The Outcome of Congenital Cardiac Surgery in Patients with Down Syndrome: Single-Center Experience.

OBJECTIVES: This study aimed to describe the outcomes of Down syndrome patients who underwent cardiac surgery for congenital heart defects and to develop risk prediction models for in-hospital mortality, recurrent hospital admission, and the need for catheter intervention among a cohort of patients. METHODS: This single-centre retrospective cohort study included consecutive Down syndrome patients who underwent cardiac surgery for congenital heart defects between January 2018 and December 2021. We reviewed the electronic medical records. Two hundred patients fulfilled the eligibility criteria with complete data reporting. The patients' perioperative data and outcomes were recorded. RESULTS: Females constituted 56.5%. Most (78.5%) patients showed accepted recovery. The incidence of all-cause in-hospital mortality was 3.0%. The rates of the need for a second operation, heart failure management, and permanent pacemaker insertion were 3.0%, 2.0%, and 2.5%, respectively. Only 8 (4.0%) patients stayed in the hospital for a long duration after chylothorax or tracheostomy (if intubated more than 2 weeks). The model had an accuracy of 99% and included the intraoperative transesophageal echocardiography (TEE) abnormalities (residual heart lesions) (adjusted odds ratio [AOR]: 26.541, p = 0.033), the duration of mechanical ventilation following the operation (AOR: 1.152, p = 0.009), and the occurrence of postoperative heart block (AOR: 76.447, p = 0.005). CONCLUSION: Surgical treatment of congenital heart defects in Down syndrome patients had good outcomes with accepted recovery (without intra-hospital or during follow-up mortality or morbidity) of 78.5% and a 3% incidence of in-hospital mortality. Though, the occurrence of chylothorax was considerably high, and resulted in a long hospital stay (more than 10 days). Repair of tetralogy of Fallot and coarctation of the aorta were associated with an increased likelihood of catheter intervention following the operation.

Diabetes Distress Among Adults With Type 1 Diabetes Mellitus in Saudi Arabia.

Background Psychological morbidity is clinically important for diabetes patients because it is often associated with worse glycemic outcomes. This study aimed to assess the prevalence of diabetes distress among adult type 1 diabetes mellitus (DM) patients in the Kingdom of Saudi Arabia (KSA). Methodology A descriptive, cross-sectional study was conducted among type 1 DM patients in KSA from 2021 to 2022. An online validated questionnaire was adopted to collect data, including demographic information, medical and social information, and Saudi Arabian Diabetes Distress Scale-17 (SADDS-17) score to assess diabetes distress. Results This study included 356 type 1 DM patients. Most patients were females (74%), with ages ranging between 14 and 62 years. More than half (53%) had a high level of diabetes distress with a mean score of 3.1 ± 1.23. Among those patients, the highest score (up to 60%) was related to regimen-related distress, the lowest score (around 42%) was related to diabetes-related interpersonal distress, and physician-related distress and emotional burden were reported among 55% and 51%, respectively. More than half (56%) of the patients treated with an insulin pen compared to 43% treated with an insulin pump had high diabetes distress (p = 0.049). The level of HbA1c was significantly higher among patients with high diabetic distress (7.93 ± 1.72 vs. 7.55 ± 1.65; p = 0.038). Conclusions Diabetes distress is prevalent among adult type 1 DM patients in KSA. Therefore, we recommend organizing a screening program for early discovery and prompt psychiatric management, incorporating diabetes education and nutrition consultation to improve their quality of life, and engaging patients in their own management to improve their glycemic control.

Toward allogenizing a xenograft: Xenogeneic cardiac scaffolds recellularized with human-induced pluripotent stem cells do not activate human naïve neutrophils.

