Exploring associations among baseline emotion regulation and change in relationship satisfaction among couples in a randomized controlled trial of emotionally focused therapy compared to usual care.

BACKGROUND: Data from a two-arm randomized controlled trial of emotionally focused therapy (EFT) compared to usual care were used to examine whether baseline emotion regulation influences relationship satisfaction for female and male partners. This is clinically relevant as clinicians have debated whether clients' initial emotion regulation skills predict positive outcomes in EFT. METHODS: Dyadic multilevel modeling was used to determine whether baseline emotion regulation predicted both initial levels and change in relationship satisfaction and whether that relationship differed by treatment group (i.e., EFT or usual care). RESULTS: Baseline emotion regulation difficulties were associated with lower initial relationship satisfaction. However, baseline emotion regulation difficulties were not associated with change in relationship satisfaction over the course of treatment and this relationship did not differ by treatment group. CONCLUSIONS: Results demonstrate that partners with diverse presentations of emotion regulation at baseline may benefit from couple therapy.

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study.

Introduction Budd-Chiari syndrome (BCS) is a rare cause of ascites in children, and its clinical manifestation depends upon the extent and rapidity of the occlusion of hepatic veins. This study aimed to identify the clinical manifestations, causes, treatment options, and outcomes of BCS in children. Materials and methods A retrospective descriptive study of BCS in children under 15 years of age was conducted. This study was approved by the Pakistan Kidney and Liver Institute and Research Centre on June 23, 2023, with approval number 0128. The patients' medical records from December 2020 to July 2023 were obtained from Sisoft Healthcare Information System. In this study, we employ a set of predetermined questions to retrieve relevant data retrospectively and then organise it in Excel spreadsheets. SPSS version 26 (Armonk, NY: IBM Corp.) was used to analyse the data. Categorical variables are shown as frequencies (%), while continuous variables are reported as mean±SD. Results Of 37 (n) patients diagnosed with BCS, 19 (51.35%) were male and 18 (48.65%) were female. The mean age of presentation was 9.8±4.1 years. Ascites are the predominant clinical manifestation (100%), followed by hepatomegaly (37.8%). A total of 45.9% of patients had deranged liver function tests. Chronic BCS is the predominant mode of presentation. Protein C deficiency was present in nine patients (24.3%), two patients (5.4%) had protein S deficiency and two patients (5.4%) had antithrombin III deficiency. Hepatic veins exhibited the highest incidence of obstruction (73.0%). Liver biopsies were done in 15 (40.54%) patients to determine the staging of fibrosis. Eight patients (21.62%) had undergone radiological interventions, two patients had liver transplants and the rest were treated with medications, including anticoagulants. Conclusion BCS can present in acute, subacute or chronic forms. Ascites and hepatomegaly should raise the suspicion of BCS in children. Common radiological findings are non-visualisation of the hepatic veins. BCS has a wide range of aetiologies and treatment options. Protein C deficiency is the most predominant procoagulant disorder. Radiological interventions during the acute and subacute forms of BCS usually have excellent results. Liver transplant remains the definite treatment.

Evidence that donors with variant RH genotypes are associated with unexpected Rh antibodies.

