71 Adrenal Insufficiency among Children treated with Hormonal Therapy for Infantile Spasms

2021 ◽  
Vol 26 (Supplement_1) ◽  
pp. e51-e51
Author(s):  
Gabrielle Doré-Brabant ◽  
Geneviève Laflamme ◽  
Maude Millette ◽  
Nicolas Chrestian ◽  
Bradley Osterman

Abstract Primary Subject area Endocrinology and Metabolism Background Hormonal therapy is a standard treatment for children with infantile spasms. However, the high doses given and long treatment duration exposes patients to the potential risk of adrenal insufficiency (AI). There is, presently, limited data on occurrence of AI after hormonal therapy in this population. Objectives This study aims to quantify the incidence of AI among children with infantile spasms treated with high-dose corticosteroids and/or adrenocorticotropic hormone (ACTH). Design/Methods A retrospective chart review of patients less than 2 years old treated for infantile spasms was performed between January 2009 to March 2020 in one pediatric specialized hospital. Variables collected included sex, age, etiology, age at treatment initiation, type of hormonal treatment, dose and duration of treatment, concomitant medication and illness, other corticosteroid uses, hydrocortisone replacement therapy use, adrenal function testing, and signs of AI. Analysis included descriptive statistics, such as incidence and bivariate analysis. Results Thirty-one patients met the inclusion criteria and received a total of 33 separated courses of hormonal treatment. Adrenal function following each course of treatment was evaluated in all patients, either by tests or by the presence of adrenal crisis. Oral hydrocortisone replacement therapy was received by 32/33 (97%) children. AI occurred in 25/33 (76% [95% CI 58-89]) children. There was no predictive factor for adrenal insufficiency after hormonal treatment, and no drug regimen (duration or total received dose) was safe. Two patients (6%) presented to the emergency room with an acute adrenal crisis the day following the weaning off of hormonal treatment. They were the youngest children of the cohort, with an age of 1,6 and 2,7 months at the initiation of treatment. All other patients were aged 4 months and older. Conclusion This study suggests that adrenal suppression is frequent after standard hormonal therapy regimen for infantile spasms. This can lead to serious complications, such as adrenal crisis, if not supplemented. A routine laboratory assessment of adrenal function should be done after hormonal therapy for all patients. We suggest that hydrocortisone replacement therapy should be given at the end of hormonal therapy and until testing results for adrenal function are obtained, particularly for younger patients.

Author(s):  
G Doré-Brabant ◽  
G Laflamme ◽  
M Millette ◽  
B Osterman ◽  
N Chrestian

Background: Hormonal therapy is a standard treatment for infantile spasms. The high doses given and long treatment duration expose patients to the risk of adrenal insufficiency (AI). This study aims to quantify the incidence of AI among children with infantile spasms treated with corticosteroids and/or adrenocorticotropic hormone (ACTH). Methods: A retrospective chart review of patients treated for infantile spasms was performed between January 2009 to March 2020 in one pediatric specialized hospital. Variables collected included patient and treatment characteristics, risk factors of AI and adrenal function testing. Analysis included descriptive statistics. Results: Thirty-one patients met the inclusion criteria and received a total of 33 separated courses of treatment. Adrenal function following each course of treatment was evaluated in all patients. AI occurred in 25/33 (76% [95CI 58-89]) children. There was no predictive factor of AI. No drug regimen was deemed safe. The two patients (6%) with an acute adrenal crisis were the youngest of the cohort. Conclusions: Adrenal suppression is frequent and can lead to adrenal crisis after standard hormonal therapy for infantile spasms. A routine laboratory assessment of adrenal function should be done for all patients. Hydrocortisone replacement therapy should be given until testing results are obtained, particularly for younger infants.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A108-A109
Author(s):  
Nani Oktavia ◽  
Chici Pratiwi ◽  
Jerry Nasaruddin ◽  
Muhammad Ikhsan Mokoagow ◽  
Marina Epriliawati ◽  
...  

