Salivary Cortisol Measurement Contamination After Oral Hydrocortisone: A Randomized Crossover Trial.

The study aimed to evaluate salivary cortisol (SC) contamination and determine the associated factors in secondary adrenal insufficiency (SAI) patients treated with hydrocortisone (Hc). A randomized crossover trial involved SAI patients. SC was measured before the morning Hc dose, then at one, two, and four hours after. The procedure was performed twice on two days of a week: one day while taking Hc in tablet form (tablet set) and one day while taking Hc in capsule form (capsule set). Area under the curve (AUC) of SC levels over time was calculated in each participant for the two sets. SC contamination was defined as AUCtablet above the 95th percentile of AUCcapsule. Thirty-four patients (24 females and 10 males) with a median age of 48 years were enrolled. Post-Hc dose SC levels were higher in tablet than in capsule set, particularly at one hour. Prevalence and extent of SC contamination were estimated to 32% and 88%, respectively. In capsule set, SC measured two hours after Hc intake showed the strongest correlation with AUC (r=0.88, p<0.001). In multivariate analysis, serum potassium≥3.9 mEq/l was the only predictor for SC contamination [multi-adjusted OR (95% CI): 7.1 (1.4-36.1); p=0.018]. SC measured during the two hours after Hc intake is inaccurate for glucocorticoid replacement therapy assessment in SAI patients treated with Hc in tablet form.

Long-term complications of permanent hypoparathyroidism in adults: prevalence and associated factors.

INTRODUCTION: Patients with Chronic hypoparathyroidism (CHPT) receiving conventional treatment are exposed to several long-term complications including basal ganglia calcifications, posterior subcapsular cataract, kidney stones, and renal insufficiency. The aim of this study was to assess the prevalence and the associated factors of these complications in patients with CHPT. METHODS: We conducted a cross-sectional study including 58 patients with CHPT. All participants underwent physical examination, biochemical assessment (total serum calcium, serum phosphorus, serum albumin, intact-PTH, serum magnesium, 25-hydroxy-vitamin D, serum creatinine, thyroid stimulating hormone (TSH), and 24-hour urinary calcium), slit lamp examination, brain computed tomography scan (CT-scan), and renal ultrasound. RESULTS: Participants had a mean age of 52.6 ± 16.4 years and a gender ratio (women/men) of 3.5. Fahr syndrome, cataract, urolithiasis, and renal failure were found in 55%, 62%, 12%, and 17% of cases, respectively. CHPT duration >15 years (Adjusted-OR = 43.1, 95-CI: 2.63-703.06, p = 0.008) and poor adherence to treatment (Adjusted-OR = 8.04, 95%-CI: 1.52-42.42, p = 0.014) were independently associated with the risk of Fahr syndrome. Age >55 years (adjusted-OR = 5.07, 95-CI: 1.10-23.42, p = 0.037), disease duration >15 years (adjusted-OR = 20.21, 95-CI: 1.54-265.84, p = 0.022), and magnesium level <0.8 mmol/l (adjusted-OR = 36.46, 95-CI: 3.75-354.08, p = 0.002) were independently associated with the risk of subcapsular cataract. Only hypercalciuria (Adjusted-OR = 21.27, 95-CI: 2.31-195.91, p = 0.007) was an independent risk factor for kidney stones. Renal failure was not associated with kidney stones (p = 1). However, creatinine clearance was negatively correlated with age (r = -0.784; p < 10-3) and disease duration (r = -0.352; p = 0.007). CONCLUSION: Our results revealed high prevalences of neurological, ocular, and renal complications in patients with CHPT and emphasized the importance of regular biological monitoring, therapeutic adjustments, screening, and adherence to treatment in the prevention of these complications.

Assessment of quality of life in patients with permanent hypoparathyroidism receiving conventional treatment.