The limited availability of human donor organs suitable for transplantation has resulted in ever-increasing patient waiting lists globally. Xenotransplantation is considered a potential option, but is yet to reach clinical practice. Although remarkable progress has been made in overcoming immunological rejection, issues with functionality are still to be resolved. Bioengineering approaches have been used to create cardiac tissues with optimized functions. The use of decellularized xenogeneic cardiac tissues seeded with donor-derived cardiac cells may prove to be a viable strategy as supporting structures of the native tissue such as vasculature can be utilized. Here we used sequential perfusion to decellularize adult rat hearts. The acellular scaffolds were reseeded with human endothelial cells, human fibroblasts, human mesenchymal stem cells, and cardiac cells derived from human-induced pluripotent stem cells. The ability of the resultant recellularized rat scaffolds to activate human naïve neutrophils in vitro was investigated to measure xenogeneic recognition. Our results demonstrate that in contrast to cadaveric xenogeneic hearts, acellular and recellularized xenogeneic scaffolds did not activate human naïve neutrophils and suggest that decellularization removes the xenogeneic antigens that lead to human naïve neutrophil activation thus allowing human cells to populate the now "allogenized" xenogeneic scaffolds.

Mass screening for hepatitis B and C viruses in a population of persons with disabilities with and without HIV status in Cameroon.

Hepatitis viral infections are one of major threat to public health worldwide. The vast majority of people infected with viral hepatitis are found in resources limited countries of Africa and Asia. There is a lack of accurate data to better determine the burden of this disease in Cameroon, moreover among vulnerable people. The aim of this study was to estimate the seroprevalence of HBV and HCV viruses among persons with disabilities (PwD) with or without HIV status.

First experience of the use of a generic of plerixafor in peripheral blood stem cell mobilization in multiple myeloma and lymphoma patients.

Mobilization failure in patients is a major therapeutic concern which makes subsequent ASCT impossible. A new growth factor called Plerixafor (Mozobil®) developed by the pharmaceutical industry (Sanofi-aventis, France), is a chemoreceptor antagonist, CXCR4 type, which disrupts the interaction of SDFI and CXCR4, thereby enhancing the effect of G-CSF mobilization and is especially indicated for mobilization failure. Currently, there is a generic of plerixafor developed by the pharmaceutical industry (Hetero Drugs Ltd, India). The brand name of this medicine is Mozifor®. The objective of this study was to evaluate if generic plerixafor has the same efficacy and safety as originator plerixafor when used with G-CSF in the mobilization of PBSCs for autologous ASCT in multiple myeloma (MM) and lymphoma failure patients. The 32 patients received plerixafor were divided in two groups. The first group concerns the 11 consecutive patients prospectively received generic plerixafor (Mozifor®) in the period between January to July 2020. These were compared with a retrospective control cohort (second group n = 21) who had been treated between 2009 and 2019 with originator plerixafor (Mozobil®). For the Mozifor® group, the mean CD34+ was 4.54x106/kg(1.56-6.79), the median time to achieve an absolute neutrophil count >0.5 G/L was 13 days (range: 8-21). The median time to self-sustained platelet count >20 G/L was 15 days (range: 8-24). For the Mozobil® group, the mean CD34+ was 3.1x106/kg (0.56-8.91) (p=0.86), the median time to achieve an absolute neutrophil count >0.5 G/L was 10 days (range 7-23). The median time to self-sustained platelet count >20 G/L was 13 days (range: 7-29). Our study showed that the generic of plerixafor was practically identical to that of the originator (Mozobil®) with no significant difference (p = 0.52). This study demonstrates the safety and feasibility of mobilization PBSC with generic plerixafor in ASCT in MM and lymphoma. Although these outcomes are encouraging, prospective comparison with other traditional auto-HCT regimens used for patients with MM and lymphoma is warranted.

Stent or Shunt, What Could be Better for Children with Duct Dependent Pulmonary Circulation?

