Clinical manifestations and expression of CD18 to guide the diagnosis of leukocyte adhesion deficiency type 1: Mexico experience.

BACKGROUND: Leukocyte adhesion deficiency type 1 (LAD-1) is an inborn error of immunity characterized by a defect in leukocyte trafficking. METHODS: Patients with clinical suspicion of LAD-1 were referred to our institution. Complete blood count and flow cytometric analysis, to identify the expression of CD18, CD11b, and the lymphocyte population phenotyping, were performed, and statistical analysis was completed. RESULTS: We report clinical manifestations and immunological findings of six Mexican patients diagnosed with LAD-1. The diagnosis was based on typical clinical presentation, combined with laboratory demonstration of leukocytosis, and significant reduction or near absence of CD18 and its associated molecules CD11a, CD11b, and CD11c on leukocytes. We found atypical manifestations, not described in other countries, such as early-onset autoimmunity or infections caused by certain microorganisms. CONCLUSIONS: Patients with LAD-1 may present with atypical manifestations, making flow cytometry an indispensable tool to confirm the diagnosis. We present the first report of LAD-1 patients in a Latin American country.

Cyclosporin for treatment of refractory multisystemic inflammatory syndrome in a child.

Multisystemic inflammatory syndrome in children is an inflammatory condition with multiorgan dysfunction that manifest late in the course of Severe acute respiratory syndrome coronavirus 2 infection. We present a 12-year-old boy with a history of fever, vomiting, diarrhoea, and abdominal pain. He developed shock with ventricular dysfunction and pericardial effusion. He was diagnosed with multisystemic inflammatory syndrome in children and treatment with intravenous immunoglobulins, corticosteroids, and tocilizumab proved to be ineffective. Eventually, the patient responded to cyclosporin-A treatment. Multisystemic inflammatory syndrome in children has been treated with immunoglobulins and glucocorticoids and in refractory cases biologics and cyclosporin-A have been used. Intravenous and oral cyclosporin-A seems to be a safe and effective alternative treatment for refractory multisystemic inflammatory syndrome in children patients.

Towards a liquid healthcare: primary care organisational and management strategies during the COVID-19 pandemic - a qualitative study.

BACKGROUND: The COVID-19 pandemic has changed the organisational and management strategies of healthcare institutions such as primary care centres. Organisational culture as well as leadership style are key issues for the success of these institutions. Due to the multidimensional nature of identity processes, it is necessary to explore the changes experienced by health professionals from these perspectives. This study explores health professionals' organisational and management strategies in primary care settings during the COVID-19 pandemic. DESIGN: Qualitative, exploratory study based on the analysis of participants' accounts within a hermeneutic phenomenologicaly approach. METHODS: Research was conducted in primary care settings in two neighbouring Spanish healthcare regions. The sample included participants with different demographics (gender, age), professional roles (practice managers, general practitioners, paediatricians), employment status (permanent, temporary, zero-hours), and years of experience (under or over ten years' experience). Data were collected between July and December 2020 through focus groups and in-depth, semi-structured individual interviews. RESULTS: A total of 53 primary care workers participated in the study, of which 38 were individually interviewed and 15 participated in three focus groups. Of these, 78.4% were healthcare professionals, 49% were female nurses, and 70.5% had more than 10 years of work experience in primary care. Two main themes emerged: "liquid" healthcare and "the best healthcare system in the world". During the first wave of the COVID-19 pandemic, new, more fluid organisational and management models were implemented in primary care settings, which have remained in place since. Primary care workers' perceived a lack of appreciation and inclusion in decision-making that risked their alienation and disengagement. CONCLUSION: Primary care workers' professional identity became gradually blurred due to shifting perceptions of their professional roles in a context of increasing improvisation and flexible working practices. This affected their professional performance. TRIAL REGISTRATION: The study was approved by the Clinical Research Ethical Committee of the Talavera de la Reina Integrated Management Area (CEIm del AGI de Talavera de la Reina in Spain, Hospital Nuestra Señora del Prado, ref: 23/2020).

Kawasaki disease shock syndrome in the COVID-19 pandemic.

