Experience of X-linked hypophosphatemic rickets in the Gulf Cooperation Council countries: case series.

Summary: X-linked hypophosphatemic rickets (XLH), the most prevalent form of inherited hypophosphatemic rickets, is caused by loss-of-function mutations in the gene encoding phosphate-regulating endopeptidase homolog, X-linked (PHEX). This case series presents 14 cases of XLH from Gulf Cooperation Council (GCC) countries. The patients' medical history, biochemical and radiological investigative findings, as well as treatment responses and side effects from both conventional and burosumab therapy, are described. Cases were aged 2-40 years at diagnosis. There were two male cases and 12 female cases. All cases were treated with conventional therapy which resulted in a lack of improvement in or worsening of the clinical signs and symptoms of rickets or biochemical parameters. Side effects of conventional therapy included nausea, diarrhea, abdominal pain, nephrocalcinosis, and hyperparathyroidism, which affected the patients' quality of life and adherence to treatment. In the 10 patients treated with burosumab, there was a marked improvement in the biochemical markers of rickets, with a mean increase in serum phosphate of +0.56 mmol/L and tubular maximum phosphate reabsorption (TmP) to glomerular filtration rate (GFR) ratio (TmP/GFR) of +0.39 mmol/L at 12 months compared to baseline. Furthermore, a mean decrease in serum alkaline phosphatase (ALP) of -80.80 IU/L and parathyroid hormone (PTH) of -63.61 pmol/L at 12 months compared to baseline was observed in these patients. Additionally, patients treated with burosumab reported reduced pain, muscle weakness, and fatigue as well as the ability to lead more physically active lives with no significant side effects of treatment. Learning points: Conventional therapy resulted in a suboptimal response, with a lack of improvement of clinical signs and symptoms. Side effects of conventional therapy included nausea, diarrhea, abdominal pain, nephrocalcinosis, and hyperparathyroidism, which affected the patients' quality of life and adherence to treatment. Burosumab demonstrated marked improvements in the biochemical markers of rickets, in addition to reducing pain, muscle weakness, and fatigue. There were no significant side effects associated with burosumab therapy.

Perception of primary caregiver toward virtual pediatric clinics for type 1 diabetes mellitus during COVID-19 pandemic in Jeddah, Saudi Arabia: A cross-sectional study.

Background: Virtual phone clinics were adopted at King Abdulaziz Medical City, Jeddah, Saudi Arabia, as an alternative to in-person routine outpatient visits amid the COVID-19 pandemic. This study aimed to assess primary caregiver perceptions of the virtual phone clinics towards routine care of pediatric patients with type 1 diabetes mellitus (T1DM). Methods: A cross-sectional research design was employed through a web-based research tool where the primary caregivers of pediatric patients with T1DM participated. Data were analyzed descriptively and statistically using independent t-tests and one-way analysis of variance. Results: In total, 214 participants were included in this study. The average primary caregiver perception value towards virtual phone clinics was 66.71 ± 23.1%, which suggested a satisfactory perception of their experience. Statistically significant differences (p > 0.05) were not found between the mean perception values and demographic characteristics. Higher mean primary caregiver perception values were associated with those who: (1) agreed or strongly agreed to be contented with their current state of health, (2) did not ask their physicians to be physically seen during a virtual visit, (3) preferred having four virtual visits or more, and (4) preferred having single to no in-person visits at all. Conclusion: This study revealed a generally acceptable primary caregiver perception regarding virtual clinics. Telemedicine is recommended for routine treatment alongside regular in-clinic appointments to improve the quality of care for pediatric patients with T1DM.

Use of continuous glucose monitoring trend arrows in the younger population with type 1 diabetes.

