scholarly journals Tofacitinib effectiveness in Blau syndrome: a case series of Chinese paediatric patients

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Song Zhang ◽  
Zhe Cai ◽  
Xiaolan Mo ◽  
Huasong Zeng
Keyword(s):  
Clinical Manifestations ◽  
Case Series ◽  
Janus Kinase ◽  
Blau Syndrome ◽  
Reactive Protein ◽  
Paediatric Patients ◽  
Imaging Results ◽  
Therapeutic Measures ◽  
Clinical Triad ◽  
Oligomerization Domain

Abstract Objective Blau syndrome (BS), a rare, autosomal-dominant autoinflammatory syndrome, is characterized by a clinical triad of granulomatous recurrent uveitis, dermatitis, and symmetric arthritis and associated with mutations of the nucleotide-binding oligomerization domain containing 2 (NOD2) gene. Aim of this study was to assess the efficacy of tofacitinib in Chinese paediatric patients with BS. Methods Tofacitinib was regularly administered to three BS patients (Patient 1, Patient 2, and Patient 3) at different dosages: 1.7 mg/day (0.11 mg/kg), 2.5 mg/day (0.12 mg/kg), and 2.5 mg/day (0.33 mg/kg). The clinical manifestations of the patients, magnetic resonance imaging results, serological diagnoses, therapeutic measures and outcomes of treatments are described in this report. Results The clinical characteristics and serological diagnoses of all BS patients were greatly improved after the administration of tofacitinib treatment. All patients reached clinical remission of polyarthritis and improvements in the erythrocyte sedimentation rate (ESR) and levels of C-reactive protein (CRP) and inflammatory cytokines. Conclusion Tofacitinib, a Janus kinase (JAK) inhibitor, is a promising agent for BS patients who have unsatisfactory responses to corticosteroids, traditional disease-modifying antirheumatic drugs, and biological agents.

scholarly journals COVID-19 in Neonates, A Case Series Study From Tertiary Neonatal Centers in Iran

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Sedigheh Rafiei Tabatabaei ◽  
Minoo Fallahi ◽  
Abbas Boskabadi ◽  
Naeeme Taslimi Taleghani ◽  
Fatemeh Pajouhandeh ◽  
...  
Keyword(s):  
Respiratory Symptoms ◽  
Neonatal Intensive Care ◽  
Heart Surgery ◽  
Open Heart Surgery ◽  
Clinical Manifestations ◽  
Case Series ◽  
Reactive Protein ◽  
Radiologic Findings ◽  
Case Series Study ◽  
Novel Coronavirus

Introduction: The Novel coronavirus, sars-cov-2, is responsible for the recent pandemic. Although it mostly affects adults, children of all ages, including neonates, can become ill with Covid-19, as well. The real prevalence rate of coronavirus disease 2019 (COVID-19) in children is unknown. However, the severity of symptoms in children and neonates is less than in adults. Regarding the new presentation of this disease, the current study has reported a case series of COVID-19 in neonates. Case Presentation: In this article, 10 neonates with COVID- 19 admitted to our neonatal intensive care units are reported. All reported neonates had general suspicious symptoms of COVID- 19 with positive results for SARS-CoV-2 assessed by polymerase chain reaction (PCR) from the nasopharynx area or nose of the patients. All neonates, except for two of them, were term neonates. One case had open-heart surgery for congenital heart disease (transposition of the great arteries (TGA)). The patients aged 22 days on admission. The most frequent symptom was fever. Severe respiratory symptoms were reported in two cases. Also, abnormal radiologic findings in the chest x-ray were detected in two cases. Regarding the lack of significant respiratory symptoms in most of the patients, the lung computed tomography (CT) scan was taken just from one neonate. Leukopenia (WBC < 5000/mm3) was detected in one case, with no lymphopenia in all neonates. The positive C-reactive protein test was not found in all cases. No patient was treated by special anti-viral agents for COVID-19, and usual antibiotic treatment for neonatal sepsis was administered for all cases. All patients, except for one, survived with no significant sequela of the disease. Conclusions: This study demonstrated that clinical manifestations, as well as laboratory and radiologic findings of COVID-19, are milder in neonates than in the older ages. Hence, it can be argued that the prognosis of COVID-19 in the neonatal period is generally good.

scholarly journals Impact of C-Reactive Protein Levels on Differentiating of Severe Fever With Thrombocytopenia Syndrome From Japanese Spotted Fever

