Association between genotype, clinical presentation, and severity of congenital adrenal hyperplasia: a review.

Congenital adrenal hyperplasia (CAH) applies to a family of inherited disorders of steroidogenesis caused by an abnormality in one of the five enzymatic steps necessary in the conversion of cholesterol to cortisol. The enzyme defects are transmitted as an autosomal recessive trait. Patients with a "classical" form of CAH usually present during the neonatal and early infancy period with adrenal insufficiency, which could be associated with a salt- losing pathology. Females usually have genital ambiguity. Approximately 67% of classical CAH patients are classified as "salt-losing", while 33% have "non-salt-losing" or the "simple-virilizing" form, reflecting the degree of aldosterone deficiency. Non-classic 21-hydroxylase deficiency (NC 21-OHD) refers to the condition in which partial deficiencies of 21-hydroxylation produce less extreme hyperandrogenemia and milder symptoms. Females do not demonstrate genital ambiguity at birth. The gene for adrenal 21-hydroxylase, CYP21, is located on chromosome 6p in the area of human leukocyte antigen (HLA) genes. Specific mutations may be associated with a certain degree of enzymatic compromise and the clinical form of 21-hydroxylase deficiency (21-OHD). NC 21-OHD patients are predicted to have mild mutations on both alleles and one severe or one mild mutation of the 21-OH locus (compound heterozygote). This review aims to describe the association between the genotype and clinical presentations and severity of CAH.

The association of extreme body weight with bone mineral density in Saudi children.

Objective: The objective to investigate the effect of extreme body weight; obesity and undernutrition, on bone mineral density (BMD). Methodology: This study is a descriptive cross-sectional study carried between January and June of 2019, and included 224 children and adolescents without any comorbidities or chronic disease. Important data collected included anthropometrics, past medical and surgical history, history of medication intake, level of physical activity and pubertal assessment. Data entry and analysis were conducted using Statistical Package for Social Sciences version 24. Results: Gender distribution showed 48.2% were male and 51.8% were female. The mean age was 9.98 ± 3.5 years. Mean weight was 34.89 ± 18.2 kg. Mean BMD z-score was - 1.64 ± 1.4. Weight was considered to significantly correlate with BMD stature at a P = 0.014. Subjects who were underweight showed a lower mean BMD z-score of <-2 than those who were overweight/obese (mean BMD z-score = -1.60). Conclusion: Both extremes of weight are considered a significant risk factor for the development of low BMD in children. We recommend the early recognition of weight aberrations and consequent aggressive intervention with strict lifestyle modifications to promote the development of maximum peak bone mass.

Résumé Objectif: étudier l'effet du poids corporel extrême; l'obésité et la dénutrition, sur la densité minérale osseuse. Méthodologie: Cette étude est une étude transversale descriptive réalisée entre les mois de janvier et juin 2019, et a inclus 224 enfants et adolescents sans aucune comorbidité ni maladie chronique. Les données importantes recueillies comprenaient l'anthropométrie, les antécédents médicaux et chirurgicaux, les antécédents de prise de médicaments, le niveau d'activité physique et l'évaluation pubertaire. La saisie et l'analyse des données ont été effectuées à l'aide du progiciel statistique pour les sciences sociales (SPSS) version 24. Résultats: La répartition par sexe a montré que 48,2% étaient des hommes et 51,8% étaient des femmes. L'âge moyen était de 9,98 ± 3,5 ans. Le poids moyen était de 34,89 ± 18,2 kg. Le score z moyen de DMO était de -1,64 ± 1,4. On a considéré que le poids était significativement corrélé à la stature de la DMO à une valeur p de 0,014. Les sujets présentant une insuffisance pondérale ont présenté un score z de DMO moyen inférieur à <-2 que ceux qui étaient en surpoids/obèses (score z moyen de DMO = -1,60). Conclusion: Les deux extrêmes de poids sont considérés comme un facteur de risque important pour le développement d'une faible DMO chez les enfants. Nous recommandons la détection précoce des aberrations de poids et une intervention agressive conséquente avec des modifications strictes du mode de vie afin de favoriser le développement d'une masse osseuse maximale. Mots-clés: insuffisance pondérale; en surpoids; indice de masse corporelle; densité minérale osseuse; les enfants.