Abstract Background: Adrenal crisis is an emergency condition in endocrinology that commonly found in primary adrenal insufficiency but also occur in chronic adrenal insufficiency triggered by various conditions such as sepsis, infection, trauma, burns, surgery, and myocardial infarction. In chronic adrenal insufficiency, adrenal crisis can be induced by excessive reductions or inadequate discontinuation of steroid treatment. Case Illustration: A 40-year old-man admitted with chief complaint abdominal pain since seven days before admission. He felt pain in the umbilical area and slowly radiated to all the part of abdomen. Other than that, he also felt nausea, had vomitus, fever, and constipation for five days. He was immobilized for four months, with muscles weakness and atrophy. He was diagnosed with Focal Segmented Glomerulosclerosis and had a high dose of methylprednisolone (48 mg) for 6 months, 40 mg for the next 2 months and methylprednisolone 12 mg for the last 2 months. The last two month, he began to have severe general weakness and hypotension. On physical examination we found hypotension, fever (38.1o C), pale conjunctiva, moon face, buffalo hump, slightly distended and tenderness of abdomen, normal bowel sound, and purple striae all over the abdomen and extremities. On laboratory examination, Hb was 8.2 (n 11.7 – 15.5 g/dl), leukocytes 10,400 (5.00 – 10.00 x 103/μL), Na 123 (n 135 – 147 mmol/L), random blood glucose 74 (n 70 – 140 mg/dL). On abdominal X ray, there was prominent faecal material and no signs of ileus. No sign of infection found in urinalysis. He had sodium correction, packed red cell transfusion, symptomatic therapy including laxative, methylprednisolone 12 mg, but no improvement of signs and symptoms beside be able to defecate. The abdominal ultrasound gave a normal result. The morning cortisol level was then examined, with the result 14.4 (n 3.7–19.4). The patient was then diagnosed with adrenal crisis based on the clinical manifestations and had hydrocortisone therapy 100 mg a day for 2 consecutive days. After hydrocortisone administration, the symptoms improved, no fever and abdominal pain, he had normotension, increased sodium level 132 (n 135 – 147 mmol/L) and blood glucose level 118 (n 70 – 140 mg/dL). On the third day the patient discharged with oral hydrocortisone 15 mg in the morning and 10 mg in the afternoon. Conclusion: Adrenal crisis was generally found in primary adrenal insufficiency but could also occur in secondary adrenal insufficiency due to inappropriate tapering off process of long term glucocorticoid use.


2018 ◽  
Vol 104 (3) ◽  
pp. 779-784 ◽  
Author(s):  
Carla Colombo ◽  
Simone De Leo ◽  
Marta Di Stefano ◽  
Guia Vannucchi ◽  
Luca Persani ◽  
...  

Abstract Context Two tyrosine kinase inhibitors (TKIs), lenvatinib and vandetanib, are often used to treat advanced radioiodine-refractory differentiated thyroid cancer (RAI-R DTC) and medullary thyroid cancer (MTC), respectively. Fatigue is a common adverse event during treatment with these and other TKIs and a common cause of drug discontinuation or dosage reduction. Cases Description We evaluated the basal and stimulated adrenal function in 12 patients with advanced RAI-R DTC and MTC treated with lenvatinib or vandetanib, respectively. Ten patients complaining of fatigue showed a progressive ACTH increase with normal cortisol levels. Moreover, six of 10 patients had a blunted cortisol response after ACTH stimulation, thus confirming the diagnosis of primary adrenal insufficiency (PAI). The causal relationship between TKIs and PAI onset was also demonstrated by the repeated testing of adrenal function before and during treatment. Patients with PAI received cortisone acetate replacement therapy, with a substantial and prompt improvement in the degree of fatigue, as assessed by the Common Terminology Criteria for Adverse Events version 4.03, thus supporting the major impact of impaired adrenal function in the genesis of this adverse event. Conclusions We show that the occurrence of PAI may be a common cause of fatigue during lenvatinib and vandetanib treatment, and we therefore recommend testing adrenal function for a prompt start of replacement therapy to avoid treatment discontinuation, dosage reduction, and potentially severe PAI complications.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Masanori Murakami ◽  
Noriko Matsushita ◽  
Rie Arai ◽  
Naohiro Takahashi ◽  
Ryuki Kawamura ◽  
...  

