Shrimp allergy: clinical characteristics and food challenge outcome in a centre in the north of Portugal

Abstract Shwachman-Diamond syndrome (SDS) is an inherited marrow failure syndrome associated with exocrine pancreatic dysfunction and an increased risk of myelodysplasia and leukemia. The majority of individuals with SDS carry biallelic SBDS gene mutations, however a subset of patients remain genetically undefined. The objective of this study was to compare the clinical characteristics of patients with and without SBDS mutations. To address these questions, we conducted a retrospective study of patients enrolled on the North American Shwachman-Diamond Syndrome Registry (SDSR). Clinical data from the SDSR were available for 55 individuals with biallelic SBDS mutations and 16 individuals who fulfilled clinical diagnostic criteria for SDS but lacked biallelic mutations in the SBDS gene. Study subject ages for SBDS mutation positive and negative cohorts span 2-52.4 and 2.8-21.4 years with median ages of 12.4 and 10.9 years respectively. Cytopenias were present for both SBDS mutation positive and negative cohorts, with neutropenia the most common event in 94% and 81% respectively. Bone marrow hypocellularity was reported in 91% of those with SBDS mutations and 69% of those without. Marrow dysplasia was reported in 65% of those with SBDS mutations and none of those without. Clonal abnormalities were present in 44% and 25% of those with and without SBDS mutations with median age of initial appearance at 9 years (0.8-45.1) and 7 years (1.2-14) respectively. Abnormalities included del7q and del20q in both groups as well as iso7q, trisomy 8 and others in the SBDS mutation positive group. Clonal abnormalities were all transient in the SBDS mutation negative cohort. One SBDS mutation positive individual developed AML. None of the SBDS mutation negative individuals developed malignancy or progressed to require HSCT thus far. Pancreatic dysfunction determined by low serum trypsinogen or pancreatic isoamylase was similar in both cohorts 79% vs 80%. However, only 27% (15/55) of SBDS mutation positive individuals reported requiring enzyme therapy with 33% (18/55) documenting failure to thrive, in contrast to 75% (12/16) of SBDS mutation negative individuals with 73% (11/15) having failure to thrive. A broad spectrum of congenital anomalies were reported in 55% and 56% of SBDS mutation positive and negative individuals respectively, with skeletal anomalies being the most common in both groups. Medical comorbidities commonly reported in both groups included eczema and endocrinopathies. Elevated liver transaminases were seen in 27% of SBDS mutation positive individuals but this was not seen in the SBDS mutation negative cohort. Conclusion: Patients with genetically undefined (SBDS mutation negative) SDS share clinical characteristics with SBDS mutation positive patients; however, the risk of leukemia in the genetically undefined patients remains unclear due to low patient numbers with short follow-up. Further studies of this young cohort are required to inform medical management and to advance our understanding of genetic etiology, mechanism, disease pathophysiology and treatment of these marrow failure disorders. Disclosures Dale: Amgen: Consultancy, Honoraria, Research Funding, Speakers Bureau.

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Clinical characteristics and drug sensitivity analysis of bloodstream infection caused by Klebsiella pneumoniae in the north of Henan province

10.1101/2021.06.15.21258957 ◽

2021 ◽

Author(s):

Yanchao Wang ◽

Shanmei Wang ◽

Wenjuan Wang ◽

Youhua Yuan ◽

Junzheng Yang

Keyword(s):

