Health-Related Quality of Life in Sudanese Children with Nephrotic Syndrome: A Comparative Cross-Sectional Study.

Background: Nephrotic syndrome (NS) is an essential chronic disease in children that has a major impact on a child's health-related quality of life (HRQoL). This study aimed to evaluate the HRQoL of Sudanese children with NS and clinical parameters that can influence their HRQoL. Methods: This study was a descriptive cross-sectional of children with NS conducted in Khartoum state hospitals. A standardized PedsQLTM 4.0 Scale Score evaluated the HRQoL of the participants. Patients' socio-demographics, clinical data, and disease complications were collected using a data collection sheet. This study assessed the HRQoL of children with NS and compared it with apparent age and sex-matched to three groups (healthy children, children with chronic diseases, and kidney-transplanted children). Results: 80 children with NS were recruited from April to August 2021. Children over eight years old represented (63.8%) of the study subjects. The total mean HRQoL scores of nephrotic children were significantly lower than those of healthy children (78.46 ± 24.01) (p = 0.001) and those with other chronic diseases (78.45 ± 24.01) (p= 0.006); however, it was not significantly different from those with kidney transplantation. Socio-demographics did not significantly affect the total mean HRQoL scores of children with NS. Clinical parameters such as the duration of illness, "less than one year" (p= 0.006), and the minimum change nephropathy histopathology (p= 0.035) significantly lowered the total mean HRQoL scores of NS children. Regression analysis further confirmed that edema, proteinuria, and hospital admission had a high impact on the total mean HRQoL. Conclusion: The total mean HRQoL scores of children with NS were low and significantly lower than healthy children. Parameters such as the patient's socio-demographics and phenotype of NS had no significant effect on the total mean HRQoL scores of children with NS. However, other clinical parameters significantly lowered their total mean HRQoL scores.

Heart surgery in a pediatric patient with congenital heart disease and hemophilia B: A case report from Sudan.

Key Clinical Message: A multidisciplinary team approach, careful hemostasis, and factor replacement therapy are important in the perioperative management of hemophiliac patients undergoing pediatric cardiac surgery. Abstract: The combination of congenital heart diseases (CHD) and hemophilia is rare; furthermore, heart surgery and perioperative management of such cases is challenging. This report illustrates the challenges of pediatric cardiac surgery in an infant with both hemophilia B and CHD. Multidisciplinary team approach, careful hemostasis, and factor replacement therapy were key to success without hemorrhagic complications before, during and after surgery.

Left anterior descending artery aneurysm in a young patient with familial retinal arterial macroaneurysm: A case report.

Purpose: Familial retinal arterial macroaneurysm (FRAM) is a rare genetic disorder caused by a gene mutation in the insulin-like growth factor binding protein 7 (IGFBP7). Observations: We report a 30-year-old male with FRAM and IGFBP7 gene mutation who presented with an acute coronary syndrome (ACS). Invasive coronary angiography revealed a large aneurysm at the proximal part of the left anterior descending (LAD) artery. Conclusions and Importance: Few cases with systemic vascular involvement in patients with FRAM have been described before; however, our case represents the first documentation of a LAD artery aneurysm in a patient with FRAM and IGFBP7 gene mutation.

The Hidden Cause of Maculopapular Rash in Interleukin-12 Deficiency.

Interleukin-12 RB1 (IL12RB1) deficiency falls under the Mendelian susceptibility to mycobacterial disease. It is a rare genetic disease with autosomal recessive inheritance. It is characterized by recurrent infections with otherwise weak bacteria, such as mycobacteria and Salmonella. Often, when encountering a maculopapular eruption, a drug-related cause comes to mind. However, we report a case of IL12RB1 deficiency presenting with a maculopapular eruption, proven by a skin biopsy to be leukocytoclastic vasculitis. The patient was given antibiotics, which improved her skin lesions. Vasculitis should be considered in the differential diagnosis in patients with IL12RB1 deficiency presenting with a cutaneous eruption.

Correlation of 25-Hydroxyvitamin D and Serum Lipid Levels Among Patients With Type 2 Diabetes.