Polycystic Ovary Syndrome Among Female Adolescents With Congenital Adrenal Hyperplasia.

OBJECTIVES: Polycystic ovary syndrome is a common endocrine disease in adolescent females that is usually diagnosed based on clinical and hormonal abnormalities. Female adolescents with poorly controlled congenital adrenal hyperplasia are at increased risk of developing polycystic ovary syndrome. This study aimed to determine the prevalence of polycystic ovary syndrome and assess its relationship with hormonal control among adolescents with congenital adrenal hyperplasia. METHODS: This retrospective descriptive study included 40 pubertal female adolescents aged between 12 and 20 years with at least two years after menarche diagnosed with classical congenital adrenal hyperplasia since birth who were screened routinely for polycystic ovary syndrome via pelvic ultrasonography between 2012 and 2020 at King Abdul-Aziz University Hospital, Jeddah, Saudi Arabia. Serum adrenocorticotropic hormone, 17-hydroxy -progesterone, testosterone, dehydroepiandrosterone sulfate, luteinizing hormone, and follicle-stimulating hormone levels were measured. RESULTS: Polycystic ovary syndrome was detected via routine pelvic ultrasonography in 12/40 (30%) females. The median age of the affected females was 16.6 years, with the youngest female aged 12.5 years. The bone age of the patients had advanced ≤3 years. Further, serum adrenocorticotropic hormone was determined to be an independent factor affecting polycystic ovary syndrome development, indicating poor hormonal control (P = 0.005). CONCLUSION: Polycystic ovary disease is likely a complication of poorly controlled congenital adrenal hyperplasia disease. Therefore, increasing the awareness of polycystic ovary disease among congenital adrenal hyperplasia females via routine ultrasonography screening is advisable to facilitate the early diagnosis and improve disease management.

Testicular adrenal rest tumors in children with congenital adrenal hyperplasia.

OBJECTIVES: To identify testicular adrenal rest tumors (TARTs) prevalence among children with congenital adrenal hyperplasia (CAH) and to assess hormonal control role as a contributing factor for TART development. Testicular adrenal rest tumors are benign tumors complicating CAH. It affects adult males with CAH commonly, with a reported prevalence of 40% on average, and up to 94%. There is insufficient data regarding their prevalence in children aged above 4 years and adolescents. METHODS: This descriptive study included 21 children and adolescents with CAH (17 salt wasting type, 4 non salt wasting type) aged 4-20 years who were screened for TARTs by routine testicular ultrasonography from October 2012 to December 2020 at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Serum adrenocorticotropic hormone, 17-hydroxy progesterone, testosterone, dehydroepiandrosterone, and luteinizing hormone levels were measured. RESULTS: Testicular adrenal rest tumors were detected in 6 (28%) patients (median age: 12.5 years). The youngest affected child was 8 years old. All patients with TARTs were asymptomatic with impalpable testicular masses at the time of detection. Bilateral involvement was observed in 5 patients, while one patient had unilateral involvement. All patients with TARTs had poor hormonal control, and 4 patients had advanced bone age. CONCLUSION: Testicular adrenal rest tumors development has been shown to correlate with poor hormonal control. Annual screening of young children with CAH for TART development is crucial, even for asymptomatic or with impalpable testicular masses.

Impact of COVID-19 lockdown on glycemic control in children and adolescents.

