammation during PFAPA flares. We carried out anti-mCRP testing (in-house ELISA) in a single-center, prospective cohort of 30 PFAPA patients (12 girls). We found a high prevalence (43.3%) of anti-mCRP antibodies in PFAPA patients during their febrile episodes, which implies the possible involvement of anti-mCRP antibodies in PFAPA pathogenesis.

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Comparative Morbidity Profile of Elective vs Therapeutic Neck Dissection

Otolaryngology ◽

10.1177/01945998211008915 ◽

2021 ◽

pp. 019459982110089

Author(s):

Quinn Dunlap ◽

James Reed Gardner ◽

Amanda Ederle ◽

Deanne King ◽

Maya Merriweather ◽

...

Keyword(s):

Neck Dissection ◽

Surgical Complications ◽

Chart Review ◽

Care Center ◽

Tertiary Care ◽

Retrospective Chart Review ◽

Neck Surgery ◽

Continuous Variables ◽

Significant Difference ◽

History Of

Objective Neck dissection (ND) is one of the most commonly performed procedures in head and neck surgery. We sought to compare the morbidity of elective ND (END) versus therapeutic ND (TND). Study Design Retrospective chart review. Setting Academic tertiary care center. Methods Retrospective chart review of 373 NDs performed from January 2015 to December 2018. Patients with radical ND or inadequate chart documentation were excluded. Demographics, clinicopathologic data, complications, and sacrificed structures during ND were retrieved. Statistical analysis was performed with χ2 and analysis of variance for comparison of categorical and continuous variables, respectively, with statistical alpha set a 0.05. Results Patients examined consisted of 224 males (60%) with a mean age of 60 years. TND accounted for 79% (n = 296) as compared with 21% (n = 77) for END. Other than a significantly higher history of radiation (37% vs 7%, P < .001) and endocrine pathology (34% vs 2.6%, P < .001) in the TND group, no significant differences in demographics were found between the therapeutic and elective groups. A significantly higher rate of structure sacrifice and extranodal extension within the TND group was noted to hold in overall and subgroup comparisons. No significant difference in rate of surgical complications was appreciated between groups in overall or subgroup analysis. Conclusion While the significantly higher rate of structure sacrifice among the TND population represents an increased morbidity profile in these patients, no significant difference was found in the rate of surgical complications between groups. The significant difference seen between groups regarding history of radiation and endocrine pathology likely represents selection bias.

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ANALYSIS AND INTERPRETATION OF SPIROMETRY OF PATIENTS ATTENDING RESPIRATORY MEDICINE OPD IN A TERTIARY CARE CENTER: A RETROSPECTIVE STUDY

10.36106/ijsr/0507201 ◽

2021 ◽

pp. 39-41

Author(s):

Vidhi Jobanputra ◽

Hafiz Deshmukh ◽

Ashish Deshmukh ◽

Sunil Jadhav ◽

Shivprasad Kasat ◽

...

Keyword(s):

Retrospective Study ◽

Bronchial Asthma ◽

Lung Diseases ◽

Care Center ◽

Tertiary Care ◽

Respiratory Medicine ◽

Smoking History ◽

Tertiary Care Center ◽

Operative Evaluation ◽

History Of

OBJECTIVE: To study the various spirometry ndings and look for its association with smoking history and use of biomass fuels. METHODS: Spirometry ndings of 375 patients who attend Respiratory medicine OPD from year 2017- 2019 were studied. RESULTS: Out of 375 patients, 95 patients were diagnosed as COPD, 62 as bronchial asthma, 149 had normal spirometry and 69 had restriction. CONCLUSION: In this study we concluded that spirometry plays an intergral role in the diagnosis of bronchial asthma, COPD and restrictive lung diseases. It gives an overview of the decline of lung function with increasing age, smokers, occupational lung exposures, family history of bronchial asthma. Restrictive patterns were most commonly seen in patients who came for pre operative evaluation.

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Frequency and clinical manifestations of post-poliomyelitis syndrome in a brazilian tertiary care center

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012000800002 ◽

2012 ◽

Vol 70 (8) ◽

pp. 571-573 ◽

Cited By ~ 4

Author(s):

Abrahão Augusto Juviniano Quadros ◽

Mônica Tilli Reis Pessoa Conde ◽

Luis Fabiano Marin ◽

Helga Cristina Almeida Silva ◽

Tatiana Mesquita e Silva ◽

...