Early control of glycaemia is key to reduce vascular complications in individuals with Type 1 diabetes. Therefore, encouraging children and adolescents with T1DM to take responsibility for controlling glucose levels is an important yet a challenging task. The rapid expansion of continuous glucose monitoring (CGM) systems has allowed for more comprehensive analysis of glycaemia in T1D. Moreover, CGM devices have the ability to calculate rate of change in glucose levels and display the information as trend arrows. In turn, this can help to take evasive actions to return glucose levels to near physiological glycaemia, which can be highly motivating for young people with T1DM. In the absence of standardised, evidence-based guidance, this consensus document, generated by experts from the Arab Society of Paediatric Endocrinology and Diabetes and international advisors, summarises recent literature on the use of trend arrows in young people with T1DM. The use of trend arrows in different CGM systems is reviewed and their clinical significance is highlighted. Adjusting insulin doses according to trend arrows is discussed while also addressing special situations, such as exercise, fasting, nocturnal hypoglycaemia and menstruation. Adequate understanding of trend arrows should facilitate optimisation of glycaemic control in the T1D population.

Diagnosis and management of X-linked hypophosphatemia in children and adolescent in the Gulf Cooperation Council countries.

INTRODUCTION: X-linked hypophosphatemia (XLH) is a rare inherited cause of hypophosphatemic rickets and osteomalacia. It is caused by mutations in the phosphate-regulating endopeptidase homolog, X-linked (PHEX). This results in increased plasma fibroblast growth factor-23 (FGF23), which leads to loss of renal sodium-phosphate co-transporter expression leading to chronic renal phosphate excretion. It also leads to low serum 1,25-dihydroxyvitamin D (1,25(OH)2D), resulting in impaired intestinal phosphate absorption. Chronic hypophosphatemia in XLH leads to impaired endochondral mineralization of the growth plates of long bones with bony deformities. XLH in children and adolescents also causes impaired growth, myopathy, bone pain, and dental abscesses. XLH is the most frequent inherited cause of phosphopenic rickets/osteomalacia. Hypophosphatemia is also found in calcipenic rickets/osteomalacia as a result of secondary hyperparathyroidism. Thus, chronic hypophosphatemia is a common etiologic factor in all types of rickets. RESULTS: There is considerable overlap between symptoms and signs of phosphopenic and calcipenic rickets/osteomalacia. Wrong diagnosis leads to inappropriate treatment of rickets/osteomalacia. Nutritional rickets and osteomalacia are common in the Gulf Cooperation Council countries which include Saudi Arabia, United Arab Emirates, Kuwait, Qatar, Bahrain, and Oman. Due to high levels of consanguinity in the region, genetic causes of phosphopenic and calcipenic rickets/osteomalacia are also common. CONCLUSION: This guideline was developed to provide an approach to the diagnosis of XLH, especially where there is no family history of the disease, and that other related conditions are not mistaken for XLH. We also guide the medical management of XLH with conventional treatment and with burosumab, a recombinant human IgG1 monoclonal antibody to FGF23.

Quality of Life and Glycemic Control in Saudi Children with Type 1 Diabetes at Different Developmental Age Groups.

BACKGROUND: Children with type 1 diabetes (T1D) at different stages of development have age-specific needs, which can influence their perception of quality of life (QoL). In our study, we aimed to emphasize these age-specific needs and assess the perception of QoL in Saudi children with T1D, as well as their parents correlating QoL scores with children's glycemic control. METHODS: This is a cross-sectional study in which children with T1D and their parents from 2 tertiary institutes in Saudi Arabia have answered a standard diabetes-specific QoL questionnaire (PedsQL™ 3.0 diabetes module, translated in Arabic). We also reported glycated hemoglobin (HbA1c) results for these children within a month of completing the questionnaire. The QoL total aggregate and domain scores for self (children) and proxy (parents') reports were compared and correlated with children's HbA1c. RESULTS: A sample was 288 self and proxy reports from 144 children with T1D of 3 age groups: 5 to 7 years (7%), 8 to 12 years (49%), and 13 to 18 years (44%), and their parents. QoL differed significantly between self and proxy reports in the total aggregate and domain scores (P-values range from .02 to <.001). The impact on QoL was significantly higher in female patients (P = .043). Insulin pump users had better HbA1c (P = .007), and HbA1c level was worse in those who intended to fast at Ramadan (P = .005). CONCLUSION: Children with T1D at different developmental age groups perceive QoL differently than their parents. Adjusting management as per age-specific challenges could potentially improve these children's QoL and glycemic control.

Quality of Life in Children With Diabetes Treated With Insulin Pump Compared With Multiple Daily Injections in Tertiary Care Center.

