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Abstract High-density lipoprotein cholesterol (HDL-C) has pleiotropic effects in immunoregulation. We study HDL-C levels in patients who visit the emergency room (ER) with infections and meet the criteria for the European Society for Immunodeficiencies (ESID). During the study period, 1600100 patients applied to the ER, and 4406 patients were identified who met the ESID criteria. There were 631 patients with HDL-C levels, and 54.3% had low HDL-C levels. Patients with low HDL-C have higher inflammation compared to patients with normal HDL-C. The low HDL-C group more frequently required hospitalization than the normal HDL-C group (Y.4 vs. @.6, p=0.007). Age, HDL-C, Hgb, LDH, CRP, and ferritin were associated with hospital admission (p<0.05 for all). HDL-C levels are associated with the need and duration of hospitalization in patients with ESID criteria. Patients with ID and low HDL-C are at increased risk for frequent ER visits and hospitalizations. HDL-C affects patient outcomes and healthcare utilization associated with ID traits.</p

A Case of Severe Spontaneous Pneumomediastinum in Acquired Immunosuppressed Child with Respiratory Syncytial Virus and Human Bocavirus Co-infection

Spontaneous pneumomediastinum is an uncommon, benign, and self-limiting disease based on the presence of free air in the mediastinal cavity. It can occur after a forced Valsalva’s maneuver due to a cough, emesis or during an asthma exacerbation. We present a 4-month-old girl infant with an autoimmune hemolytic anemia and lymphopenia, who was admitted to the pediatric intensive care unit due to respiratory distress. A suspected pneumomediastinum image on chest X-ray was documented by the chest tomography. A polymerase chain reaction of the nasopharyngeal secretions was positive for a respiratory syncytial virus and human bocavirus. Additionally, she was examined for an immune deficiency due to her lymphopenia. The patient required mechanical ventilatory support for 26 days. She was treated with antibiotics, antiviral, and antifungal agents and corticosteroids. Intravenous immunoglobulin, trimethoprim sulfamethoxazole, and fluconazole prophylaxis were continued because of the ongoing lymphopenia. At the end of five weeks, the patient was discharged with a normal lymphocyte count and without any respiratory distress. In immunosuppressed patients, respiratory diseases may be severe and long. Therefore, complications including pneumomediastinum can be seen more frequently in this group of patients

Atypical Localization of Eczema Discriminates DOCK8 or STAT3 Deficiencies from Atopic Dermatitis

PurposeAutosomal recessive dedicator of cytokinesis 8 (DOCK8(-/-)) and autosomal dominant signal transducer and activator of transcription 3 (STAT3(-/+)) deficiencies are inborn errors of immunity (IEI) disorders present with the classic features of eczema and create a dilemma during differentiation from atopic dermatitis (AD). Therefore, an appropriate approach is required for eczema to diagnose DOCK8(-/-) and STAT3(-/+) early. Here, we described a set of clinical and immunological variables, including atypical AD localizations and lymphocyte subsets, to differentiate DOCK8(-/-) or STAT3(-/+) from AD.MethodsThis multicenter study involved 100 patients with DOCK8(-/-) and STAT3(-/+) and moderate/severe AD. We recruited disease manifestations, including detailed localizations of eczema, infections, and allergy. Principle component analysis (PCA) was used to discriminate DOCK8(-/-) or STAT3(-/+) from AD.ResultsThere were 43 patients with DOCK8(-/-), 23 with STAT3(-/+), and 34 with AD. Pneumonia, severe infections, mucocutaneous candidiasis, and skin abscesses were commonly observed in DOCK8 and STAT3 deficiencies. Atypical skin involvement with neonatal rash, retro auricular, axillary, sacral, and genital eczema discriminate DOCK8(-/-) and STAT3(-/+) from AD with high specificity ranges between 73.5 and 94.1% and positive predictive index ranges between 55 and 93.1%. Together with using absolute numbers of CD3(+), CD4(+), and CD8(+) T cells, the combined clinical and laboratory features showed perfect differentiation between DOCK8(-/-) or STAT3(-/+) and AD via PCA.ConclusionsThe described features can be easily implemented by physicians providing early diagnosis of DOCK8 and STAT3 deficiencies.This work was supported by the Scientific and Technological Research Council of Turkey to S.B. (318S202).Scientific and Technological Research Council of Turkey [318S202

A novel gain-of-function mutation in STAT5Bis associated with treatment-resistant severe atopic dermatitis.

