Crisis adrenal neonatal secundaria a hipoplasia hipofisaria / Neonatal Adrenal Crisis Secondary to Pituitary Hypoplasia

Introducción: La insuficiencia adrenal hipotálamo hipofisaria usualmente se manifiesta secundaria a tumores y, cuando resulta congénita se asocia, con frecuencia, con otras deficiencias hormonales. La crisis adrenal suele presentarse en su debut y puede resultar potencialmente mortal. Objetivo: Examinar el caso de una paciente con insuficiencia adrenal central que debutó con una crisis adrenal congénita. Presentación del caso: Recién nacida a término, padres no consanguíneos, hospitalizada a los 9 días de vida por clínica de una semana con múltiples episodios eméticos y apnea. Ingresó con deshidratación severa, hipotensa y estuporosa. Además, se encontró acidosis metabólica severa, hipoglucemia persistente, hiponatremia e insuficiencia prerrenal. Ante la no mejoría de su estado hemodinámico, a pesar del uso de cristaloides y vasopresores, finalmente mejoró con la administración de dosis altas de hidrocortisona. El diagnóstico de deficiencia de cortisol de origen central se realizó con un test dinámico de insulina y la resonancia magnética nuclear hipofisaria. Conclusiones: La crisis adrenal se debe tener presente como diagnóstico diferencial en episodios agudos con inestabilidad hemodinámica persistente e hipoglucemia de difícil manejo. Adicionalmente, hay que considerar que existen otras causas menos comunes de insuficiencia adrenal en neonatos como la hipoplasia hipofisaria(AU)

Introduction: Hypothalamic-pituitary adrenal insufficiency usually manifests secondary to tumors and, when congenital, is often associated with other hormonal deficiencies. Adrenal crisis usually occurs at its onset and can be life threatening. Objective: To review the case of a patient with central adrenal insufficiency who had an onset with a congenital adrenal crisis. Case presentation: Term newborn, non-consanguineous parents, hospitalized at 9 days of life for a week-long clinical presentation with multiple emetic episodes and apnea. She was admitted with severe dehydration, hypotensive and stuporous. In addition, severe metabolic acidosis, persistent hypoglycemia, hyponatremia and prerenal failure were found. Given the lack of improvement of her hemodynamic status, despite the use of crystalloids and vasopressors, she finally improved with the administration of high doses of hydrocortisone. The diagnosis of cortisol deficiency of central origin was made with a dynamic insulin test and pituitary nuclear magnetic resonance imaging. Conclusions: Adrenal crisis should be kept in mind as a differential diagnosis in acute episodes with persistent hemodynamic instability and difficult-to-manage hypoglycemia. Additionally, other less common causes of adrenal insufficiency in neonates, such as pituitary hypoplasia, should be considered(AU)

Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia

ABSTRACT Primary adrenal insufficiency (PAI) is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Addison's disease (AD) and congenital adrenal hyperplasia (CAH) are the most frequent disorders in adults and children, respectively. Despite the diagnostic advances and the availability of glucocorticoid and mineralocorticoid replacements, adrenal crisis (AC) is still a potentially lethal condition contributing to the increased mortality, not only during the first year of life, but also throughout life. Failure in increasing glucocorticoid doses during acute stress, when greater amounts of glucocorticoids are required, can lead to AC and an increase morbimortality rate of PAI. Considering a mortality rate of 0.5 per 100 patient years, up to 1,500 deaths from AC are expected in Brazil in the coming decade, which represents an alarming situation. The major clinical features are hypotension and volume depletion. Nonspecific symptoms such as fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain are common. The main precipitating factors are gastrointestinal diseases, other infectious disease, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. Suspected AC requires immediate therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if necessary. AC is best prevented through patient education, precocious identification and by adjusting the glucocorticoid dosage in stressor situations. The emergency card, warning about acute glucocorticoid replacement, has high value in reducing the morbidity and mortality of AC.

Recognition and treatment of adrenal crisis complicated with nephrotic syndrome in children / 中国小儿急救医学

Up to 60% of children with nephrotic syndrome may occur secondary adrenal insufficiency, and even adrenal crisis.Adrenal crisis is one of the serious complications of nephrotic syndrome in children.The mortality rate is high and it seriously threatens the life.Adrenal crisis is often lack of specific manifestations, and usually begin with gastrointestinal symptoms, accompanied by circulatory dehydration, neuropsychiatric symptoms, hyponatremia, hyperkalemia and hypoglycemia.Glucocorticoid replacement therapy is important.Active prevention can reduce the incidence of crisis and improve prognosis.

