p.p1 patients had a normal height, about (25.6%) suffered

Topic: Parenting
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Last updated: July 9, 2019

p.p1 {margin: 0.0px 0.

0px 12.0px 0.0px; font: 12.0px ‘Times New Roman’; -webkit-text-stroke: #000000}span.s1 {font-kerning: none}span.s2 {font: 8.0px ‘Times New Roman’; font-kerning: none}This study describes the long-term effect on patients with classic 21OHD and non-classical CAH at a single institute.

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In an effort to address suboptimal growth in those patients, replacement therapy is the only salvage to optimize their growth and maintain their life. Glucocorticoid therapy remains the standard treatment for patients with CAH, with a goal to reach the optimal level of adrenal steroid suppression.     The optimal therapeutic range is difficult to attain, and often short an long-term complication arises, reflecting over or under treatment 9,10. Growth suppression in CAH children is multifactorial, and it’s still not clear which factor contributes the most to these complications.

Many researchers have extensively studied the possible causes; starting from the clinical presentation, age at start of therapy, glucocorticoids dosage, and control Glucocorticoids alone are widely known to suppress the linear growth 11, 12. Moreover, the chronic treatment with glucocorticoid, even at therapeutic doses, has been associated with poor growth 12. A study published in BMJ suggests, high hydrocortisone dosage used during early childhood, especially during infancy period, lead to growth impairment and might be linked to a permanent loss of final length 13, 14. Moreover, CAH if not treated, the disease itself will accelerate the velocity of fusion of epiphysis resulting in a limitation in the height potential in those patients 9, 15.  In this study, most of the patients had a normal height, about (25.6%) suffered from short stature.

It could be attributed to familial short stature in those patients because their target height falls below the 3rd percentile, and the mean of mid-parental height who their offsprings suffered from short stature is 159.8 cm. Evidence derived from observational studies suggests that patients with congenital adrenal hyperplasia CAH especially classical CAH usually reach a reduced in final adult height opposed to their calculated target height. While there is general agreement on reduced final height in this population, there are yet undiscovered factors affecting growth and optimal approaches to improve final height in CAH patients. Retrospective studies have shown that the adult height of treated patients is independent of the level of hormonal regulation, which might suggest that hypercortisolism contributes to the observed short stature. Moreover, final height SDS and height SDS were not significantly associated with age at diagnosis, gender, mid-parenteral height (?=0.

415), the dose of hydrocortisone, and disease control. Most children who were compliant with medication had a normal height (p-value= 0.013). Furthermore, in our institute; the 5 years follow up rate was approximately 71.9% in CAH patients, with 65.

5% follow up rate. Mean of mid-parental height who their children not suffering from short stature is 162.3 cm, which may suggest that patients with CAH diagnosed early, regularly followed and treated exclusively with hydrocortisone can have a satisfactory final height prognosis and limit short stature to some extent.

 CAH children can also suffer from obesity, the cause of obesity among those patients remains uncertain but many factors play a major role in it. It is widely speculated that obesity is linked to glucocorticoid dosage 16. Moreover, an interesting hypothesis states that chronic adrenal hypofunction with decreased adrenaline synthesis may also have a role in the development of obesity 17. Patients with classic 21-hydroxylase deficiency display significantly lower plasma concentrations of epinephrine and metanephrine as well as urinary epinephrine excretion than normal healthy subjects 17. Underweight subjects were 13 (19.1%), 39 (57.

4%) were normal weight, 4 (5.9%) were overweight and 12 (17.6%) were obese. Underweight children may be attributed to a poor nutritional status of these patients and low socioeconomic status. Obese children had at least one BMI measurement ?95 percentile and had at least one reading  ?85 percentile. Our data showed a significant association between hydrocortisone dose, BMI, and BMI SDS. We can note that most obese children were taking more than 15 mg/m2 /day of hydrocortisone (p-value= 0.

008, and p-value= 0.027 respectively). Another study that was conducted in US had a similar result, about 50% of their sample size had a BMI reading above >95 percentile 17.

It’s essential to carefully adjust HC dosing in CAH children, especially during early childhood to prevent increased weight gain and an early adiposity rebound 18. The adiposity rebound is defined as the second increase in BMI that happens in preschoolers until the age of  7. An early age at adiposity rebound is a risk factor for adult obesity 19. Compliance and regular follow up and monitor is one of the important factors that can affect the final height dramatically. Properly treated children with excellent compliance show better results than non-compliant patients 9, 15.

The dose of steroid was quite variable, i.e. <12 mg/m2 /day and more than >15 mg/m2 /day of hydrocortisone, averaging between 12 to 15 mg/m2 /day, 38% were clinically controlled on that dose.

35.7% controlled on the minimal dose of <12mg/m2/day of hydrocortisone and 26.2% were controlled with a higher dosage >15mg/m2/day. Mean of the age of clinically controlled were = 17.7 ± 25.8 months. Mean of age among of un-clinically controlled= 5.5 ± 5.

9 months. Different clinical presentations in young CAH may contribute to control. The earlier the presentation, the earlier to initiate the treatment. Family history is also an important factor to consider in those patients, as such, family history of obesity was found in (8.7%) of obese children.

On the other hand, other endocrine disease plays a major role in gaining weight. Hypothyroidism was a common finding in (13.6%) of obese children. Also, other factors can affect the weight drastically, is the lifestyle. Saudi Arabia suffers from high obesity rate, and recently obesity has been a major concern in the country. Latest studies have revealed that obesity in both genders is 28.7% of the total population (prevalence among (33.

5%) of females and (24.1%) of males) 20. It’s considered one of the highest countries in the world. This could be attributed to westernization of the society, high caloric consumption, and decreased physical activity 10.

Family history of consanguinity was a common finding in CAH patients; about 31.1% of all patients had a positive consanguinity. However, family history of consanguinity is not associated with a stronger variant of CAH. There’s no association between consanguinity with control, initial of presentation, sever presentations (shock or salt-losing crisis), response to medication, or frequent hospital admission.

            We conclude that personalized treatment approach can prevent growth failure. Good follow up and compliance is an important factor. As well as, close monitoring and treatment which can affect the height, weight, health status, and quality of life for the patient. Majority of our patients had normal growth parameters due to good follow up and compliance with treatment; while a quarter had an abnormality in weight and height.               We highly recommend educating the parents, caregivers, children about the potential outcomes of the disease and side effects of medications.  Global neonatal screening should be available all over the hospital for early detection and intervention.

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