Self-advocates with Down syndrome research the lived experiences of COVID-19 lockdowns in Aotearoa New Zealand

Background

Individuals with Down syndrome are particularly vulnerable to COVID-19 because they are recognised as significantly immunocompromised. Yet their voices regarding their lived experiences of pandemic lockdowns have not been sought or heard.

Aim

This study aims to describe the lived experiences of people with Down syndrome during the pandemic lockdowns in Aotearoa New Zealand to add evidence in order to inform systemic advocacy.

Method

A mixed-methods approach positioned within an inclusive research paradigm was used, in which a group of self-advocates with Down syndrome co-designed a structured interview schedule and conducted 40 face-to-face interviews. Key themes were identified by using content analysis.

Results

Despite the difficulties associated with lockdowns and participants not receiving their usual supports and having to make significant adjustments, they remained positive, adapted well, and demonstrated a high level of resilience and adaptability.

Conclusions

The findings add to the limited research on the lived experiences of people with Down syndrome during pandemic lockdowns. This research has given them a voice to contribute to policy, government initiatives, and service providers; particularly on issues around support during lockdown and staying connected with others.     

Differential effects of heparin on the early and late phases of hepatic ischemia and reperfusion injury in the pig.

The mechanisms by which heparin protects the liver during induced episodes of liver ischemia-reperfusion are poorly understood. Previous work in a swine model demonstrated that serum levels of glycohydrolases and lipid peroxide peaked within 3 h after 45 minutes of hepatic ischemia followed by reperfusion. Serum levels of lactate dehydrogenase and aspartate aminotransferase peaked 20-24 h later. The aim of this study was to evaluate the effect of heparin on these two-phases of enzyme release, using a pig model of hepatic ischemia-reperfusion injury. Twenty male swine were divided into control (n = 8) and heparin (n = 12) groups. In the heparin group, heparin was administered prior to and concurrent with ischemia-reperfusion. Following 45 min of hepatic ischemia, the levels of beta-galactosidase, beta-glucosidase, acid phosphatase, purine nucleoside phosphorylase, lipid peroxides, lactate dehydrogenase, and aspartate aminotransferase in serum were monitored for up to 166 h and compared to pre-ischemic and control levels. With heparin infusion, the peak levels of beta-galactosidase, beta-glucosidase, and the lipid peroxide were reduced to 50-60% of the control levels. Acid phosphatase and purine nucleoside phosphorylase activities in serum were reduced to 25% and 60%, respectively. The peak concentrations of lactate dehydrogenase and aspartate aminotransferase were reduced to about 25% of the control level. In addition, the serum enzymes of control pigs did not return to pre-ischemic levels until 2 weeks after hepatic ischemia, while they normalized in less than 1 week in the heparin-treated animals. Systemic heparinization had different protective effects on the first and secondary phases of liver injury. These differences may reflect heparin protection of different types of liver cells. The protection of the parenchymal cells may be the combined result of reduced sinusoidal cell injury and the anticoagulant properties of heparin.     

Lysosomal enzymes in preeclamptic women in northern Nigeria

BACKGROUND: The incidence of preeclampsia is high in northern Nigeria, as it is in many other developing countries, and preeclampsia is associated with significant maternal and fetal morbidity and mortality. We inquired if proteinuria or hypertension alone could account for the altered concentrations of urinary lysosomal hydrolases that have been reported in preeclamptic women and pregnant women without preeclampsia. METHODS: The activities of urinary beta-hexosaminidase and beta-galactosidase were determined fluorometrically in pregnant women assigned to one of four groups: Group I: 41 preeclamptic women; Group II: 31 hypertensive aproteinuric women; Group III: 44 normotensive proteinuric women; and Group IV: 52 healthy pregnant women (controls). RESULTS: The urinary beta-hexosaminidase concentrations were decreased in the preeclamptic women (P<0.005) and proteinuric women (P<0.001) when compared to the healthy pregnant controls. There was no significant difference in beta-hexosaminidase concentrations between the hypertensive women and the healthy pregnant controls. The urinary beta-galactosidase concentrations for preeclamptic, hypertensive, and proteinuric women did not differ significantly versus healthy pregnant controls. CONCLUSIONS: The reduced urinary excretion of beta-hexosaminidase in preeclamptic women is associated with proteinuria, but not hypertension. Measuring urinary concentrations of lysosomal hydrolases alone or in conjunction with urinary protein concentrations is not likely to be useful in predicting or monitoring the clinical course of preeclampsia; however, it might prove important in gaining a more complete understanding of the pathogenesis of renal tubular epithelial cell injury and proteinuria that occurs in preeclampsia.     

Symptomatic Hyperglycemia in a Patient with Dialysis Ascites

An anuric woman with ascites rapidly developed extreme hyperglycemia and seizures after hemodialysis. During development of hyperglycemia, the decrease in serum sodium concentration (Δ[Na]) was nearly twice the value predicted by a formula accounting for the degree of hyperglycemia and the intracellular-to-extracellular volume ratio. The prediction assumed that ascitic fluid is part of the extracellular volume. Potential contributors to the development of seizures include the rapid development of severe hypertonicity, a remote history of seizure disorder and development of dialysis disequilibrium syndrome. Observations in peritoneal dialysis suggest that fluid with sodium concentration lower than in the ascitic fluid is transferred from the abdominal cavity into the blood during rapid development of hyperglycemia. In this case, Δ[Na], which determines the tonicity level expected after correction of hyperglycemia, resulted from exit of both intracellular and ascitic fluid into the extracellular compartment and, therefore, ascitic fluid functions as an extension of the intracellular fluid.     