BACKGROUND: Continuous Subcutaneous Insulin Infusion (CSII) and Multiple Daily Injections (MDI) have been widely used as options in treating diabetes in childhood. Glycemic control is important to reduce diabetes complications; however, more focus needs to be on patients' Quality of Life (QoL). Diabetes and QoL have strong associations in terms of patients' overall health including their psychology, physical well-being, compliance with medication. A previous systematic review stressed that strong evidence to deny or prove the benefits of insulin pump therapy on health-related quality of life is deficient. The aim of this study is to assess the health-related quality of life and the psychological impacts of children with diabetes who use CSII and MDI treatment. METHODS: A cross-sectional study included 68 pediatric patients with type 1 Diabetes (T1DM) who were treated in a tertiary center in Jeddah, Saudi Arabia. We used the Pediatric Quality of Life Inventory 3.0 Diabetes Module and this module assesses the health-related quality of life of children with diabetes. RESULTS: Thirty-four (50%) participants found to be on MDI, of which 21 (61.8%) are males, compared to 34 (50%) patients using CSII, of which 12 (35.2%) are females. Participants using CSII had statistically significant better symptom control, less treatment difficulties but were more worrisome than MDI participants. CONCLUSION: CSII group had better quality of life in almost all aspects even though they were more worrisome. Further studies with a larger sample size are needed to give comprehensive generalizations.

Persistent Hypoglycemia in Seven-year-old Saudi Child: A Case Report.

Hypoglycemia is a frequent problem in infants and children, causing a significant dilemma to reach the correct diagnosis and perform the appropriate management. Congenital hyperinsulinism is the most common cause of hypoglycemic hyperinsulinemia in infants and is due to beta-cell hyperplasia caused by genetic defects. This is a well-known genetically and clinically heterogeneous condition causing severe hypoglycemia in infants. Insulin-secreting tumors (insulinoma) are rare findings during childhood. In contrast, insulinoma is the most common form of endogenous hypoglycemic hyperinsulinemia in the adult population. Here we present a successful diagnosis and treatment of a nine-year-old Saudi child who presented for the first time with severe episodes of hypoglycemia at age seven. Critical samples at the time of hypoglycemia confirmed the associated hyperinsulinemia state. Initially, the child responded well to anti-insulin medications at small doses, but with time the disease became progressive in severity requiring a high dose of anti-insulin medications, frequent glucagon injections, and hospital admission for intravenous dextrose infusion. After two years of seeking therapy in many hospitals, the final diagnosis was confirmed to be an insulinoma, which was removed surgically, resulting in a complete cure and full recovery. Here we report the first published case of insulinoma in a young child aged < 15 years old in Saudi Arabia, their disease course, final diagnostic steps, and curative therapy. We conclude that hypoglycemia in children is challenging in terms of diagnosis and management. Although insulinoma is very rare in children, it requires significant time and effort by a pediatrician, pediatric endocrinologist, patients, and parents to reach the final diagnosis and carefully preserve the integrity of the neurological state of those children.

Symptoms of Daytime Sleepiness and Sleep Apnea among Pregnant Women.

OBJECTIVES: Despite the fact that sleep disturbances have been associated with poor maternal and neonatal health outcomes in pregnancy, no studies have assessed excessive daytime sleepiness or the risk for sleep apnea among pregnant Saudi Arabian women. We sought to estimate the prevalence of excessive daytime sleepiness (EDS) and the high risk for sleep apnea (OSA) in a sample of pregnant Saudi women. METHODS: An anonymous self-report questionnaire was completed by 517 pregnant women who attended obstetric outpatient clinics at King Abdulaziz Medical City, Riyadh, Saudi Arabia, for a routine pregnancy check. We collected demographic and clinical data for all patients and used the Berlin Questionnaire and the Epworth Sleepiness Scale to determine the primary outcomes. RESULTS: A high risk of OSA was found in 37.1% of women (95% confidence interval (CI): 33.00%-41.50%), and EDS was found in 32.1% (95% CI: 28.10%-36.30%). The presence of both (EDS and a high risk of OSA) was found in 14.9% of women (95% CI: 11.90%-18.30%). We found increased odds of EDS in women who reported pain three times or more per week (adjusted odds ratio (aOR) = 2.59) and insomnia (aOR = 1.65). Older women (≥ 37 years) (aOR = 3.00), those who reported pain once a week (aOR = 1.99), pain twice a week (aOR = 2.75), three times or more a week (aOR = 2.57), and insomnia (aOR = 1.95) increased the odds of high risk for OSA. CONCLUSIONS: EDS and a high risk for OSA affected a large portion of the pregnant women included in the study, primarily those who reported pain and insomnia. Our study provides important information for gynecologists to help promote healthy sleep and manage the issues arising from sleep disturbances among pregnant women as part of their daily practice.