BACKGROUND: We previously reported unexpected Rh antibodies in the plasma of patients with sickle cell disease (SCD) that demonstrated common Rh specificities in the absence of transfusion of RBCs positive for that antigen. We hypothesize that these antibodies might result from transfusion of antigen-negative donor units with variant RH genotypes. METHODS: Plasma testing by tube and IgG gel, extended RBC phenotyping, and HEA and RH genotyping were by standard methods. CASE: A 6-year-old female with SCD, phenotype D + C-c + E-e + K- undergoing exchange transfusion with CEK- and Fy(a-) units, presented with anti-C in the plasma, a + DAT and warm autoantibody (WAA) in the eluate. Her RH genotype was unremarkable: RHD*D/DAU0 and RHCE*ce/ce(48C). Units (n = 10) transfused over the prior 6 months were confirmed CEK- by serology and DNA testing. Most (n = 7) were Rh-negative. A unit with variant RH, RHD*DIIIa/weak partial 4.0, RHCE*ceVS.03/ceVS.02, was transfused 5 weeks prior. Anti-C and + DAT continued to demonstrate for 25 weeks. Total hemoglobin and % Hgb S did not deviate from her established baseline. CONCLUSION: We show direct association of plasma anti-C with transfusion of a C-negative unit with variant RH encoding partial D and uncommon V/VS+ hrB - phenotype. The antibody was transient, without evidence of compromised survival of transfused RBCs. The +DAT and WAA complicated workups and selection of units, and it is uncertain whether donors of the same genotype should be avoided. Minority donors are important for CEK-matching to avoid depleting Rh-negative supplies. Consideration of patient and donor RH genotypes may avoid unexpected antibodies and improve allocation of rare donations.

Therapeutic plasma exchange for multiorgan dysfunction among critically ill pediatric patients with leukemia: A single-institutional experience.

INTRODUCTION: Therapeutic plasma exchange (TPE) has well-documented applications in the adult population, outlined by the American Society for Apheresis (ASFA) 2019 guidelines. Limited data exist regarding the use of TPE in critically ill pediatric patients; however, these reports rarely include patients with oncological diseases. METHODS: We highlight the use of TPE in the acute management of seven pediatric patients with leukemia treated at Children's Mercy Hospital ICU from 2015 to 2020, including TPE specifications, baseline and interval laboratory evaluation, and outcome measures. Analysis compared those who did (n = 4) and did not (n = 3) survive 30 days post-TPE. RESULTS: Multiorgan failure (MOF) prompted the initiation of TPE in 85.7% of patients, 57.1% of whom were also diagnosed with hemophagocytic lymphohistiocytosis (HLH). Baseline laboratory evaluations prior to initiation of TPE were similar between the two groups. With subsequent TPE treatments, C-reactive protein (CRP) and lactic acid decreased. CRP and lactic acid following the last TPE treatment were significantly higher for those deceased 30 days post-TPE (P = .023 and .031, respectively). No TPE-associated adverse events necessitated discontinuation of TPE treatment. Several surviving patients required chemotherapy dose reductions or alterations. CONCLUSION: Our cohort demonstrated MOF and HLH consistently as indications for TPE, currently ASFA category III indications (optimal role of apheresis is not yet established). Recognition of this treatment modality earlier in the clinical course for critically ill oncological patients may lead to the development of formal protocols that may result in earlier initiation of TPE and improved outcomes.

The invisible pain of women: sexual coercion, psychological distress and somatic symptoms.

INTRODUCTION: Sexual coercion among married women is a widely concealed and a serious public health concern that may impede physical, sexual and psychological health of women. PURPOSE: We aimed to investigate the associations between sexual coercion, psychological distress and somatic symptoms among married women. METHODS: This cross-sectional study was carried out from September 2018 to March 2019 at the International Islamic University, Pakistan. A sample of 200 married women with equal proportion of working and home bound women was selected whilst using non-probability purposive sampling technique from the cities of Rawalpindi and Islamabad (Pakistan). Information about study variables were gathered through Sexual Coercion in Intimate Relationship Scale, Kessler Psychological Distress Scale and Somatic Symptoms Scale. Linear regression analysis, a moderation analysis and a t-test was carried out using SPSS 23. RESULTS: Sexual coercion was positively related to psychological distress and somatic symptoms among married women (p<.001). As a predictor variable, sexual coercion explained a variance of 53% in psychological distress and 35% in somatic symptoms. Additionally, home bound married women were more prone to suffer from sexual coercion, distress of psychological nature and somatic symptoms than married working women (p<.001). DISCUSSION: Overall, women who experience sexual coercion have poorer psychological health. Study findings support the notion that husbands may act coercively to acquire or retain an impersonal sense of control and power more on home bound women. Additionally, working status might be a reason that work will positively contribute to the mental health of working women that needs further exploring.

