Childhood Steroid Sensitive Nephrotic Syndrome: Characteristics and Predictors of Relapses; A study at a Single Center in Khartoum

Background: Childhood steroid-sensitive nephrotic syndrome (SSNS) usually has a favorable outcome in spite of its relapsing course. The objective of the authors was to study the demographic and clinical characteristics, outcome and risk factors for relapses in children with SSNS at a single center in Khartoum, Sudan. Material and Methods: In this cross-sectional, facility-based study, the authors retrospectively reviewed all the records of children with SSNS, followed at the Pediatric Renal Unit, Soba University Hospital, Khartoum between 2001 and 2014. SSRNS was defined as the remission of proteinuria within 4–6 weeks of corticosteroids. Relapse is therecurrence of proteinuria after remission; frequent if ≥ 2 within initial six months or ≥ 4 within one year, and steroid dependence if 2 during therapy or within 14 days after stopping it. Results: 330 children (males 220; 66.7%) with SSNS were studied with a mean age of 5.2 ± 3.5 years of whom 42.4% aged 1–5 years. At the presentation, hypertension was detected in 31.8% and hematuria in 19.1%. Serum cholesterol was elevated in all patients (mean 347.34 ± 117.87 mg/dl) and serum creatinine in 7.27% (mean 1.4 ± 0.35 mg/dl). Renal histology showed mesangioproliferative glomerulonephritis (MesPGN) in 57.5%, minimal change disease (MCD) in 35.5%, and focal segmental glomerulosclerosis (FSGS) and IgM nephropathy in 3.5% each. During the course of the illness, 10.3% achieved long-term remission, 89.7% relapsed— of whom 52.3% had frequent relapsing/steroid-dependent (FR/SD) course and 37.7% had infrequent relapses. Risk of frequent relapses were age of onset and low/moderate socioeconomic status (P = 0.015 and 0.019, respectively). Infectionswere recorded in 71.8%, but not significantly associated with the risk of frequent relapses (P > 0.05). Conclusions: The majority children with SSNS at this single center in Khartoum had a relapsing course with the majority being FR or SD. Predictors of frequent relapses were young age at onset and low socioeconomic status

Nutritional and hematological status of Sudanese women of childbearing age with steady-state sickle cell anemia

We sought to investigate the nutritional and hematological status of Sudanese women of childbearing age with sickle cell anemia (SCA). Anthropometry and hematology were used to assess nutritional status and health and disease conditions, respectively. Women with steady-state (HbSS, n = 39; age = 19.0±2.7) and without (HbAA, n = 36; age, 19.8±2.7) SCA were recruited during a routine visit to the Hematology Clinic, Ibn-Auf Teaching Hospital, Khartoum, Sudan. The two groups of women lived in similar environmental conditions and ate similar diets three times a day. However, despite taking regular meals, the women with sickle anemia were thinner and lighter ( 0.050). The low anthropometric (height, weight, and body mass index) and abnormal hematological values in the women with SCA in steady-state reflect sustained nutritional insults inflected by the disease and poverty. Tailored nutritional counseling/advice must be an integral part of managing patients with SCA. Such advice is particularly vital for women of childbearing age because of the adverse effects of prepregnancy nutritional deficiency on outcomes

Adverse pregnancy outcomes in sickle cell trait: a prospective cohort study evaluating clinical and haematological parameters in postpartum mothers and newborns