Top Ethical Issues Concerning Healthcare Providers Working in Saudi Arabia.

BACKGROUND: Healthcare providers working in Saudi Arabia come from various nationalities, cultures, and training backgrounds. This study aimed to assess the perceptions of healthcare providers working in Riyadh hospitals about ethical dilemmas and solutions. METHODS: This is a cross-sectional study among physicians working in Riyadh's private and governmental hospitals between June and December 2017. The study collected information on demographics, knowledge about medical ethics, the sources of such knowledge, and common ethical issues in general and the top ethical issues and dilemmas encountered in their daily practice. RESULTS: A total of 455 physicians from government and private hospitals were enrolled in the study. The mean age of the participants was 34.29 ± 10.5 years, females were 29.7% and mean years of practice was 13.0 ± 11.5. The top ethical issues identified by the participants were "disagreement with the patients' relatives about treatment" (91%), patient disagreement with decisions made by professionals (84%), treating the incompetent patient (79%), conflict with administration policy and procedures (77%), scarcity of resources (72%), and making decision about do-not-resuscitate or life-sustaining treatment (68%). There were significant differences in dealing with ethical issues in relation to gender, confidence about ethical knowledge, nationality, seniority, training site, and private or government hospitals academic and nonacademic. CONCLUSION: Healthcare providers in Riyadh hospitals face multiple ethical challenges. In addition to improvement in ethics knowledge through educational program among healthcare professional, there is a valid need for healthcare professionals and other sectors within society to engage in serious and continuous dialogue to address these issues and propose recommendations.

Awareness, Self-Use, Perceptions, Beliefs, and Attitudes toward Complementary and Alternative Medicines (CAM) among Health Professional Students in King Saud bin Abdulaziz University for Health Sciences Jeddah, Saudi Arabia.

BACKGROUND: Around 75% of the world's population relies on the use of complementary and alternative medicines (CAM) for their healthcare. Thus, we aimed at assessing the attitude and perception of health professional students about CAM and correlate the results with their demographical data. METHODS: An observational cross-sectional study was conducted at King Saud bin Abdulaziz University for Health Sciences (KSAU-HS), Jeddah, Saudi Arabia, by enrolling 350 students. A self-administered questionnaire was used for data collection. Descriptive and inferential statistical analysis was performed using SPSS. RESULTS: Results of our data suggested that majority of students were aware of the CAM modalities and considered prayers/spirituality (83.6%), massage (72.5%), nutritional supplements (74.1%), cupping (68.5%), herbal medicine (66.2), and yoga (61.6%) as most effective and least harmful CAM modalities. The students acquired more information about CAM from media (55%), books (56%), friends/relatives (59.7%), and other health professionals (58.4%), however, very little information from formal CAM courses or training (36.7%), which shows the lack of courses and trained health professional in the field of CAM. Despite utilizing nonreliable sources of CAM information, the majority of students had positive attitudes and perceptions about CAM usage. Majority of the students (75.8%) believe in the use of CAM for the mental and spiritual aspect of health and think that CAM providers give good information on maintaining a healthy lifestyle. The data also showed a significant (P < 0.05) association between awareness about CAM and gender. A significantly higher percentage of female students were using yoga and aromatherapy, while cupping was mostly used by male students. Similarly, a significant association was found among the different colleges and level of students in most of the CAM modalities. However, no association was found between the awareness and use of CAM with the educational level of parents or relatives in health sector except for acupuncture and cupping, where student's awareness about acupuncture and cupping was found to have a significant positive association with mother educational level. On the other hand, a significant inverse association was found between acupuncture, yoga, cupping, and aromatherapy with family income. CONCLUSION: In conclusion, despite the lack of proper courses and professionally trained healthcare providers in the field of CAM, students demonstrated positive attitudes and beliefs toward the safety and effectiveness of CAM.