Sheehans syndrome: the hidden nemesis

Sheehan’s syndrome is the name given to postpartum hypopituitarism, usually precipitated by massive uterine haemorrhage and hypovolemic shock during or after childbirth resulting into pituitary injury in varying degrees. Most cases are diagnosed very late in life and the first presentation may be a life-threatening situation like shock or cardiorespiratory collape. This hidden nemesis in women compromises their quality of life and may result into mortality if not suspected early. In this article we present 3 patients who presented in the ICU in a state of complete cardio-respiratory collapse and later discharged in full health.

Management of Infants with Congenital Adrenal Hyperplasia

Treatment of congenital adrenal hyperplasia (CAH) requires lifelong replacement of glucocorticoids with regular follow up to manageassociated morbidities. The current review focuses on follow-up and management of infants diagnosed with classical CAH pertinent toIndian context. Early initiation of oral hydrocortisone in divided doses is recommended after diagnosis in newborn period, infancy andchildhood. Fludrocortisone is recommended for all infants with classical CAH. All infants should be monitored as per protocol fordisease and treatment related complications. The role of prenatal steroids to pregnant women with previous history of CAH affectedinfant for prevention of virilization of female fetus is controversial.

Clinical review of secondary adrenocortical insufficiency after kidney transplantation / 中华器官移植杂志

Objective:To explore the clinical characteristics, diagnosis and treatment of secondary adrenocortical insufficiency(SACI)after kidney transplantation.Methods:Retrospective analysis was conducted for clinical data of 12 recipients with SACI after transplantation from March 2018 to November 2019(observation group). Meanwhile, 10 healthy subjects(control group)were randomly selected for in-hospital physical reexaminations during the same period. General data and morning cortisol levels of adrenocorticotropic hormone(ACTH)and aldosterone were compared between two groups.Results:In observation group, there were 8 male and 4 female with an average age of (43.67±8.81) years. Six cases(50.0%)of SACI occurred during recovery period within 30 days and 3 cases(25.0%)within 30 to 90 days post-transplantation. Deceased citizen donation(DCD)was performed in 9 cases(75.0%)and re-transplanted in 3 cases(25.0%). Oral immunosuppressive regiments were administered in a low-dose prednisone-based triple/quadruple regimen. The mean eGFR of observation group was(54.08±20.03)ml/min. The first patient had adrenal crisis, the fourth had sole symptom of fatigue and the remainder stayed asymptomatic. All of them had persistent hyperkalemia and hyponatremia. The average level of plasma cortisol was(62.24±24.16)mmol/L and it was much lower than normal in all patients at 8 am. The determination of plasma ACTH at 8 am showed that 7 patients(58.33%)were lower than normal and the remaining 5 slightly surpassed the low limit of normal. The average level of plasma cortisol at 8 am was significantly lower in observation group than that in control group(141.34±26.28)nmol/L( t=-7.349, P<0.001). The average ACTH level of observation group at 8 am was(1.08±0.515)pmol/l and it was significantly lower than that of control group(2.53±1.06)pmol/L( t=-4.178, P<0.001). The level of aldosterone was normal in both groups and showed no significant difference. All patients in observation group received an intravenous injection of hydrocortisone with satisfactory outcomes. Conclusions:Transplant surgeons should be on a high alert for an occurrence of SACI in renal transplant recipients. Serum potassium and sodium levels may be the predictors of SACI.

Adrenal insufficiency after cardiovascular surgery with cardiopulmonary bypass / 中国胸心血管外科临床杂志

@#Relative/absolute adrenal insufficiency is not uncommmon in the patients undergoing cardiovascular surgery with cardiopulmonary bypass. However, this complication was rarely reported due to its unspecific clinical presentations and the unawareness of ICU physicians and cardiac surgeons. However, this puts them on a higher risk of hemodynamic instability and cardiovascular adverse events during hospitalization. Systematic reviews concentrated on it are rarely reported currently. We aim to review the etiology, risk factors, potential pathogenesis and related research progress for this complication.