We report a 65-year-old woman with isolated adrenocorticotropic hormone (ACTH) deficiency. The patient was transported to the emergency outpatient department by ambulance complaining of malaise and nausea. Because her laboratory data revealed hyponatremia, we performed endocrinological examinations and diagnosed isolated ACTH deficiency. After admission, she went into a delirious state and suffered from takotsubo cardiomyopathy due to adrenal insufficiency. Replacement therapy with hydrocortisone sufficiently improved her delirium and cardiomyopathy. We conclude that her unstable mental state and myocardial dysfunction were closely related to adrenal insufficiency and suggest that adrenal crisis may cause delirium and Takotsubo cardiomyopathy.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5862-5862 ◽  
Author(s):  
Ahmad Hatem Mattour ◽  
Shatha Farhan ◽  
Nalini Janakiraman ◽  
Edward Peres

Abstract Background High dose chemotherapy (HDC) followed by autologous stem cell transplant (ASCT) is considered the standard of care for patients with multiple myeloma (MM). With most patients receiving induction therapy that includes corticosteroids. The combined effect of prior therapy for myeloma and ASCT related complications may result in hypotension and require intensive medical treatment. To date the incidence of adrenal insufficiency in the setting of ASCT is unknown. The effects of this underlying disorder in regards to post transplant outcome remain unknown as well. We set out to compare the outcomes of patients with multiple myeloma who underwent ASCT with adrenal insufficiency compared to those with sufficient adrenal function. Methods We undertook a prospective study in 13 consecutive patients with Multiple Myeloma admitted for high dose chemotherapy and autologous stem cell transplant at Henry Ford Hospital between February 2014 through June 2014, with the first patient sample being obtained in on 2/14/2014 and last sample recorded in 6/23/2014. Random cortisol levels were obtained on the day of admission or day -2 (figure 1), prior to the start of high dose chemotherapy. All prior therapies included corticosteroids and consisted of the following RVD, DCEP, RD, VD, DT-PACE. Patients were classified into two groups those with cortisol levels > 5 and those patients who had cortisol levels <5. Endpoints analyzed included hypotension, septic shock, and duration of antibiotic therapy. Results Of the 13 patients analyzed the median age was 60 years old (range 53-78), gender 8 male patients 5 female. All patients underwent high dose chemotherapy with Melphalan and Autologous stem cell transplant. Of the 13 patients in which data was obtained 3/13 23% had adrenal insufficiency prior to high dose Melphalan and ASCT. With a median cortisol level of 3.2 in the patient cohort who were found to be adrenal insufficient. The incidence of hypotension was 2/3 66% in the adrenal insufficient patients compared 1/10 10% in the cohort with sufficient adrenal function. Septic shock occurred in 2/3 66% of the adrenal insufficient compared to 0/10 in the adrenal sufficient group. The median duration of antibiotic therapy was 5 days in the adrenal insufficient cohort compared to 2 days in the patients with adequate adrenal function. Conclusion In this small cohort of consecutive patients from a single center, we found that there was a high incidence of adrenal insufficiency 23% in patients undergoing ASCT. The treatment regimens varied in the study group with all patients receiving corticosteroid therapy in there induction regimen. Given the unexpectedly high incidence of adrenal insufficiency a consideration should be given to check a random cortisol level prior to the initiation of HDC. Supplemental therapy or treatment should be considered in this high risk group to avoid unnecessary complications and prolonged antibiotic use and supportive care in this patient cohort. Figure 1. Random Cortisol levels obtained prior to Autologous stem cell transplant Figure 1. Random Cortisol levels obtained prior to Autologous stem cell transplant Disclosures No relevant conflicts of interest to declare.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A121-A121
Author(s):  
Joseph Raco ◽  
Norhan Shamloul ◽  
Rohit Jain