Introduction Type 2 diabetes mellitus (T2DM) is a well-known health care problem. The is a growing interest in the role of vitamin D in metabolism including glucose and lipid metabolism. This study aims to investigate the possible association between 25-hydroxyvitamin D levels and serum lipid levels among patients with T2DM. Method A cross-sectional study was done at the King Faisal University Health Care Center in the Eastern Region of Saudi Arabia. Ethical approval was obtained from the Ethics and Research Committee at the College of Medicine, King Faisal University. We obtained the clinical and laboratory data of patients with T2DM by searching the electronic files of patients attending the center during the period between 2014 and 2021. Data collected included age, gender, nationality, vitamin D levels, HbA1c, and lipid levels. The chi-square and independent sample t-tests were applied when appropriate, for comparisons between groups to determine significance. A P-value of less than 0.05 was considered statistically significant. Result The study included 191 diabetic patients, 137 (71.7%) from Saudi Arabia, and 54 (28.3%) from other countries. Patient ages ranged from 21 to 100 years with a mean age of 56.2 ± 11.8 years. Cholesterol levels were observed to be high among 61 (32.3%) patients. Considering vitamin D, the average level among male patients was 26.526 ng/ml compared to 26 ng/ml% among females (P = 0.742).  Conclusion Further long-term and more comprehensive randomized controlled trials are needed to make a firmer conclusion and stronger evidence on this beneficial role of vitamin D treatment on T2DM.

Impact of Self-Esteem and Self-Perceived Body Image on the Acceptance of Cosmetic Surgery.

Backboard Cosmetic surgery is the preservation, rebuilding, or improvement of the physical appearance of an individual through surgical and non-surgical methods. In the last few years, an increase in the number of cosmetic procedures was noticed worldwide. This increase suggests due to multifactorial changes in people's attitudes towards cosmetic surgery and concern about their physical appearance. This study aims to assess the impact of self-esteem and self-perceived body image on the acceptance of cosmetic surgery and other related factors in the Eastern province of Saudi Arabia. Material and methods This was a cross-sectional study carried out in the Eastern region of Saudi Arabia. The study was conducted between May and August 2021. A self-administrated questionnaire was distributed to all the participants who are attending plastic surgery clinics and online through social media. Three valid and reliable scales were used [Acceptance of Cosmetic Surgery Scale (ACSS), Body Appreciation Scale (BAS), Rosenberg Self-Esteem Scale (RSE)] to assess the relationship between these variables and other factors. The data were analyzed by using two-tailed tests. P-value less than 0.05 was statistically significant. Correlation analysis was done by using the Pearson correlation coefficient (r). Results A total of 1008 participants were included in the study with a response rate of 67%. Participant's ages ranged from 18 to 54 years with a mean age of 34.7 ± 11.2 years old. The study participants showed an average level of acceptance with a mean score % of 55.4% comparing to body appreciation; it was 74.2% higher with a more than average level of self-esteem, 24.7 out of 40 points for self-esteem with a mean score of 61.8%. Participants with a history of cosmetic surgery had significantly higher acceptance score than who did not (mean score of 72.6 compared to 57.1; P=0.001). Male participants had better body appreciation than females (mean score of 50.2 vs. 47.6, respectively; P=0.013). A weak positive correlation with no significance was found between participants' self-esteem and their acceptance of cosmetic surgery. Conclusion A better understanding of the acceptance of cosmetic surgery from a different cultural perspective and other related factors including social, psychological, and self-esteem are crucial for the plastic surgeon to ensure patient satisfaction.

Neonatal sepsis in Sana'a city, Yemen: a predominance of Burkholderia cepacia.