Multisystem inflammatory syndrome in children is a new entity in association with SARS-CoV2. Clinical features of Kawasaki disease were noted from the first reported cases of MIS-C. Before the COVID-19 pandemic, Kawasaki disease shock syndrome was considered to be a distinct and unique form of KD. We present a representative case that prove the current difficulty in clearly distinguishing MIS-C from pre-COVID-19-KDSS and emphasie the overlap of the diagnostic criteria.

Single-Walled Carbon Nanotubes Encapsulated within Metallacycles.

Mechanically interlocked derivatives of carbon nanotubes (MINTs) are interesting nanotube products since they show high stability without altering the carbon nanotube structure. So far, MINTs have been synthesized using ring-closing metathesis, disulfide exchange reaction, H-bonding or direct threading with macrocycles. Here, we describe the encapsulation of single-walled carbon nanotubes within a palladium-based metallosquare. The formation of MINTs was confirmed by a variety of techniques, including high-resolution transmission electron microscopy. We find the making of these MINTs is remarkably sensitive to structural variations of the metallo-assemblies. When a metallosquare with a cavity of appropriate shape and size is used, the formation of the MINT proceeds successfully by both templated clipping and direct threading. Our studies also show indications on how supramolecular coordination complexes can help expand the potential applications of MINTs.

Magnetic, Mechanically Interlocked Porphyrin-Carbon Nanotubes for Quantum Computation and Spintronics.

Atomic-scale reproducibility and tunability endorse magnetic molecules as candidates for spin qubits and spintronics. A major challenge is to implant those molecular spins into circuit geometries that may allow one, two, or a few spins to be addressed in a controlled way. Here, the formation of mechanically bonded, magnetic porphyrin dimeric rings around carbon nanotubes (mMINTs) is presented. The mechanical bond places the porphyrin magnetic cores in close contact with the carbon nanotube without disturbing their structures. A combination of spectroscopic techniques shows that the magnetic geometry of the dimers is preserved upon formation of the macrocycle and the mMINT. Moreover, the metallic core selection determines the spin location in the mMINT. The suitability of mMINTs as qubits is explored by measuring their quantum coherence times (Tm). Formation of the dimeric ring preserves the Tm found in the monomer, which remains in the µs scale for mMINTs. The carbon nanotube is used as vessel to place the molecules in complex circuits. This strategy can be extended to other families of magnetic molecules. The size and composition of the macrocycle can be tailored to modulate magnetic interactions between the cores and to introduce magnetic asymmetries (heterometallic dimers) for more complex molecule-based qubits.

COVID-19 in the Context of Inborn Errors of Immunity: a Case Series of 31 Patients from Mexico.

INTRODUCTION: Patients with inborn errors of immunity (IEI) have a compromised or inappropriate immune response. Although they might be considered a high-risk group for severe SARS-CoV-2 infection, the reported impact of COVID-19 in these patients has been reassuring, while the differential susceptibility of distinct types of IEI remains unclear. OBJECTIVE: We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19. METHODS: In a retrospective study from March 2020 to February 2021, four centers in Mexico collected clinical, laboratory, and genetic data from pediatric and adult patients with known diagnoses of IEI who presented with COVID-19, based on compatible symptoms and positive SARS-CoV-2 testing or known household exposure. RESULTS: We report 31 patients with known IEI from Mexico who presented with SARS-CoV-2 infection. Seventy-four percent were male, 52% were pediatric, and 81% survived. Their ages ranged from 5 months to 56 years, with a median of 17 years. Sixty-five percent had predominant antibody deficiencies, 48% were hospitalized, and 26% required ICU. Pediatric patients had a higher hospital admission rate than adults. Inpatient mortality was 40%, and ICU mortality rate was 63%. Forty-eight percent developed pneumonia, while 36% had evidence of hyperinflammation (4 adults and 7 children). Predominant laboratory features were lymphopenia and thrombocytopenia, seen in 70 and 44% of patients, respectively. The serum D-dimer median value was 2.6 (0.5-20.6) µg/mL, and the median highest ferritin value was 1015 (32-10,303) ng/mL. Intravenous immunoglobulin was used in 80% of patients. Other treatments included macrolides (39%) and corticosteroids (29%). Six patients died from secondary infection or uncontrolled systemic inflammation. DISCUSSION: Although impaired immunity due to IEI may be a predisposing factor for severe COVID-19, most of our patients with IEI who acquired the SARS-CoV-2 infection developed a well-tolerated infection and survived, as have more than 80% of worldwide reported patients to date. An impaired immune or inflammatory response may be a predisposing factor for some and a protective factor for others. A systematic review of the literature could help identify those patients at risk of severe disease and complications. Healthcare-associated infections should be aggressively prevented.