BACKGROUND: Systemic to pulmonary shunt (Shunt) is offered for children with duct dependent pulmonary circulation to augment pulmonary flow. Recently patent ductus arteriosus (PDA) stent (Stent) is widely used as an alternative method. We aimed to compare post intervention outcomes in children underwent either procedure. METHODS: Infants under 3 months who had an initial palliation by Shunt or Stent were retrospectively reviewed between 2008 and 2016, then followed till the second intervention or 1 year whichever earlier. RESULTS: 187 patients (110 Shunt and 77 Stent) were included. Initial weight and pulmonary artery (PA) branches size were similar between the groups. Shunt patients had more shock preoperatively and required more emergency intervention. Stent group showed less ICU stay 4 (1-8) vs 13 (7-23) days, p < 0.0001 and less positive pressure ventilation days 1 (0-2) vs 5.5 (3-11), p < 0.0001. However, Stent group had more symptomatic arterial and deep venous thromboses. In Stent patients the branch PAs growth was better and more homogeneous. At follow-up, no difference between groups regarding cumulative readmission days to hospital, hemoglobin levels and the weight percentile for age. Mortality was not different with a tendency to be higher in the Shunt group (13%) compared to the Stent group (5%), p 0.1. CONCLUSIONS: The implantation of PDA stent in patients with duct dependent pulmonary circulation results in a smoother ICU course and a shorter hospital stay, with higher risk of vascular injury. Shunt and Stent procedures have a good outcome for PA growth, somatic growth and survival.

The efficacy and safety of percutaneous balloon angioplasty for aortic coarctation in children. Acute and mid-term results in a single center experience.

OBJECTIVES: To assess the efficacy and safety of balloon angioplasty (BAP) procedure for treatment of coarctation of the aorta (CoA) in children. Methods: A retrospective study included 27 consecutive children, underwent BAP for either native-CoA (Na-CoA) or recoarctation (Re-CoA). Medical records, echocardiographic findings, angiographic and hemodynamic data were collected from the hospital database. Follow­up was scheduled at 1, 3, 6, 12 months after the procedure. The study took place over a period of 4.5 years, from April 2014 to January 2019, in Madinah Cardiac Center, Madinah, Northwest region, Saudi Arabia. RESULTS: The mean age of patients was 11.86±8.96 months. Seven children had Na-CoA and 20 children had Re-CoA. The success rate of the procedure was achieved in 23 children (85%), as BAP reduced the mean systolic pressure gradient across the CoA (Na-CoA: from 45.28± 18.3 to 9.8± 6.57 mm Hg, p=0.0009), and in Re-CoA groups (from 42.48±16.7 to 10.9±8.5 mm Hg, p less than 0.0001). In mid-term follow-up, the need for re-intervention occurred in 8 children of the cohort (3 children [42.8%] from the Na-CoA group, and 5 children [25%] from the Re-CoA group).  Conclusions: Balloon angioplasty is considered a safe procedure for the management of CoA, but its efficacy remains questionable especially for young infants with Na-CoA type. However, it is a reliable option for managing Re-CoA children, with a lower rate of future re-intervention.

Transcatheter device closure of ventricular septal defects in children: a retrospective study at a single cardiac center.

BACKGROUND: Ventricular septal defect (VSD) is the most common congenital heart disease in the pediatric population. Nowadays, trans-catheter closure is considered a feasible method of therapy for most muscular and some perimembranous types of VSDs. OBJECTIVE: Assess the safety, efficacy and outcome of percutaneous transcatheter closure of VSDs in children. DESIGN: Retrospective, single center study. SETTING: Madinah Cardiac Center, Madinah, Saudi Arabia. PATIENTS AND METHODS: The study included all consecutive children who underwent transcatheter closure of isolated VSD during the period from December 2014 to January 2019. The data were collected from hospital database medical records. Transthoracic echocardiography (TTE) and an electrocardiogram (ECG) were done before and after the procedure in all the patients. The device was implanted by the retrograde or antegrade approach. All patients were subjected to follow-up evaluation at 1, 3, 6, 12 months, and annually thereafter with TTE and ECG. MAIN OUTCOME MEASURES: Procedure success rate, clinical follow-up, TTE. SAMPLE SIZE: 70 children. RESULTS: The mean (standard deviation) age of patients was 10.2 (4.1) years (range: 2-18 years), and their mean body weight was 30.9 (13.9) kg (range: 7.0-57.7 kg). Forty-eight (68.6%) children had muscular VSD (mVSD), and 22 (31.4%) children had perimembranous VSD (pmVSD). The majority of defects were closed via the retrograde approach using the Amplatzer muscular occluder device. At 24 hours after the procedure, the success rate was 90%. Only four (5.7%) cases had major adverse events including complete atrioventricular block, hemolysis, and thrombus formation. CONCLUSION: Transcatheter closure is a safe and feasible procedure in VSDs of various morphologies, with a low adverse event rate. LIMITATIONS: Retrospective design, single-center study, absence of control group. CONFLICT OF INTEREST: None.