2020 ◽  
Vol 7 (11) ◽  
Author(s):  
Takeshi Kawaguchi ◽  
Kunihiko Umekita ◽  
Atsushi Yamanaka ◽  
Seiichiro Hara ◽  
Tetsuro Yamaguchi ◽  
...  
Keyword(s):  
Spotted Fever ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Case Series ◽  
Positive Likelihood Ratio ◽  
Hemorrhagic Fever ◽  
Spotted Fever Group ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Severe Fever

Abstract Background Severe fever with thrombocytopenia syndrome (SFTS) is an emerging viral hemorrhagic fever in China, Korea, and Japan. Japanese spotted fever (JSF), which belongs to spotted fever group rickettsioses, is also endemic to Western Japan. Patients with SFTS and those with JSF display many of the same clinical manifestations. Sudden fever, rash, tick bite, and neurological and gastrointestinal symptoms may be seen in both infections, but the frequency and severity of each disease have not been compared and studied. Because laboratory confirmation of pathogens takes time, it is important to predict diagnosis of SFTS vs JSF based on the features of the clinical characteristics at the initial presentation, particularly in primary care settings. Methods We conducted a case series review at 4 medical facilities in Miyazaki, Japan. Based on the medical records, clinical and laboratory characteristics were compared between patients with SFTS and those with JSF. Results Eighty-one patients were enrolled in this study, including 41 with SFTS and 40 with JSF. The absence of rash (P &lt; .001), leukopenia (P &lt; .001), and normal C-reactive protein (CRP) levels (P &lt; .001) were the variables distinguishing SFTS from JSF. Normal CRP levels (≤1.0 mg/dL) had a 95% sensitivity (84%–99%) and 97% specificity (87%–100%) for SFTS, with a positive likelihood ratio of 37.1 (5.35–257). Conclusions Normal serum CRP levels were shown to differentiate SFTS from JSF with a very high probability.

scholarly journals Thrombocytopenia and Intracranial Venous Sinus Thrombosis after “COVID-19 Vaccine AstraZeneca” Exposure

2021 ◽  
Vol 10 (8) ◽  
pp. 1599
Author(s):  
Marc E. Wolf ◽  
Beate Luz ◽  
Ludwig Niehaus ◽  
Pervinder Bhogal ◽  
Hansjörg Bäzner ◽  
...  
Keyword(s):  
Sinus Thrombosis ◽  
Clinical Manifestations ◽  
Venous Sinus ◽  
European Medicines Agency ◽  
Coagulation Disorder ◽  
Platelet Factor ◽  
Venous Sinus Thrombosis ◽  
Antiplatelet Antibodies ◽  
Imaging Results ◽  
Venous Thromboembolic Events

Background: As of 8 April 2021, a total of 2.9 million people have died with or from the coronavirus infection causing COVID-19 (Corona Virus Disease 2019). On 29 January 2021, the European Medicines Agency (EMA) approved a COVID-19 vaccine developed by Oxford University and AstraZeneca (AZD1222, ChAdOx1 nCoV-19, COVID-19 vaccine AstraZeneca, Vaxzevria, Covishield). While the vaccine prevents severe course of and death from COVID-19, the observation of pulmonary, abdominal, and intracranial venous thromboembolic events has raised concerns. Objective: To describe the clinical manifestations and the concerning management of patients with cranial venous sinus thrombosis following first exposure to the “COVID-19 vaccine AstraZeneca”. Methods: Patient files, laboratory findings, and diagnostic imaging results, and endovascular interventions of three concerning patients were evaluated in retrospect. Results: Three women with intracranial venous sinus thrombosis after their first vaccination with “COVID-19 vaccine AstraZeneca” were encountered. Patient #1 was 22 years old and developed headaches four days after the vaccination. On day 7, she experienced a generalized epileptic seizure. Patient #2 was 46 years old. She presented with severe headaches, hemianopia to the right, and mild aphasia 13 days after the vaccination. MRI showed a left occipital intracerebral hemorrhage. Patient #3 was 36 years old and presented 17 days after the vaccination with acute somnolence and right-hand hemiparesis. The three patients were diagnosed with extensive venous sinus thrombosis. They were managed by heparinization and endovascular recanalization of their venous sinuses. They shared similar findings: elevated levels of D-dimers, platelet factor 4 antiplatelet antibodies, corona spike protein antibodies, combined with thrombocytopenia. Under treatment with low-molecular-weight heparin, platelet counts normalized within several days. Conclusion: Early observations insinuate that the exposure to the “COVID-19 vaccine AstraZeneca” might trigger the expression of antiplatelet antibodies, resulting in a condition with thrombocytopenia and venous thrombotic events (e.g., intracranial venous sinus thrombosis). These patients’ treatment should address the thrombo-embolic manifestations, the coagulation disorder, and the underlying immunological phenomena.