Adenoviral-induced rash and mucositis: Expanding the spectrum of reactive infectious mucocutaneous eruption.

Mucocutaneous eruptions associated with respiratory pathogens, specifically Mycoplasma pneumoniae (MP), has recently been described as a MIRM (MP-induced rash and mucositis). The term reactive infectious mucocutaneous eruption (RIME) has been proposed, since non-MP pathogens may also cause a similar rash and mucositis. We report two cases with clinical manifestations suggestive of MIRM/RIME, both with documented adenovirus infection.

Kawasaki disease presenting with hoarseness: A multinational study of the REKAMLATINA network.

BACKGROUND: Recently, hoarseness affecting the supraglottic structure has been reported in Kawasaki disease (KD). The objective of this study was to characterize the frequency of hoarseness in acute KD patients in Latin America. METHODS: We used prospective data from the multinational Red de Enfermedad de Kawasaki en America Latina (REKAMLATINA) network. A total of 865 patients from 20 countries were enrolled during the 3 year study period. Data on hoarseness were available in 858 (99.2%) patients. The clinical and laboratory characteristics between hoarse and non-hoarse KD were compared. RESULTS: Hoarseness was documented in 100 (11.6%) patients. Hoarse patients were younger than those with KD without hoarseness (median age 18 vs 26 months; P = 0.002) and presented with lower hemoglobin (10.7 g/dL vs 11.3 g/dL; P = 0.040) and hematocrit levels (32% vs 33%, P = 0.048). CONCLUSIONS: Hoarseness was found to be prevalent as a presenting sign of acute KD in younger children. Anemia may indicate the presence of active inflammation.

Kawasaki disease mimickers.

BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects patients younger than 5 years. In the absence of an available, affordable diagnostic test, detailed clinical history and physical examination are still fundamental to make a diagnosis. METHODS: We present five representative cases with KD-like presentations: systemic onset juvenile idiopathic arthritis, mycoplasma-induced rash and mucositis, staphylococcal scalded skin syndrome, BCGosis, and the recently described multisystemic inflammatory syndrome in children (MIS-C) associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) virus. RESULTS: Rash, fever, and laboratory markers of inflammation can be present in several childhood diseases that may mimic KD. CONCLUSION: The term 'Kawasaki syndrome' instead of 'Kawasaki disease' may be more appropriate. Physicians should consider an alternative diagnosis that may mimic KD, particularly considering MIS-C during the present pandemic, as an aggressive diagnostic and therapeutic approach is needed.

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease.

BACKGROUND: Kawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance. METHODS: A retrospective, descriptive study of children with KDSS in two hospitals was performed. Relevant articles about KD and shock were collected, and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature. RESULTS: Seventy-two patients presented with complete KD (69.9%), and 30.1% (31/103) had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS; n = 20). Sixteen of the 20 had coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 65% of the patients. The mortality rate was 6.8%. CONCLUSION: The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.

Band-Gap Opening in Metallic Single-Walled Carbon Nanotubes by Encapsulation of an Organic Salt.

The encapsulation of viologen derivatives into metallic single-walled carbon nanotubes (SWNTs) results in the opening of a band gap, making the SWNTs semiconducting. Raman spectroscopy, thermogravimetric analysis, and aberration-corrected high-resolution transmission electron microscopy confirm the encapsulation process. Through the fabrication of field-effect transistor devices, the change of the electronic structure of the tubes from metallic to semiconducting upon the encapsulation is confirmed. The opening of a gap in the band structure of the tubes was not detected in supramolecular controls.

Role of re-growth interface preparation process for spectral line-width reduction of single InAs site-controlled quantum dots.

