Disorders of sexual differentiation as seen at Kenyatta National Hospital

Background: Disorders of sexual differentiation (DSD) are a group of congenital anomalies characterised by discordance between genetic, gonadal and phenotypic sex. There has been remarkable evolution in management over the last decade, including nomenclature, diagnosis and management. There has also been increased awareness and interest from patients and the public, including legal opinion. There has however been no local study to document and evaluate management in Kenya.Objective: To describe management of patients presenting with DSD at Kenyatta National Hospital (KNH) over a 10 year period.Design: Retrospective descriptive study.Setting: Kenyatta National Hospital.Subjects: Patients diagnosed with DSD.Results: A total of 30 patients whose charts were available were reviewed. Age ranged from birth to 19 years (median 5 months) at diagnosis. Presumed gender was assigned at birth in 28 patients. Karyotyping was available in 23(76%) patients. Other common tests included blood tests (23, 76%), ultrasound scan (14, 47%), contrast studies (3, 10%) and exploratory laparoscopy or laparotomy. 46XX and 46XY DSD were the most common conditions encountered (13, 43% and 7, 23% respectively). The commonest operations were correction of hypospadias and orchidopexy (55%), followed by feminising genitoplasty (16%). Only two patients had gender reassignment. Fifteen patients were asked how they feel about their current gender, and 11 expressed satisfaction.Conclusion: DSD is a relatively rare condition. There are also no strict protocols being followed. Management at KNH is acceptable although there is a lack of facilities to carry out many requisite investigations

Posttransplant malignancies and their relationship with human leukocyte antigens in kidney allograft recipients.

INTRODUCTION: Kidney transplant recipients are at increased risk of cancers, most frequently skin cancers, and in some regions, Kaposi sarcoma and non-Hodgkin lymphoma. We sought to investigate the associate of the most frequent malignancies among our patients with human leukocyte antigens (HLAs). MATERIALS AND METHODS: We performed a retrospective study on 44 kidney allograft recipients who had posttransplant malignancy and 44 kidney allograft recipients without malignant lesions (control group). All of the patients had been treated by immunosuppressive regimens including cyclosporine plus prednisolone or cyclosporine, prednisolone, and mycophenolate mofetil. Data on HLA typing were achieved from their transplant records. RESULTS: There were 15 patients (34.1) with Kaposi sarcoma; 13 (29.6) with non-Hodgkin lymphoma, 6 (13.6) with skin cancer, 2 (4.5) with ovary cyst adenocarcinoma, and 8 (18.2) with other tumors. The mean interval from transplantation to diagnosis of malignancy was 15.3 month. Twelve patients died of cancer during the follow-up (mean, 12.3 years). No significant difference was noted in the age, sex, and time of transplantation between these patients and those in the control group. Kaposi sarcoma was associated with HLA-CW4 (P = .03) with an odds ratio of 4.96 (95 confidence interval, 2.90 to 8.12). CONCLUSIONS: We found HLA-CW4 as a risk factor of Kaposi sarcoma in kidney allograft recipients. Screening for malignancies after kidney transplantation sounds very important with special attention to the specific environmental and genetic factors in each population

Posttransplant malignancies and their relationship with human leukocyte antigens in kidney allograft recipients.

INTRODUCTION: Kidney transplant recipients are at increased risk of cancers, most frequently skin cancers, and in some regions, Kaposi sarcoma and non-Hodgkin lymphoma. We sought to investigate the associate of the most frequent malignancies among our patients with human leukocyte antigens (HLAs). MATERIALS AND METHODS: We performed a retrospective study on 44 kidney allograft recipients who had posttransplant malignancy and 44 kidney allograft recipients without malignant lesions (control group). All of the patients had been treated by immunosuppressive regimens including cyclosporine plus prednisolone or cyclosporine, prednisolone, and mycophenolate mofetil. Data on HLA typing were achieved from their transplant records. RESULTS: There were 15 patients (34.1) with Kaposi sarcoma; 13 (29.6) with non-Hodgkin lymphoma, 6 (13.6) with skin cancer, 2 (4.5) with ovary cyst adenocarcinoma, and 8 (18.2) with other tumors. The mean interval from transplantation to diagnosis of malignancy was 15.3 month. Twelve patients died of cancer during the follow-up (mean, 12.3 years). No significant difference was noted in the age, sex, and time of transplantation between these patients and those in the control group. Kaposi sarcoma was associated with HLA-CW4 (P = .03) with an odds ratio of 4.96 (95 confidence interval, 2.90 to 8.12). CONCLUSIONS: We found HLA-CW4 as a risk factor of Kaposi sarcoma in kidney allograft recipients. Screening for malignancies after kidney transplantation sounds very important with special attention to the specific environmental and genetic factors in each population

Digitising diabetes education for a safer Ramadan:Design, delivery, and evaluation of massive open online courses in Ramadan-focused diabetes education

Aims: Ramadan-focused diabetes education is critical to facilitate safer Ramadan fasting amongst Muslim people living with diabetes. We present the design, delivery, and evaluation of two parallel massive open online courses (MOOCs) in Ramadan-focused diabetes education for people with diabetes and HCPs. Methods: Two Ramadan-focused diabetes education MOOCs were developed and delivered for Ramadan 2023: one for HCPs in English, and another for people with diabetes in English, Arabic and Malay. A user-centred iterative design process was adopted, informed by user feedback from a 2022 pilot MOOC. Evaluation comprised a mixed-methods evaluation of pre- and post-course user surveys. Results: The platform was utilised by people with diabetes and their family, friends and healthcare professionals. Overall, a total of 1531 users registered for the platform from 50 countries, 809 started a course with a 48% subsequent completion rate among course starters. Qualitative analysis showed users found the course a user-friendly and authoritative information source. In the HCP MOOC, users reported improved post-MOOC Ramadan awareness, associated diabetes knowledge and ability to assess and advise patients in relation to their diabetes during Ramadan (p&lt;0.01). Conclusions: We demonstrate the potential of MOOCs to deliver culturally tailored, high-quality, scalable, multilingual Ramadan-focused diabetes education to HCPs and people with diabetes.</p

