Jump to content

Search the Community

Showing results for tags 'child'.

The search index is currently processing. Current results may not be complete.
  • Search By Tags

    Type tags separated by commas.
  • Search By Author

Content Type


Forums

  • Welcome!
    • Introduce Yourself
    • Guest Questions
    • Cushing's Basics
    • News Items and Research
    • Announcements
    • Questions about how these boards work?
  • Get Active!
    • Meetings, events and information
    • Fundraising Ideas
    • Cushing's Awareness Day, April 8
    • Spread the Word
    • Marathons
    • Cushing's Clothes Closet
    • Cushing's Library
    • Cushing's Store
  • Cushing's
    • Resources
    • Types of Cushing's
    • Symptoms
    • Tests
    • Treatments
  • Miscellaneous
    • Other Diseases
    • Good News / Attitude of Gratitude
    • Inspirational / Motivational
    • Quotes and Affirmations
    • Lighten Up!
    • Word Games
    • Miscellaneous Chit Chat
    • Current Events
    • Cushie Commerce
    • Internet Classes
    • Recipes

Calendars

  • Cushie Calendar

Blogs

  • MaryO'Blog
  • Christy Smith's Blog
  • rooon55's Blog
  • LLMart's Blog
  • regina from florida's Blog
  • terri's Blog
  • Canasa's Blog
  • Tberry's Blog
  • LisaMK's Blog
  • diane177432's Blog
  • Jen1978's Blog
  • GreenGal's Blog
  • Yada Yada Yada
  • Jinxie's Blog
  • SherryC's Blog
  • stjfs' Blog
  • kalimae7371's Blog
  • Kristy's Blog
  • kathieb1's Blog
  • Yavanna's Blog
  • Johnni's Blog
  • AutumnOMA's Blog
  • Will Power
  • dropsofjupiter's Blog
  • Lorrie's Blog
  • DebMV's Blog
  • FarWind's Blog
  • sallyt's Blog
  • dseefeldt's Blog
  • ladylena's Blog
  • steffie's Blog
  • Lori L's Blog
  • mysticalsusan1's Blog
  • cathy442's Blog
  • Kathy711's Blog
  • Shannonsmom's Blog
  • jack's Blog
  • Kandy66's Blog
  • mars72's Blog
  • singlesweetness33's Blog
  • michelletm's Blog
  • JC_Adair's Blog
  • Lisa-A's Blog
  • Jen3's Blog
  • tammi's Blog
  • Ramblin' Rose (Maggie's)
  • monicaroni77's Blog
  • monicaroni's Blog
  • Saz's Blog
  • alison
  • Thankful for the Journey
  • Judy from Pgh's Blog
  • Addiegirl's Blog
  • candlelite2000's Blog
  • Courtney likes to talk......
  • Tanya's Blog
  • smoketooash's Blog
  • meyerfamily8's Blog
  • Sheila1366's Blog
  • A Guide to Blogging...
  • Karen's Blog
  • barbj222222's Blog
  • Amdy's Blog
  • Jesh's Blog
  • pumpkin's Blog
  • Jazlady's Blog
  • Cristalrose's Blog
  • kikicee's Blog
  • bordergirl's Blog
  • Shelby's Blog
  • terry.t's Blog
  • CanadianGuy's Blog
  • Mar's Cushie Couch
  • leanne's Blog
  • honeybee30's Blog
  • cat lady's Blog
  • Denarea's Blog
  • Caroline's Blog
  • NatalieC's Blog
  • Ahnjhnsn's Blog
  • A journey around my brain!
  • wisconsin's Blog
  • sonda's Blog
  • Siobhan2007's Blog
  • mariahjo's Blog
  • garcia9's Blog
  • Jessie's Blog
  • Elise T.'s Blog
  • glandular-mass' Blog
  • Rachel Bridgewater's Blog
  • judycolby's Blog
  • CathyM's Blog
  • MelissaTX's Blog
  • nessie21's Blog
  • crzycarin's Blog
  • Drenfro's Blog
  • CathyMc's Blog
  • joanna27's Blog
  • Just my thoughts!
  • copacabana's Blog
  • msmith3033's Blog
  • EyeRishGrl's Blog
  • SaintPaul's Blog
  • joyce's Blog
