Objectives: To present a prospective report on the characteristics of autoimmune manifestations in patients with primary immunodeficient children registered in the Kuwait National PIDs Registry (KNPIDR).Methods: The data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of January 2004 to December 2019.Results: A total of 286 PID children were registered in KNPIDR during the study period with a predominance of immunodeficiencies affecting cellular and humoral immunity followed by combined immunodeficiencies with associated syndromic features and diseases of immune dysregulation. Fifty-seven (19.9%) patients presented with a total of 107 autoimmune manifestations. There was no significant statistical association between autoimmune manifestations and gender. Patients with autoimmune manifestations were older at onset of PID symptoms compared to those with no such manifestations, but this did not reach level of significance. The diagnosis delay was longer in patients with autoimmune manifestations compared to those with no such manifestations (p = 0.038). Forty-seven percent of these manifestations were among the presenting symptoms while 53% were documented later during the course of the disease. Fifty-seven percent of the patients developed 1 autoimmune manifestation, 30% developed 2 such manifestations, and 16% had ≥3 autoimmune manifestations. The most common autoimmune manifestation was cytopenia, followed by gastrointestinal manifestations and manifestations of the skin, hair, and nails. Autoimmune cytopenia were more common in patients with immune dysregulation syndromes, while gastrointestinal and skin manifestations predominate in patients with immunodeficiencies affecting cellular and humoral immunity and endocrine manifestations were more common in immune dysregulation syndromes. There were significant statistical associations between developing autoimmune manifestations and death as well as PID categories, being more common in patients with immune dysregulation. The frequency of autoimmunity was high among patients with RAG, WAS, STAT5b, NF-κB2, Fas, FasL, LRBA, APECED, IL-10, and C4 deficiencies.Massaad et al. Autoimmunity in Primary ImmunodeficienciesConclusions: Autoimmunity is frequent in patients with PIDs in Kuwait. This should prompt the suspicion of a PID in patients who present initially with autoimmunity, especially autoimmune cytopenia. Such patients should be managed with extra care since they are at a higher risk of death.

PurposeThis paper analyses the impact of innovation orientation on the family business performance in Kuwait. Further, the aim of this paper is to understand the innovation culture among Kuwaiti family businesses and the innovation orientation concept and its importance for these businesses.Design/methodology/approachThe study will use a quantitative research methodology in order to investigate the relationship between independent variables: creativity, risk-taking, future orientation, openness to change and proactiveness with Kuwaiti business performance as a dependent variable. A well-structured questionnaire is distributed to 150 family businesses that operate in Kuwait, where 114 of the respondents responded positively. Structural equation modeling (SEM) analysis was used for evaluating the proposed model.FindingsThe results show that creativity, risk-taking and future orientation have significant relationship with business performance, while openness to change and proactiveness do not have any significant relationship with business performance.Research limitations/implicationsThis research is conducted during the period of initial months of COVID-19 pandemic, March–July 2020 respectively, and responses might be influenced by the current situation.Originality/valueThe correlation between family businesses and innovation orientation is not studied so far in Kuwait. There is a lack of studies about family business in general in Kuwait since there are only very few research studies. This is the first research about this topic that covers Kuwait as a country.

Background and ObjectivesReports on skin manifestations in inborn errors of immunity (IEI) are based on retrospective analysis, small series, or isolated case reports. The present prospective study aimed to determine the spectrum of skin manifestations in children with IEI and their relevance to specific molecular defects.Materials and MethodsThe data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of 2004–2020.ResultsA total of 313 pediatric cases of IEI, 71% diagnosed at molecular level, were registered with a cumulative follow-up period of 29,734 months. Skin manifestations were seen in 40.3% of the patients, and they were among the presenting manifestations in 33%. Patients with skin manifestations were older at both onset and diagnosis ages of IEI symptoms, but this was statistically significant for the latter only. The diagnosis delay was significantly longer in patients with skin manifestations. There was a statistically significant association between having skin manifestations and IEI category, being more common in patients with complement deficiencies, combined immunodeficiencies, and diseases of immune dysregulation. There was no statistically significant association between having skin manifestations and both gender and survival. Skin infections were the most frequent manifestations followed by eczema and autoimmune associations. Among IEI with more than 10 cases, skin lesions were a consistent finding in dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome, ataxia-telangiectasia, and recombination activation gene (RAG)1 deficiency.ConclusionsSkin manifestations are common in IEI patients, and they had significant diagnosis delay and referral to specialists. Improvement of awareness about IEI is needed among pediatricians and dermatologists.

This survey demonstrates that there is universal deficiency in both the knowledge and practice of pediatricians in the field of PID. Implementation of strategies to improve the awareness of pediatricians about PID is critical so early therapeutic interventions can be done to improve the health and prevent morbidity and mortality.

The focal aim of the project was to assess the economic anxiety (EA) and the performance of small and medium enterprises (SMEs) during partial and full lockdowns in Kuwait. The challenges facing the SMEs during COVID-19 and the potential solutions were also explored. The call for this vital investigation was due to the global economic fallout and the shocking drop within the marketplace caused by the COVID-19 pandemic. A descriptive approach was used for online survey design to collect datasets from 147 SMEs spanning all governorates of Kuwait in the period between March and June 2021. It included sociodemographic data, economic anxiety perception, potential challenges and solutions to SMEs, and SMEs’ performance. The data analysis using SPSS 25 showed that 78.2% of the SMEs were affected directly by the COVID-19 pandemic, and about 83% were affected negatively by the COVID-19 pandemic. In comparison, only 12.2% experienced a positive impact, mainly medical, technology, social media, food supplies, and delivery or logistics industries. With great concerns of SMEs for all dimensions related to economic anxiety (with an average of around 3.95), the greatest concerns were the financial and cash flow, followed by labor shortage (an average between 4.51 and 5.00). The results also showed that most of the performance indicators for the SMEs were low (with an average of less than or equal to 2.5), and more than 66% of them worked fewer hours during the pandemic; the number of operating hours was dropped dramatically. More than 74% of the SMEs used technology in more than 20% of their activities, representing an increase in using technologies of about 44%, and about 25.2% used social networks in more than 80% of their activities. The performance of SMEs is also found to be significantly and positively correlated with the economic anxiety levels, with a correlation coefficient of 0.186. The findings revealed significant and crucial outcomes for policymaking, decision-makers, and governmental agencies to build recovery plans and proper actions needed to manage the consequences caused by the disaster against the economic and other developments within the context of SMEs. Overall, there is a clear need to find ways and customize operations to adapt to the new work modes that require social distancing, online operations, and site management. In addition, new alternative modes of SMEs work follow to compensate for the lower working hours from the office and increased online working from home.