We present growth and optical characterization measurements of single InAs site-controlled quantum dots (SCQDs) grown by molecular beam epitaxy on GaAs (001) patterned substrates by atomic force microscopy oxidation lithography. InAs SCQDs directly grown on the patterned surface were used as a seed layer and strain template for the nucleation of optically active single InAs SCQDs. The preservation of the initial geometry of the engraved pattern motifs after the re-growth interface preparation process, the lack of buffer layer growth prior to InAs seed layer deposition and the development of suitable growth conditions provide us an improvement of the SCQDs' active layer optical properties while retaining a high ratio of single occupation (89%). In this work a fivefold reduction of the average optical line-width from 870 µeV to 156 µeV for InAs SCQDs located 15 nm from the re-growth interface is obtained by increasing the temperature of the initial thermal treatment step of the re-growth interface from 490 °C to 530 °C.

Pseudologia fantastica: forensic and clinical treatment implications.

Pseudologia fantastica, also known as mythomania, or pathological lying, is a psychiatric phenomenon that is a mixture of fact and fiction involving fantasized events and self aggrandizing personal roles. It has been recognized in the field for over a century. In this case report we discuss three different cases, two of them presented in the acute inpatient setting and one outpatient setting. All three presented with the common theme of extensive and 'pathological lying' in a manner that was notably very destructive to them and posing significant challenges to the treatment team. In an attempt to shed light into some of the clinical and legal/forensic challenges it creates when faced in the clinical settings, we also raise the need for a better definition and classification of this symptom in the DSM.

Mechanically interlocked single-wall carbon nanotubes.

Extensive research has been devoted to the chemical manipulation of carbon nanotubes. The attachment of molecular fragments through covalent-bond formation produces kinetically stable products, but implies the saturation of some of the C-C double bonds of the nanotubes. Supramolecular modification maintains the structure of the SWNTs but yields labile species. Herein, we present a strategy for the synthesis of mechanically interlocked derivatives of SWNTs (MINTs). In the key rotaxane-forming step, we employed macrocycle precursors equipped with two π-extended tetrathiafulvalene SWNT recognition units and terminated with bisalkenes that were closed around the nanotubes through ring-closing metathesis (RCM). The mechanically interlocked nature of the derivatives was probed by analytical, spectroscopic, and microscopic techniques, as well as by appropriate control experiments. Individual macrocycles were observed by HR STEM to circumscribe the nanotubes.

Salivary Gland Scintigraphy in Sjögren's Syndrome: A Retrospective Study of Diagnostic Accuracy and Correlation With Histological and Immunological Biomarkers.

Introduction Sicca syndrome, characterized by xerophthalmia and xerostomia, is associated with various autoimmune and non-autoimmune conditions, posing diagnostic challenges. Sjögren's syndrome (SS) is the most prevalent systemic autoimmune disease linked to sicca symptoms. This study evaluates the diagnostic accuracy of salivary gland scintigraphy (SGS) in distinguishing SS from non-Sjögren's sicca conditions, alongside other diagnostic tests. Methods A retrospective analysis was conducted at Hospital Universitario La Paz from December 2019 to March 2023, including 142 patients diagnosed with sicca syndrome. Correlations between qualitative and quantitative SGS data (GE Healthcare, Chicago, Illinois) and multiparametric sicca evaluations were assessed. Results Among the 142 patients, 84 (59.15%) were classified as having SS, with 55 (65.48%) seropositive for anti-Ro antibodies. Abnormal SGS results were found in 135 (95.07%) patients. Qualitative SGS categorized seven (4.93%) as mild, 53 (37.32%) as moderate, 50 (35.21%) as severe, and 21 (14.79%) as functionally annulled. Moderate or worse impairment had a sensitivity of 0.88 and a specificity of 0.17. Functional annulment had a sensitivity of 0.17 and a specificity of 0.97. Quantitative SGS using ejection fraction thresholds of ≤30% and ≤20% had sensitivities of 0.35 and 0.18 and specificities of 0.84 and 0.94, respectively. Quantitative SGS metrics correlated with unstimulated whole salivary flow (WUSF; 0.243; p=0.003) and inversely with lymphocytic infiltration (-0.281; p=0.001). The 2016 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for Sjögren's syndrome demonstrated an area under the curve (AUC) of 0.932, which improved to 0.951 with the inclusion of SGS parameters. Conclusions SGS is a significant diagnostic tool in the multiparametric evaluation of sicca syndrome, showing strong correlations with histological and immunological markers. Its integration into diagnostic criteria enhances the differentiation between SS and non-Sjögren's sicca conditions, suggesting its potential inclusion in future classification frameworks.