Self-Efficacy,Depression, and Self-Care Activities in Adult Jordanians with Type 2 Diabetes: The Role of Illness Perception

Diabetes mellitus is reaching epidemic levels worldwide. In a developing country like Jordan, type 2 diabetes mellitus (T2DM) has reached a prevalence rate of 17.1%. This cross-sectional study examined the relationship between self-care activities and: illness perception, depression, social support, religiosity and spiritual coping, and self-efficacy among patients with T2DM. A random sample of 220 patients with T2DM, who attended Jordan University Hospital in Jordan were enrolled. The data were collected through a structured interview and the medical files. The instruments consisted of a sociodemographic and clinical standardised questionnaires: Brief Illness Perception Questionnaire, Patients’ Health Questionnaire-9; ENRICH Social Support Instrument; Religious and Spiritual Coping Subscale; Diabetes Management Self-Efficacy Scale; and Summary of Diabetes Self-Care Activities. Bivariate analysis investigated the relationship between variables. Structure Equation Modelling (SEM) was performed to test the proposed conceptual model. The study found that approximately 70% of the respondents suffered some form of depressive symptoms. The SEM showed a direct relationship between self-efficacy and self-care activities (β = 0.40; p < 0.001). Depression was indirectly related to self-care activities through self-efficacy (β = –0.20; p = 0.003); nevertheless, it was directly related to perception of: treatment control, consequences, and emotional representations. Overall, the sequence between illness perception and self-efficacy was mediated by depression. Strategies to promote self-efficacy and illness perception are vital in customising a diabetes health plan to meet Arabic cultural expectations.     

Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency

Homocysteine can be methylated to form methionine by the cobalamin- (Cbl) and folate-dependent enzyme, methionine synthase; serum levels of total homocysteine are elevated in greater than 95% of patients with either Cbl or folate deficiency. Homocysteine can also condense with serine to form cystathionine in a pyridoxal phosphate-dependent reaction catalyzed by cystathionine beta-synthase. Cystathionine is subsequently cleaved to cysteine and alpha-ketobutyrate by the pyridoxal phosphate-dependent enzyme gamma-cystathionase. To assess levels of cystathionine in Cbl and folate deficiency, we developed a new capillary gas chromatographic-mass spectrometric assay and measured cystathionine in the serum of normal subjects and patients with clinically confirmed deficiencies of these vitamins. The normal range for serum cystathionine was 65 to 301 nmol/L (median = 126 nmol/L) for 50 normal blood donors. In 30 patients with clinically confirmed Cbl deficiency, values for cystathionine ranged from 208 nmol/L to 2,920 nmol/L (median = 816 nmol/L) and 26 (87%) had levels above the normal range. In 20 patients with clinically confirmed folate deficiency, cystathionine concentrations ranged from 138 nmol/L to 4,150 nmol/L (median = 1,560 nmol/L) and 19 (95%) had values above the normal range. Five homozygotes for cystathionine beta-synthase deficiency had high values for serum-total homocysteine and low or low-normal values for serum cystathionine that ranged from 30 nmol/L to 114 nmol/L even though they were on treatment with pyridoxine and had partially responded. One patient with a defect in the synthesis of 5-CH3- tetrahydrofolate and five patients with defects in the synthesis of CH3- Cbl had high values for serum-total homocysteine and high values for cystathionine that ranged from 311 nmol/L to 1,500 nmol/L even though they were on treatment with folic acid and Cbl, respectively, and had partially responded. We conclude that levels of cystathionine are evaluated in the serum of most patients with Cbl and folate deficiency and that they are useful in the differential diagnosis of an elevated serum-total homocysteine level.     

Association of the subtype 2 of the Epstein-Barr virus with T-cell non- Hodgkin's lymphoma of the midline granuloma type [see comments]

Lethal midline granuloma (LMG) is associated with Epstein-Barr virus (EBV). The latter has at least two subtypes with different biological properties. The subtypes can be identified by their genomic configuration. Using EBV-RNA (EBER) in situ hybridization and EBV polymerase chain reaction (PCR), we have looked for the presence of EBV in six LMGs and six non-Hodgkin's lymphomas (NHLs) located in the nasopharyngeal region, and determined the subtype of EBV. Six of six LMGs were positive by PCR and EBER in situ hybridization, whereas NHLs were either negative or, in three of six cases, showed few EBER- positive cells considered to be nonneoplastic lymphocytes. The subtype 2 was found in LMG lesions of three of six patients; the remaining three of six patients with LMG had the generally occurring subtype 1. The results indicate that the association of EBV with NHL may depend more on tumor type than on its localization. The occurrence of the rare subtype 2 in LMG may relate to a covert immune defect.