OBJECTIVES: To identify how children and adolescents with type 1 diabetes were coping with their condition during the COVID-19 lockdown, by detecting differences in blood glucose control and in lifestyle, including diet, physical activity, and mood deterioration, before and during the lockdown. METHODS: This descriptive, cross-sectional study was conducted between April and June 2020 at King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia. Data were collected from interviews, using various forms of telecommunication. RESULTS: The total sample size was 150 patients, 48 (28%) of whom were males and 102 (72%) females. The mean age of the patients was 12.45 years. The lockdown was associated with a significant increase in patients' weight (p=0.001), body mass index (p=0.001), and blood glucose readings (p=0.007) compared to their values before the lockdown. Conclusion: A negative impact of the COVID-19 lockdown was found on blood glucose values and BMI, which may correlate with a lack of physical activity, increased consumption of carbohydrates and fast food, and mood deterioration.

The relationship between body weight and dietary habits with respect to the timing of puberty among saudi children and adolescents.

Objective: The objective is to investigate the timing of pubertal onset as determined by the development of secondary sexual characteristics in relation to body weight and dietary patterns among Saudi females. Methods: Children/adolescents visiting general and endocrinology pediatric clinics in King AbdulAziz University Hospital were invited to participate in this study. Female subjects between the ages of 5 and 20 years were included in this study, and those with syndromic disease, chronic comorbidities, endocrinopathies, organic causes of precocious puberty, positive family history of early pubertal onset, and under chronic medication were excluded from the study. Data were collected through clinical interviews with the consent of the legal guardians, and physical examinations were conducted. Results: A.total of 164 females were investigated. The mean age of thelarche, adrenarche, and menarche was 10, 11.3, and 12.2 years, respectively. We found a significant correlation between higher weight standard deviation and an earlier age of both thelarche and adrenarche. In addition, daily consumption of fast foods was significantly associated with an earlier menarchal age. Consumption of nonorganic poultry was linked to early thelarche. Conclusion: Efforts should be directed to increase public and community awareness that fast food consumption, inorganic poultry, and higher body weight are important modifiable factors that lead to an earlier onset of female puberty across different parameters: breast development, adrenarche, and menstruation.

RésuméObjectif: Étudier le moment de l'apparition de la puberté tel que déterminé par le développement de caractères sexuels secondaires en relation avec le poids corporel et les habitudes alimentaires chez les femmes saoudiennes. Méthodes: Les enfants / adolescents visitant les cliniques pédiatriques générales et d'endocrinologie de l'hôpital universitaire King AbdulAziz ont été invités à participer à cette étude. Les sujets de sexe féminin âgés de 2 à 18 ans ont été inclus dans cette étude, et ceux atteints de maladie syndromique, de comorbidités chroniques, d'endocrinopathies, de causes organiques de puberté précoce, d'antécédents familiaux d'apparition pubertaire précoce et sous traitement chronique ont été exclus de l'étude . Les données ont été recueillies lors d'entretiens cliniques avec le consentement des tuteurs légaux et des examens physiques ont été effectués. Résultats: Un total de 163 femmes ont été étudiées. L'âge moyen de la larche, de l'adrénarche et de la ménarche était respectivement de 10, 11,3 et 12,2 ans. Nous avons trouvé une corrélation significative entre un écart-type de poids plus élevé et un âge plus précoce de la larche et de l'adrénarche. De plus, la consommation fréquente de fast-foods était significativement associée à un âge ménarché plus précoce. Une consommation plus élevée de volaille non biologique était liée à la croissance précoce. Conclusion: Des efforts devraient être déployés pour sensibiliser le public et la communauté aux habitudes alimentaires et au poids corporel en tant que facteurs modifiables importants qui favorisent l'apparition précoce de la puberté féminine, indépendamment de leurs effets les uns sur les autres.

Endocrinopathies complicating transfusion-dependent hemoglobinopathy.

