Kawasaki disease with retropharyngeal edema: case series from a single center experience.
Ontology highlight
ABSTRACT:
SUBMITTER: Au DCY
PROVIDER: S-EPMC6759096 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:BackgroundSilvery Hair Syndromes (SHS), an autosomal recessive inherited disorder, includes Chediak-Higashi syndrome (CHS), Griscelli syndrome (GS), Hermansky-Pudlak syndrome (HPS), and Elejalde syndrome. Associated immunological and neurological defects and predilection for hemophagocytic lymphohistiocytosis (HLH) makes them a distinctive entity in pediatric practice. Thorough clinical examination, bedside investigations such as peripheral blood smear (PBS) and hair microscopy, and bone marrow (BM) examination are inexpensive and reliable diagnostic tools.MethodsWe report 12 cases with SHS (CHS, n = 06; GS, n = 04; HPS, n = 02).Results8 out of 12 SHS children (CHS-05, GS-03) presented with HLH. Out of 5 cases of CHS with HLH, 2 died, 3rd is stable post-chemotherapy; 4th completed chemotherapy, underwent matched related hematopoietic stem cell transplant (HSCT), and is stable 8 months off treatment. The 5th child completed chemotherapy and is in process of transplant. One CHS child without HLH is thriving without any treatment. Of the 4 GS cases, 3 presented with HLH and received chemotherapy (HLH 2004 protocol). One lost follow-up after initial remission; another had recurrence 7 months off treatment and discontinued further treatment. The third child had recurrence 1.5 years after initial chemotherapy; HLH 2004 protocol was restarted followed by HSCT from matched sibling donor; is currently well, 2.5 years post-transplant. One child with GS had neurological features with no evidence of HLH and did not take treatment. Of 2 children with HPS, one presented with severe sepsis and the other with neurological problems. They were managed symptomatically.ConclusionIn SHS with HLH, chemotherapy followed by allogeneic hematopoietic stem cell transplantation is a promising curative option.
| S-EPMC9455113 | biostudies-literature
Project description:IntroductionCoronavirus disease 2019 (COVID-19) pandemic, is a newly conducted respiratory disease caused by infection with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2). The current study aims to estimate the neurological diseases which develop after COVID-19 infection.MethodThis is a single center retrospective case series conducted in seven months. the patients were collected in an out-patient clinic. Diagnosis of COVID-19 and the way of diagnosis is confirmed through either polymerase chain reaction (PCR) test for COVID-19 and/or typical findings on chest computed tomography scan (CT scan). Patients developed neurological symptoms after being infected with COVID-19. Symptoms have to be developed within less than 6 months of recovery, or developed during illness and persisted after recovery.ResultA total number of 59 patients infected with SARS-CoV2 were included. The majority of the patients had mild symptoms 32 (54%), 12 (20%) patients developed severe symptoms. Headache was the most common presenting symptom 27(46%) followed by fatigue in 8 (13.5%). The majority of the patients 55 (91.6%) presented with no focal signs. MRI was done for 27 (46%) patients without abnormal finding in 22 cases. Nearly 22 (37.3%) cases were diagnosed as recurrent episodes of migraine or new onset of migraine. All patients were managed according to the underlying pathology, only (28, 47.5%) patients were known to be completely recovered.ConclusionSARS-CoV2 can invade and cause inflammation in the central and peripheral nervous systems. It is responsible for many neurological problems. More studies are necessary to analyze the long term effect of the virus on the nervous system.
| S-EPMC8957714 | biostudies-literature
Project description:BackgroundIn the year 1724, Hermann Boerhaave reported a case of a Dutch admiral who died due to spontaneous rupture of the esophagus following vomiting. The aim of this retrospective study is to analyze the therapeutic modality, morbidity and mortaliy of a group of patients with spontaneous esophageal rupture treated in our hospital.MethodsTen patients were treated from March 1990 to August 2016. Seven patients were males and three were females. The age range was, 52-72 years, with an average of 66.2 years. In four patients, the diagnosis and posterior treatment were performed within 24 h (Group I) and the remaining six patients after 24 h (Group II).ResultsThe mean hospital stay was 36.6 days (range 17-62 days). The mortality rate was 50%, which was due to septic shock and the morbidity of patients who survived was 40% due to pneumonia in one case and fistula in another.DiscussionsThis condition has a high mortality rate with a lethality that depends on the time between recognition of symptoms and proper surgical treatment.