Background: Sickle cell trait (SCT) is a congenital condition caused by the inheritance of a single allele of the abnormal haemoglobin beta gene, HbS. Carriers of SCT are generally asymptomatic, and they do not manifest the clinical and haematological abnormalities of sickle cell anaemia (SCA). However, there is evidence that they display some symptoms in stressful situations. Pregnancy is a stressful physiological event, and it is not clear if SCT adversely affects pregnancy outcomes, particularly in those from developing countries where people regularly suffer from nutritional insufficiency. Objective: This study aims to investigate pregnancy outcomes in Sudanese women with SCT. Subjects and methods: Pregnant women with (HbAS, n=34) and without (HbAA, n=60) SCT were recruited during their first trimester at El Obeid Hospital, Kordofan, Western Sudan. Following appropriate ethical approval and informed consent from the participants, detailed anthropometric, clinical, haematological, obstetric, and birth outcome data were registered. In addition, blood samples were collected at enrolment and at delivery. Results: At enrolment in the first trimester, the SCT group did not manifest SCA symptoms, and there was no difference in the haematological parameters between the SCT and control groups. However, at delivery, the women with SCT, compared with the control group, had lower levels of hemoglobin (Hb, p=0.000), packed cell volume (PCV, p=0.000), mean corpuscular haemoglobin (MCH, p=0.002) and neutrophil counts (p=0.045) and higher mean corpuscular volume (MCV, p=0.000) and platelet counts (p=0.000). Similarly, at delivery, the babies of SCT women had lower birth weight (p=0.000), lower Hb (p=0.045), PCV (p=0.000), MCH (p=0.000), and higher neutrophil (p=0.004) and platelet counts (p=0.000) than the babies of the healthy control group. Additionally, there were more miscarriages, stillbirths, and admissions to the Special Care Baby Unit (SCBU) in the SCT group. Conclusions: The study revealed that SCT is associated with adverse pregnancy outcomes, including maternal and neonatal anaemia, low birth weight, and increased risk of stillbirth, miscarriage, and admission to SCBU. Therefore, pregnant women with SCT should be given appropriate pre-conceptual advice and multidisciplinary antenatal and postnatal care

Coexistence of HBsAg/Anti-HBs and HBeAg/Anti-HBe in Sudanese Patients with Chronic Hepatitis B Virus Infection: A Cross-Sectional Study

Background: Seroconversion of hepatitis B surface antigen (HBsAg) to hepatitis B surface antibody (anti-HBs) is a recognized goal of HBV therapy. This dynamic transition responsible for the coexistence of HBsAg and anti-HBs is rarely detected in clinical cases. However, with vaccination and the use of various antiviral drugs, as well as the development of new medical technologies, recognizing the coexistence of HBsAg and anti-HBs has become more common. In addition, mutations in viral genomes, immune status, and human genetic factors may also contribute to such coexistence. The current study was designed to determine the prevalence of the coexistence of HBsAg and anti-HBs and HBeAg and anti-HBe in CHB patients in Sudan. Methods and Results: This was a descriptive cross-sectional study conducted in Khartoum state from November 2018 to January 2019. The study included 70 HBV-infected patients who were positive for HBsAg for more than six months. Blood samples were tested for HBsAg/Anti-HBs and HBeAg/Anti-HBe using Commercial ELISA Kits (Foresight, United Kingdom) and (PRECHEK, USA). Demographic data were collected using a structured questionnaire, and any antiviral agent and laboratory results were also recorded for each participant. The current study showed that one case (1.4%) was reactive for the coexistence of HBsAg/HBsAb and two cases (2.8%) for the coexistence of HBeAg/HBeAb. There was no statistical difference between the coexistence of HBsAg/HBsAb and HBeAg/HBeAb with age, gender, residence, and treatment status. Conclusion: Our study indicates that the frequencies of the coexistence of HBsAg/HBsAb and HBeAg/HBeAb among Sudanese patients with chronic HBV infection were low compared to previous studies in a different population

Association of respiratory symptoms and lung function with occupation in the multinational Burden of Obstructive Lung Disease (BOLD) study

Background Chronic obstructive pulmonary disease has been associated with exposures in the workplace. We aimed to assess the association of respiratory symptoms and lung function with occupation in the Burden of Obstructive Lung Disease study. Methods We analysed cross-sectional data from 28 823 adults (≥40 years) in 34 countries. We considered 11 occupations and grouped them by likelihood of exposure to organic dusts, inorganic dusts and fumes. The association of chronic cough, chronic phlegm, wheeze, dyspnoea, forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1)/FVC with occupation was assessed, per study site, using multivariable regression. These estimates were then meta-analysed. Sensitivity analyses explored differences between sexes and gross national income. Results Overall, working in settings with potentially high exposure to dusts or fumes was associated with respiratory symptoms but not lung function differences. The most common occupation was farming. Compared to people not working in any of the 11 considered occupations, those who were farmers for ≥20 years were more likely to have chronic cough (OR 1.52, 95% CI 1.19–1.94), wheeze (OR 1.37, 95% CI 1.16–1.63) and dyspnoea (OR 1.83, 95% CI 1.53–2.20), but not lower FVC (β=0.02 L, 95% CI −0.02–0.06 L) or lower FEV1/FVC (β=0.04%, 95% CI −0.49–0.58%). Some findings differed by sex and gross national income. Conclusion At a population level, the occupational exposures considered in this study do not appear to be major determinants of differences in lung function, although they are associated with more respiratory symptoms. Because not all work settings were included in this study, respiratory surveillance should still be encouraged among high-risk dusty and fume job workers, especially in low- and middle-income countries.publishedVersio