Introduction: Patients with permanent hypoparathyroidism suffer from multiple complaints and are exposed to long-term complications that might compromise their well-being. The aim of this study was to assess the quality of life (QoL) in patients with permanent hypoparathyroidism receiving conventional therapy and to determine the associated factors. Methods: This was a cross-sectional matched case-control study including 53 patients with permanent hypoparathyroidism and 53 matched controls. Biochemical blood parameters (calcium, phosphate, albumin, magnesium, 25-hydroxy-vitamin D, creatinine, TSH, and PTH) and 24-hours calciuria were measured in patients with hypoparathyroidism. QoL was assessed in all participants using the Short Form 36 Health Survey (SF-36). Results: The study included 53 patients (41 women and 12 men) with hypoparathyroidism receiving conventional therapy.Their mean age was 52.8 ± 16.5 years. In comparison with controls, patients with hypoparathyroidism had significantly lower scores in all eight domains of SF-36 (p < 10- 3). Patients with poor socioeconomic conditions had lower SF-36 scores than those with good conditions.The etiology of hypoparathyroidism, the disease duration, the control of the disease, and the body mass index did not significantly interfere with SF-36 scores. SF-36 total score was negatively correlated with age (r=-0.619, p < 10- 3) and symptoms of hypocalcemia (r=-0.284, p = 0.039), and positively correlated with creatinine clearance (r = 0.559, p < 10- 3), magnesium level (r = 0.345, p = 0.011), and 25 hydroxy-vitamin D level (r = 320, p = 0.021). No significant correlations were found between SF-36 scores and other biological parameters such as calcemia, phosphatemia, phosphocalcic product, PTH, TSH, and calciuria. Conclusion: Patients with permanent hypoparathyroidism had impairment in their QoL. Age, socioeconomic conditions, renal function, magnesium level, and 25 hydroxy-vitamin D level may interfere in the decline of the QoL of these patients.

Metabolic disorders during endogenous Cushing's syndrome: prevalence, associated factors, and outcome after remission.

Objective. The prognosis of Cushing's syndrome (CS) is related to a higher cardiovascular morbidity and mortality. This study aimed to determine the prevalence of metabolic disorders in patients with CS, the associated factors, and the rate of remission of these disorders after the remission from CS. Methods. It is a retrospective study including 75 cases of CS followed up at the university hospital La Rabta of Tunis from 1987 to 2018. Clinical and paraclinical data were collected from medical files. Results. The mean age of the patients was 44.1±18.9 years and the sex ratio was 0.39. At CS diagnosis, the frequencies of obesity, hypertension, diabetes, dyslipidemia, and metabolic syndrome were 52, 75, 43, 83, and 73%, respectively. The age, gender, body mass index, waist circumference, and baseline serum cortisol level were not associated with the presence of diabetes, hypertension or dyslipidemia. Forty-eight patients were operated on. At one year, 38 patients were in remission from CS. The remission rates of hypertension, diabetes, and dyslipidemia were respectively 58% (p<0.001), 76% (p<0.001), and 17% (NS). Conclusion. Metabolic disorders were frequent during CS and their frequencies decreased after the remission from the syndrome.

Spontaneous bladder rupture after non-traumatic vaginal delivery: a rare case report.

Spontaneous bladder rupture (SBR) is a rare condition and often missed diagnosis, especially after a non traumatic vaginal delivery. A 32-year-old para 3 woman, consulted for abdominal pain and anuria two days after instrumental vaginal delivery with forceps for foetal distress in second sate of labour. Blood tests were suggestive of an acute renal failure. An abdominocentesis revealed a clear fluid looking like ascites. The ultrasound and computed tomography (CT) scan showed a large abdominal effusion. An exploratory laparoscopy revealed a bladder perforation which was sutured after laparotomy. SRB is extremely rare after a non traumatic vaginal delivery. It is associated with significant morbidity and mortality. Symptoms are mostly non-specific. It is suspected when post partum abdominal pain is associated with an effusion and renal failure signs. If suspected, the uroscanner remains the gold standard for diagnostic. Laparotomy is the standard surgical approach in this condition. Abdominal pain with elevated serum creatinine should be suspicious of SBR in post-partum.

Thyroid hormone resistance syndrome caused by a novel mutation in the thyroid hormone receptor-beta gene (THRB, GLU457LYS) treated with methimazole.

A 15-year-old girl presented with hyperactivity and behavior disorders. She had tachycardia and no goiter. Thyroid hormones were high and TSH normal. A novel mutation GLU457LYS in THRB gene was observed. Methimazole and propranolol improved clinical symptoms but increased TSH level.

Management of patients with cardiovascular diseases during Ramadan.

During the month of Ramadan, over one billion Muslims observe a water and food fast from sunrise to sunset. The practice of this religious duty causes marked changes in eating and sleeping habits. With the increasing incidence of cardiovascular (CV) risk factors, the number of patients with CV pathologies who wish to fast is increasing worldwide, and in Tunisia, which is ranked as a high CV risk country. If fasting has been shown to be beneficial for the improvement of some metabolic parameters, its practice in patients with CV pathology remains debated. The Tunisian Society of Cardiology and Cardiovascular Surgery (STCCCV) in consultation with the National Instance of Evaluation and Accreditation in Health (INEAS) has established this document in the form of a consensus after having analysed the literature with the aim of addressing these questions: -What is the impact of fasting in patients with CV pathologies? -How to stratify the risk of fasting according to CV pathology and comorbidities? -How to plan fasting in patients with CV diseases? -What are the hygienic and dietary measures to be recommended during fasting in patients with CV pathologies? -How to manage medication during the month of Ramadan in patients with CV diseases?