OBJECTIVES: To investigate the prevalence and significance of different endocrinopathies in children and adolescents with transfusion-dependent thalassemia and sickle-cell anemia. METHODS: This is a descriptive, retrospective study between January 2010 and July 2018 in King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Data was collected through reviewing electronic hospital medical records then filling out data collection sheets and was interpreted through the IBM SPSS Statistics for Windows version 20.0 (IBM Corp, Armonk, NY, USA). Results: The total sample size was 119 patients, gender equality was almost achieved with 55.5% being male and 45.5% being female. The most common endocrinopathies were identified in the following order of short stature (39.5%), diabetes mellitus (29.4%), hypogonadism (12.6%), osteopenia (12.6%), osteoporosis (9.2%), hypothyroidism (9.2%), hypocortisolism (3.4%), and hypoparathyroidism (2.5%). All of which were statistically significant in their relationship to hemoglobinopathies with the exception of osteopenia and osteoporosis. Hypogonadism and hypocortisolism were found to be statistically significant in their relationship to a positive history of splenectomy at p=0.026 and p=0.012. Short stature was found to be statistically significant in its relationship to the male gender with a p=0.001. Conclusion: Endocrinopathy is a frequent complication of hemoglobinopathies, for which the most common were found to be short stature, diabetes mellitus,  and low bone mineral density.

Quantitative ultrasound screening of bone mineral density on children with short stature.

OBJECTIVES: To assess bone mineral density (BMD) of children with short stature using quantitative ultrasound (QUS) and compare it to children with normal height. Methods: We conducted a descriptive, cross-sectional controlled study between May 2018 and February 2019 at various pediatric clinics in Jeddah, Saudi Arabia. In total, 219 children were included: 100 had short stature, and 119 were of normal height. Data were collected from one-on-one interviews, and BMD was measured using quantitative ultrasound. Results: Children with short stature had significantly lower BMD z-scores than children with normal height (pless than 0.05). The use of vitamin D supplements was related to higher BMD z-scores in children with short stature (p less than 0.05). A significant association was found between higher BMD z-scores, and both age (p=0.05) and height (p=0.02). Through a further division of children with short stature into those with and those without growth hormone deficiencies, we show that growth hormone deficiency was positively associated with lower BMD z-scores; however, the p-value was 0.06. Conclusions: Compared with children of normal height, those with short stature had lower BMD. Height, vitamin D supplementation, and age were all significantly correlated with higher BMD, while growth hormone deficiency was correlated with lower BMD.

Determinants of bone mineral density through quantitative ultrasound screening of healthy children visiting ambulatory paediatric clinics.

OBJECTIVES: To investigate the determinants of bone mineral density through screening healthy children using a non-invasive quantitative ultrasound measurement device. METHODS: A descriptive cross-sectional study carried out at King AbdulAziz University Hospital, Jeddah, Kingdom of Saudi Arabia. between May 2018 and January 2019 through interviewing, examining, and screening healthy children visiting general paediatric. Total sample size encompassed 450 children. The inclusion criteria were healthy children between the ages of 2 and 20 years. Exclusion criteria were previous pathological fractures, chronic medical diseases, or long-term medications. Data entry and analysis was conducted using Statistical Package for Social Sciences version 24 (IBM Corp, Armonk, NY, USA). Chi-square tests were used to determine the association between categorical variables, with calculated p<0.05 considered significant. With one-way Anova testing to study the relationship between categorical variables and continuous variables. Results: A significant association with bone mineral density (BMD) was found during first 2 years with height (p=0.015), vitamin D supplementation (p=0.03), and breastfeeding (p=0.025). A directly proportional relationship with BMD was found with pubertal status, physical activity, diet, sun exposure, and calcium supplement intake. CONCLUSION: This is a novel study in the investigation of the dietary, lifestyle and demographic determinants of bone mineral density in the healthy middle-eastern child otherwise unaffected by chronic medical or metabolic disease or exposed to long term medications that could have affected bone metabolism.

Impact of body mass index on high blood pressure among obese children in the western region of Saudi Arabia.