Cohort Profile: Burden of Obstructive Lung Disease (BOLD) study

The Burden of Obstructive Lung Disease (BOLD) study was established to assess the prevalence of chronic airflow obstruction, a key characteristic of chronic obstructive pulmonary disease, and its risk factors in adults (≥40 years) from general populations across the world. The baseline study was conducted between 2003 and 2016, in 41 sites across Africa, Asia, Europe, North America, the Caribbean and Oceania, and collected high-quality pre- and post-bronchodilator spirometry from 28 828 participants. The follow-up study was conducted between 2019 and 2021, in 18 sites across Africa, Asia, Europe and the Caribbean. At baseline, there were in these sites 12 502 participants with high-quality spirometry. A total of 6452 were followed up, with 5936 completing the study core questionnaire. Of these, 4044 also provided high-quality pre- and post-bronchodilator spirometry. On both occasions, the core questionnaire covered information on respiratory symptoms, doctor diagnoses, health care use, medication use and ealth status, as well as potential risk factors. Information on occupation, environmental exposures and diet was also collected

Childhood Steroid Sensitive Nephrotic Syndrome: Characteristics and Predictors of Relapses; A Study at A Single CENTER in Khartoum

Background: Childhood steroid-sensitive nephrotic syndrome (SSNS) usually has a favorable outcome in spite of its relapsing course. The objective of the authors was to study the demographic and clinical characteristics, outcome and risk factors for relapses in children with SSNS at a single center in Khartoum, Sudan. Material and Methods: In this cross-sectional, facility-based study, the authors retrospectively reviewed all the records of children with SSNS, followed at the Pediatric Renal Unit, Soba University Hospital, Khartoum between 2001 and 2014. SSRNS was defined as the remission of proteinuria within 4–6 weeks of corticosteroids. Relapse is therecurrence of proteinuria after remission; frequent if ≥ 2 within initial six months or ≥ 4 within one year, and steroid dependence if 2 during therapy or within 14 days after stopping it. Results: 330 children (males 220; 66.7%) with SSNS were studied with a mean age of 5.2 ± 3.5 years of whom 42.4% aged 1–5 years. At the presentation, hypertension was detected in 31.8% and hematuria in 19.1%. Serum cholesterol was elevated in all patients (mean 347.34 ± 117.87 mg/dl) and serum creatinine in 7.27% (mean 1.4 ± 0.35 mg/dl). Renal histology showed mesangioproliferative glomerulonephritis (MesPGN) in 57.5%, minimal change disease (MCD) in 35.5%, and focal segmental glomerulosclerosis (FSGS) and IgM nephropathy in 3.5% each. During the course of the illness, 10.3% achieved long-term remission, 89.7% relapsed— of whom 52.3% had frequent relapsing/steroid-dependent (FR/SD) course and 37.7% had infrequent relapses. Risk of frequent relapses were age of onset and low/moderate socioeconomic status (P = 0.015 and 0.019, respectively). Infectionswere recorded in 71.8%, but not significantly associated with the risk of frequent relapses (P > 0.05). Conclusions: The majority children with SSNS at this single center in Khartoum had a relapsing course with the majority being FR or SD. Predictors of frequent relapses were young age at onset and low socioeconomic status

Clinical Implications of Krüpple-like Transcription Factor KLF-14 and Certain Micro-RNA (miR-27a, miR-196a2, miR-423) Gene Variations as a Risk Factor in the Genetic Predisposition to PCOS

