scholarly journals Current Insights Into Adrenal Insufficiency in the Newborn and Young Infant

2020 ◽  
Vol 8 ◽  
Author(s):  
Federica Buonocore ◽  
Sinead M. McGlacken-Byrne ◽  
Ignacio del Valle ◽  
John C. Achermann
Keyword(s):  
Adrenal Insufficiency ◽  
Young Infant ◽  
Adrenal Androgen ◽  
Androgen Excess ◽  
The Family ◽  
Threatening Condition ◽  
Life Threatening ◽  
Glucocorticoid Deficiency ◽  
Poor Weight Gain ◽  
Prolonged Jaundice

Adrenal insufficiency (AI) is a potentially life-threatening condition that can be difficult to diagnose, especially if it is not considered as a potential cause of a child's clinical presentation or unexpected deterioration. Children who present with AI in early life can have signs of glucocorticoid deficiency (hyperpigmentation, hypoglycemia, prolonged jaundice, poor weight gain), mineralocorticoid deficiency (hypotension, salt loss, collapse), adrenal androgen excess (atypical genitalia), or associated features linked to a specific underlying condition. Here, we provide an overview of causes of childhood AI, with a focus on genetic conditions that present in the first few months of life. Reaching a specific diagnosis can have lifelong implications for focusing management in an individual, and for counseling the family about inheritance and the risk of recurrence.

scholarly journals A Rare Case of Autoimmune Polyglandular Syndrome Type 2 in a Child With Persistent Fatigue

2019 ◽  
Vol 6 ◽  
pp. 2333794X1984507 ◽  
Author(s):  
Ryan Kenneth Smith ◽  
Peter M. Gerrits
Keyword(s):  
Adrenal Insufficiency ◽  
Autoimmune Polyglandular Syndrome ◽  
Threatening Condition ◽  
Viral Illness ◽  
Persistent Fatigue ◽  
Life Threatening ◽  
Local Emergency Department ◽  
Autoimmune Polyglandular Syndrome Type ◽  
High Index Of Suspicion

Adrenal insufficiency is a rare, potentially life-threatening condition whose diagnosis requires a high index of suspicion. Adrenal insufficiency may be primary, secondary, or tertiary with varied etiologies. Primary insufficiency may be part of a cluster of autoimmune diseases, referred to as autoimmune polyglandular syndrome(s) (APS). We describe a case of a 15-year-old male who presents to a local emergency department complaining of fatigue, fever, abdominal pain, nausea, and vomiting for a few days with a preceding viral illness. The patient was hyponatremic and hyperkalemic with skin hyperpigmentation, raising concern for adrenal insufficiency. Laboratory workup confirmed autoimmune primary adrenal insufficiency, with subsequent laboratory studies revealing autoimmune thyroiditis and celiac disease. Concomitant Addison’s and Hashimoto’s diseases led to a diagnosis of APS type 2. The patient was started on steroid replacement with rapid clinical improvement.

scholarly journals Acute Primary Adrenal Insufficiency after Hip Replacement in a Patient with Acute Intermittent Porphyria

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Adele Latina ◽  
Massimo Terzolo ◽  
Anna Pia ◽  
Giuseppe Reimondo ◽  
Elena Castellano ◽  
...  
Keyword(s):  
Postmenopausal Woman ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Genetic Disease ◽  
Hip Replacement ◽  
Acute Intermittent Porphyria ◽  
Adrenal Hemorrhage ◽  
Primary Adrenal Insufficiency ◽  
Threatening Condition ◽  
Life Threatening

Adrenal insufficiency is a potentially life-threatening condition when it occurs acutely, as in adrenal hemorrhage. Generally it is not reversible and requires chronic replacement therapy. Acute intermittent porphyria (AIP) is a rare genetic disease characterized by alterations in heme biosynthesis that result in accumulation of precursors in tissues. A crisis can be triggered by many conditions such as surgery and infections. Symptoms are similar to those of acute hypoadrenalism. Moreover, both conditions are characterized by hyponatremia. We describe the case of a postmenopausal woman known to be affected by AIP who developed after surgery a primary adrenal insufficiency associated with adrenal enlargement; the latter completely reverted in six months.