| S-EPMC6651841 | biostudies-literature
Project description:IntroductionRetropharyngeal calcific tendinitis (RCT) is a self-limiting aseptic inflammation of the tendon of the longus colli muscle, which can be clinically and radiologically misdiagnosed as abscess formation. This is a particular challenge for ENT specialists. However, articles about RCT are highly underrepresented in ENT journals and existing articles in ENT journals almost exclusively report overtreatment.MethodsThis study presents five patients, in which the diagnosis of RCT was delayed and of which one patient underwent incision and draining of a suspected retropharyngeal abscess under general anesthesia. In addition, the literature on the reported cases of RCT, between 1990 and 2020 was reviewed. For each case, epidemiological characteristics, complaints on presentation, symptoms, imaging and laboratory finding and treatment were summarized and compared to our own findings.ResultsIn all the five patients, the correct diagnosis was delayed. One patient underwent incision and draining of a suspected RA under general anesthesia. All patients received antibiotic treatment. The literature review revealed a total of 116 reported cases of RCT. A total of 99 CT scans and 72 MRI showed soft tissue swelling in 89.6% and calcifications in 91.4% of the cases, 6.9% received invasive treatment.ConclusionThis article emphasizes the importance of knowledge about RCT and its management to avoid invasive and potentially harmful treatment. The focus in establishing the correct diagnosis of RCT is the identification and correct interpretation of clinical symptoms together with the specific radiological findings.
| S-EPMC7410824 | biostudies-literature
Project description:ObjectiveTo report the stabilization of urinary glycosaminoglicans (GAG) excretion and clinical improvements in patients with mucopolysaccharidosis type I (MPS I) under an alternative dose regimen of laronidase of 1.2 mg/kg every other week.MethodsWe participated in a dose-optimization trial for laronidase in MPS-I patients using four alternative regimens: 0.58 mg/kg every week, 1.2 mg/kg every two weeks, 1.2 mg/kg every week and 1.8 mg/kg every other week (EOW). After the trial ended, the patients resumed the recommended dose and regimen of 0.58 mg/kg every week. Under this regimen, some patients presented difficulties in venous access and were unable to commute weekly to the treatment center. Therefore, we used an alternative regimen that consisted of 1.2 mg/kg EOW in eight patients. A retrospective study of medical records of MPS-I patients who underwent both enzyme replacement therapy (ERT) regimens, of 0.58 mg/kg every week and 1.2 mg/kg EOW, was done.ResultsPatients remained clinically stable under the alternative regimen, did not present elevation of urinary GAG nor any adverse event.Conclusions: The switch of dose regimen to 1.2 mg/kg EOW of laronidase was safe, and did not cause any clinical worsening in patients who had been previously under standard dose ERT.
| S-EPMC6868559 | biostudies-literature
Project description:Kawasaki disease (KD) is most common among East Asian children. There is a lack of data from Middle Eastern countries. We conducted a retrospective study of 27 paediatric patients with KD in Dubai, United Arab Emirates, 2012-2020. The majority of the patients were male, Asian, aged 1-5 years and presented with typical (complete) KD. Timely high-dose intravenous immunoglobulins were administered to 18 patients. Twelve patients did not develop any cardiac complications, 12 had a coronary artery aneurysm and 2 patients developed serious cardiac complications . No patient experienced non-cardiac complications or died. Paediatric patients with KD in Dubai were similar to those from other countries.
| S-EPMC9716952 | biostudies-literature
Project description:Immune checkpoint inhibitor anemias (ICI-A) are a rare entity which can be potentially life-threatening without prompt identification. The goal of the study is to characterize the presentation, evaluation, and outcomes of ICI therapy in early phase clinical trial setting to guide future research and to develop standardized care guidelines. Retrospective chart review of 333 patients who participated in early phase clinical trials at the University of Texas MD Anderson Cancer Center revealed four cases with ICI-A between 2016 and 2020. We identified a spectrum of four cases which included ICI-related autoimmune hemolytic anemias, hemophagocytic lymphohistiocytosis and thrombotic microangiopathy as a result of combinatory investigational therapies involving ICI. Patient presentation, evaluation, bone marrow pathology, interventions, and clinical course were reviewed. The median time to onset of hematological immune-related adverse events (heme-irAEs) in this retrospective series was 3.5 weeks (2 - 6 weeks). One patient had pre-existing untreated chronic lymphocytic leukemia. Glucocorticoids are an effective first-line treatment in most patients although most patients were not rechallenged but successfully had complete recovery and pursued further non-immunotherapy-based therapies. Cognizance of ICI-A in clinical trial setting is paramount to early recognition of heme-irAEs. Further research is needed to identify and stratify risk factors during clinical trial enrollment and optimal management strategies for immune-mediated hematologic toxicities.