Additional information about study methods and findings are available in the following repository

Mycotoxigenic and phylogenetic perspective to the yeasts and filamentous moulds in mould-matured Turkish cheese

This study was conducted to determine the diversity of yeasts and filamentous moulds in mould-matured cheese (MMC) consumed in Turkey. Overall, 120 samples were collected from 12 different geographical locations between March 2016 and April 2017. The morphological observation was applied in combination with matrixassisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) and molecular analyses to determine yeasts and filamentous moulds in the cheeses. High-performance liquid chromatography (HPLC) technique was used to evaluate the ability of mycotoxins production of fungal isolates and the presence of mycotoxins in cheese samples. A total of 241 fungi (81 filamentous moulds and 160 yeast) were recovered, and Penicillium roqueforti and Debaryomyces hansenii were the most frequently isolated species in all cheese samples. The rep-PCR results indicated a high level of genetic diversity among fungal isolates, regardless of isolation source or geographical origin. Filamentous mould strains isolated from MMC were found to synthesize at least one mycotoxin (Aflatoxin B1, B2, G1 and G2, citrinine, cyclopiazonic acid, mycophenolic acid, ochratoxin A, penicillic acid and roquefortine C). Although mycotoxin producing ability was observed from all isolates, none of the cheese samples were found positive for these mycotoxins. AFM1 was detected in 8 (6.6%) MMC samples from which 2 (1.6%) were above the legal limits (0.05 mu g/kg) set by the Turkish Food Codex (TFC) and European Commission (EC). In conclusion, Turkish MMCs were found to be contaminated with toxigenic fungi, so a potential public health risk, while low, exists. Therefore, the selection of nontoxigenic filamentous mould strains for cheese manufacturing and control of the ripening conditions is a critical need to ensure the quality and safety of Turkish MMC

Clonal diversity and antifungal susceptibility of Candida spp. recovered from cow milk

The aim of this study was the isolation, identification, phylogenetic analysis and antifungal susceptibility of Candida spp. from milk samples of healthy and mastitic cows in Kayseri/Turkey. Milk samples from 300 cows were found to be negative/positive for mastitis with the California Mastitis Test. Candida spp. was isolated by using the Brillance Candida Agar Base. Phenotypic tests, Matrix Assisted Laser Desorption Ionization - Time of Flight (MALDI-TOF), and VITEK-2 analyses were applied to confirm the obtained isolates. Resistance to flucytosine, fluconazole and caspofungin antifungals of the isolates were determined by Etest and VITEK-2. The genetic homologies of Candida spp. isolates were determined by Repetitive Extragenic Palindromic-PCR (RepPCR). In this study 62 (from 53 healthy, 9 mastitic) yeast isolates were obtained and 37 (59.6 %) were identified as non-albicans Candida (NAC) species. Phenotypic tests revealed that out of 62 isolates, 29 (46.7 %), 4 (6.5 %), 3 (4.8 %), 1 (1.6 %) and, 24 (38.7 %) were identified as Candida lusitaniae, Candida catenulate, Candida tropicalis, Candida silvicola and other yeast species, respectively. Only one sample (1.6 %) was identified as Candida albicans by MALDI-TOF however, according to VITEK-2, the agent was not confirmed as C. albicans. According to antifungal susceptibility testing by VITEK-2, one (2.7 %) of the isolates was resistant to fluconazole, one (2.7 %) was resistant to caspofungin, and 4 (10.8 %) were resistant to flucytosine. However, using E test, 10 isolates (27 %) were resistant to flucytosine. Using Rep-PCR, eight genotypic clones were observed. Genotype F (13.8 %) and G (13.8 %; 2 subtypes) were common clones in this study. In conclusion, NAC species were detected in healthy and mastitic cow milk samples. Epidemiological studies need to be conducted to track effectively the main source and to understand the diversity and distribution of the agent. It is necessary to consider the potential risks of yeast contamination in milk for public health. It is essential to focus on adequate sanitation procedures and storage conditions of milk