Effects of a hospital-based food security programme for children with complex diseases in an upper-middle-income country: a before-and-after study.

BACKGROUND: To reduce health inequities in paediatric patients with complex diseases, our hospital developed a food security programme in 2022. The programme aims to mitigate food insecurity (FI) in paediatric patients with oncological, transplantation and congenital cardiovascular diagnoses, by providing a monthly nutritious food supply that covers up to 50% of the patient's family food intake, accompanied by social and nutritional follow-up. In this study, we aimed to assess the effect of the programme on FI and nutritional status and describe its implementation. METHOD: We conducted a before-and-after study of patients who entered the programme in a 14-month period. We used the Escala Latinoamericana y Caribeña de Seguridad Alimentaria (ELCSA) scale score, FI level and nutritional status measures to assess the effect of the programme. We used the Wilcoxon and McNemar tests to assess changes in scores and proportions of patients with moderate and severe FI, respectively, 31.5%-14.4% (p=0.0008) and of moderate FI from 68.5% to 36.9%. RESULTS: 111 patients were included. They had a baseline median (IQR) ELCSA score=8 (7-11) that changed to 6 (4-9) (p<0.0001). Severe FI according to ELCSA changed from 31.5% to 14.4% (p<0.001) and moderate from 68.5% to 36.9% (p<0.001). We found no differences in nutritional status regarding height for age (49.5% vs 51.3%, p=0.76), weight for height (42.5% vs 59.1%, p=0.75) or body mass index for age (38% vs 46%, p=0.42) CONCLUSION: The programme reduced FI in families by improving its level to mild or moderate. Children who entered the programme maintained an appropriate nutritional status despite the considerable risk of malnutrition described for oncological paediatric patients and paediatric solid organ transplantation receptors.

Hospital padrino: a collaborative strategy model to tackle maternal mortality: a mixed methods study in a middle-income region.

Background: Reducing maternal mortality ratio (MMR) remains a paramount goal for low- and middle-income countries (LMICs), especially after COVID-19's devastating impact on maternal health indicators. We describe our experience implementing the Hospital Padrino Strategy (HPS), a collaborative model between a high-complexity hospital (Fundación Valle del Lili) and 43 medium- and low-complexity hospitals in one Colombian department (an administrative and territorial division) from 2021 to 2022, to sustain the trend towards reducing MMR. The study aimed to assess the effects of implementing HPS on both hospital performance and maternal health indicators in Valle del Cauca department (VCD). Methods: A mixed-methods study was conducted, comprising two phases. In the first phase, we investigated a cohort of hospitals through prospective follow-up to assess the outcomes of HPS implementation on hospital performance and maternal health indicators in VCD. In the second phase, qualitative data were collected through focus groups with 131 health workers from 33 hospitals to explore the implications of the HPS implementation on healthcare personnel. All data were obtained from records within the HPS implementation and from the Health Secretary of VCD. Findings: Evidence shows that in the context of HPS, 51 workshops involved 980 healthcare workers, covering the entire territory. Substantial improvements were observed in hospital conditions and healthcare personnel's technical competencies when providing obstetric care. Seven hundred eighty-five pregnant women with obstetric or perinatal emergencies received care through telehealth systems, with a progressive increase in technology adoption. Nine percent required Intensive Care Unit (ICU) admission, and none died. The MMR decreased from 78.8 in 2021 to 12.0 cases per 100,000 live births by 2022. Improvements in indicators and conducted training sessions instilled confidence and empowerment among the healthcare teams in the sponsored hospitals, as evidenced in focus groups derived from a sample of 131 healthcare workers from 33 hospitals. Interpretation: Implementing the Hospital Padrino Strategy led to a significant MMR reduction, and consolidated a model of social healthcare innovation replicable in LMICs. Funding: The Hospital Padrino Strategy was funded by the Fundación Valle del Lili and the Health Secretary of Valle del Cauca. Furthermore, this study received funding from a general grant for research from Tecnoquimicas S.A.