BACKGROUND: Neonatal sepsis is a global concern with increasing morbidity and mortality. The burden of neonatal sepsis is highest in developing countries, especially in those lacking proper surveillance systems. The causative pathogens and their drug-resistance levels vary between countries with emergence of multidrug resistance organisms. Thus, accurate records on the recent trends of organisms causing neonatal sepsis will provide vital information for appropriate intervention. We aimed to investigate neonatal sepsis, identify its associated factors and causative pathogens and to assess the antibiotic susceptibility patterns in Sana'a city, Yemen. METHODS: A cross-sectional study was conducted on neonates admitted to intensive care units of six hospitals in Sana'a city, Yemen, in the period from January 15, to March 30, 2020. Natal and prenatal medical data were collected using well-structured questionnaire. Neonates were subjected to sepsis work-up including blood culture, complete blood count and C-reactive protein. Organisms were identified by Gram staining and analyzed by the VITEK II system for bacterial bio-typing and antibiotic susceptibility testing. FINDINGS: Of the 199-neonates with suspected neonatal sepsis, 154 (77.38%) had culture-proven sepsis. Early-onset neonatal sepsis (EOS) was higher (50.25%; 100/199) than late-onset neonatal sepsis (LOS) (27.13%; 54/199). Multivariable analysis identified vaginal delivery as an independent risk factor for neonatal sepsis p = 0.005. Majority of isolated bacteria (74.39%) were gram-negative with Burkholderia cepacia (39%) and Klebsiella oxytoca (13%) being the most common pathogens of EOS and LOS. The most common gram-positive pathogens were Staphylococcus haemolyticus (9.1%) and Staphylococcus epidermidis (7.1%). B. cepacia showed multidrug resistance except for cefepime. All Klebsiella species isolates (100%) and most Pantoea species (93%) were ESBL and carbapenemase positive. All Escherichia coli and Acinetobacter baumannii isolates were ESBL positive. A significant number of gram-positive bacteria showed resistance to vancomycin. CONCLUSION: The study findings show a high proportion of neonatal sepsis among neonates admitted to hospitals in Sana'a city with antibiotic-resistant B. cepacia being the single most common pathogen causing EOS and LOS. Findings also emphasize the emerging threat of multidrug-resistant bacteria in neonatal units and will help develop evidence-based management of neonatal sepsis in Yemen.

25(OH)D Serum Level in Non-Diabetic and Type II Diabetic Patients: A Cross-Sectional Study.

Background Diabetes mellitus is a major disease worldwide. In Saudi Arabia, it is considered to be the most common disease in the country. Diabetes mellitus has been also found to be associated with 25(OH)D (vitamin D) deficiency. In Saudi Arabia, sunlight is considered a major source for vitamin D. Saudi Arabia is popular for sunny weather most of the year, in which people can get vitamin D from the sun. However, vitamin D deficiency is common in Saudi Arabia, and its deficiency can increase blood glucose levels. We conducted a study to determine the reason for vitamin D deficiency in Saudi Arabia and to assess the relationship of diabetes mellitus with vitamin D. Aim of the work  This study is aimed to assess the incidence of vitamin D deficiency in non-diabetic and type II diabetic patients in the King Faisal University (KFU) Health Center in the Al-Ahsa region. Methods Our study is a cross-sectional study that was carried out at the KFU Health Center in Saudi Arabia. Ethical approval was obtained from the Ethics and Research Committee at the College of Medicine at King Faisal University. The study period was from January 2016 to April 2016. We collected each patient's vitamin D serum level, glycosylated hemoglobin (HbA1c), and fasting blood glucose at the same time for each patient's particular visit to the hospital. Data were analyzed using the Statistical Package for Social Sciences (SPSS) (IBM SPSS Statistics, Armonk, NY).  Results Our results showed that 89.53% of the patients had a vitamin D level below the normal range. There was a higher incidence of vitamin D deficiency in females (81.67%) than in males (65.27%) (p-value = 0.001). The incidence of vitamin D deficiency was greater in Saudi (82.19%) than non-Saudi patients (68.40%) (p-value = 0.001), as well as in diabetics (89.68%) than non-diabetics (76.12%) patients (p-value = 0.001). Within each group, the incidence of vitamin D deficiency was higher in females than in males. The incidence of vitamin D deficiency was highest in the age group of 21 to 40 years old (86.19%) and lowest in the age group of one to 20 years old (66.1%). The results showed an inverse relationship between the vitamin D level and both fasting blood glucose and HbA1c (independent sample t-test) were used for correlation. The mean fasting glucose was higher in the deficiency group (165.55) as compared to the insufficiency group (118.67). Also, the mean HbA1c was higher in the deficiency group (8.06) as compared to the insufficiency group (7.23) (p-value = 0.030).  Conclusions There was a high incidence of vitamin D deficiency among KFU Health Center patients. The vitamin D level was inversely proportional to the level of fasting glucose and HbA1c. There is an evident role of vitamin D deficiency on glucose tolerance in diabetic patients.

Coexistence of diverse heavy metal pollution magnitudes: Health risk assessment of affected cattle and human population in some rural regions, Qena, Egypt.