Drug Resistance ◽

Antibiotic Resistance ◽

Klebsiella Pneumoniae ◽

Bloodstream Infection ◽

Clinical Characteristics ◽

Drug Sensitivity ◽

Resistance Rate ◽

Henan Province ◽

The North ◽

Drug Sensitivity Test

Objectives To analyze clinical characteristics of bloodstream infection caused by Klebsiella pneumoniae and antibiotic resistance of Klebsiella pneumoniae in the north of Henan province, provide the basis for rational selection of antimicrobial drugs. Methods Klebsiella pneumoniae was isolated from 195 patients with bloodstream infection caused by Klebsiella pneumoniae in 2017 in the north of Henan Province, Phoenix100 blood culture and identification system were used for bacterial identification and drug sensitivity test was used for antibiotic resistance detection, and WHONET 5.6 software was used for data analysis of antibiotic resistance and antibiotic sensitivity; the medical history of patients, antibiotic use and laboratory examination results of 195 cases of patients with bloodstream infection caused by Klebsiella pneumoniae were also retrospectively analyzed. Results The patients with bloodstream infection caused by Klebsiella pneumoniae were mainly distributed in ICU, surgical department and Internal medicine department. There were 110 patients with bloodstream infection caused by Klebsiella pneumoniae accompanied with underlying diseases, accounting for 56.41% in 195 patients with bloodstream infection caused by Klebsiella pneumoniae, and 87 (87/110, 77.3%) patients accompanied with hypertension and diabetes. Drug sensitivity test showed that in 195 patients with bloodstream infection caused by Klebsiella pneumoniae, the top three antibiotics of the drug resistance rate of Klebsiella pneumoniae were cefazolin (74%), amoxicillin/clavulanic acid (70.1%), ampicillin /sulbactam (68.5%); the lower three antibiotics of drug resistance rate were imipenem (52%), cefepime (53%) and amikacin (33%); there were 81 strains of Klebsiella pneumoniae produced ESBLs, accounting for 41.53% in 195 strains of Klebsiella pneumoniae. The drug resistance rate of ESBLs positive strains was significantly higher than that of ESBL negative strains. It should be pointed out that the resistance of ESBLs positive strains of Klebsiella pneumoniae to cefazole reached 100%, followed by gentamicin (71.4%), ciprofloxacin (70.4%) and levofloxacin (69.1%); The resistance of ESBLs negative strains to cefazole was 18.2%, followed by gentamicin (3.5%), ciprofloxacin (3.0%) and levofloxacin (19.3%). Conclusions The number of total bacteria isolated from departments with large number of patients is relatively large, and the number of pneumonia patients caused by Klebsiella is also increased; ESBLs positive strains in this hospital are still the main reasons for the drug resistance of Klebsiella pneumoniae, reducing the antibiotics use of cefazol, gentamycin, ciprofloxacin and levofloxacin can effectively reduce the resistance of Klebsiella pneumoniae in the hospital. At the same time, we should pay attention to some contraindications for treating hypertension, diabetes drugs and antibiotics; the clinical staff should pay attention to the timely blood culture test, rational drug use can reduce the emergence of drug-resistant strains and prevent the outbreak of nosocomial infection.

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Demographic and clinical characteristics of UK military veterans attending a psychological therapies service

Psychiatric Bulletin ◽

10.1192/pb.bp.113.046474 ◽

2014 ◽

Vol 38 (6) ◽

pp. 270-275 ◽

Cited By ~ 5

Author(s):

Clarissa M. Giebel ◽

Paul Clarkson ◽

David Challis

Keyword(s):

Physical Disability ◽

Clinical Characteristics ◽

Post Treatment ◽

Depression And Anxiety ◽

North West ◽

Symptoms Of Depression ◽

Psychological Therapies ◽

The North ◽

Anxiety Depression ◽

Probable Depression

Aims and methodTo investigate the demographic and clinical characteristics of subgroups of UK veterans attending a dedicated psychological therapies service following the Improving Access to Psychological Therapies (IAPT) treatment model. Veterans accessing a newly established service in the north-west were categorised into three groups: early service leavers, those with a physical disability, and substance and/or alcohol misusers. Anxiety, depression and social functioning were measured pre- and post-treatment.ResultsVeterans vary in their demographic and clinical characteristics as well as in treatment efficacy, as measured by the post-treatment scores on probable depression and anxiety. Therapy appears to be most effective in early service leavers, whereas veterans with a physical disability or a substance or alcohol misuse problem tend not to do as well in terms of symptoms of depression or anxiety.Clinical implicationsThis study highlights the importance of targeting different veteran subgroups for dedicated psychological therapy.

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