  • Tara Lou's Blog
  • penybobeny's Blog
  • From Where I Sit
  • Questions..
  • jennsarad's Blog
  • looking4answers2's Blog
  • julie's blog
  • cushiemom's Blog
  • greydragon's Blog
  • AmandaL's Blog
  • KWDesigns: My Cushings Journey
  • cushieleigh's Blog
  • chelser245's Blog
  • melissa1375's Blog
  • MissClaudie's Blog
  • missclaudie92's Blog
  • EEYORETJBD's Blog
  • Courtney's Blog
  • Dawn's Blog
  • Lindsay's Blog
  • rosa's Blog
  • Marva's Blog
  • kimmy's Blog
  • Cheryl's Blog
  • MissingMe's Blog
  • FerolV's Blog
  • Audrey's (phil1088) Blog
  • sugarbakerqueen's Blog
  • KathyBair's Blog
  • Jenn's Blog
  • LisaE's Blog
  • qpdoll's Blog
  • blogs_blog_140
  • beach's Blog
  • Reillmommy is Looking for Answers...
  • natashac's Blog
  • Lisa72's Blog
  • medcats10's Blog
  • KaitlynElissa's Blog
  • shygirlxoxo's Blog
  • kerrim's Blog
  • Nicki's Blog
  • MOPPSEY's Blog
  • Betty's Blog
  • And the beat goes on...
  • Lynn's Blog
  • marionstar's Blog
  • floweroscotland's Blog
  • SleepyTimeTea's Blog
  • Shelly3's Blog
  • fatnsassy's Blog
  • gaga's Blog
  • Jewels' Blog
  • SusieQ's Blog
  • kayc6751's Blog
  • moonlight's Blog
  • Sick of Being Sick
  • Peggy's Blog
  • kouta5m's Blog
  • TerryC's Blog
  • snowii's Blog
  • azZ9's Blog
  • MaMaT333's Blog
  • missaf's Blog
  • libertybell's Blog
  • LyssaFace's Blog
  • suzypar2002's Blog
  • Mutley's Blog
  • superc's Blog
  • lisajo42's Blog
  • alaustin's Blog
  • Tina1962's Blog
  • Ill never complain a single word about anything.. If I get rid of Cushings disease.
  • puddingtoast's Blog
  • AmberC's Blog
  • annacox
  • justwaiting's Blog
  • RachaelB's Blog
  • MelanieW's Blog
  • My Blog
  • FLHeather's Blog
  • HollieK's Blog
  • Bonny777's Blog
  • KatieO's Blog
  • LilDickens' Mini World
  • MelissaG's Blog
  • KelseyMichelle's Blog
  • Synergy's Blog
  • Carolyn1435's Blog
  • Disease is ugly! Do I have to be?
  • A journey of a thousand miles begins with a single wobble
  • MichelleK's Blog
  • lenalee's Blog
  • DebGal's Blog
  • Needed Answers
  • Dannetts Blog
  • Marisa's Blog
  • Is this cushings?
  • alicia26's Blog
  • happymish's Blog
  • mileymo's Blog
  • It's a Cushie Life!
  • The Weary Zebra
  • mthrgonenuts' Blog
  • LoriW's Blog
  • WendyG's Blog
  • khmood's Blog
  • Finding Answers and Pissing Everyone Off Along the Way
  • elainewwjd's Blog
  • brie's Blog
  • dturner242's Blog
  • dturner242's Blog
  • dturner242's Blog
  • Stop the Violins
  • FerolV's Internal Blog
  • beelzebubble's Blog
  • RingetteLUVR
  • Eaglemtnlake's Blog
  • mck25's Blog
  • vicki11's Blog
  • vicki11's Blog
  • ChrissyL's Blog
  • tpatterson757's Blog
  • Falling2Grace's Blog
  • meeks089's Blog
  • JustCurious' Blog
  • Squeak's Blog
  • Kill Bill
  • So It Begins ! Cushings / Pituitary Microadenoma
  • Crystal34's Blog
  • Janice Barrett

Categories

  • Helpful Articles
    • Links
    • Research and News
    • Useful Information
  • Pages
  • Miscellaneous
    • Databases
    • Templates
    • Media

Categories

  • New Features
  • Other

Find results in...

Find results that contain...


Date Created

  • Start

    End


Last Updated

  • Start

    End


Filter by number of...