Impact on the family

2021 ◽  
pp. 111-125
Author(s):  
Veronica Dussel ◽  
Barbara Jones
Keyword(s):  
Social Care ◽  
Family Members ◽  
Well Being ◽  
Family Unit ◽  
Health And Social Care ◽  
The Family ◽  
Threatening Condition ◽  
Life Threatening ◽  
Limiting Condition ◽  
The Impact

In this chapter, we will focus on the importance of caring for the family of a child with a life-limiting condition (LLC) or life-threatening condition as a unit, each of the family members being integral to the well-being and care of the others. We recognize that the family unit itself is embedded within a wider context including the health and social care system, and more broadly within its society and culture. We discuss the concept of family, exploring the impact of having a child with an LLC, and how families adjust to this. We then expand on considerations about how to offer effective and timely support and help. We have included parents’ narratives with the aim of adding depth to the discussion, and in recognition of the truth of families’ own experiences.

scholarly journals The incidence of adrenal crisis in the postoperative period of HPA axis insufficiency after surgical treatment for Cushing’s syndrome

2019 ◽  
Vol 181 (2) ◽  
pp. 201-210 ◽  
Author(s):  
Leonie H A Broersen ◽  
Femke M van Haalen ◽  
Tina Kienitz ◽  
Olaf M Dekkers ◽  
Christian J Strasburger ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Crisis ◽  
Pituitary Hormone ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Life Threatening ◽  
Glucocorticoid Deficiency

Background Adrenal crisis, the most feared complication of adrenal insufficiency, is a potentially life-threatening state of acute glucocorticoid deficiency. After successful surgery for Cushing’s syndrome, many patients develop (transient) adrenal insufficiency. The incidence of adrenal crisis in patients treated for hypercortisolism is unknown. Methods Cohort study included consecutive patients with Cushing’s syndrome with adrenal insufficiency after surgery from Leiden and Berlin from 2000 to 2015. We summarized the incidence of adrenal crisis, compared patients with and without adrenal crisis regarding potential risk factors for its occurrence and assessed the effect of better education in time on incidence of adrenal crisis. Results We included 106 patients, of whom 19 patients had a total of 41 adrenal crises. There were 9.0 crises per 100 patient-years at risk (95% confidence interval (CI): 6.7–12.0). All crises occurred while on hydrocortisone replacement. The risk ratio for a recurrent crisis was 2.3 (95% CI: 1.2–4.6). No clear change in incidence of adrenal crisis due to better education in time was observed. There was no difference in recurrence rate between patients with, and without any crisis, but patients with adrenal crisis had more often pituitary deficiencies. Conclusions The incidence of adrenal crises after treatment for Cushing’s syndrome is substantial, and patients who suffered from an adrenal crisis have higher risk for recurrent crisis. Adrenal crisis tends to present early after remission of Cushing’s syndrome, which is probably the period of severest HPA axis suppression, despite in general higher hydrocortisone replacement doses for withdrawal complaints in this period. Additional pituitary hormone deficiencies may be a risk marker for increased risk of adrenal crisis. However, further risk factor analysis is needed to identify risks for a first crisis. Effective education methods to prevent adrenal crises should be identified and implemented, including stress instructions by trained nursing staff before hospital discharge.

Assessment of autoantibodies against 21-hydroxylase in the diagnosis of primary autoimmune adrenal insufficiency

2020 ◽  
Author(s):  
Leila Sozaeva ◽  
Nadezhda Makazan ◽  
Larisa Nikankina ◽  
Natalya Malysheva ◽  
Ekaterina Kuvaldina ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Patient Survival ◽  
Adrenal Crisis ◽  
Primary Adrenal Insufficiency ◽  
Adrenal Steroidogenesis ◽  
Threatening Condition ◽  
Life Threatening ◽  
The One

Primary adrenal insufficiency is manifested by a deficiency of adrenal cortex hormones and can lead to a life-threatening condition. Early diagnosis is key to patient survival. Auto-antibodies to one of the adrenal steroidogenesis enzymes, 21-hydroxylase, are an immunological marker of autoimmune adrenal insufficiency. On the one hand, the study of antibodies to 21-hydroxylase is a method that helps establish the etiology of the disease the autoimmune genesis of adrenal gland damage. On the other hand, the determination of autoantibodies to 21-hydroxylase is the only prognostic factor of the risk of adrenal insufficiency, which makes it possible to prevent the development of acute adrenal crisis. The article provides a brief literature review on autoantibodies to 21-hydroxylase and the pathogenesis of autoimmune adrenal insufficiency, and a series of clinical cases that illustrates the significant role of autoantibodies to 21-hydroxylase in diagnosis of adrenal insufficiency.

Perioperative Adrenal Crisis

2017 ◽  
Author(s):  
Jing Tao ◽  
Jeffrey J. Schwartz
Keyword(s):  
Adrenal Insufficiency ◽  
Adrenal Crisis ◽  
Life Saving ◽  
Distributive Shock ◽  
Threatening Condition ◽  
Life Threatening ◽  
High Doses ◽  
Patient At Risk ◽  
Stress Dose ◽  
Timely Treatment

Perioperative adrenal insufficiency (AI) (adrenal crisis) is an uncommon life-threatening condition manifested by distributive shock that is poorly responsive to the administration of fluids and vasopressors. Timely treatment with high doses of glucocorticoids can be life saving. The difficulty is in recognizing the patient at risk, suspecting the diagnosis, and distinguishing it from other forms of shock. The incidence of adrenal crisis is rare, in large part, due to the liberal use of perioperative “stress dose” steroids in patients already receiving steroids. In this chapter we review the pathophysiology involved in both primary and secondary AI, and provide step-by-step treatment recommendations for patients as risk for this condition.

scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Hyperandrogenism after menopause

2015 ◽  
Vol 172 (2) ◽  
pp. R79-R91 ◽  
Author(s):  
Marios C Markopoulos ◽  
Evanthia Kassi ◽  
Krystallenia I Alexandraki ◽  
George Mastorakos ◽  
Gregory Kaltsas
Keyword(s):  
Postmenopausal Women ◽  
Imaging Modalities ◽  
Adrenal Androgen ◽  
Androgen Excess ◽  
Endocrine Disease ◽  
Diagnostic Certainty ◽  
Cardiovascular Morbidity And Mortality ◽  
Need To Evaluate ◽  
Life Threatening

Postmenopausal hyperandrogenism is a state of relative or absolute androgen excess originating from either the adrenals and/or the ovaries, clinically manifested as the appearance and/or increase in terminal hair growth or the development of symptoms/signs of virilization. In either settings, physicians need to evaluate such patients and exclude the presence of the relatively rare but potentially life-threatening underlying tumorous causes, particularly adrenal androgen-secreting tumors. It has been suggested that the rapidity of onset along with severity of symptom and the degree of androgen excess followed by relevant imaging studies may suffice to identify the source of excessive androgen secretion. However, up to date, there is no consensus regarding specific clinical and hormonal indices and/or imaging modalities required for diagnostic certainty. This is particularly relevant as the aging population is increasing and more cases of postmenopausal women with clinical/biochemical evidence of hyperandrogenism may become apparent. Furthermore, the long-term sequels of nontumorous hyperandrogenism in postmenopausal women in respect to cardiovascular morbidity and mortality still remain unsettled. This review delineates the etiology and pathophysiology of relative and absolute androgen excess in postmenopausal women. Also, it attempts to unravel distinctive clinical features along with specific hormonal cut-off levels and/or appropriate imaging modalities for the facilitation of the differential diagnosis and the identification of potential long-term sequels.

scholarly journals A case of severe rhabdomyolysis associated with secondary adrenal insufficiency and autoimmune hepatitis

2019 ◽  
Vol 12 (3) ◽  
pp. e227343 ◽  
Author(s):  
Lisa Kennedy ◽  
Sureshkumar Nagiah
Keyword(s):  
Autoimmune Hepatitis ◽  
Adrenal Insufficiency ◽  
Review Of The Literature ◽  
Secondary Adrenal Insufficiency ◽  
Autoimmune Hypophysitis ◽  
Endocrine Disturbances ◽  
Threatening Condition ◽  
Life Threatening ◽  
New Diagnosis

Rhabdomyolysis is a serious and life-threatening condition which has many established causes including endocrine disturbances. Of those, thyroid, adrenal and pituitary deficiencies are the most commonly seen. Most cases of rhabdomyolysis with adrenal insufficiency that have been reported have been primary. Here, we report an encounter with a patient who presented with her second case of severe rhabdomyolysis in the setting of secondary adrenal insufficiency. The cause for corticotropic suppression was most likely autoimmune hypophysitis given the presence of other autoimmune comorbidities including a new diagnosis of autoimmune hepatitis. In addition to her case, we present a brief review of the literature pertaining to cases of rhabdomyolysis attributed to adrenal insufficiency.

scholarly journals Rare Bilateral Adrenal Haemorrhage with Addisonian Crisis: When Risk Factors Come in Droves

2020 ◽  
Vol 2020 ◽  
pp. 1-4 ◽  
Author(s):  
Anders Boisen Jensen ◽  
Elise Durand ◽  
Vojtech Pavlicek
Keyword(s):  
Risk Factors ◽  
Tract Infection ◽  
Adrenal Insufficiency ◽  
Orthopedic Surgery ◽  
Anticoagulation Therapy ◽  
Rare Condition ◽  
Precipitating Factors ◽  
Adrenal Haemorrhage ◽  
Life Threatening ◽  
Glucocorticoid Deficiency

Addisonian crisis is the life-threatening acute manifestation of adrenal insufficiency due to absolute or relative glucocorticoid deficiency. Adrenal haemorrhage is a rare condition of unknown incidence with the risk of adrenal insufficiency and death, not uncommonly first being diagnosed on the pathologists table. We report the case of a 68-year-old female patient with respiratory tract infection suffering acute life-threatening adrenal insufficiency caused by bilateral adrenal haemorrhages following orthopedic surgery while taking anticoagulation therapy. The patient rapidly deteriorated with hypotension, showing how important it is to consider a possible Addisonian crisis when this scenario occurs, especially with precipitating factors such as anticoagulant therapy, sepsis, or surgery.

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