scholarly journals 766. Everything Old is New Again-A Case Series of New World Leishmaniasis in African Children in Portland, Maine

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S428-S428
Author(s):  
Jennifer Jubulis ◽  
Amanda Goddard ◽  
Elizabeth Seiverling ◽  
Marc Kimball ◽  
Carol A McCarthy
Keyword(s):  
Latin America ◽  
Cutaneous Leishmaniasis ◽  
New World ◽  
Pediatric Patients ◽  
Data Extraction ◽  
Clinical Manifestations ◽  
Case Series ◽  
Surgical Excision ◽  
Skin Lesions ◽  
Travel History

Abstract Background Leishmaniasis has many clinical manifestations and treatment regimens, dependent on species and host. Old world leishmaniasis is found primarily in Africa and Asia, and is associated with visceral disease, while new world disease, seen primarily in Latin America, is more commonly mucocutaneous. We present a case series of pediatric African patients with New World cutaneous leishmaniasis (NWCL). Methods Data extraction was performed via chart review, analyzing travel history, clinical presentation, diagnosis, and management in children with cutaneous leishmaniasis presenting to the pediatric infectious diseases clinic in Portland, ME. Biopsy specimens were sent to the federal CDC for identification by PCR and culture. Results Five cases of NWCL were diagnosed in pediatric patients in Maine from November 2018 through February 2020. Median age of patients was 10 years (range 1.5-15 years). Four cases (80%) occurred in children from Angola or Democratic Republic of Congo, arriving in Maine via Central/South America, with one case in a child from Rwanda who arrived in Maine via Texas. Three patients had multiple skin lesions and two had isolated facial lesions. Leishmaniasis was not initially suspected resulting in median time to diagnosis of 5 months (range 1-7 months). Four patients were initially treated with antibacterials for cellulitis and one was treated with griseofulvin. After no improvement, patients underwent biopsy with 2 patients diagnosed with L panamensis, 1 with L braziliensis, 1 with mixed infection (L panamensis and L mexicana), and 1 with Leishmania species only. One patient was managed with surgical excision, 3 with ketoconazole, and 1 was observed off therapy. Four patients were referred to otolaryngology. All continue to be followed in infectious disease clinic. Conclusion We present five cases of new world cutaneous leishmaniasis in African pediatric patients arriving to Maine through Latin America or Texas. Patients were diagnosed with cellulitis, tinea corporis or atopic dermatitis initially, underscoring importance of high index of suspicion in migrant patients. Detailed travel history and epidemiologic knowledge is essential to diagnosis, as patients may present with illness not congruent with country of origin. Optimal therapy remains unclear. Disclosures All Authors: No reported disclosures

scholarly journals Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review

2021 ◽  
Author(s):  
Videha Sharma ◽  
Zia Moinuddin ◽  
Angela Summers ◽  
Mohan Shenoy ◽  
Nicholas Plant ◽  
...  
Keyword(s):  
Case Series ◽  
Wound Management ◽  
Supplementary Information ◽  
Operative Morbidity ◽  
Post Surgery ◽  
Paediatric Patients ◽  
Stoma Formation ◽  
Gut Function ◽  
Health And Development ◽  
Secondary Closure

Abstract Background Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique. Methods Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. Results Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / − stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient’s stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants. Conclusions This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

scholarly journals Clinical features and predictors of severity in COVID-19 patients with critical illness in Singapore

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ser Hon Puah ◽  
◽  
Barnaby Edward Young ◽  
Po Ying Chia ◽  
Vui Kian Ho ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Clinical Features ◽  
Operating Characteristic ◽  
Characteristic Curve ◽  
Case Series ◽  
Plateau Pressure ◽  
Apache Ii Score ◽  
Multivariable Logistic Regression Model ◽  
Reactive Protein ◽  
Ventilatory Management