Attitudes and behaviours of physicians towards the relationship with the pharmaceutical industry in Saudi Arabia.

BACKGROUND: The relationship and interactions between physicians and the pharmaceutical industry can affect patient care. A physician's practice can be influenced by this relationship. It is believed that these interactions are common among doctors in Saudi Arabia. AIMS: This study was undertaken to assess the frequency of such relationships and physicians' attitudes and behaviours toward them. METHODS: This was a cross-sectional questionnaire survey completed by practicing physicians at four Saudi government and private tertiary care centres in Riyadh, Saudi Arabia. The questionnaire addressed the frequency of meetings with representatives of pharmaceutical companies (PRs) and of receiving gifts and considered the physicians' attitudes and behaviours towards PRs. RESULTS: A total of 300 completed questionnaires were obtained. Among the physicians surveyed, 223 (74.3%) met PRs one to three times per month. Up to 191 (64%) of physicians admitted receiving gifts. More than two thirds of physicians-192 (63%) have been invited to activities sponsored by pharmaceutical companies. Among the physicians, 239 (80%) agreed that PRs use promotional techniques in their approach and 251 (84%) of them stressed the need for expert physicians to attend presentations by PRs to correct the facts. CONCLUSION: The frequent meetings between physicians and PRs and the use of promotional techniques by PRs are concerning. Future studies should assess the impact of this involvement on medical practice and drugs prescription in Saudi Arabia.

Effect of growth hormone treatment on children with idiopathic short stature (ISS), idiopathic growth hormone deficiency (IGHD), small for gestational age (SGA) and Turner syndrome (TS) in a tertiary care center.

OBJECTIVES: To assess the long-term effect of growth hormone (GH) therapy in a large cohort of short children with different etiologies. PATIENTS AND METHODS: We evaluated retrospectively the anthropometric data of 252 short children [height SDS <-2: 154 children with growth hormone deficiency (GHD), 63 with idiopathic short stature (ISS), 26 with SGA, and 9 with Turner syndrome (TS)] who were treated, in our center, with GH between 1-2007 and 1-2018. Before and during recombinamt growth-hormone (recGH) treatment, auxological parameters including height (Ht), weight (Wt), Ht - Z score (HtSDS), body mass index (BMI) and BMISDS were recorded every 6 months; bone age (BA) was assessed every 12 months. RESULTS: At the end of first year of rhGH therapy and after an average of 3 years treatment all groups of short children had significant increase in HtSDS, which was higher in GHD compared to other groups. Children with GHD, SGA, ISS and TS increased their HtSDS by an average of 2.2, 1.46, 0.6 and 0.99 SD, respectively at the end of follow up period (for all groups, p: <0.001). The bone age/chronological age (BA/CA) ratio did not differ significantly among ISS, GHD and SGA groups after GH therapy. The HtSDS gain was higher in children with GHD compared to other ISS, SGA and TS groups (p:< 0.01; p: 0.015 and p: 0.029, respectively).  HtSDS improvement occurred during the first 3 years of rhGH therapy. The BMISDS increased significantly in children with GHD, after 3 years of rhGH therapy (p: < 0.001). After rhGH treatment, the BMISDS decreased significantly in children with ISS and SGA (p: < 0.01 and < 0.001, respectively) but did not change in children with TS (p: 0.199). CONCLUSIONS: Children with GHD, SGA, ISS and TS exhibited significant increases in HtSDS when treated with rhGH for 3 years. The HtSDS gain was higher in children with GHD compared to other groups.

Prevalence of Obesity and Overweight among School-Aged Children in Saudi Arabia and Its Association with Vitamin D Status.