Keyword(s):

Joint Pain ◽

Care Center ◽

Neuromuscular Disorders ◽

Tertiary Care ◽

Clinical Manifestations ◽

Paralytic Poliomyelitis ◽

Prior History ◽

Cold Intolerance ◽

Infection Age ◽

History Of

OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders. METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms. RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%). CONCLUSIONS: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

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COVID-19 Conversion after Exposure in a Semiprivate Room at a Tertiary Care Center in Iowa, July–December 2020

Antimicrobial Stewardship & Healthcare Epidemiology ◽

10.1017/ash.2021.37 ◽

2021 ◽

Vol 1 (S1) ◽

pp. s20-s21

Author(s):

Alexandra Trannel ◽

Takaaki Kobayashi ◽

Oluchi Abosi ◽

Kyle Jenn ◽

Holly Meacham ◽

...

Keyword(s):

Exposure Time ◽

Care Center ◽

Tertiary Care ◽

Tertiary Care Center ◽

University Of Iowa ◽

Subsequent Conversion ◽

Risk Of Exposure ◽

Polymerase Chain ◽

The University

Background: Hospital semiprivate rooms may lead to coronavirus disease 2019 (COVID-19) patient exposures. We investigated the risk of COVID-19 patient-to-patient exposure in semiprivate rooms and the subsequent risk of acquiring COVID-19. Methods: The University of Iowa Hospitals & Clinics is an 811-bed tertiary care center. Overall, 16% of patient days are spent in semiprivate rooms. Most patients do not wear masks while in semiprivate rooms. Active COVID-19 surveillance included admission and every 5 days nasopharyngeal SARS-CoV-2 polymerase chain reaction (PCR) testing. We identified inpatients with COVID-19 who were in semiprivate rooms during their infectious periods during July–December 2020. Testing was repeated 24 hours after the first positive test. Cycle threshold (Ct) values of the two tests (average Ct <30), SARS-CoV-2 serology results, clinical assessment, and COVID-19 history were used to determine patient infectiousness. Roommates were considered exposed if in the same semiprivate room with an infectious patient. Exposed patients were notified, quarantined (private room), and follow-up testing was arranged (median seven days). Conversion was defined as having a negative test followed by a subsequent positive within 14 days after exposure. We calculated the risk of exposure: number of infectious patients in semiprivate rooms/number of semiprivate patient-days (hospitalization days in semiprivate rooms). Results: There were 16,427 semiprivate patient days during July–December 2020. We identified 43 COVID-19 inpatients who roommates during their infectious periods. Most infectious patients (77%) were male; the median age was 67 years; and 22 (51%) were symptomatic. Most were detected during active surveillance: admission testing (51%) and serial testing (28%). There were 57 exposed roommates. The risk of exposure was 3 of 1,000 semiprivate patient days. In total, 16 roommates (28%) did not complete follow-up testing. Of 41 exposed patients with follow-up data, 8 (20%) converted following their exposure. Median time to conversion was 5 days. The risk of exposure and subsequent conversion was 0.7 of 1,000 semiprivate patient days. Median Ct value of the source patient was 20 for those who converted and 23 for those who did not convert. Median exposure time was 45 hours (range, 3–73) for those who converted and 12 hours (range, 1–75) for those who did not convert. Conclusions: The overall risk of exposure in semiprivate rooms was low. The conversion rate was comparable to that reported for household exposures. Lower Ct values and lengthier exposures may be associated with conversion. Active COVID-19 surveillance helps early detection and decreases exposure time.Funding: NoDisclosures: None

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Accidental tramadol ingestion in children admitted in tertiary care centre

International Journal of Basic & Clinical Pharmacology ◽

10.18203/2319-2003.ijbcp20195200 ◽

2019 ◽

Vol 8 (12) ◽

pp. 2661

Author(s):

Vadlakonda Sruthi ◽

Annaladasu Narendra

Keyword(s):

Pediatric Population ◽

Care Center ◽

Tertiary Care ◽

Ventilatory Support ◽

Case Series ◽

Pediatric Intensive Care ◽

Hemodynamic Instability ◽

Tertiary Care Centre ◽

Medical College ◽

History Of

Background: Tramadol use has been increasing in the adult and pediatric population. Practitioners must be alert because Tramadol misuse can lead to severe intoxication in which respiratory failure and seizures are frequent. Overdoses can lead to death. We report 47 pediatric cases with history of accidental tramadol exposure in children.Methods: An observational, retrospective, single center case -series of children with a history of accidental tramadol exposure in children admitted in pediatric intensive care unit of tertiary care center, Niloufer Hospital (Osmania Medical College) Hyderabad, Telangana India.Results: Of 47 children, 22 (47%) are male and 25 (53%) were female. At presentation 11 (23%) had loss of consciousness, 14 (29%) seizures, 17 (36%) hypotonia was noted. Pupils were miotic in 22 (47%) mydriatic in 2 (4.2%) normal in rest of children. Hemodynamic instability noted in 13 (27.6%). Serotonin syndrome (tachycardia, hyperthermia, hypertension, hyper reflex, clonus) was noted on 5 (10.6%) children. Respiratory depression was seen in 4 (8%) children who needed ventilatory support. Antidote Naloxone was given in 7 children. No adverse reaction was noted with Naloxone. All 47 children were successfully discharged.Conclusions: Overdoses can lead to death and practitioners must be alert because of the increasing use of tramadol in the adult and pediatric population. The handling of the tramadol should be explained to parents and general population and naloxone could be efficient when opioid toxicity signs are present.