Management of severe hypercalcaemia secondary to primary hyperparathyroidism: The efficacy of saline hydration, furosemide, and zoledronic acid.

INTRODUCTION: Severe hypercalcaemia is a life-threatening condition that should be managed urgently. The aim of this study was to assess the efficacy of saline hydration, furosemide, and zoledronic acid in the management of severe hypercalcaemia secondary to primary hyperparathyroidism (PHPT). METHODS: We conducted a retrospective analysis of the management of 65 patients with severe hypercalcaemia (≥3 mmol/L) secondary to PHPT. The efficacy of each therapeutic agent was evaluated according to the variation in serum calcium level calculated as Δ calcium = initial calcium level - minimal calcium level reached after the administration of each agent. RESULTS: The mean age of patients was 56.4 ± 13.8 years. At baseline, the mean total serum calcium level was 3.42 ± 0.40 mmol/L. After normal saline hydration, calcium level decreased from 3.25 ± 0.21 to 2.98 ± 0.2 mmol/L (p < 10-3 ) in 3.1 ± 1.7 days. Normalization of calcium level did not occur in any patient. Furosemide was prescribed in 35 patients. It resulted in a serum calcium increase of 0.09 ± 0.21 mmol/L. Calcium levels did not reach the normal range in any patient. Forty-five patients received intravenous zoledronic acid. The mean maximal reduction in serum calcium level was 0.57 ± 0.27 mmol/L (from 3.25 ± 0.26 mmol/L to 2.68 ± 0.22 mmol/L, p-value <10-3 ). Normalization of calcium levels occurred in 27 patients (60%). CONCLUSIONS: Our results show the absence of a significant additional effect of furosemide on calcium levels in patients with severe hypercalcaemia secondary to PHPT when compared with the effect of saline hydration alone. However, zoledronic acid was more potent. Thus, appropriate normal saline hydration with immediate intravenous bisphosphonates infusion should be considered in the management of severe hypercalcaemia in patients with PHPT.

A case of Hashimoto's thyroiditis following Graves' disease.

Graves' disease is characterized by the presence of circulating autoantibodies that stimulate the TSH receptor, inducing hyperthyroidism and goiter. Hashimoto's thyroiditis is an autoimmune disease leading to thyroid tissue destruction by cell and antibody-mediated immune processes. The occurrence of Hashimoto's thyroiditis following Graves' disease has been rarely reported. Its pathogenesis is not clear. Herein, we report the case of a 40-year-old woman who was referred to our department for thyrotoxicosis. Laboratory tests revealed overt hyperthyroidism. Thyroid scintigraphy showed an enlarged gland with diffusely increased tracer uptake, confirming the diagnosis of Graves's disease. The patient was treated with propranolol and thiamazole. Two months later, she received radioactive iodine therapy. Three years and 9 months later, the patient presented with hypothyroidism and very high levels of thyroperoxidase antibodies consistent with the diagnosis of Hashimoto's thyroiditis. She was treated with levothyroxine. The shift from Graves' disease to Hashimoto's thyroiditis was reported in the literature. However, its pathogenesis has not been clearly elucidated.

Professional practices in the management of adrenal insufficiency in two tertiary referral hospitals (Tunis, Tunisia).

BACKGROUND: Adrenal insufficiency (AI) is a rare and life-threatening disease. Glucocorticoid replacement therapy and patient education are crucial. Few is known about physician practice in this topic. AIMS: To describe physician practice in the management of AI and to identify the associated factors. METHODS: the physicians, all grades and specialties, from two university hospitals in Tunis, were invited to respond to a paper-based 16- multiple choice item-questionnaire about the management of AI and the prevention of acute AI. Each question was scored 1 if correct or 0 if incorrect. The global score was calculated by adding the score of the first 15 questions. RESULTS: 200 physicians responded to the questionnaire, sex ratio: 0.47, mean age: 29.0 ± 5.8 years (24 - 60). The overall rate of correct answers was 59.6%. The rate of correct responses was good for the type of replacement therapy (92%), the lifelong duration of treatment (88%), the symptoms of overtreatment (73.5%), the type of diet indicated (77%), and the necessity of special measures during the peri operative period (100%). However, the rate of correct responses was low for the half-life of hydrocortisone (12.5%), biological signs suggesting acute AI (17.5%), situations during which an increase in the dose of glucocorticoid is required (26.5%) and the risks of intermittent fasting (2%). Endocrinology specialty and overall medical specialties were independently associated with a better global score. CONCLUSION: physician practice in the management of AI need to be improved.