OBJECTIVES: To evaluate the impact of body mass index (BMI) on high blood pressure among obese children and adolescents in western region, Saudi Arabia.  Methods: Cross-sectional data were obtained from 306 (female: 140, male: 166) child, between August 2016 and March 2017. A questioner was filled by health professionals at ambulatory pediatric clinic followed by waist-hip circumference, height, weight, and blood pressure measurement. Diastolic blood pressure (DBP) and systolic blood pressure (SBP) were adjusted to gender, height, and age. World Health Organization growth standards were used to calculate BMI z-scores. Results: The mean age of subjects was 10.1 years. Body mass index increased SBP by 1.722 mmHg (p=0.001), and DBP by 0.901 mmHg (p=0.006) in boys, and 0.969 mmHg (p=0.036), and DBP by 0.704 mmHg (p=0.045) in girls. Waist hip ratio showed significant difference p=0.041, (p=0.0001) between male and female. Of the baseline characteristics, age greater than 11 years showed significant difference. Symptomatic manifestation of high blood pressure, family history of hypertension, level of activity, income level and post-secondary education in parents, did not show any significant results. Conclusion: Elevated BMI is associated with significantly increased diastolic and systolic blood pressure in obese children, especially in children older than 11 years.

Growth characteristics in children with congenital adrenal hyperplasia.

OBJECTIVES: To evaluate the growth parameters in congenital adrenal hyperplasia patients in Jeddah, Saudi Arabia.  Methods: This is a descriptive retrospective study over the period of 5 years. Data analysis was using Statistical Package for Social Science. The study included 90 participants in which 61 were girls and 29 were boys aged 0 to 18 years . They were evaluated in Pediatric Endocrinology Clinic at King Abdulaziz University Hospital in Jeddah, Saudi Arabia, between January  2012 and January 2017. RESULTS: A total of 90 subjects, of which 67.8% were females and 32.2% were males. Subjects who were underweight  constituted 19.1% of the population, while those who were obese were estimated up to 17.6% of the population. Of the children, 25.7% were suffering from short stature and 74.3% had normal height. Approximately 11.8% of the children who suffered from short stature also suffered from hypothyroidism. Mid-parental height of those who suffered from short stature is 159.8 cm. CONCLUSION: This study showed a significant effect of congenital adrenal hyperplasia on both height, weight, and body mass index. Risk factors includes glucocorticoids dosage, compliance to treatment, and regular follow up. Personalized treatment approach should be followed with all patients diagnosed with congenital adrenal hyperplasia as well as close monitoring and targeted therapy.

Flash glucose monitoring system may benefit children and adolescents with type 1 diabetes during fasting at Ramadan.

OBJECTIVES: To assess the benefit of using the flash glucose monitoring system (FGMS) in children and adolescents with type 1 diabetes mellitus (T1DM) during Ramadan fasting. Methods: A prospective pilot study of 51 participants visited the pediatric diabetes clinic at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia from between June until and July 2016. The FreeStyle® Libre™ FGMS (Abbott Diabetes Care, Alameda, CA, USA) was used. Hypoglycemia was defined as glucose values of less than 70 mg/dL, while hyperglycemia as glucose values of more than 150 mg/dL for all participants based on our institute's protocol. Results: Participants were able to fast for 67.0% of the total days eligible for fasting, whereas they did not fast on 33% of the days due to either hypoglycemia (15.4%) or non-diabetes-related reasons (17.6 %). None of the participants developed severe hypoglycemia. The mean number of hyperglycemic episodes during fasting hours was 1.29, per day, which was higher than that of hypoglycemic episodes (0.7). None of the participants developed diabetic ketoacidosis (DKA). Glycemic control with mean of estimated hemoglobin A1C reading during Ramadan (8.16 ± 1.64% [pre study]) to 8.2 ± 1.63% [post study] p=0.932. Conclusions: Children and adolescents with T1DM who use the FGMS could fast without the risk of life-threatening episodes of severe hypoglycemia (namely seizure, coma), or DKA during Ramadan. Adequate education and good glycemic control prior to Ramadan are important strategies in combination with the use of an FGMS to achieve better outcome.