AbstractWe aim to describe a case series of critically and non-critically ill COVID-19 patients in Singapore. This was a multicentered prospective study with clinical and laboratory details. Details for fifty uncomplicated COVID-19 patients and ten who required mechanical ventilation were collected. We compared clinical features between the groups, assessed predictors of intubation, and described ventilatory management in ICU patients. Ventilated patients were significantly older, reported more dyspnea, had elevated C-reactive protein and lactate dehydrogenase. A multivariable logistic regression model identified respiratory rate (aOR 2.83, 95% CI 1.24–6.47) and neutrophil count (aOR 2.39, 95% CI 1.34–4.26) on admission as independent predictors of intubation with area under receiver operating characteristic curve of 0.928 (95% CI 0.828–0.979). Median APACHE II score was 19 (IQR 17–22) and PaO2/FiO2 ratio before intubation was 104 (IQR 89–129). Median peak FiO2 was 0.75 (IQR 0.6–1.0), positive end-expiratory pressure 12 (IQR 10–14) and plateau pressure 22 (IQR 18–26) in the first 24 h of ventilation. Median duration of ventilation was 6.5 days (IQR 5.5–13). There were no fatalities. Most COVID-19 patients in Singapore who required mechanical ventilation because of ARDS were extubated with no mortality.

Wernicke’s encephalopathy in women with hyperemesis gravidarum – Case series and literature review

2021 ◽  
pp. 004947552110143
Author(s):  
Makarand Anil Rane ◽  
Hari Kishan Boorugu ◽  
Usha Ravishankar ◽  
S Tarakeswari ◽  
Hemamalini Vadlamani ◽  
...  
Keyword(s):  
Hyperemesis Gravidarum ◽  
Rare Complication ◽  
Case Series ◽  
Wernicke’S Encephalopathy ◽  
Wernicke's Encephalopathy ◽  
Chronic Alcohol Abuse ◽  
Delayed Treatment ◽  
Altered Mental State ◽  
Short And Long Term ◽  
Clinical Triad

Whilst nausea affects around 80% of pregnant women, hyperemesis gravidarum, an extreme form of the same, affects only 0.5% to 3%, but may lead to severe nutritional deficiency. Wernicke’s encephalopathy is an acute neuropsychiatric disorder which occurs due to thiamine deficiency and needs emergency treatment to prevent neurological morbidity and mortality. Wernicke’s encephalopathy is characterised by a clinical triad of oculomotor abnormalities, cerebellar dysfunction and altered mental state. Korsakoff’s psychosis is a chronic condition and consequence of Wernicke’s encephalopathy, resulting from its delayed treatment. Wernicke’s encephalopathy is a well-known complication of chronic alcohol abuse. Not many are aware of its association with hyperemesis gravidarum. Although it is a rare complication, if not diagnosed and treated promptly, it may result in permanent and irreversible neurological sequelae. The objective of our retrospective observational study was to analyse the clinical profile and outcome (short and long term) in a rare yet preventable complication of pregnancy.

P1279INSULIN RESISTANCE IN HEMODIALYSIS PATIENS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Pavel Filinyuk ◽  
Aleksander Rumyantsev
Keyword(s):  
Insulin Resistance ◽  
Systemic Inflammation ◽  
Clinical Manifestations ◽  
Biological Response ◽  
Fold Increase ◽  
Atherogenic Dyslipidemia ◽  
Model Assessment ◽  
Peripheral Tissues ◽  
Reactive Protein ◽  
Homeostatic Model Assessment