Polycystic ovary syndrome (PCOS) is a disorder with a symptomatic manifestation of an array of metabolic and endocrine impairments. PCOS has a relatively high prevalence rate among young women of reproductive age and is a risk factor for some severe metabolic diseases such as T2DM, insulin insensitivity, and obesity, while the most dominant endocrine malfunction is an excess of testosterone showing hyperandrogenism and hirsutism. MicroRNAs have been implicated as mediators of metabolic diseases including obesity and insulin resistance, as these can regulate multiple cellular pathways such as insulin signaling and adipogenesis. Genome-wide association studies during the last few years have also linked the Krüpple-like family of transcription factors such as KLF14, which contribute in mechanisms of mammalian gene regulation, with certain altered metabolic traits and risk of atherosclerosis and type-2 DM. This study has characterized the biochemical and endocrine parameters in PCOS patients with a comprehensive serum profiling in comparison to healthy controls and further examined the influence of allelic variations for miRNAs 27a (rs895819 A > G), 196a2 (rs11614913 C > T), 423 (rs6505162C > A), and transcription factor KLF14 (rs972283 A > G) gene polymorphism on the risk and susceptibility to PCOS. The experimental protocol included amplification refractory mutation-specific (ARMS)-PCR to detect and determine the presence of these polymorphic variants in the study subjects. The results in this case–control study showed that most of the serum biomarkers, both biochemical and endocrine, that were analyzed in the study demonstrated statistically significant alterations in PCOS patients, including lipids (LDL, HDL, cholesterol), T2DM markers (fasting glucose, free insulin, HOMA-IR), and hormones (FSH, LH, testosterone, and progesterone). The distribution of Krüppel-like factor 14 rs972283 G > A, miR-27a rs895819 A > G, and miR-196a-2 rs11614913 C > T genotypes analyzed within PCOS patients and healthy controls in the considered population was significant (p A genotypes (p > 0.05). The study found that in the codominant model, KLF14-AA was strongly associated with greater PCOS susceptibility (OR 2.35, 95% CI = 1.128 to 4.893, p p p < 0.009). Moreover, allele A of KLF-14 and allele T of miR-196a2 were strongly associated with PCOS susceptibility in the considered population

Clinical Implications of Kr&uuml;pple-like Transcription Factor KLF-14 and Certain Micro-RNA (miR-27a, miR-196a2, miR-423) Gene Variations as a Risk Factor in the Genetic Predisposition to PCOS

Polycystic ovary syndrome (PCOS) is a disorder with a symptomatic manifestation of an array of metabolic and endocrine impairments. PCOS has a relatively high prevalence rate among young women of reproductive age and is a risk factor for some severe metabolic diseases such as T2DM, insulin insensitivity, and obesity, while the most dominant endocrine malfunction is an excess of testosterone showing hyperandrogenism and hirsutism. MicroRNAs have been implicated as mediators of metabolic diseases including obesity and insulin resistance, as these can regulate multiple cellular pathways such as insulin signaling and adipogenesis. Genome-wide association studies during the last few years have also linked the Kr&uuml;pple-like family of transcription factors such as KLF14, which contribute in mechanisms of mammalian gene regulation, with certain altered metabolic traits and risk of atherosclerosis and type-2 DM. This study has characterized the biochemical and endocrine parameters in PCOS patients with a comprehensive serum profiling in comparison to healthy controls and further examined the influence of allelic variations for miRNAs 27a (rs895819 A &gt; G), 196a2 (rs11614913 C &gt; T), 423 (rs6505162C &gt; A), and transcription factor KLF14 (rs972283 A &gt; G) gene polymorphism on the risk and susceptibility to PCOS. The experimental protocol included amplification refractory mutation-specific (ARMS)-PCR to detect and determine the presence of these polymorphic variants in the study subjects. The results in this case&ndash;control study showed that most of the serum biomarkers, both biochemical and endocrine, that were analyzed in the study demonstrated statistically significant alterations in PCOS patients, including lipids (LDL, HDL, cholesterol), T2DM markers (fasting glucose, free insulin, HOMA-IR), and hormones (FSH, LH, testosterone, and progesterone). The distribution of Kr&uuml;ppel-like factor 14 rs972283 G &gt; A, miR-27a rs895819 A &gt; G, and miR-196a-2 rs11614913 C &gt; T genotypes analyzed within PCOS patients and healthy controls in the considered population was significant (p &lt; 0.05), except for miR-423 rs6505162 C &gt; A genotypes (p &gt; 0.05). The study found that in the codominant model, KLF14-AA was strongly associated with greater PCOS susceptibility (OR 2.35, 95% CI = 1.128 to 4.893, p &lt; 0.022), miR-27a-GA was linked to an enhanced PCOS susceptibility (OR 2.06, 95% CI = 1.165 to 3.650, p &lt; 0.012), and miR-196a-CT was associated with higher PCOS susceptibility (OR 2.06, 95% CI = 1.191 to 3.58, p &lt; 0.009). Moreover, allele A of KLF-14 and allele T of miR-196a2 were strongly associated with PCOS susceptibility in the considered population