- Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation. [Journal Article]
- EDEndocrinol Diabetes Metab Case Rep 2018; 2018
- We report on a boy of Albanian descent with the history of juvenile myelomonocytic leukemia (JMML). JMML was diagnosed at the age of 17 months and treated by hematopoietic stem cell transplantation (...
We report on a boy of Albanian descent with the history of juvenile myelomonocytic leukemia (JMML). JMML was diagnosed at the age of 17 months and treated by hematopoietic stem cell transplantation (HSCT). At the age of 14.3 years, about 12 years after HSCT, he was hospitalized with an adrenal crisis. Hormone findings were consistent with primary adrenal insufficiency. Autoimmune adrenalitis was confirmed by positive autoantibodies against 21-hydroxylase and adrenal tissue. Since autoimmune Hashimoto thyroiditis was already known from the age of 9 years, we assume that both diseases are part of the spectrum of autoimmune polyglandular syndrome (APS) type 2. APS type 2 is a rare endocrine disease characterized by Addison's disease along with autoimmune thyroid disease and/or type 1 diabetes.
- [Canine hypoadrenocorticism - an update on pathogenesis, diagnosis and treatment]. [Journal Article]
- TPTierarztl Prax Ausg K Kleintiere Heimtiere 2018; 46(3):163-175
- Canine hypoadrenocorticism (HoAC) results from a loss of functional adrenal cortex, the most common etiology of which is an immune-mediated destruction leading to an inadequate production of glucocor...
Canine hypoadrenocorticism (HoAC) results from a loss of functional adrenal cortex, the most common etiology of which is an immune-mediated destruction leading to an inadequate production of glucocorticoids and mineralocorticoids. The term "atypical" HoAC is used for a subgroup of dogs with either an isolated glucocorticoid deficiency or a combined glucocorticoid and mineralocorticoid deficiency but normal electrolytes. Dogs with HoAC can present with a large variety of clinical signs, ranging from shaking, weakness, and mild gastrointestinal signs to seizures, hypovolemic shock, and collapse. Routine clinicopathologic and diagnostic imaging findings are usually nonspecific and frequently mimic those of other common diseases. However, the absence of a stress leukogram, eosinophilia, hyponatremia, hyperkalemia, and azotemia and small adrenal glands on abdominal ultrasound are characteristic findings in dogs with HoAC. The ACTH stimulation test is currently the gold standard method for diagnosing HoAC. Other endocrine laboratory diagnostics, including the quantification of endogenous ACTH, basal and ACTH-stimulated aldosterone levels, cortisol:ACTH ratio, and aldosterone:renin ratio, may further aid in differentiating between primary, secondary, and "atypical" HoAC. Aggressive intravenous fluid therapy is the cornerstone of treatment in paients with an acute Addisonian crisis because it restores normovolemia and normal blood electrolytes. Maintenance therapy consists of glucocorticoid (e.g., prednisolone) and mineralocorticoid (e.g., des- oxycortone pivalate) supplementation and aims for stable electrolyte concentrations and a clinically well dog. The optimal dose of desoxy- cortone pivalate for a specific dog is determined based on blood so- dium and potassium concentrations by using a standardized protocol. Regular reevaluation of blood electrolytes is required for early identifi- cation of a mineralocorticoid deficiency in dogs with "atypical" HoAC. The long-term prognosis for dogs with HoAC is excellent provided that patients receive adequate treatment and there is good owner com- pliance.
- Evaluation of Hypothalamic-Pituitary-Adrenal Axis Suppression following Cutaneous Use of Topical Corticosteroids in Children: A Meta-Analysis. [Journal Article]
- HRHorm Res Paediatr 2018 Jun 13; :1-8
- CONCLUSIONS: There is a low rate of reversible HPA axis suppression with the use of mid- to low-potency TCS compared to more potent formulations. In pediatric clinical practice, the limited use of mid- to low-potency TCS is rarely associated with clinically significant adrenal insufficiency or adrenal crisis. In the absence of signs and symptoms of adrenal insufficiency, there is little need to test the HPA axis of these patients.
- Mortality in children with classic congenital adrenal hyperplasia and 21-hydroxylase deficiency (CAH) in Germany. [Journal Article]
- BEBMC Endocr Disord 2018 Jun 08; 18(1):37
- CONCLUSIONS: According to the assessments by the attending centres, almost all deaths could be related to an inadequate administration of stress doses of hydrocortisone. Since no deceased CAH children were reported in Germany from 2005 on, we assume the effectiveness of educational programs over the past years.
- Relative adrenal insufficiency in adults with sickle cell disease. [Journal Article]
- PAPan Afr Med J 2018; 29:30
- Rheological modifications observed in sickle cell anemia are associated with ischemic complications that can cause target organ functional impairment. The objective was to investigate adrenal functio...