Safety & Efficacy of Cyclic Zoledronic Acid Therapy on Pediatric Secondary Osteoporosis.

BACKGROUND/AIM: Osteoporosis is a systemic disease characterized by decreased bone density and increased tendency to develop fractures. Osteoporosis in children and adolescents is a rare disease usually secondary to Medical conditions or medications given to children. The condition affects normal bone growth and development and carries with it multiple morbidities (physical and psychological) if not corrected promptly. This study aims to share our experience with Zoledronic Acid Therapy in Pediatric patients with secondary osteoporosis. METHOD: A retrospective study which included 46 patients aged 3 to 18 years. All patients received specific doses of Zoledronic acid and were followed up at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. Clinical and laboratory data were collected for each patient from their files. Adverse events were also recorded. RESULTS: The use of Zoledronic Acid in children and adolescents appears to be statically significant reduce fracture rate (p=0.005), bone turnover markers (Osteocalcin p= 0.003, CTX p= 0.008) and pain frequency in symptomatic individuals (p=0.000). Careful selection of cases is required to provide maximum benefits compared to risks associated with therapy. CONCLUSION: This study demonstrates that Zoledronic acid has positive effects on clinical outcome and bone marker level as well as quality of life for Pediatric patients with Osteoporosis and their families, with no long-term side effects.

The association between body mass index and duration spent on electronic devices in children and adolescents in Western Saudi Arabia.

OBJECTIVES: To evaluate the relationship between body mass index (BMI) and the duration spent on electronic devices, and to assess the factors that can cause obesity among children. METHODS: A cross-sectional study including 541 participants. Data was collected from March to June 2015 via ambulatory pediatric clinics in Jeddah, Kingdom of Saudi Arabia. The BMI standard deviation was calculated based on Center of Disease Control and Prevention (CDC) standards. RESULTS: The mean age of the participants was 10.1 years. Children who spent ≥2 hours daily on electronic devices showed an increased BMI, and made up 68.4% of the sample. CONCLUSION: An increased BMI was more common among children who spent ≥2 hours daily on electronic devices. The relationship between BMI, reduced physical activity, and eating during television viewing was determined.

Correlation between obesity and emotional, social, and behavioral problems associated with physical limitation among children and adolescents in Western Saudi Arabia.

OBJECTIVES: To evaluate the impact of body mass index (BMI) on limitation of physical activity and the associated effect on behavioral, school, and social problems among obese children in Western Saudi Arabia. METHODS: A cross-sectional obesity survey was conducted in Jeddah, Saudi Arabia. Data were collected between August 2014 and February 2015 from 281 obese children aged between 2-18 (girls: 130, boys: 151). Participants were selected randomly to represent different economic status, level of education, and family structure.  RESULTS: The mean age of participants was 10.5 years for girls and 10.6 years for boys. Higher BMI was associated with physical activity problems in walking (50.6%), running (55.8%), and exercise (44.3%), with school problems in attention (37.4%) and follow-up duties fade (31.3%), and with social problems in doing things other children could do (42.8%), playing with others (46%), and continuing play (33.4%). There was no significant relationship between BMI and emotional problems and school attendance.  CONCLUSION: Higher BMI significantly increased physical limitation, problems maintaining attention in school, and social problems among obese children. However, emotional problems and school attendance did not show a significant correlation with BMI.

Diverse etiology of hyperlipidemia among hospitalized children in Western region of Saudi Arabia.

OBJECTIVES: To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system.  Results: Of the 253 children who were reviewed, those who have shown to have abnormal lipid metabolism with nephrotic syndrome were 35.6%, diabetes mellitus 17.8%, primary/idiopathic hyperlipidemia 19.4%, hypothyroidism 7.1%, obesity 4.3%, metabolic syndrome 2.8%, chronic liver disease 2% and chronic renal failure 1.2%. The body mass index relative to gender and age in this group of children showed that 23.2% were underweight, 38.4% were normal weight, 8.9% were overweight, and 29.5% were obese.  Conclusion: The highest prevalence of hyperlipidemia was in nephrotic syndrome, followed by primary/idiopathic hyperlipidemia and diabetes mellitus.