Relationship of adrenal insufficiency with metabolic crisis and sudden death / 中国实用儿科杂志

Adrenal insufficiency(AI)is a type of adrenocortical hormone-deficient lesion caused by primary or secondary factors. Because of its lack of specificity in clinical manifestations,it is difficult to identify early,which may lead to delayed diagnosis. If the treatment is not timely,it may cause adrenal crisis(AC)or even sudden death. Sudden death in children due to adrenal insufficiency may be directly related to hyperkalemia caused by adrenal crisis. Gastrointestinal illness and infections are common precipitant for an adrenal crisis. Despite it being a treatable condition for now,failure of adequate preventive measures or delayed treatment has often led to unnecessary deaths. Therefore,more measures are needed to prevent the adrenal crisis and to ensure that appropriate emergency medical services are established after the crisis to reduce the rate of sudden death.

Conduct protocol in emergency: Acute adrenal insufficiency / Protocolo de condutas em emergência: insuficiência adrenal aguda

Summary Introduction: Acute adrenal insufficiency or addisonian crisis is a rare comorbidity in emergency; however, if not properly diagnosed and treated, it may progress unfavorably. Objective: To alert all health professionals about the diagnosis and correct treatment of this complication. Method: We performed an extensive search of the medical literature using specific search tools, retrieving 20 articles on the topic. Results: Addisonian crisis is a difficult diagnosis due to the unspecificity of its signs and symptoms. Nevertheless, it can be suspected in patients who enter the emergency room with complaints of abdominal pain, hypotension unresponsive to volume or vasopressor agents, clouding, and torpor. This situation may be associated with symptoms suggestive of chronic adrenal insufficiency such as hyperpigmentation, salt craving, and association with autoimmune diseases such as vitiligo and Hashimoto's thyroiditis. Hemodynamically stable patients may undergo more accurate diagnostic methods to confirm or rule out addisonian crisis. Delay to perform diagnostic tests should be avoided, in any circumstances, and unstable patients should be immediately medicated with intravenous glucocorticoid, even before confirmatory tests. Conclusion: Acute adrenal insufficiency is a severe disease that is difficult to diagnose. It should be part of the differential diagnosis in cases of hypotensive patient who is unresponsive to vasoactive agents. Therefore, whenever this complication is considered, health professionals should aim specifically at this pathology.

Resumo Introdução: a insuficiência adrenal aguda ou crise addisoniana é uma comorbidade rara na emergência; porém, se não diagnosticada e tratada de forma correta, pode evoluir de maneira desfavorável. Objetivo: alertar a todos os profissionais da saúde sobre o diagnóstico e tratamento corretos dessa complicação. Método: foi realizada uma ampla pesquisa na literatura médica, por meio de ferramentas específicas, sendo selecionados 20 artigos sobre o tema. Resultados: a crise addisoniana é de difícil diagnóstico pela inespecificidade de seus sinais e sintomas. No entanto, pode ser suspeitada em pacientes que chegam à emergência com queixa de dor abdominal, hipotensão não responsiva a volume ou a agentes vasopressores, obnubilação e torpor. Esse quadro pode vir associado a sintomas sugestivos de insuficiência adrenal crônica, como hiperpigmentação e avidez por sal, bem como a doenças autoimunes, como vitiligo e tireoidite de Hashimoto. Pacientes estáveis hemodinamicamente podem passar por métodos diagnósticos mais apurados para se confirmar ou descartar a crise addisoniana. Os exames diagnósticos não podem retardar, em hipótese alguma, o tratamento de pacientes instáveis, que deve ser iniciado imediatamente com glicocorticoide endovenoso, inclusive antes das provas confirmatórias. Conclusão: a insuficiência adrenal aguda é uma patologia grave e de difícil diagnóstico, que deve fazer parte do diagnóstico diferencial do médico ao atender um paciente hipotenso sem reposta à infusão de drogas vasoativas. Logo, na suspeita dessa complicação, o profissional não deve tardar a agir especificamente nessa patologia.

X-linked Congenital Adrenal Hypoplasia with a Novel NR0B1/DAX Gene Mutation.

Background: The etiology of primary adrenal insufficiency has implications for further management of the condition. Case characteristics: A 5-year-old boy presented in adrenal crisis with glucocorticoid and mineralocorticoid deficiency. Observation: Investigations confirmed primary adrenal insufficiency and ruled out the common etiologies. Genetic testing identified a novel NR0B1/DAX gene mutation. Message: A genetic diagnosis in children with primary adrenal insufficiency is useful to provide genetic counselling.

A 36-year-old man with vomiting, pain abdomen, significant weight loss, hyponatremia, and hypoglycemia.

