Adverse Effects of Ramadan Fasting in a Girl with Salt- Losing Congenital Adrenal Hyperplasia

Abstract

Introduction: Congenital Adrenal Hyperplasia (CAH) is the most common cause of adrenal insufficiency in pediatrics. Chronic glucocorticoid replacement is the mainstay of treatment in the classic forms of CAH and mineralocorticoids replacement therapy is mandatory in salt-wasting form. Fasting is a mild stressor, which can expose to dehydration, hypotension, hypoglycemia and acute adrenal crisis in patients with adrenal insufficiency.

Case: We report the case of an adolescent affected by classic form with salt-losing CAH, who observed Ramadan for 30 days, without individualized therapeutic management plan. After Ramadan, a dramatic increase of ACTH level (1081 pg/ml), reduced cortisolemia, tendency to hypotension and weight loss was recorded. She experienced insomnia, intense thirst, asthenia and headache. The symptoms disappeared restarting the previous therapy schedule and increasing the total hydrocortisone daily dose with progressive restoring of hormonal control.

Conclusion: Our case confirm that patients with CAH are vulnerable, especially during fasting in Ramadan, with a higher risk of acute adrenal crisis. CAH patients should not be encouraged to fast, but if they do so, they will need individualized therapeutic plan and careful monitoring.

Keywords: Congenital adrenal hyperplasia; Ramadan; Fasting; Acute adrenal crisis; Personalized treatment

Introduction

Congenital adrenal hyperplasia is the most common cause of adrenal insufficiency in pediatrics [1]. CAH comprises a family of autosomal recessive disorders that impairs adrenal steroidogenesis; the main form is due to 21-hydroxylase deficiency associated with mutations in the 21-hydroxylase gene (6p21). In the case of 21-OHD, the phenotypic spectrum ranges from virilization associated to saltlosing, to simple virilizing forms, to mild forms. The “classic form” includes salt-losing and simple virilizing forms. The mild form is also known as “late-onset or non-classic form” [1]. However, this classification system is somewhat artificial because disease severity due to a continuum based on residual enzyme activity [2]. Chronic glucocorticoid replacement is the mainstay of treatment in the classic forms of CAH. Mineralocorticoid replacement is also necessary in salt-wasting form. Patients in glucocorticoid treatment must double or triple hydrocortisone dose during intercurrent illness (fever, gastrointestinal illness), stressful life events, surgery, or trauma in order to prevent adrenal crisis [3].

Fasting is usually considered as a mild stressor. It can expose to dehydration, hypotension, hypoglycemia and acute adrenal crisis patients with adrenal insufficiency. Ramadan fasting is the fourth pillar of Islam. During this holy month, Muslims must abstain from drinking, eating and even taking oral medications, from sunrise to sunset. This practice is request to all the Muslims, beginning from adolescence, excluding pregnant women and ill subjects in which Ramadan can be dangerous [4]. Nevertheless, some people with chronic conditions still want to adhere fasting during Ramadan. Here we report the case of an adolescent affected by classic form with salt-losing CAH, who observed Ramadan for 30 days, without individualized therapeutic management plan.

Case Report

Patient is a 13 years old girl, third child of non-consanguineous parents, born at the end of a normal course pregnancy, by eutocic birth. Increased 17-OHP level (252 nmol/L) was found at Guthrie neonatal screening and confirmed by a second test (440 nmol/L). Classical CAH form with salt losing was diagnosed and replacement therapy with hydrocortisone, acetate fludrocortisone and NaCl oral supplementation was therefore started. During infancy and childhood, there were no remarkable events in her medical history except for episodes of febrile convulsions; secondary enuresis at the age of 6 was treated with oxybutynin for 6 months. Growth and neuropsycomotory development were normal. Menarche occurs at 12 years. During the follow-up (10 years), therapeutic adjustment in medications were related to weight gain and stress periods (fever). Despite the adherence of the therapy was not always optimal, major complications never occurred. The data at the last check-up visit [4] months before Ramadan beginning) was reported in (Table 1). On May 5^th 2019, without consulting our Clinic or agreeing on therapeutic plans, the girl begins Ramadan, which she completes on June 3^rd 2019 (30 total days). During Ramadan, the girl observed fasting from sunrise to sunset, having dinner at 9pm, a snack at midnight and an abundant breakfast at 4am. Consequently, the patient modified therapy schedule as reported in (Table 1). At the periodic endocrinological evaluation, which almost coincided with the end of Ramadan (June 6^th 2019), we found dramatic increase of ACTH level, reduced cortisolemia, tendency to hypotension and important weight loss (-12.5% of body weight), (Table 1).

TABLECREATED

Table 1: Hormonal panel (8am), blood pressure, body weight and therapy of our patient before, during and after Ramadan.