Abstract Background: Adrenal insufficiency (AI) is a life-threatening disorder that is generally caused by primary adrenal failure or by hypothalamic-pituitary impairment of the corticotropic axis. Patients often present with fatigue, hypotension, anorexia, and weight loss. There are few reported cases of AI following bariatric surgery; a type of procedure designed to cause weight loss and anorexia - two of the cardinal symptoms of AI. Establishing a diagnosis of AI in patients who have undergone bariatric surgery can be challenging due to symptom overlap, but it is of critical importance given that an acute stressor has the potential to precipitate adrenal crisis with risk of death. Clinical Case: A 42-year-old woman presented 15-months after sleeve gastrectomy with biliopancreatic diversion and duodenal switch with nausea, vomiting, decreased oral intake, weight loss, weakness and fatigue. Her symptoms were severe, requiring admission to the hospital and were initially thought to be a result of her bariatric surgery. Initial intake exam and labs were notable for mild hypotension, persistent hypoglycemia with hemoglobin A1c &lt;3.4%. Further evaluation revealed a low AM cortisol (5.5 μg/dL) and diagnosis of AI was confirmed by a failed cosyntropin stimulation test with cortisol levels of 7.0 μg/dL and 10.6 μg/dL 30- and 60-minutes after cosyntropin administration, respectively. Her diagnosis remained confounded by the presence of concomitant micronutrient deficiencies including copper, zinc, vitamin D and vitamin B6. Furthermore, she was found to have low insulin and c-peptide levels of 1 μU/mL and 0.4 ng/ml, respectively, despite persistent hypoglycemia. The patient was treated with enteral nutrition via nasojejunal feeds and glucocorticoid replacement therapy with daily oral hydrocortisone and fludrocortisone. Each of her symptoms improved after initiation of steroid replacement therapy and she was discharged home. Conclusion: Clinicians should keep a high degree of suspicion for adrenal insufficiency in patients who have undergone bariatric surgery due to the degree of symptom overlap in this population.


2019 ◽  
Vol 104 (12) ◽  
pp. 5814-5822
Author(s):  
Reiko Hayashi ◽  
Daisuke Tamada ◽  
Masahiko Murata ◽  
Tetsuhiro Kitamura ◽  
Kosuke Mukai ◽  
...  

Abstract Context Low serum adiponectin and high-density lipoprotein–cholesterol (HDL-C) levels are risk factors for cardiovascular disease. Patients with primary adrenal insufficiency are at higher risk of cardiovascular complications compared with healthy subjects. However, there is no information on the relationship between adiponectin and glucocorticoid replacement therapy in patients with secondary adrenal insufficiency (SAI). Objective To determine the effects of intrinsic adrenal function and glucocorticoid replacement therapy on serum adiponectin levels and lipid profile in patients with SAI. Design Part 1: a cross-sectional study. Part 2: a randomized, double-blind, crossover study. Setting Osaka University Hospital, Osaka, Japan. Patients Part 1: 58 patients diagnosed with nonfunctioning pituitary adenoma who underwent insulin tolerance test (ITT) for assessment of adrenal function. Part 2: 12 SAI patients randomly received hydrocortisone replacement therapy at a dose of 10, 20, or 30 mg/d for 4 weeks per term for three terms. Outcome Measurements Part 1: we analyzed the relationship between serum cortisol levels during ITT and serum adiponectin levels and the lipid profile. Part 2: serum adiponectin levels and lipid profile were measured every 4 weeks. Results Serum levels of adiponectin and HDL-C correlated significantly with peak cortisol levels after ITT. Serum adiponectin and HDL-C levels were significantly lower in patients with SAI than non-SAI. Serum levels of adiponectin and HDL-C increased in a hydrocortisone dose-dependent manner. Conclusions Glucocorticoid replacement therapy increased serum levels of adiponectin, an adipose-derived anti-atherogenic factor, and HDL-C in patients with SAI.


1994 ◽  
Vol 28 (6) ◽  
pp. 779-791 ◽  
Author(s):  
Stuart T. Haines ◽  
Daniel T. Casto