Interest of the waist-to-height ratio to predict metabolic syndrome in type 2 diabetic patients.

INTRODUCTION: Metabolic syndrome (MetS) is defined as a cluster of risk factors for cardiovascular disease. AIM: To determine the optimal cut-off point of the waist-to-height ratio (WHtR) at which MetS can be identified with maximum sensitivity and specificity in a sample of Tunisian type 2 diabetic patients. METHODS: We enrolled 457 type 2 diabetic patients in a cross-sectional study. Blood pressure, anthropometric indices, fasting glucose, and lipid profile were measured. WHtR was calculated. MetS was defined according to the IDF criteria. Receiver operating characteristic (ROC) curve analysis was used to identify the optimal cut-off value of WHtR in MetS screening with maximum sensitivity and specificity. RESULTS: The overall prevalence of MetS was 79.8%, it was higher in women than in men (85.5% vs 61.4%; p.

Diabetic patient satisfaction on the quality of outpatient health care services.

AIM: To assess the degree of satisfaction of diabetic patients with health care services and to determine the factors that influence it. METHODS: It was a cross sectional study conducted in 2018 among diabetic outpatients of the department of endocrinology of the university hospital La Rabta. Patients general characteristics were noted. Each patient responded to a questionnaire, asked orally, in Tunisian dialect, by two physicians, about health care services including 20 questions grouped into three items. Each question was scored from 1 to 4 according to the degree of satisfaction. The global score as well as the scores of the items were calculated by adding the scores of the corresponding questions. RESULTS: 150 diabetic patients responded to the questionnaire; mean age: 58.9±12.2 years [18-88], sex-ratio: 0.51. Cronbach coefficient was 0.78. The overall score was 62.2±6.5 [46-80]. Ninety-seven patients (64.7%) had a score ≥60. The item concerning 'human contact and communication' was considered good in 98% of cases. The item concerning 'premises, cleanliness, comfort and safety' was considered intermediate in 64% of cases. Patients were very satisfied with the accessibility of the department inside the hospital (76.7%), the attitude of the agents during the administrative formalities (74%) and particularly the behaviour of the doctor (96%). However, they were dissatisfied with the noise (72%) and overcrowding (67.4%) of the waiting room. There were no statistically significant associations between the overall score and the studied data. CONCLUSION: The diabetic patients were overall satisfied with the provided services. Actions must be taken to reduce overcrowding and long waiting times.

Anti Mullerian hormone as a diagnostic tool for polycystic ovary syndrome in women of reproductive age with morbid obesity.

OBJECTIVES: The aim of the study was to analyze the performance of the anti-mullerian hormone (AMH) level for the diagnosis of polycystic ovary syndrome in women with morbid obesity. STUDY DESIGN: A single-centre cross-sectional study was conducted in 50 women of reproductive age with a body mass index (BMI) ≥ 40 kg/m2. Each patient underwent a clinical examination, biological and hormonal assays, and an ovarian ultrasound between the third and the fifth day of the menstrual cycle. Polycystic ovary syndrome was diagnosed according to the Rotterdam's criteria. RESULTS: The mean age of participants was 34.2 ± 7.5 years. Polycystic ovary syndrome was diagnosed in 20 women (40%). Age and anthropometric parameters did not differ between women with and without polycystic ovary syndrome. The mean AMH level was significantly higher in women with polycystic ovary syndrome (3.4 ± 3.6 vs 1.3 ± 1.2 ng/ml, p=0.010). It was positively correlated with the Ferriman and Gallwey score (r=0.496, p=0.016), total testosterone level (r=0.524, p < 10-3) and the LH/FSH ratio (r=0.290, p=0.046). In women aged between 35 and 45 years, the optimum cut-off level for the diagnosis of polycystic ovary syndrome was 0.81 ng/mL, providing a sensitivity and a specificity of 90 and 71%, respectively with an area under the ROC curve of 0.857. CONCLUSIONS: AMH level was significantly higher in morbid obese women with polycystic ovary syndrome compared with those without polycystic ovary syndrome. Specific thresholds for this population must be assessed to improve the sensitivity and specificity of AMH for the diagnosis of polycystic ovary syndrome.