BACKGROUND: Childhood obesity is a significant global public health problem. Worldwide data showed an increasing trend over the years. We aimed to explore the prevalence of obesity, and its association with vitamin D status. METHODS: This cross-sectional study was conducted during the period from 2016 to 2017. The study included 3613 schoolchildren aged 6-19 years in the western, central, and eastern regions in Saudi Arabia. Anthropometric data including age, sex and body mass index (BMI) was collected and the serum 25OH- vitamin D (25OHD) was measured. Age-sex standardized BMI Z-scores using the 2007 World Health Organization growth standards were applied. RESULTS: Data from 3613 school-aged children (females = 51.8%), with equal percentage of participants from each region were analyzed. Prevalence of obesity and overweight was estimated at 7.1% and 14.4% respectively. An increasing trend was detected with age (p: 0.006). Obesity started to increase at the age of 10 years and continued to increase until 19 years. Most of the obese children (64.2%) had deficient vitamin D levels, compared to 33.7% of them with suboptimal levels and only 2.0% with optimal levels (p: <0.001). CONCLUSIONS: This large cohort study revealed a high prevalence (21.5%) of obesity and overweight in school children aged between 6-19 years with increasing trend with age.  Children with high BMI showed extremely high prevalence of VDD and VDI. These findings are alarming and point to the need for effective national interventions that include improving and encouraging access to physical activity and exposure to sunlight, educational activities for students, parents, and schoolteachers and possible enrichment of staple food with vitamin D.

Epidemiology of Preventable Drug-Related Problems (DRPs) Among Hospitalized Children at KAMC-Jeddah: a Single-Institution Observation Study.

AIM: Drug-related problems (DRPs) "are the unwanted effects of drugs that potentially lead to a harmful outcome" thereby requiring considerable attention. Hospitalized pediatric patients, in particular, represent a population at risk of DRPs. The epidemiology of preventable DRPs among children in Saudi Arabia remains scarce, which thus poses distinct challenges to all healthcare professionals. We aim to characterize preventable DRPs among hospitalized children at KAMC-Jeddah. METHODS: A prospective observational study of children (≤15 years) admitted to pediatric units (excluding cancer units) at KAMC-Jeddah over a 3-month period (May 29 to August 30, 2016) is carried out to determine the incidence of preventable DRPs and investigate the possible associated factors (gender, age, admission location, type of admission, and number of medications). RESULTS: A total of 319 DRPs were identified among 235 patients, of which 280 DRPs (87.8%, 280/319) were deemed preventable. The majority of preventable DRPs were related to dose selection (78%, 219/280). None of the preventable DRPs were life threatening or fatal, and the majority were assessed as moderate in severity (94.3%, 264/280). There was no significant difference between DRP incidences with age mean 3.5 (P=0.389), gender mean (P=0.436), and weight mean 13.47 (P=0.323). Younger children (age ≤2years) admitted to PICU were more likely to have DRP (OR 4.44, 95% CI, 1.87 to 10.52, P=0.00001). Scheduled admissions were 2.89 times more likely to be exposed to DRP compared to transferred admissions (OR 2.8, 95% CI, 1.83 to 4.70, P=0.005). Additionally, DRP incidences increased proportionally to the number of medications. CONCLUSION: Our data suggest that establishing appropriate prevention strategies towards improvement and safety in medicine use among this vulnerable patient population is a high priority.

Common medical ethical issues faced by healthcare professionals in KSA.

OBJECTIVE: There are growing concerns about ethical issues in the healthcare system. This study was conducted to determine the nature of common ethical issues faced by healthcare providers in a tertiary-care hospital in KSA. METHOD: This cross-sectional study comprised a self-administered questionnaire given to the physicians working at King Abdulaziz Medical City Hospital-Riyadh, Ministry of National Guard Health Affairs (KAMC-RD, MNGHA). We used a convenience sampling technique during symposia and conferences. RESULTS: We distributed 240 questionnaires amongst the physicians and recorded a response rate of 80%; 68% (136) of the respondents were men, while 82.5% were Saudis. The mean age of the group was 34.08 ± 10.43 years. Only 69% (138) of the physicians had ever received any formal teaching in bioethics. Most physicians (77.5%) demanded clear guidelines to help them to take appropriate ethical decisions on therapeutic futility, whereas 54% felt that they sometimes overtreat their patients. CONCLUSION: This study reported a lack of knowledge in certain healthcare-related ethical issues in a significant proportion of the physicians. There is a need for a standard educational agenda for medical ethics for healthcare providers, not only during medical school but also after graduation and during clinical rounds.