OBJECTIVE: To summarize and evaluate the literature regarding the clinical features, epidemiology, etiology, pathophysiology, and treatment of infantile spasms. DATA SOURCES: A literature search of articles from January 1966 to July 1993 using MEDLINE, EM-Base, and Current Concepts/Life Sciences, as well as bibliographies of relevant articles. STUDY SELECTION: All identified original and review publications regarding the clinical features, epidemiology, etiology, pathophysiology, and treatment of infantile spasms were reviewed. Emphasis was placed on original studies published since 1975. DATA EXTRACTION: Data from published research were extracted and evaluated according to study design, sample size, dosing regimen, outcome measures, and treatment efficacy and safety. DATA SYNTHESIS: Infantile spasms constitute a rare epileptic syndrome with a poor long-term prognosis for normal intellectual development. The spasms are characterized by a brief symmetric contraction of the muscles of the neck, trunk, and/or extremities, often occurring in a series of 2 to more than 100 spasms during a single episode. The disorder is age-specific, with the peak onset of symptoms occurring between 2 and 8 months of age. Spasms of no identifiable cause in infants with normal development prior to the onset of infantile spasms are classified as cryptogenic or idiopathic, whereas those with an identifiable cause are classified as symptomatic. Long-term prognosis is best in cryptogenic cases, with 30–70 percent attaining normal intellect compared with 5–19 percent in symptomatic cases. The etiology and pathophysiology are not well understood. Recent theory postulates that infantile spasms may be caused by an excess of corticotropin-releasing hormone activity during infancy. The suspected association between the whole-cell pertussis vaccine and infantile spasms is coincidental. Few well-designed, prospective, controlled clinical trials for the treatment of infantile spasms have been conducted. CONCLUSIONS: Standard anticonvulsants such as phenytoin, the barbiturates, carbamazepine, and the succinimides have been ineffective. Of the anticonvulsants, only the benzodiazepines, valproic acid, and vigabatrin have shown efficacy in reducing spasm frequency and severity. Hormonal therapy with adrenocorticotropic hormone (ACTH) and/or prednisone has been the most frequently studied treatment modality and appears to be the most effective. Hormonal therapy achieves complete spasm control in 50?75 percent of infants within four weeks of initiation. Opinions differ regarding the relative efficacy between ACTH and prednisone, the need for early initiation of hormonal treatment, and the benefits of high dosages of ACTH (>40 units/d). No treatment has been shown conclusively to improve the long-term intellectual development of these infants. Neurosurgery may be the treatment of choice in select cases when a localized central nervous system abnormality can be demonstrated. Well-designed, blind, prospective clinical trials are needed to answer definitively many lingering questions regarding the treatment of infantile spasms.


Author(s):  
Catarina Roque ◽  
Ricardo Fonseca ◽  
Carlos Tavares Bello ◽  
Carlos Vasconcelos ◽  
António Galzerano ◽  
...  

Summary Primary adrenal lymphoma is a rare malignancy. It frequently presents bilaterally and with symptoms of adrenal insufficiency. Amiodarone may induce secondary organ dysfunction, and thyrotoxicosis develops in 15% of cases. The symptomatology of both conditions is nonspecific, especially in the elderly, and a high suspicion index is necessary for appropriate diagnosis. A 78-year-old female presented to the emergency department with confusion, nausea and vomiting. She had recently been to the emergency department with urinary tract infection, vomiting and acute hypochloremic hyponatremia. Upon re-evaluation, the leukocyturia persisted and because of TSH 0.01 µU/mL and free-T4 68 (10–18) pmol/L, she was admitted to the Endocrinology ward. Further evaluation supported amiodarone-induced thyroiditis type 2. Sepsis ensued, in the setting of nosocomial pneumonia. Hemodynamic instability, hyponatremia, hypoglycemia and vomiting raised the suspicion of adrenocortical insufficiency. Fluid resuscitation and hydrocortisone led to clinical improvement, and adrenal insufficiency was admitted. The thoracoabdominal tomography suggested an endobronchic primary lesion with hepatic and adrenal secondary deposits (6.6 and 7 cm), but this was confirmed neither on pleural effusion nor on bronchofibroscopic fluid analyses. The adrenals were not accessible for biopsy. Despite high-dose hydrocortisone maintenance, the patient died before definite diagnosis. The autopsy confirmed primary non-Hodgkin lymphoma. Learning points: Primary adrenal lymphoma is a rare cause of adrenal insufficiency, but progression can be fast and fatal. Hyperpigmentation is frequently absent. The presenting symptoms are nonspecific and might mimic infection. Disproportion of the general state with signs of specific organ symptomatology is a diagnostic clue. Infection may precipitate adrenal crisis and worsen thyroid function with further adrenal insufficiency exacerbation. In the context of thyrotoxicosis, there may be little clinical response to a therapeutic trial with standard dose glucocorticoids. High-dose glucocorticoid substitution may be required to achieve clinical stability in thyrotoxic patients.


Sign in / Sign up

Export Citation Format

Share Document