| S-EPMC9275440 | biostudies-literature
Project description:We conducted a retrospective, observational study of 42 children with intracranial empyema admitted to a pediatric neurosurgical center over a 9-year period. Intracranial empyema is rare, but causes significant morbidity and mortality. Twenty-eight cases had neurosurgical source control, more commonly for subdural collections. Streptococcus anginosus group bacteria are important pathogens in subdural empyema, whose isolation predicts more complicated postoperative courses.
| S-EPMC10569674 | biostudies-literature
Project description:PurposeThe number of newly diagnosed inflammatory bowel disease (IBD) cases such as ulcerative colitis (UC), Crohn's disease (CD), and indeterminate colitis (IC) is rapidly increasing in Gulf countries and Saudi Arabia. The aim of this study was to investigate the incidence and prevalence of IBD in patients who have attended the Salmaniya Medical Complex, Bahrain, between the years 1984 and 2014.Patients and methodsAll patients who had attended the Salmaniya Medical Complex, Bahrain, and had been diagnosed with UC, CD, or IC, between the years 1984 and 2014, were included in the analysis. Data collected were: patient demographics, symptoms, clinical signs, complications, surgical interventions, extent of disease, endoscopic findings, histopathology, and lab measurements.ResultsA total of 187 cases were included; 61 had CD, 123 had UC, and a further 3 cases presented with IC. A clear increase in the incidence and prevalence of IBD can be seen in this cohort. The prevalence of IBD was calculated to be 26.25/105 cases. The average number of IBD cases increased from 3 cases (average for the years 1984-2001) to 12 cases (average for the years 2002-2014). A number of factors correlate positively or negatively with CD and UC. In the current study, a link between gastrointestinal complications in CD cases and the use of steroids as a treatment was noted (p-value -0.02). Age also had a significant influence on the need for surgery in CD cases (p-value -0.04), and a family history of UC was statistically linked to surgical intervention (p-value -0.05).ConclusionsIBD can no longer be considered a rare disease in Bahrain. The incidence of both UC and CD is steadily increasing. There is a need for increasing awareness of the Bahraini public to IBD in order for proper medical care to be given.
| S-EPMC5524702 | biostudies-literature
Project description:PurposeThe diagnosis of pulmonary blastomycosis is usually delayed because of its non-specific presentation. We aimed to assess the extent of diagnostic delay in hospitalized patients and detect the step in the diagnostic process that requires the most improvement.MethodsAdult patients diagnosed with pulmonary blastomycosis during a hospital admission between January 2010 through November 2021 were eligible for inclusion. Patients who did not have pulmonary involvement and who were diagnosed before admission were excluded. Demographics and comorbid conditions, specifics of disease presentation, and interventions were evaluated. The timing of the diagnosis, antifungal treatment, and patient outcomes were noted. Descriptive analytical tests were performed.ResultsA total of 43 patients were diagnosed with pulmonary blastomycosis during their admissions. The median age was 47 years, with 13 (30%) females. Of all patients, 29 (67%) had isolated pulmonary infection, while 14 (33%) had disseminated disease, affecting mostly skin and musculoskeletal system. The median duration between the initial symptoms and health care encounters was 4 days, and the time to hospital admission was 9 days. The median duration from the initial symptoms to the diagnosis was 20 days. Forty patients (93%) were treated with empirical antibacterials before a definitive diagnosis was made. In addition, corticosteroid treatment was empirically administered to 15 patients (35%) before the diagnosis, with indications such as suspicion of inflammatory processes or symptom relief. In 38 patients (88%), the first performed fungal diagnostic test was positive. Nineteen patients (44%) required admission to the intensive care unit, and 11 patients (26%) died during their hospital stay.ConclusionThere was a delay in diagnosis of patients with pulmonary blastomycosis, largely attributable to the lack of consideration of the etiological agent. Novel approaches to assist providers in recognizing the illness earlier and trigger evaluation are needed.
| S-EPMC10208673 | biostudies-literature