Digitising diabetes education for a safer Ramadan:Design, delivery, and evaluation of massive open online courses in Ramadan-focused diabetes education

AIMS: Ramadan-focused diabetes education is critical to facilitate safer Ramadan fasting amongst Muslim people living with diabetes. We present the design, delivery, and evaluation of two parallel massive open online courses (MOOCs) in Ramadan-focused diabetes education for people with diabetes and HCPs.METHODS: Two Ramadan-focused diabetes education MOOCs were developed and delivered for Ramadan 2023: one for HCPs in English, and another for people with diabetes in English, Arabic and Malay. A user-centred iterative design process was adopted, informed by user feedback from a 2022 pilot MOOC. Evaluation comprised a mixed-methods evaluation of pre- and post-course user surveys.RESULTS: The platform was utilised by people with diabetes and their family, friends and healthcare professionals. Overall, a total of 1531 users registered for the platform from 50 countries, 809 started a course with a 48% subsequent completion rate among course starters. Qualitative analysis showed users found the course a user-friendly and authoritative information source. In the HCP MOOC, users reported improved post-MOOC Ramadan awareness, associated diabetes knowledge and ability to assess and advise patients in relation to their diabetes during Ramadan (p&lt;0.01).CONCLUSIONS: We demonstrate the potential of MOOCs to deliver culturally tailored, high-quality, scalable, multilingual Ramadan-focused diabetes education to HCPs and people with diabetes.</p

Physical activity and fat-free mass during growth and in later life

Physical activity may be a way to increase and maintain fat-free mass (FFM) in later life, similar to the prevention of fractures by increasing peak bone mass.A study is presented of the association between FFM and physical activity in relation to age.In a cross-sectional study, FFM was analyzed in relation to physical activity in a large participant group as compiled in the International Atomic Energy Agency Doubly Labeled Water database. The database included 2000 participants, age 3–96 y, with measurements of total energy expenditure (TEE) and resting energy expenditure (REE) to allow calculation of physical activity level (PAL = TEE/REE), and calculation of FFM from isotope dilution.PAL was a main determinant of body composition at all ages. Models with age, fat mass (FM), and PAL explained 76\% and 85\% of the variation in FFM in females and males &lt; 18 y old, and 32\% and 47\% of the variation in FFM in females and males ≥ 18 y old, respectively. In participants &lt; 18 y old, mean FM-adjusted FFM was 1.7 kg (95\% CI: 0.1, 3.2 kg) and 3.4 kg (95\% CI: 1.0, 5.6 kg) higher in a very active participant with PAL = 2.0 than in a sedentary participant with PAL = 1.5, for females and males, respectively. At age 18 y, height and FM–adjusted FFM was 3.6 kg (95\% CI: 2.8, 4.4 kg) and 4.4 kg (95\% CI: 3.2, 5.7 kg) higher, and at age 80 y 0.7 kg (95\% CI: −0.2, 1.7 kg) and 1.0 kg (95\% CI: −0.1, 2.1 kg) higher, in a participant with PAL = 2.0 than in a participant with PAL = 1.5, for females and males, respectively.If these associations are causal, they suggest physical activity is a major determinant of body composition as reflected in peak FFM, and that a physically active lifestyle can only partly protect against loss of FFM in aging adults

Total energy expenditure is repeatable in adults but not associated with short-term changes in body composition

Low total energy expenditure (TEE, MJ/d) has been a hypothesized risk factor for weight gain, but repeatability of TEE, a critical variable in longitudinal studies of energy balance, is understudied. We examine repeated doubly labeled water (DLW) measurements of TEE in 348 adults and 47 children from the IAEA DLW Database (mean ± SD time interval: 1.9 ± 2.9 y) to assess repeatability of TEE, and to examine if TEE adjusted for age, sex, fat-free mass, and fat mass is associated with changes in weight or body composition. Here, we report that repeatability of TEE is high for adults, but not children. Bivariate Bayesian mixed models show no among or within-individual correlation between body composition (fat mass or percentage) and unadjusted TEE in adults. For adults aged 20-60 y (N = 267; time interval: 7.4 ± 12.2 weeks), increases in adjusted TEE are associated with weight gain but not with changes in body composition; results are similar for subjects with intervals >4 weeks (N = 53; 29.1 ± 12.8 weeks). This suggests low TEE is not a risk factor for, and high TEE is not protective against, weight or body fat gain over the time intervals tested

Human total, basal and activity energy expenditures are independent of ambient environmental temperature

ower ambient temperature (Ta) requires greater energy expenditure to sustain body temperature. However, effects of Ta on human energetics may be buffered by environmental modification and behavioral compensation. We used the IAEA DLW database for adults in the USA (n = 3213) to determine the effect of Ta (−10 to +30°C) on TEE, basal (BEE) and activity energy expenditure (AEE) and physical activity level (PAL). There were no significant relationships (p > 0.05) between maximum, minimum and average Ta and TEE, BEE, AEE and PAL. After adjustment for fat-free mass, fat mass and age, statistically significant (p < 0.01) relationships between TEE, BEE and Ta emerged in females but the effect sizes were not biologically meaningful. Temperatures inside buildings are regulated at 18–25°C independent of latitude. Hence, adults in the US modify their environments to keep TEE constant across a wide range of external ambient temperatures