Current Management and Outcome of Pregnancies in Women With Adrenal Insufficiency: Experience from a Multicenter Survey.

CONTEXT: Appropriate management of adrenal insufficiency (AI) in pregnancy can be challenging due to the rarity of the disease and lack of evidence-based recommendations to guide glucocorticoid and mineralocorticoid dosage adjustment. OBJECTIVE: Multicenter survey on current clinical approaches in managing AI during pregnancy. DESIGN: Retrospective anonymized data collection from 19 international centers from 2013 to 2019. SETTING AND PATIENTS: 128 pregnancies in 113 women with different causes of AI: Addison disease (44%), secondary AI (25%), congenital adrenal hyperplasia (25%), and acquired AI due to bilateral adrenalectomy (6%). RESULTS: Hydrocortisone (HC) was the most commonly used glucocorticoid in 83% (97/117) of pregnancies. Glucocorticoid dosage was increased at any time during pregnancy in 73/128 (57%) of cases. In these cases, the difference in the daily dose of HC equivalent between baseline and the third trimester was 8.6 ± 5.4 (range 1-30) mg. Fludrocortisone dosage was increased in fewer cases (7/54 during the first trimester, 9/64 during the second trimester, and 9/62 cases during the third trimester). Overall, an adrenal crisis was reported in 9/128 (7%) pregnancies. Cesarean section was the most frequent mode of delivery at 58% (69/118). Fetal complications were reported in 3/120 (3%) and minor maternal complications in 15/120 (13%) pregnancies without fatal outcomes. CONCLUSIONS: This survey confirms good maternal and fetal outcome in women with AI managed in specialized endocrine centers. An emphasis on careful endocrine follow-up and repeated patient education is likely to have reduced the risk of adrenal crisis and resulted in positive outcomes.

Dopamine agonist-responsive Cushing's disease.

A 47-year-old Caucasian man was referred to our clinic with a severe clinical and biochemical phenotype of endogenous hypercortisolism for further evaluation and treatment. In addition to confirming adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, we found left temporal hemianopsia, massively increased prolactin, increased growth hormone/insulin-like growth factor 1 values, hypogonadotropic hypogonadism and central hypothyroidism. As the cause of these abnormalities we revealed an invasive macroadenoma of the pituitary secreting ACTH, prolactin and growth hormone, resulting not only in a clinically predominant picture of Cushing's syndrome but also causing hypogonadotropic hypogonadism and central hypothyroidism. The patient responded surprisingly well to dopamine agonist treatment leading not only to normalisation of prolactin levels but also to clinical and biochemical remission of Cushing's syndrome. Tumour size decreased successively in follow-up MRI scans. Despite lacking immunohistochemical analysis of tumour tissue, we assume plurihormonal secretion of ACTH, prolactin and growth hormone from pituitary macroadenoma, which fortunately responded well to dopamine agonist treatment.

Perioperative Management of Endocrine Active Adrenal Tumors.

Endocrine active adrenal tumors are associated with a relevant risk of complications during surgery, either due to direct hemodynamic effects of the hormone excess or due to hormone related comorbidities. Over the last decades, careful preoperative evaluation and improved peri-interventional medical management of affected patients has resulted in a significant reduction of perioperative complications. In addition, improvement in anesthesia and surgical techniques with the feasibility of laparoscopic adrenalectomy have contributed to reduce morbidity. Nevertheless, there are still several challenges to be considered in the perioperative care of these patients. Due to the rarity of functionally active adrenal tumors, there are no prospective data available to guide clinical management. Accordingly, most recommendations are based on retrospective data analysis, expert opinion or carry weak evidence based on small series or case reports. The aim of this review is to summarize the current knowledge and to suggest practical approaches to reduce perioperative complications in endocrine active adrenal tumors. This review exclusively deals with data from adult patients with functionally active adrenal tumors.

MANAGEMENT OF ENDOCRINE DISEASE: Fertility, pregnancy and lactation in women with adrenal insufficiency.