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Clinical trends of scalp alopecia areata: a tertiary care hospital based observational study

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20200607 ◽

2020 ◽

Vol 6 (2) ◽

pp. 193

Author(s):

Pallavi Singh ◽

Milind A. Patvekar ◽

Bhavika Shah ◽

Alisha Mittal ◽

Asmita Kapoor

Keyword(s):

Family History ◽

Observational Study ◽

Alopecia Areata ◽

Tertiary Care Hospital ◽

Past History ◽

Tertiary Care ◽

Disease Onset ◽

Epidemiological Data ◽

Care Hospital ◽

History Of

Background: Alopecia areata is one of the commonest types of non-scarring alopecia involving the scalp and/or body. As there is paucity of recent epidemiological data from our country, this study was conducted to determine the latest clinical and epidemiological trends of scalp alopecia areata.Methods: A hospital-based observational study consisting of 100 cases of clinically diagnosed scalp alopecia areata who reported to the Dermatology OPD, Dr. D.Y. Patil Medical College, Pune, was conducted for a period of six months. Socio-demographic and clinical information was collected and clinical examination was performed on all patients. The data was evaluated using appropriate statistical methods.Results: Out of the 100 cases enrolled, males (64%) outnumbered females (36%). The commonest presenting age group was 21-30 years (44%). Disease onset was sudden in 80% patients and 59% cases had a progressive disease course. Majority (75%) had a disease duration of less than 3 months. Majority cases were asymptomatic (80%) with no precipitating factors (90%). Past history and family history of alopecia areata were present in 13% and 9% cases, respectively. Personal and family history of associated diseases were present in 27% and 22% patients, respectively. Most patients had single (61%), patchy (83%) lesions with occiput (45%) being the commonest initial site. Nail changes were present in 22% cases, of which pitting (13%) was the commonest nail finding.Conclusions: This study reflects the clinical profile of scalp alopecia areata in a tertiary care hospital.

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Psychiatric Illnesses among the Patients Admitted for Self-poisoning in a Tertiary Care Hospital of Nepal

Journal of Advances in Internal Medicine ◽

10.3126/jaim.v2i1.7630 ◽

2013 ◽

Vol 2 (1) ◽

pp. 10-13 ◽

Cited By ~ 1

Author(s):

Ajay Risal ◽

Pushpa Prasad Sharma ◽

Rajkumar Karki

Keyword(s):

Tertiary Care Hospital ◽

Organophosphorus Compounds ◽

Care Center ◽

Tertiary Care ◽

Psychiatric Evaluation ◽

Care Hospital ◽

Age Group ◽

Psychiatric Illnesses ◽

History Of ◽

One Year

Background and Aims- Self-poisoning is the commonest mode of suicide in our part of the world. Patients attempting suicide by self-poisoning usually land up in the Emergency. They are admitted for management of medical complications and subsequently referred to Psychiatry for evaluation of possible Psychiatric illnesses. The aim of this study was to explore the patterns of psychiatric illnesses in the patients admitted for self-poisoning in a tertiary care center in Nepal. Methods- The study population included those patients who were admitted and being managed for self-poisoning and brought for psychiatric evaluation during the period of one year (1st June 2011- 30th June 2012) at Dhulikhel Hospital, Nepal. Each patient underwent a detailed psychiatric evaluation by a consultant psychiatrist once they were medically stable. Details including sociodemographic data, psychiatric diagnosis, and treatment offered and outcome was tabulated and analyzed using SPSS-16. Results- Among the total patients (N=100), 43 were in the age group 21-40 years, median age being 27.5. There was almost equal gender distribution. Majority was of Mongolian ethnicity, homemaker by occupation and married. More than 90% were single-attempters, suicidal attempt using organophosphorus compounds. Almost 50% had depression; family dispute (19%) and marital disharmony (17%) were the most common psychosocial precipitant. Conclusion- Patients with history of self-poisoning are commonly brought to the Emergency Department of any tertiary care hospital. It is widely prevalent on younger age group. It is usually by the use of Organophosphorus compound in our setting and most commonly associated with depression. Hence, psychiatric care is essential for these patients. Journal of Advances in Internal Medicine 2013;02(01):10-13 DOI: http://dx.doi.org/10.3126/jaim.v2i1.7630

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Abstract 359: Lack Of Blood Pressure Augmentation During Stress Testing Is Associated With Increased Mortality.