OBJECTIVE: The purpose of this study was to measure the mean concentrations of heavy metals including aluminum (Al), arsenic, nickel (Ni), mercury, lead (Pb), and cadmium (Cd) and to assess the health hazards due to the exposure of cattle/human population to a distinct or the mixture of heavy metals through various sources. MATERIALS AND METHODS: A total of 180 samples including water sources, animal feed, and raw cows' milk from rural regions in Qena, Egypt, were examined using the inductively coupled plasma emission spectrometer (ICP; iCAP 6200). RESULTS: The data highlighted heavy metal pollution with variable concentrations among most of the investigated regions. All concentrations of Al, Ni, and Cd detected in the feeding stuff showed a strong correlation to their respective levels in milk rather than those detected in water (R 2= 0.072 vs. 0.039, 0.13 vs. 0.10, and 0.46 vs. 0.014, respectively) (p < 0.05). Anisocytosis and poikilocytosis with a tendency to rouleaux formation were evident, and basophilic stippling was a pathognomic indicator for heavy metal toxicity, especially Pb. Leukopenia and macrocytic anemia were shown in 50% and 65% of examined cattle, respectively. The target hazard quotients values were more than one (>1) for all heavy metals from water intake for both children and adults and Al and Cd in milk for children, and the hazard index values were indicated higher for noncarcinogenic health hazards. The target cancer risk values predispose people in the surveyed villages to higher cancerous risks due to exposures to the mixture of heavy metal through the consumption of water and milk. CONCLUSION: The bioaccumulation and transmission of heavy metal mixtures from water sources and feeding material have detrimental influences on milk pollution and cattle health which seem to be a serious issue affecting public health in those rural communities.

T- and B-cell immunosuppressive activity of novel α-santonin analogs with humoral and cellular immune response in Balb/c mice.

In continuation of our endeavours to synthesize immunosuppressive agents from α-santonin, we report herein the design and synthesis of a new series of α-santonin derived O-aryl/aliphatic ether, ester and amide analogs and the evaluation of their immunosuppressive activities. The in vitro studies led to several analogs with significant immunosuppressive effects by inhibiting ConA and LPS stimulated T- and B-cell proliferation in a dose dependent manner. The more significant compounds 4d, 4e, 4f, 4h, 6a and 6b displayed potent inhibitory activity on the mitogen-induced T- and B-cell proliferation in comparison to α-santonin 1. Compound 4e displayed stupendous in vitro immunosuppressive effects with ∼80% suppression of B and ∼75% suppression of T lymphocyte proliferation, respectively. The in vivo investigation on BALB/c mice revealed that non-cytotoxic compound 4e suppresses both humoral and cellular immunity.

Synthesis of α-santonin derived acetyl santonous acid triazole derivatives and their bioevaluation for T and B-cell proliferation.

A new series of α-santonin derived acetyl santonous acid 1,2,3-triazole derivatives were synthesised using Huisgen 1,3-dipolar cyclo-addition reaction (click chemistry approach) and evaluated for their in vitro inhibition activity on concanavalin A (ConA) induced T cell proliferation and lipopolysaccharide (LPS) induced B cell proliferation. Among the synthesised series, compounds 2-10 and 19 exhibited significant inhibition against ConA and LPS stimulated T-cell and B-cell proliferation in a dose dependent manner. More significantly compounds 4, 9-10 and 19 exhibited potent inhibition activity with remarkably lower cytotoxicity on the mitogen-induced T cell and B cell proliferation at 1 µM concentration. The compound 6 displayed potent immunosuppressive effects with ∼89% against LPS induced B-cell and ∼83% against ConA stimulated T-cell proliferation at 100 µM concentration without cytotoxicity. Compound 10 was more selective against B cell proliferation and exhibited 81% and 69% suppression at 100 and 1 µM concentration respectively. The present study led to the identification of several santonin analogs with reduced cytotoxicity and strong inhibition activity against the cell proliferation induced by the mitogens.

Disruption of cellular homeostasis induces organelle stress and triggers apoptosis like cell-death pathways in malaria parasite.

A regulated protein turnover machinery in the cell is essential for effective cellular homeostasis; any interference with this system induces cellular stress and alters the normal functioning of proteins important for cell survival. In this study, we show that persistent cellular stress and organelle dysfunction because of disruption of cellular homeostasis in human malaria parasite Plasmodium falciparum, leads to apoptosis-like cell death. Quantitative global proteomic analysis of the stressed parasites before onset of cell death, showed upregulation of a number of proteins involved in cellular homeostasis; protein network analyses identified upregulated metabolic pathways that may be associated with stress tolerance and pro-survival mechanism. However, persistent stress on parasites cause structural abnormalities in endoplasmic reticulum and mitochondria, subsequently a cascade of reactions are initiated in parasites including rise in cytosolic calcium levels, loss of mitochondrial membrane potential and activation of VAD-FMK-binding proteases. We further show that activation of VAD-FMK-binding proteases in the parasites leads to degradation of phylogenetically conserved protein, TSN (Tudor staphylococcal nuclease), a known target of metacaspases, as well as degradation of other components of spliceosomal complex. Loss of spliceosomal machinery impairs the mRNA splicing, leading to accumulation of unprocessed RNAs in the parasite and thus dysregulate vital cellular functions, which in turn leads to execution of apoptosis-like cell death. Our results establish one of the possible mechanisms of instigation of cell death by organelle stress in Plasmodium.