Therapeutic efficacy of a newly synthesized benzimidazole compound BTP-OH against murine schistosomiasis mansoni.

Because of the increasingly emerging praziquantel resistance, there is a crucial need to develop new anti-schistosomal agents. This work was conducted to assess the therapeutic efficacy of a new benzimidazole compound (BTP-OH) in mice experimentally infected with Schistosoma mansoni. A total of 40 Swiss albino female mice were divided into an infected untreated group and three infected treated groups (using praziquantel and BTP-OH). The compound activity was evaluated through parasitological, histopathological and scanning electron microscopy studies. Praziquantel and BTP-OH at both doses significantly reduced male (75%, 42.67% and 61.08%, respectively), female (71.45%, 48.94% and 68.13%, respectively) and total worm burden (75.21%, 42.42% and 62.28%, respectively), as well as tissue egg load in the liver (71.22%, 42.12% and 66.04%, respectively). In oogram, praziquantel significantly increased the percentage of dead eggs (65.89%), while BTP-OH significantly reduced the percentage of immature eggs (30.43% and 19.64%). BTP-OH significantly diminished granuloma count (33.87% and 44.77%) and diameter (39.23% and 49.40%), and caused ultrastructural changes in the tegument of adult schistosomes. This study provides evidence for the schistosomicidal efficacy of BTP-OH. However, future studies are needed to elucidate the full mechanisms of action and effects of BTP-OH on other human schistosomes.

Effect of a newly synthesized quinoline-based compound (PPQ-8) on murine schistosomiasis mansoni.

Schistosomiasis represents a public health problem and praziquantel is the only drug used for treatment of all forms of the disease. Thus, the development of new anti-schistosomal agents is of utmost importance to increase the effectiveness, reduce side effects and delay the emergence of resistance. The present study was conducted to report the therapeutic efficacy of PPQ-8, a new synthetic quinoline-based compound against Schistosoma mansoni. Mice were treated with PPQ-8 at day 49 post infection using two treatment regimens (20 and 40 mg/kg). Significant reductions were recorded in hepatic (62.9% and 83.6%) and intestinal tissue egg load (57.4% and 73.5%), granuloma count (75.4% and 89.1%) and diameter (26.2% and 47.3%), in response to the drug regimens, respectively. In addition, both treatment regimens induced significant decrease in liver (23.3% and 32.8%) and spleen (37.5% and 45.3%) indices. Also, there were significant reductions in mature ova, total worm and female count, which were more prominent with the higher dose. The reduction in the level of nitric oxide in the liver by both therapeutic regimens to 22.5% and 47.2% indicates the anti-oxidant activity of PPQ-8. Bright field microscopic examination of worms recovered from infected and PPQ-8-treated mice showed nearly empty intestinal caeca with no observable changes in the tegument. Our findings hold promise for the development of a novel anti-schistosomal drug using PPQ-8, but further in vitro and in vivo studies are needed to elucidate the possible mechanism/s of action and to study the effect of PPQ-8 on other human schistosomes.

Inflammatory myofibroblastic tumor of heart in a 4-month-old infant.

Primary tumors of heart are rare in infants. We report a case of inflammatory myofibroblastic tumor in a 4-month-old baby girl who presented with a massive pericardial effusion. She underwent complete surgical excision of the tumor. This is the first reported case from the Middle Eastern region. The clinical behavior and management of these rare infantile cardiac tumors are discussed briefly in this report.

Persistent fifth aortic arch diagnosed by echocardiography and confirmed by angiography: Case report and literature review.