With the introduction of hormonal substitution therapy in the 1950s, adrenal insufficiency (AI) has been turned into a manageable disease in pregnant women. In fact, in the light of glucocorticoid replacement therapy and improved obstetric care, it is realistic to expect good maternal and fetal outcomes in patients with AI. However, there are still a number of challenges such as establishing the diagnosis of AI in pregnant women and optimizing the treatment of AI and related comorbidities prior to as well as during pregnancy. Clinical and biochemical diagnoses of a new-onset AI may be challenging because of overlapping symptoms of normal pregnancy as well as pregnancy-induced changes in cortisol values. Physiological changes occurring during pregnancy should be taken into account while adjusting the substitution therapy. The high proportion of reported adrenal crisis in pregnant women with AI highlights persistent problems in this particular clinical situation. Due to the rarity of the disease, there is no prospective data-guiding management of pregnancy in patients with known AI. The aim of this review is to summarize the maternal and fetal outcomes based on recently published case reports in patients with AI and to suggest a practical approach to diagnose and manage AI in pregnancy.

Severe hypophosphataemia after intravenous iron administration.

Iron deficiency is common and can be effectively treated with parenteral iron infusion. We report a case of an iron-deficient and vitamin D-deficient woman who developed severe symptomatic hypophosphataemia following intravenous ferric carboxymaltose administration. We stress the need of increased awareness of this potential complication among physicians. Patients should be informed of this complication and instructed to report for follow-up if they experience new musculoskeletal symptoms or worsening of tiredness. As severe hypophosphataemia is usually symptomatic, we recommend screening symptomatic patients for this complication. Recognising and treating the possible exacerbating factors, especially vitamin D deficiency, might be a simple measure to mitigate this complication.

Feasibility of breath monitoring in patients undergoing elective colonoscopy under propofol sedation: A single-center pilot study.

AIM: To determine whether a newly developed respiratory rate monitor can practically and accurately monitor ventilation under propofol sedation in combination with standard monitoring. METHODS: Patients [American Society of Anesthesiologists (ASA) Classification I-III] scheduled for elective colonoscopy under propofol sedation were monitored with a new device that measures the respiratory rate based on humidity in expired air. Patients with clinically significant cardiac disorders or pulmonary disease and patients requiring emergency procedures were excluded from study participation. All of the patients also received standard monitoring with pulse oximetry. This was a single-center study conducted in a community hospital in Switzerland. After obtaining written informed consent from all subjects, 76 patients (51 females and 25 males) were monitored during colonoscopy under propofol sedation. The primary endpoint was the occurrence of any respiratory event (apnea or hypopnea). Apnea was defined as the cessation of breathing for a minimum of 10 s. Significant apnea was defined as the cessation of breathing for more than 30 s. Hypopnea was defined as a reduction in the respiratory rate below 6/min for a minimum of 10 s. Any cases of significant apnea triggered interventions by the endoscopy team. The interventions included withholding propofol, verbal stimulation of the patients, and increased oxygen supplementation or the chin lift maneuver. A secondary endpoint was the correlation of apnea or hypopnea with hypoxemia (measured as a decrease in SaO2 of at least 5% from baseline or less than 90%). RESULTS: At least one respiratory event was detected in thirty-seven patients (48.7%). In total, there were 73 respiratory events, ranging from one to six events in a single patient. Significant apnea (> 30 s) occurred in five patients (6%). Only one episode of apnea led to a relative SaO2 reduction (from 98% to 93%) after a 50 s lag time. No event requiring assisted ventilation was recorded. Our analysis revealed that the total propofol dose was an independent risk factor for respiratory events (P = 0.01). Artifacts developed with the same frequency with the new device as with conventional pulse oximetry. Compared with pulse oximetry alone, this new monitoring device detected more respiratory events and may provide earlier warning of impending respiratory abnormalities. CONCLUSION: Apnea commonly occurs during endoscopy under sedation and may precede hypoxemia. We recommend this respiration rate monitor as an alternative to capnography to aid in detecting ventilatory problems.