Plasmodium falciparum signal recognition particle components and anti-parasitic effect of ivermectin in blocking nucleo-cytoplasmic shuttling of SRP.

Signal recognition particle (SRP) is a ubiquitous ribonucleoprotein complex that targets proteins to endoplasmic reticulum (ER) in eukaryotes. Here we report that Plasmodium falciparum SRP is composed of six polypeptides; SRP9, SRP14, SRP19, SRP54, SRP68 and SRP72 and a 303nt long SRP RNA. We generated four transgenic parasite lines expressing SRP-GFP chimeric proteins and co-localization studies showed the nucleo-cytoplasmic localization for these proteins. The evaluation of the effect of known SRP and nuclear import/export inhibitors on P. falciparum revealed that ivermectin, an inhibitor of importin α/ß mediated nuclear import inhibited the nuclear import of PfSRP polypeptides at submicromolar concentration, thereby killing the parasites. These findings provide insights into dynamic structure of P. falciparum SRP and also raise the possibility that ivermectin could be used in combination with other antimalarial agents to control the disease.

Disruption of a mitochondrial protease machinery in Plasmodium falciparum is an intrinsic signal for parasite cell death.

The ATP-dependent ClpQY protease system in Plasmodium falciparum is a prokaryotic machinery in the parasite. In the present study, we have identified the complete ClpQY system in P. falciparum and elucidated its functional importance in survival and growth of asexual stage parasites. We characterized the interaction of P. falciparum ClpQ protease (PfClpQ) and PfClpY ATPase components, and showed that a short stretch of residues at the C terminus of PfClpY has an important role in this interaction; a synthetic peptide corresponding to this region antagonizes this interaction and interferes with the functioning of this machinery in the parasite. Disruption of ClpQY function by this peptide caused hindrance in the parasite growth and maturation of asexual stages of parasites. Detailed analyses of cellular effects in these parasites showed features of apoptosis-like cell death. The peptide-treated parasites showed mitochondrial dysfunction and loss of mitochondrial membrane potential. Dysfunctioning of mitochondria initiated a cascade of reactions in parasites, including activation of VAD-FMK-binding proteases and nucleases, which resulted in apoptosis-like cell death. These results show functional importance of mitochondrial proteases in the parasite and involvement of mitochondria in programmed cell death in the malaria parasites.

Molecular diversity of the plasmid genotypes among Rhizobium gene pools of sesbanias from different habitats of a semi-arid region (Delhi).

Plasmid genotypes of root nodulating rhizobial isolates of Sesbania, sampled from six ecologically distinct habitats, were characterized. Plasmid profile analysis revealed nine different plasmid types having molecular masses ranging from 30 to 300 MDa, distributed among six profile types that grouped the isolates into six plasmid classes. The six plasmid profiles were diverged from each other and lack many common plasmid types among them. Variation in number and types of symbiotic (Sym) plasmid was assessed by hybridization of plasmid profiles with sym gene probes. Relatedness among different plasmid types was assessed by hybridization of total DNAs as well as plasmid profiles of different isolates with labelled intact plasmid. Plasticity of plasmid genotype and possible recombination between different plasmid types is suggested from the results obtained. Structural diversity among sym plasmids was assessed by PCR amplified product profiles using primer corresponding to the reiterated nif promoter consensus element (NPC-PCR). A total of 26 NPC-PCR profile types were recognized. Genetic diversity among sym plasmids of isolates belonging to the same plasmid class and having similar sym plasmid suggested recombinations and rearrangements of sequences within the sym plasmids. Cluster analysis based upon similarity among profile types sorted the isolates across the ecological gradient. We suggest that habitat heterogeneity and plasticity of plasmid genotype together contribute for the generation of genetic diversity leading to strainal differentiation in rhizobia.