Persistent fifth aortic arch is a rare congenital anomaly that can be discovered incidentally or at postmortem exam. It can be associated with major congenital heart malformations involving the systemic or the pulmonary circuits. It usually has no clinical significance but can be either, beneficial as in systemic outflow tract obstructions or cause hemodynamic compromise when associated with a significant left to right shunt. We report an infant with persistent fifth aortic arch associated with Shone's complex diagnosed accurately by transthoracic echocardiography and confirmed by cardiac catheterization and computed tomography.

Generation of 11 fs pulses by using hollow-core gas-filled fibers at a 100 kHz repetition rate.

Using self-phase modulation in a hollow-core fiber filled with xenon, we were able to produce 2.3 microJ laser pulses with a duration of 10.9 fs at a repetition rate of up to 100 kHz. We started with 45 fs, 4.4 microJ, 800 nm pulses generated by a Coherent RegA Ti:sapphire regenerative amplifier system, then spectrally broadened the 30 nm bandwidth to more than 100 nm. Dispersion compensation was achieved with two pairs of chirped mirrors. This is believed to be the first time this type of compression was achieved at a repetition rate as high as 100 kHz. This brings the advantages of few-cycle laser pulses to experiments that require high-repetition-rate, low-energy laser systems, for example, coincidence experiments.

Tissue culture based screening for selection of high biomass and phenolic producing clonal lines of lavender using pseudomonas and azetidine-2-carboxylate.

Lavender is a good source of essential oils and phenolic metabolites for food, medicine, and cosmetic applications. Due to cross-pollination, lavender has substantial plant to plant variation and therefore a high degree of genetic inconsistency in the level of phytochemicals produced for diverse applications. Tissue culture methods, using benzyladenine-induced shoot organogenesis, were used to isolate clonal lines originating from individual heterozygous seeds among a heterogeneous seed population to exploit the genetic heterogeneity. Subsequently, in a two-step method, clonal shoots of each clonal line were evaluated for the ability to tolerate Pseudomonas inoculation and various levels (0-200 microM) of proline analogue, azetidine-2-carboxylate. On the basis of tolerance to Pseudomonas and proline analogue treatments, multiple shoot forming ability, biomass, rosmarinic acid, total phenolics, and total chlorophyll, 20 separate clonal lines were screened and isolated for further vegetative propagation and evaluation. From the clonal lines isolated, lines LH-14, LH-15, LH-17, and LH-11 showed the best potential for overexpression of phenolic metabolites in response to Pseudomonas and proline analogue.

A case of unexplained oxygen desaturation during laparoscopic cholecystectomy.

The authors report a case of sudden desaturation upon creating pneumoperitoneum during laparoscopic cholecystectomy, which was most probably caused by endobronchial intubation. The literature of the previously reported cases is reviewed. Differential diagnosis and different mechanisms are discussed. Recommendations are suggested to avoid this problem.

Hypercaloric glycerol in injured patients.

BACKGROUND: This study investigates the effects of glycerol as a fuel source in the hypermetabolic patient (after injury). METHODS: Twenty-two patients were studied after multiple trauma and randomly assigned to either glucose or glycerol as the carbohydrate source (220 gm glycerol or 320 gm dextrose) during a lipid-based system of parenteral nutrition. In the immediate postoperative period, measurements were made of nitrogen balance, substrates, energy expenditure, insulin, glucagon, and liver function tests. RESULTS: In the glycerol group glucose concentrations in plasma were significantly lower, whereas glycerol levels increased nearly twentyfold. Insulin and glucagon levels increased in both groups; however, the rise in insulin level was greater in the glucose group, whereas glucagon increased in both groups to a similar degree. Nitrogen balance was restored to equilibrium in the glycerol group while remaining negative in the glucose group. No abnormalities in liver function test results or differences in serum albumin levels were noted in either group. A 12% thermic effect was noted in the glucose group but not in the glycerol group. CONCLUSIONS: Glycerol seems to be a viable fuel source in the traumatized patient, being associated with nitrogen retention and minimal thermal effect. A marked rise in plasma levels of glycerol does occur, but this does not appear to have any associated toxicity.