The remission phase in adolescents and young adults with newly diagnosed type 1 diabetes mellitus: prevalence, predicting factors and glycemic control during follow-up

ABSTRACT Objective: There is little data about the remission phase in adolescents and young adults with newly diagnosed type 1 diabetes mellitus (T1D). The aims of this study were to determine the prevalence of remission and its predicting factors among adolescents and young adults with newly diagnosed T1D and to assess the association between remission and long-term glycemic control in this population. Subjects and methods: This is a longitudinal and retrospective study including 128 type 1 diabetic patients aged between 12 and 30 years at diabetes onset. Clinical, biological and therapeutic features were collected at diagnosis and for 5 years after diagnosis. Remission was defined by an HbA1c < 6.5% with a daily insulin dose < 0.5 IU/kg/day. Results: Twenty-three patients (18%) experienced a remission. The peak of remission prevalence was at 6 months after diabetes diagnosis. An insulin dose at discharge <0.8 IU/kg/day was independently associated with remission (p=0.03, adjusted OR [CI 95%] = 0.2 [0.1-0.9]). A low socioeconomic level was independently associated with non remission (p=0.02, adjusted OR [CI 95%] = 4.3 [1.3-14.3]). HbA1c was significantly lower during the first five years of follow-up in remitters. The daily insulin dose was significantly lower during the first four years of follow-up in remitters. Conclusions: Occurrence of remission in adolescents and young adults with newly diagnosed T1D is associated with better glycemic control and lower insulin requirements during the first 5 years of follow-up. A lower initial dose of insulin was associated with a higher percentage of remission.

The remission phase in adolescents and young adults with newly diagnosed type 1 diabetes mellitus: prevalence, predicting factors and glycemic control during follow-up.

Objective: There is little data about the remission phase in adolescents and young adults with newly diagnosed type 1 diabetes mellitus (T1D). The aims of this study were to determine the prevalence of remission and its predicting factors among adolescents and young adults with newly diagnosed T1D and to assess the association between remission and long-term glycemic control in this population. Methods: This is a longitudinal and retrospective study including 128 type 1 diabetic patients aged between 12 and 30 years at diabetes onset. Clinical, biological and therapeutic features were collected at diagnosis and for 5 years after diagnosis. Remission was defined by an HbA1c < 6.5% with a daily insulin dose < 0.5 IU/kg/day. Results: Twenty-three patients (18%) experienced a remission. The peak of remission prevalence was at 6 months after diabetes diagnosis. An insulin dose at discharge <0.8 IU/kg/day was independently associated with remission (p=0.03, adjusted OR [CI 95%] = 0.2 [0.1-0.9]). A low socioeconomic level was independently associated with non remission (p=0.02, adjusted OR [CI 95%] = 4.3 [1.3-14.3]). HbA1c was significantly lower during the first five years of follow-up in remitters. The daily insulin dose was significantly lower during the first four years of follow-up in remitters. Conclusion: Occurrence of remission in adolescents and young adults with newly diagnosed T1D is associated with better glycemic control and lower insulin requirements during the first 5 years of follow-up. A lower initial dose of insulin was associated with a higher percentage of remission.

[Risk factors for major lower limb amputations in diabetic patients hospitalized for diabetic foot ulcer].

BACKGROUND: Amputations in diabetes patient lead to high postoperative morbidity and mortality . AIM: To indentify the risk factors for major lower limb amputations in diabetic patients hospitalized in endocrinology department. METHODS: It was a descriptive retrospective study including diabetic patients hospitalized in the endocrinology department of the Rabta Hospital for management of an infected foot lesion. We distributed the patients into 2 groups: group 1: patients with major amputation of the lower limb and group 2: patients who have had an amputation below the ankle or who had a conservative treatment. RESULTS: One hundred and twenty patients were included. The mean age was 59 ±11.9 years [28-97]. Twenty one (17.5%) patients had a major amputation (group 1). The frequency of obliterating arterial disease and gangrenes of the lower limbs were significantly higher in group 1. The extent of lesions> 2cm and the frequency of osteitis were comparable between the two groups. The frequency of hyperleukocytosis and mean C reactive protein were significantly higher in group 1. Antibiotic therapy prescribed during hospitalization was targeted in 30% of cases in group 1 versus 12.9% in group 2 (p = 0.05). Obliterating arterial disease, gangrenes and long duration of diabetes were independant risk factors significant on binary regression analysis. CONCLUSION: Some risk factors associated with major amputations are modifiable, such as arteriopathy obliterating of the lower limbs. A larger and prospective study would allow a better analysis of the predictive factors of major amputations.