Learning styles and satisfaction with educational activities of Saudi Health Science University Students.

OBJECTIVES: Medical knowledge is constantly changing; this puts pressure on educators to adapt instructional strategies to their students' learning styles (LSs). Therefore, identifying the LSs of medical students could help teachers to develop teaching strategies to achieve better outcomes. The purpose of this study was to determine the LSs of health science university students, and to assess the relationship between LSs and student satisfaction with educational activities. METHODS: This cross-sectional descriptive study was conducted in the Colleges of Medicine and Applied Medical Sciences, King Saud bin Abdulaziz University for Health Sciences (KSAU-HS) Jeddah. We used Kolb's LS and a modified Student Satisfaction Survey from Mott Community College, Michigan. All participants received self-administered questionnaires; LS and demographic data were used as predictor variables. Students' satisfaction levels were considered the outcome variable. RESULTS: A total of 359 students were recruited (mean age 19.0 ± 1.4 years; 53.5% males). The most popular LS was Accommodator (29.2%), followed by Assimilator (27.9%) and Diverger (25.6%), while the Converger style (17.3%) was the least preferred LS. The satisfaction scores of the students showed that they were generally satisfied with a mean score of 64.4%. Males were found to have higher satisfaction across all variables than females (p = 0.002). CONCLUSIONS: This study could not find a predominant LS or satisfaction difference across LSs among health science students. Thus, the current educational programmes in KSAU-HS meet students' LSs and satisfaction. Educators need to broaden their strategies for instruction, so as to build an effective learning environment.

Clinical Patterns and Linear Growth in Children with Congenital Adrenal Hyperplasia, an 11-Year Experience.

OBJECTIVE: An important goal in treating children with congenital adrenal hyperplasia (CAH) is to achieve a normal final adult height (FH). The aim of this study was to describe the clinical presentations and evaluate linear growth and possible factors affecting it in children with CAH. METHODS: This is a retrospective study of 56 patients with CAH followed up in a tertiary center for 11 years. Patient's data including demographics, clinical, anthropometric, and laboratory information at presentation and during follow-up period were collected from medical records. RESULTS: Fifty-six children (31 females) with CAH were seen at KAMC-Jeddah over 11-year period and 91% were 21-hydroxylase deficient. Of these, 46.4% had hyponatremia and 28.6% had hyperkalemia (21.4% had hyponatremia and hyperkalemia) at presentation. Positive family history was documented in 53.6%. Ambiguous genitalia were present in 72% of females and the majority required corrective surgery. Males had significantly decreased HtSDS versus females and females had significantly higher body mass index. The HtSDS of children who had had higher 17OHP or salt-losing crisis during treatment was significantly lower than those who had normal 17OHP and those who did not have salt-losing crisis, respectively. CONCLUSION: The final height outcome in our patients with CAH treated with glucocorticoids is lower than the population norm. Proper control of the disease clinically and biochemically through strict compliance to medical therapy as well as close clinical and laboratory monitoring is an important key to achieve normal final adult height in these patients. Side effects, including overweight, obesity, and hypertension are true risk associations and need timely diagnosis and early management.

Incidence of diabetic ketoacidosis in newly diagnosed type 1 diabetes children in western Saudi Arabia: 11-year experience.

Background A wide range of reports on the incidence of diabetic ketoacidosis (DKA) at the onset of type 1 diabetes mellitus (T1DM) in children have been published worldwide. Reports from Saudi Arabia are limited. The aim of this study was to assess the incidence, clinical pattern and severity of DKA in children with newly diagnosed T1DM and the association of autoimmune conditions with initial DKA occurrence at King Abdulaziz Medical City - Jeddah. Methods This retrospective chart review was conducted during the period 2005-2015. All newly diagnosed T1DM children during the study period were investigated (n = 390). Data were collected on the demographic characteristics, body mass index (BMI), DKA severity, length of hospital stay and follow-up data on the type of diabetes therapy. Results The incidence of DKA among newly diagnosed T1DM pediatric patients was 37.7% (n = 147). Moderate and severe DKA cases were significantly higher among female children (p = 0.04). Patients diagnosed with DKA had lower BMI (20.87 ± 5.21) than their counterparts (p = 0.03). The median length of hospital stay was higher among severe DKA compared to moderate and mild cases (5.0, 4.5 and 4.0 days, respectively). Conclusions The incidence of DKA among newly diagnosed T1DM is still high compared to developed countries; however, it is relatively lower than previous reports in Saudi Arabia. Immediate interventions, such as awareness campaigns, are vital to reduce the burden of this preventable health sequela among children with DM.