Relationship between psychological distress, quality of life and resilience among medical and non-medical students.

OBJECTIVE: To explore inherent associations between psychological distress and resilience, and its effect on quality of life among medical and non-medical students. METHODS: The cross-sectional study was conducted from December 2018 to January 2020 after approval from the Department of Psychology Ethics Committee of the International Islamic University, Islamabad, and comprised medical and non-medical students of either gender enrolled with different institutions of higher learning in Rawalpindi and Islamabad. Data were obtained using Brief Resilience Scale, Kessler Psychological Distress Scale and Quality of Life Scale. Data was analysed using SPSS. RESULTS: Of the 300 subjects, 150(50%) each were medical and non medical students. Within both groups, 75(50%) each were males and females. Psychological resilience was a positive predictor of quality of life ( =0.30) whereas psychological distress served as a negative predictor of quality of life ( =-0.54). Medical students were significantly higher on psychological distress (p=0.001), whereas non-medical students were higher on quality of life (p=0.001) and resilience (p=0.001). Female students were higher on quality of life and male students were higher on psychological distress (p=0.002). CONCLUSIONS: Resilience needs to be promoted among students, particularly medical students, as it may help in reduction and adequate management of academic distress levels.

Development and validation of perceived sexual resentment scale for women (PSRS-W).

OBJECTIVE: To develop a valid and reliable scale to asses perceived sexual resentment among married women. METHODS: The cross-sectional validation study was conducted from September 2018 to July 2019 at the International Islamic University, Islamabad, Pakistan, and had three phases. Phase I comprised initial generation/selection of items regarding sexual resentment among married women. Phase II comprised pilot testing of the perceived sexual resentment scale for women items before applying the scale on the actual sample. Phase III determined convergent validity by estimating correlation of the newly developed scale with the Sexual Coercion in Intimate Relationship Scale, and its divergent validity by finding correlation with the Satisfaction with Life Scale. Data was analysed using SPSS 23. RESULTS: The pilot study, done on 20 married women, established the internal consistency of the newly developed scale (α=0.82). Overall, three factors emerged following factor analysis. The scale had 29 items and reliability analysis of the entire scale indicated adequate internal consistency (α=0.93). The convergent validity of the scale and the Sexual Coercion in Intimate Relationship Scale was positive (p<0.001), and the correlation with the Satisfaction with Life Scale was negative (p<0.01), indicating that the newly-developed instrument had a good convergent and divergent validity. CONCLUSIONS: The Perceived Sexual Resentment Scale for Women was found to be a precise and concise 29-item self-reporting tool developed to assess sexual dislikes and displeasures among married women.

Clinical and molecular characterization of novel deletions causing epsilon gamma delta beta thalassemia: Report of two cases.

Epsilon gamma delta beta (εγδß)0 - thalassemia is a very rare disorder that results from large deletions in the ß-globin gene cluster which abolish all regional globin chain gene expression from that allele. Since it is an exceedingly rare cause of neonatal anemia and is not detected by routine newborn screening, it is usually not suspected clinically and commonly undiagnosed or misdiagnosed. In this study, we describe two patients diagnosed in our hospital with (εγδß)0-thalassemia based on the results obtained from DNA microarray analysis of their peripheral blood. The first patient of mixed European descent presented as a neonate with microcytic hemolytic anemia, hyperbilirubinemia, hypoglycemia and hypothermia, and was found to have a 2.2 Mb loss that included the entire ß-globin gene cluster and the locus control region (LCR). The second patient, also of mixed European descent, presented in the neonatal period with anemia, thrombocytopenia and cutaneous extramedullary hematopoiesis, and was found to have a 59 kb loss that included the ß-globin LCR, HBE1, HBG1, and HBG2 genes. Both cases highlight the importance of recognizing the clinical features of (εγδß)0-thalassemia and implementing appropriate testing to clarify the diagnosis and manage the condition.