Predictive factors for recurrence of Cushing's disease after pituitary surgery.

INTRODUCTION: The first-line treatment for Cushing's disease (CD) is transsphenoidal excision of the corticotropic adenoma. AIM: To identify the predictive factors of recurrence of corticotropic adenomas after pituitary surgery. METHODS: This is a retrospective and longitudinal study conducted in 28 patients operated for corticotropic adenoma between 1987 and 2014 and followed up in the department of endocrinology of La Rabta Hospital in Tunis (Tunisia). The mean duration of follow-up was 82 ± 65.9 months. The population was subdivided into two groups according to the occurrence or not of a recurrence. Recurrence was defined by hormonally confirmed postoperative recurrence of Cushing's syndrome. RESULTS: The mean age of the patients was 30.8±11.8 years (24 women and 4 men). CD was related to a microadenoma in 46% of cases (n=13) and a macroadenoma in 54% of cases (n=15). The recurrence rate was 28% (n=8/28). The mean time to diagnosis of recurrence was 5.6±4 years. The sex ratio (F/M) was 7 in the recurrence group (R) and 5.6 in the non-recurrence group (NR); p= NS. The mean age of patients in the (R) group was 27.2±11.8 years and 32.2±11.9 years in the (NR) group; p= NS. The recurrence rate was 63% in case of macroadenoma and 37% in case of microadenoma; p= NS. Postoperative cortisolemia was significantly higher in the recurrence group (23.2 ± 13.5 µg/dl vs 4.4 ± 3.9 µg/dl; p 4.4 µg/dl was significantly associated with recurrence (100% vs 26.3%; p= 0.001). The duration of corticotropic axis inertia was 67.2 ± 47.7 months in the (R) group versus 88.37 ± 72.14 months in the (NR) group; p= NS. CONCLUSIONS: Nearly a quarter of patients operated for CD develop a recurrence within five years. A postoperative cortisol level > 4.4 µg/dl is predictive of disease recurrence. Multicenter studies and a larger sample are needed to support these results.

A diffuse large B-cell thyroid lymphoma presented as a compressive goiter in a young woman with no evidence of Hashimoto's thyroiditis.

Thyroid lymphoma should be suspected in case of rapid enlargement of the thyroid gland even in a young patient with no history of Hashimoto thyroiditis. The confirmation is based on histopathology and immunohistochemistry.

Intermittent fasting in adrenal insufficiency patients: a review and guidelines for practice.

Ramadan fasting is a religious duty for adult Muslims. Even though sick people are exempted from fasting, numerous are those who insist on fasting, and seek advice from their physicians. The role of the physician is to support his patient and preserve his health. However, little is known about intermittent fasting in adrenal insufficiency. The aim of this review was to determine the risks of fasting in patients with adrenal insufficiency, the subjects at risk of complications, and the measures that can be undertaken for safe fasting. The analysis of the data of the different studies showed that there is a risk of complications during fasting in these patients. Optimal glucocorticoid replacement therapy for safe fasting has not yet been determined. Finally, a risk stratification and the modalities of the pre-Ramadan visit for adrenal insufficiency fasting patients have been suggested.

Late-Onset Isolated Corticotrope Deficiency in a Woman with Down Syndrome.

Isolated corticotrope deficiency is a rare cause of secondary adrenocortical insufficiency. Its occurrence in patients with Down syndrome is exceptional. Herein, we report a case of an isolated corticotrope deficiency diagnosed at the age of 33 years in a woman with Down syndrome and discuss its possible mechanisms. A 33-year-old woman with Down syndrome was referred to our department for the investigation of low blood pressure. She complained of asthenia, dizziness, and palpitation with arterial hypotension for the past 4 years. The thyroid function was normal and anti-thyroperoxidase antibodies were negative. The peak of cortisol level in response to the insulin-induced hypoglycemia test was 9.4 µg/dl. ACTH level was normal, indicating corticotrope deficiency. Other pituitary hormones were normal. Magnetic resonance imaging scan revealed a partially empty sella turcica. Genetic analysis showed no mutations and no copy number variants of the TBX19 and NFKB2 genes. The mechanism of isolated corticotrope deficiency is unclear, but it may be induced by autoimmune mechanism in similar to other disorders of patients with Down syndrome.