Glycemic Control in Children and Youth With Type 1 Diabetes Mellitus in Saudi Arabia.

OBJECTIVE: To determine the glycemic control and cardio-metabolic complications of children and adolescents with type 1 diabetes mellitus (T1DM) attending a tertiary care diabetes clinic in Saudi Arabia. METHODS: We conducted a retrospective cross-sectional study of children and adolescents with T1DM attending King Abdulaziz Medical City-Jeddah from 2010 to 2013. We assessed their glycemic control and diabetes management. Vitamin D status was compared with glycemic control and gender differences. RESULTS: We identified 301 subjects (53.5% females); mean age was 13.9 ± 3.8 years. The mean duration of diabetes was 7.7 ± 3.7 years, body mass index (BMI) was 21.1 ± 4.5 kg/m2, and hemoglobin A1c (HbA1c) was 9.6% ± 1.9% in both genders. There were modest gender-specific differences in Saudi patients with T1DM, with males having more symptoms than females. Mean age at diagnosis of T1DM was slightly younger in males (6.01 ± 3.65 years) than in females (6.33 ± 3.45 years). Education was the most common reason for admission in males (32.9%), whereas diabetic ketoacidosis (DKA) was the most common reason in females (38.8%). Frequency of symptomatic hypoglycemic attacks was relatively higher in males (47.1%) than in females (42.9%). The majority of our patients (83%) were on intensive insulin regimen, having 4 injections or more per day. The remaining (17%) were on conventional insulin therapy. Only 26.2% had satisfactory HbA1c (⩽8%). The mean level of 25-hydroxyvitamin D was 35.15 ± 15.9 nmol/L and cholesterol was 4.75 ± 1.1 nmol/L. Vitamin D deficiency (25-hydroxyvitamin D ⩽ 37.5 nmol/L) was detected in 63.6% males and 67.7% females. No significant correlation between HbA1c and vitamin D deficiency was observed. CONCLUSIONS: Metabolic control among Saudi children with T1DM is less satisfactory compared with other countries. The high prevalence of vitamin D deficiency in this population supports the recommendation of vitamin D supplementation in T1DM subjects. Further studies in a larger cohort are needed to confirm our findings.

Molecular genetics and phenotype/genotype correlation of 5-α reductase deficiency in a highly consanguineous population.

CONTEXT AND OBJECTIVES: 5-α reductase deficiency is a rare 46,XY disorder of sex development. We present detailed phenotypic and genotypic features of a cohort of 24 subjects from a highly consanguineous population of Saudi Arabia SUBJECTS AND METHODS: We studied the clinical presentation and hormonal profiles of 24 subjects diagnosed with 5-α reductase deficiency and performed genetic testing on DNA isolated from their peripheral blood using polymerase chain reaction and direct sequencing of the SRD5A2. RESULTS: All subjects had 46,XY karyotype and presented with atypical appearance of external genitalia ranging from clitoromegaly, micophallus with hypospadias, undescended testes to completely normally looking female genitalia. Thirteen (54%) of them had severe under virilization and were assigned female sex at birth. The other 11 subjects were raised as males. Stimulated Testosterone:Dihydrotestosterone ratio was high in all 16 subjects in whom it was measured. The genetic testing revealed 2 nonsense mutations (p.R103X and p.R227X) in 2 unrelated subjects, 3 missense mutations (p.P181L, p.A228T, p.R246Q) in 11 subjects and a splice site mutation (IVS1-2A > G) in 11 other subjects. There was significant phenotypic variability even in subjects with the same mutation and also within the same family. CONCLUSION: This is the first and largest report of the clinical and molecular genetics of 5-α reductase deficiency from the Middle East. It shows weak genotype/phenotype correlation and significant phenotypic heterogeneity. IVS1-2A > G mutation is the most common mutation and is likely to be a founder mutation in this part of the world.