Diagnosis of Strongyloides stercoralis hyperinfection can be a challenge. The key to a timely diagnosis is to have a high index of suspicion. We present a rare case of a 36-year-old human immunodeficiency virus negative male patient, who was on multidrug therapy for lepromatous leprosy and was treated for type 2 lepra reactions with steroids in the past. The patient presented with vomiting and pain abdomen, persistent hyponatremia, and terminal hypoglycemia. He had features of malnutrition and had a rapid downhill course following admission. A diagnosis of S. stercoralis hyperinfection with sepsis and multiorgan failure, adrenal hemorrhage, and syndrome of inappropriate antidiuretic hormone secretion was established on a postmortem examination.

Early recognition and treatment of adrenal crisis in children / 中国小儿急救医学

Adrenal crisis is a life-threatening emergency caused by the destruction or altered function of the adrenal gland with a primary deficit in cortisol secretion(primary adrenal insufficiency)or by hypotha-lamic-pituitary pathologies determining a deficit of adrenocorticotropic hormone(secondary adrenal insuffi-ciency).Infection and abrupt end glucocorticoid treatment are the major precipitating causes of adrenal crisis. Patients with adrenal crisis typically present with hypovolemic shock or hypotension,nausea,vomiting,and fe-ver responding well to parenteral hydrocortisone administration.The main laboratory findings include lower serum cortisol concentrations,hyponatremia,hypoglycaemia and/or hyperkalemia.Delay diagnosis of adrenal insufficiency leads to adrenal crisis which is potentially lethal complication.Empirical glucocorticoid replace-ment should be initiated as soon as the suspicious of adrenal crisis,or sooner if the patient presents in adrenal crisis in critically ill children.

Etiology, diagnosis and management of adrenal insufficiency in children / 中华实用儿科临床杂志

Adrenal insufficiency(AI) is a disorder that can result from primary adrenal failure(primary type) or secondary adrenal disease due to impairment of the hypothalamic-pituitary axis (central type).It is characterized by glucocorticoid deficiency,with or without inappropriate mineralocorticoid or androgen production.The diagnosis and management of children with AI remain challenging as clinical characteristics of AI in children maybe non-specific and acute adrenal crisis is life-threatening,needing life-long glucocorticoid replacement treatment and education of the patient and the family.New formulations of hydrocortisone which should be able to simulate the circadian rhythm of steroid secretion are supposed to improve outcome in patients with adrenal insufficiency in the near future.

Phenotypic Variability in Congenital Lipoid Adrenal Hyperplasia.

Background: Congenital lipoid adrenal hyperplasia presents with adrenal insufficiency and sex reversal in 46XY genetic males. Case characteristics: Two patients (46 XY karyotype), one having ambiguous genitalia and other having female external genitalia, presented with adrenal crisis at 6 months and 4 weeks of age, respectively. Observation: Steroidogenic Acute Regulatory Protein gene sequencing revealed homozygous mutations in both patients. Outcome: Treatment with hydrocortisone and fludrocortisone resulted in marked improvement . Message: Congenital lipoid adrenal hyperplasia should be considered in infants having female or ambiguous genitalia, and presenting with adrenal insufficiency.

Progress in the adrenal insufficient and adrenal crisis of critical children / 国际儿科学杂志

Recent studies have showed that adrenal insufficiency are common in some status of critically ill children,like systemic inflammatory response syndrome,sepsis,shock,et al.The frequency of adrenal crisis was also higher than expectation.The currrent glucocorticoid replacement therapies are inclined to lower dose [ hydrocortisone 20 ～ 25 mg/( m2· d) ].The adrenal function could be evaluated with the free cortisol index and calculated free cortisol.A new method,temperature-controlled centrifugal ultrafiltration with chemiluminescence immunoassay,has been used to detect the free cotisol,which has improved the clinical practicality.

A Case of Type II Autoimmune Polyglandular Syndrome: Acute adrenal crisis presented as the first manifestation of Addison's disease in a patient with diabetic ketoacidosis and hypgonadism / 대한내분비학회지

Type II autoimmune polyglandular syndrome typically presents in adulthood. Insulin dependent diabetes mellitus and thyroid dysfunction are the most frequent manifestations. Addison's disease is the third major endocrine component of this disorder. In this report, we described a thirty-two year-old male patient who had hypogonadism, insulin dependent diabetes mellitus, and mild Addison's disease presenting its first manifestation as an acute adrenal crisis due to diabetic ketoacidosis. The ACTH concentration will be elevated early in the course of Addisons disease even before a significant reduction in the basal cortisol level or its response to exogenous ACTH occurs. Therefore, plasma ACTH measurements serve as a valuable screening study for Addisons disease.