The association between body mass index and duration spent on electronic devices in children and adolescents in Western Saudi Arabia.

[No Abstract Available].

Osteoporosis treatment with zoledronic acid in pediatric population at a university hospital in Western Saudi Arabia. A 13-year experience.

OBJECTIVES: To highlight the clinical benefit, efficacy, and safety of zoledronic acid (ZA) therapy in children and adolescents with primary and secondary osteoporosis. METHODS: This is a retrospective observational study of 131 children and adolescents visiting the Pediatric Endocrine Clinic at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia, between January 2002 and January 2015. Clinical and laboratory data were collected for each patient and adverse events were evaluated. RESULTS: The mean patient age was 11.43 years. There was a significant decrease in the number of fractures after ZA treatment for primary osteoporosis (p=0.000) and in secondary osteoporosis (p=0.005). There was a significant decrease in both osteocalcin (p=0.001) and C-terminal telopeptide (p=0.003) in patients with primary osteoporosis, as well as osteocalcin (p=0.003) and C-terminal telopeptide (p=0.008) in patients with secondary osteoporosis after treatment. CONCLUSION: The use of ZA in children and adolescent appears to have favorable effects on fracture rate and quality of life, including pain and mobility in symptomatic individuals. Intravenous ZA is comparable to other bisphosphonate agents in its efficacy and safety and features a more convenient infusion protocol with no documented long-term complications, thus, we advise its use in pediatric population.

Glycemic control, complications, and associated autoimmune diseases in children and adolescents with type 1 diabetes in Jeddah, Saudi Arabia.

OBJECTIVES: To investigate the relationship between metabolic control, acute and long-term complications, the coexistence of autoimmune diseases, and to assess the different factors that can affect the glycemic control level among children with type 1 diabetes mellitus (T1DM). METHODS: This is a cross-sectional study that included 228 T1DM children and adolescents visiting the pediatric diabetes clinic at the King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia from January 2013 to January 2014. The clinical and laboratory characteristics of the patients were recorded. Metabolic control, complications, and associated autoimmune diseases were evaluated. RESULTS: The mean age of patients was 10.99 years, and the glycated hemoglobin (HbA1c) level was 8.8%. Acute complications included ketoacidosis in 65.4% of patients, and hypoglycemic attacks in 68.9%. Long-term complications were detected in patients including retinopathy (4.4%), microalbuminuria (16.2%), and dyslipidemia (8.3%). Autoimmune thyroiditis was noted in 14%, and celiac disease was found in 19.7% of patients. A significant difference was found in pubertal and pre-pubertal age groups in terms of glycemic control (p=0.01). CONCLUSION: The level of HbA1c was found to be higher among the pubertal age group. A relationship between autoimmune diseases and gender was determined.

Salt losing nephropathy simulating congenital adrenal hyperplasia in an infant.

Pseudo-hypoaldosteronism occurring predominately in male infants has been reported in association with a spectrum of urologic diseases including obstructive uropathy. This is thought to reflect tubule unresponsiveness to aldosterone. We report a case, which was misdiagnosed as a case of congenital adrenal hyperplasia and treated inappropriately with hydrocortisone and fludrocortisone for 12-months before he had a urinary tract infection and was discovered to have obstructive uropathy on ultrasound. He presented with vomiting, dehydration, hyperkalemia, hyponatremia and metabolic acidosis. His initial 17 hydroxyprogestrone was high. His electrolytes improved to normal after relieving the obstruction by vesicostomy and his treatment weaned slowly without complications. This case demonstrates the importance of urine culture and ultrasound examination in suspected cases of pseudo-hypoaldosteronism.