Rheological modifications observed in sickle cell anemia are associated with ischemic complications that can cause target organ functional impairment. The objective was to investigate adrenal function of adult patients with sickle cell disease. In this cross-sectional study conducted in a tertiary referral hospital of the capital city of Cameroon, we enrolled ten crisis-free adult patients with sickle cell disease (SCD) and ten age- and sex-matched healthy individuals. We assessed adrenal function by testing basal cortisol levels and 60 min after tetracosactide (Synacthen®) injection using immuno-chemiluminescence method. Post-stimulatory cortisol was defined as primary endpoint and secondary endpoints include basal cortisol levels, post-stimulatory cortisol increments and the fold increase of cortisol one hour after stimulation. Sickle cell patients had an impairment of adrenal function despite no significant difference between patients' and controls' for basal or post-stimulatory cortisol levels. In fact, one patient in two failed to achieve a two-fold increase in cortisol levels after stimulation (5/10) as opposed to 1 in 10 in the control population (1/10), P = 0.070. The percent increment of cortisol after stimulation was lower in patients versus controls (133 vs 207, P = 0.047). Relative adrenal insufficiency is frequent in sub-Saharan adult patients with sickle cell disease despite normal basal cortisol levels. Our results suggest that adrenal function require further investigation during SCD crises as these represent an important stress and may worsen the prognosis.
- Adrenal Crises in Children: Perspectives and Research Directions. [Journal Article]
- HRHorm Res Paediatr 2018 Jun 06; :1-11
- Adrenal crises (AC) are life-threatening physiological disturbances that occur at a rate of 5-10/100 patient years in patients with adrenal insufficiency (AI). Despite their seriousness, there is a p...
Adrenal crises (AC) are life-threatening physiological disturbances that occur at a rate of 5-10/100 patient years in patients with adrenal insufficiency (AI). Despite their seriousness, there is a paucity of information on the epidemiology of AC events in the paediatric population specifically, as most investigations have focused on AI and ACs in adults. Improved surveillance of AC-related morbidity and mortality should improve the delineation of AC risk overall and among different subgroups of paediatric patients with AI. Valid incidence measures are essential for this purpose and also for the evaluation of interventions aimed at reducing adverse health outcomes from ACs. However, the absence of an agreed AC definition limits the potential benefit of research and surveillance in this area. While approaches to the treatment and prevention of ACs have much in common across the lifespan, there are important differences between children and adults with regards to the physiological, psychological, and social milieu in which these events occur. Education is considered to be an essential element of AC prevention but studies have shown that ACs occur even among well-educated patients, suggesting that new strategies may be needed. In this review, we examine the current knowledge regarding AC events in children with AI; assess the existing definitions of an AC and offer a new definition for use in research and the clinic; and suggest areas for further investigation that are aimed at reducing the incidence and health impact of ACs in the paediatric age group.
- Steps to Stock: Keeping Students Safe With Fully Implemented Stock Epinephrine. [Journal Article]
- NSNASN Sch Nurse 2018 Jun 01; :1942602X18774780
- Laws have been passed across the United States to either allow or mandate the use of stock epinephrine in the school setting. The challenge remains for our schools to fully implement the use of this ...
Laws have been passed across the United States to either allow or mandate the use of stock epinephrine in the school setting. The challenge remains for our schools to fully implement the use of this life-saving medication. Barriers to implementation exist, but quality tools are available to support the school nurse.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Adrenal crisis is an acute life-threatening condition precipitated by an internal or external process in the setting of known or unknown adrenal insufficiency and corticosteroid deficiency. It is imp...
Adrenal crisis is an acute life-threatening condition precipitated by an internal or external process in the setting of known or unknown adrenal insufficiency and corticosteroid deficiency. It is important to note that there is a difference between adrenal insufficiency and adrenal crisis. Addison's disease, among other causes, is characterized by long-term adrenal insufficiency, whereas adrenal crisis is an acute process that can present with symptoms such as abdominal pain, vomiting, fever, weakness, among others. However, it is most notably accompanied by cardiovascular collapse with patients found to be in acute distress. While this disease is well described, it is often difficult to recognize, and treatment initiation may be delayed leading to notable morbidity and mortality.
- Initial presentations and associated clinical findings in patients with classical congenital adrenal hyperplasia. [Journal Article]
- JPJ Pediatr Endocrinol Metab 2018 Jun 27; 31(6):671-673
- CONCLUSIONS: CAH should be highly suspected in all females born with AG. Contrarily, affected male newborns more frequently present with a salt-losing crisis, and considering the recent institution of newborn screening in Saudi Arabia, earlier detection in these children is anticipated.
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- Myxedema Coma due to Hashimoto Thyroiditis: A Rare but Real Presentation of Failure to Thrive in Infancy. [Journal Article]
- HRHorm Res Paediatr 2018 May 04; :1-5
- CONCLUSIONS: MC may be a cause of altered mental status in infancy and may simultaneously be associated with FTT on presentation. With the presence of a normal thyroid NBS, autoimmunity should be entertained as the etiology of profound hypothyroidism, as positive thyroid antibodies may prompt an exploration for coexisting diseases which may explain other presenting features.