Circulation Cardiovascular Quality and Outcomes ◽

10.1161/circoutcomes.6.suppl_1.a359 ◽

2013 ◽

Vol 6 (suppl_1) ◽

Author(s):

Geoffrey D’Cruz ◽

Ashish Rastogi ◽

Neil Yager ◽

Amarinder Bindra ◽

Steven A Fein ◽

...

Keyword(s):

Blood Pressure ◽

Chest Pain ◽

Stress Echocardiography ◽

Stress Testing ◽

Systolic Function ◽

Stress Test ◽

Care Center ◽

Tertiary Care ◽

History Of ◽

Response To Exercise

Objective: We investigated long-term outcomes associated with hypertensive response to exercise in patients with chest pain referred for stress echocardiography. Methods: Records of 404 patients with normal baseline LV systolic function (45% females, mean age 60+/-11 years, baseline SBP 136+/-20 mmHg, 26% with CAD, 4% with CHF, 39% with hypertension, 13% with diabetes mellitus, 5% with peripheral vascular disease, 21% with history of smoking or active smoking, 43% on beta-blockers, 23% on ACE-inhibitors/ARBs) referred for chest pain evaluation with stress echocardiography at a single tertiary care center were reviewed. Demographics, clinical data, and outcomes were collected. Median length of followup was 35+/-0.3 months. Patients were divided into four groups depending on their maximum blood pressure during exercise (greater or less than 180mmHg) and whether they achieved their age-adjusted target heart rate. Results: Contrary to the expectations, hypertensive response to exercise was not associated with the increased mortality (Table). Instead, lack of blood pressure augmentation during exercise and low double product were predictive of increased mortality. History of CHF (p=0.0003) and/or PVD (p=0.001) were the strongest predictors of failure to augment blood pressure during stress testing. Conclusions: Failure to augment systolic blood pressure during exercise appears to be associated with increased mortality. Although ischemia on echocardiography and reduced exercise capacity are the stress test outcomes traditionally associated with poor prognosis, failure to augment blood pressure during exercise may be an important predictor of mortality as well. Additional studies of this subject are needed.

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Epidemiologic Characteristics of Patients Presenting with Head Injury due to Road Traffic Accident and Factors Associated with Outcome: Experience of a Tertiary Care Center in Northern Kerala

Indian Journal of Neurosurgery ◽

10.1055/s-0039-1678468 ◽

2019 ◽

Vol 08 (01) ◽

pp. 039-046

Author(s):

Mandaka Rajeev ◽

Vattaparambil Shinihas ◽

Pankaj Chauhan

Keyword(s):

Road Traffic Accident ◽

Traffic Accident ◽

Alcohol Intake ◽

Road Traffic ◽

Care Center ◽

Tertiary Care ◽

Health Resources ◽

Medical College ◽

History Of ◽

Limited Health

Abstract Background In India, most factors related to road traffic accident (RTA) causation and outcome go improperly documented, and database regarding RTA-related traumatic brain injury (TBI) seems inadequate. Two-wheeler drivers form the largest segment of people affected by RTA. The socioeconomic and neurologic burden, imposed by TBI due to RTAs (largely preventable), is overwhelmingly significant, especially for a developing country like India. Materials and Methods Descriptive study involving patients, presenting to the casualty of Government Medical College, Kozhikode, Kerala, was performed, and various demographic features were analyzed. Usage of helmet and history of alcohol intake were also noted. Patients were evaluated according to their presenting Glasgow coma scale (GCS), investigated and either operated or managed conservatively, and their outcome was assessed with Glasgow outcome score (GOS) at 3 months. Results Bike drivers formed the single largest proportion of RTA victims (53.7%). Proportion of helmet users was 17.9%, whereas 21.9% were found with history of alcohol intake. There was a consistent trend toward a favorable outcome in patients with no alcohol intake (17.08% deaths compared with 34.07% patients with alcohol intake) and with helmet usage (14.55% compared with 22.18% in patients without helmet). However, the absolute contribution of these factors cannot be inferred. Conclusion Various factors related to RTA need evaluation for pooling and compilation of data at regional and national levels. Mandatory helmet laws and strict implementation and provision of subsidized helmets (standard, full coverage) will go a long way in reducing the burden on limited health resources.

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