Abstract Background and Aims insulin resistance (IR) is a decrease in the biological response of sensitive tissues to insulin. IR is known as an adverse risk factor in cardiovascular disease, which largely determines the prognosis of patients receiving hemodialysis (HD). But this issue is not well understood. For the screening of IR, special indices have been developed that characterize the sensitivity of tissues to insulin. The aim of the study was to compare the methods of screening for IR in patients receiving HD in relation to the markers of systemic inflammation and atherogenic dyslipidemia (AtD). Method 124 patients receiving HD for 75.4 ± 44.5 months were examined including 66 men and 58 women aged 57.6 ± 13.6 years. For IR screening, the Homeostatic Model Assessment-1 and 2 indices (HOMA-1 and HOMA-2), the Quantitative Insulin Sensitivity Check Index (QUICKI) and triglycerides / glucose (Tri/G) were used. Patients were examined in accordance with the recommendations of KDIGO. Data analysis was carried out using “STATISTICA 10.0”. Results fasting insulin levels were elevated in 19% of patients. But, the calculated indices were consistent with the idea that IR is much more common. So, the IR index in the HOMA -1 model was increased in 47%, in the HOMA -2 model - in 33%, in the QUICKI model - in 36%, the TriH indicator - in 91%. The sensitivity of peripheral tissues in the HOMA-1 and HOMA-2 models was equally reduced by 35-40%. The results of the correlation analysis between indicators of IR and plasma concentration of C-reactive protein and lipid profile are presented in table 1. Informativeness of IR indicators depending on the presence of obesity is presented in table 2 We were also interested in whether insulin resistance affects the development of clinical manifestations of atherosclerosis, cardiac arrhythmias, and heart failure. An analysis of this relationship did not reveal. Only the IR index in the HOMA-1 model with a value of more than 2.7 units was associated with a 4.5-fold increase in the risk of developing clinical manifestations of atherosclerotic lesions (χ2 = 4.582 p = 0.032). Statistically significant it was only in men. Given our data, perhaps IR is one of the reasons for the higher morbidity and mortality of men at HD. Conclusion a comparison of IR models allows us to distinguish HOMA-2 as the most accurate index. The highest correlation with systemic inflammation and AtD was in the HOMA-1 and HOMA-2 indices.

scholarly journals Bacillus cereus Typhlitis in a Patient with Acute Myelogenous Leukemia: A Case Report and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
James D. Denham ◽  
Sowmya Nanjappa ◽  
John N. Greene
Keyword(s):  
Bacillus Cereus ◽  
Acute Myelogenous Leukemia ◽  
English Language ◽  
Neutropenic Patient ◽  
Hematologic Malignancy ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Case Series ◽  
Myelogenous Leukemia ◽  
Acute Myelogenous

Bacillus cereus is a Gram-positive rod that is now recognized as a rare cause of frank disease in the neutropenic hematologic malignancy patient. Because this pathogen is rarely isolated in clinical specimens, no large studies exist to guide the management of these acutely ill patients. Individual case reports and case series exist in the literature describing various clinical manifestations of B. cereus in the neutropenic patient including bacteremia/septicemia, pneumonia, meningitis/encephalitis, hepatic abscesses, and gastritis. In this report, we describe a case of typhlitis caused by B. cereus in a 74-year-old female with recently diagnosed acute myelogenous leukemia (AML), and we summarize the available English language literature to draw tentative conclusions regarding the clinical manifestations of this organism.

Role of antioxidant therapy in patients with moderate and severe COVID-19

2021 ◽  
Vol 19 (1) ◽  
pp. 159-164
Author(s):  
E.K. Shavarova ◽  
◽  
E.R. Cazakhmedov ◽  
M.V. Alekseeva ◽  
L.G. Ezhova ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Intervention Group ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Control Group ◽  
Reactive Protein ◽  
Virus Rna ◽  
Active Intervention Group ◽  
Group 2 ◽  
Novel Coronavirus

The coronavirus disease COVID-19 is characterized by high mortality and the lack of effective etiotropic therapy. Activation of oxidative stress may be one of the links in the pathogenesis of organ damage of this infection. Objective. To assess the ability of Mexidol® to influence the rate of clinical improvement in pneumonia caused by the SARSCoV-2 virus in hospitalized patients with the novel coronavirus disease COVID-19 and concomitant discirculatory encephalopathy. 62 patients over the age of 18 years with confirmed new coronavirus disease COVID-19 according to computed tomography (CT) of the lungs (stages CT1, CT2, CT3) and PCR of a swab from the nasopharynx and oropharynx for SARS-CoV-2 virus RNA were included. After randomization patients of group 1 received an infusion of Mexidol® at a dose of 1000 mg/day, patients of group 2 – an infusion of isotonic sodium chloride solution for 7 days. Compared with the control group, the patients receiving Mexidol® therapy showed a significantly more pronounced decrease in body temperature, a tendency towards a decrease in the severity of shortness of breath. In the Mexidol® group, the concentration of superoxidedismutase did not change, while in the control group there was a tendency to its decrease, C-reactive protein decreased 2.2 times more than in the control group (p = 0.09). There was a tendency for a more rapid decrease in ferritin in the active intervention group. Mexidol® therapy can have a positive effect on the clinical manifestations and severity of laboratory-inflammatory syndrome in patients with the new coronavirus disease COVID-19. Key words: coronavirus disease COVID-19, oxidative stress, Mexidol

Sign in / Sign up

Export Citation Format

Share Document