Joined

  • Start

    End


Group


AIM


MSN


Website URL


ICQ


Yahoo


Jabber


Skype


Location


Interests

Found 5 results

  1. Abstract Cushing's syndrome is a rare entity in children. Adrenal tumour is the common cause of this syndrome in young children, whereas, iatrogenic causes are more common among older children. We report a 4 year old male child diagnosed with Cushing syndrome due to a right adrenal adenoma; the child presented with obesity and increase distribution of body hair. After thorough investigation and control of hypertension and dyselectrolytemia, right adrenalectomy was performed. The patient had good clinical recovery with weight loss and biochemical resolution of Cushing's syndrome. 1. Introduction Cushing's syndrome (CS) is rarely encountered in children. The overall incidence of Cushing syndrome is approximately 2–5 new cases per million people per year. Only approximately 10% of the new cases each year occur in children [1]. Unlike in adults, a male-to-female predominance have been observed in infants and young toddlers [[1], [2], [3]]. Although iatrogenic causes are common in children above seven years of age, adrenal causes (adenoma, carcinoma or hyperplasia) are common in children of younger age [4]. We report a 4 year old boy diagnosed with Cushing syndrome caused by a right adrenal adenoma, who had presented with obesity and increase distribution of body hair. Right adrenalectomy was performed and clinical stabilization resulted in weight loss and biochemical resolution of Cushing's syndrome. (see Fig. 5) 2. Case report A 4 years old boy presented with complaints of excessive weight gain of 5 months duration and increase frequency of micturition and appearance of body hair for 4 months. There was no history of any other illness, medication or steroid intake. The child was first born at term by normal vaginal delivery and birth weight of 3 kg. Physical examination revealed a chubby boy with moon face, buffalo hump, protruding abdomen, increase body hair and appearance of coarse pubic hair (Fig. 1). His intelligent quotient (IQ) was appropriate for his age and sex. His younger sibling was in good health and other family members did not have any metabolic or similar problems. Download : Download high-res image (710KB) Download : Download full-size image Fig. 1. The child with moon face, protruded abdomen and coarse body hair. The patient's body length was 92cm (between -2SD to -3SD), weight 20kg (between 1 SD and 2 SD), weight for height >3SD, and BMI was 23.6 (BMI for age >3 SD). His blood pressure on right arm in lying position was 138/76 mm Hg (above 99th percentile for height and age). Investigations: Morning 8am serum cortisol level - 27.3 μg/dl (normal: 6–23 μg/dl). with a concurrent plasma ACTH level of < 5 pg/ml (n value < 46 pg/ml). His serum cortisol following low dose dexamethasone suppression test (1mg dexamethasone at 11pm) at 8 am next morning was 22.1 μug/dl and his 24 hours urine catecholamine fraction was within normal limit. HB % -- 10.3 gm/dl; LDDST -- 25 μg/dl; FBS -- 106 mg/dl. Serum Na+ - 140.6mmol/l; K+ - 2.83mmol/l; Ca+ - 8.7 mg/dl. S. Creatinine −0.3 mg/dl. Ultrasonography of abdomen revealed a heterogenous predominantly hypoechoic right supra renal mass. Contrast enhanced CT abdomen revealed well defined soft tissue density lesion (size −5.2 cm × 5.2 cm x 5.7cm) in right adrenal gland with calcifications and fat attenuations showing mild attenuation on post contrast study (Fig. 2). Download : Download high-res image (703KB) Download : Download full-size image Fig. 2. CECT shows right adrenal mass with calcification and mild attenuation on post-contrast study. The child was started on oral amlodipine 2.5mg 12hourly; after 5days blood pressure became normal. For hypokalemia oral potassium was given @20 meq 8 hourly and serum potassium value became normal after 4 days. Right laparoscopic adrenalectomy was planned. but due to intra operative technical problems it was converted to an open adrenalectomy with right subcostal incision. A lobulated mass of size 9 cm × 5 cm x 4 cm with intact capsule was excised. The tumour weighed 230 gm. There was no adhesion with adjacent organs, three regional nodes were enlarged but without any tumour tissue. Inferior vena cava was spared. Histopathology report was consistent with adrenal adenoma (Fig. 3) (see Fig. 4). Download : Download high-res image (427KB) Download : Download full-size image Fig. 3. Cut section of tumour shows fleshy mass with fatty tissue. Download : Download high-res image (618KB) Download : Download full-size image Fig. 4. Microphotograph (100 × 10) showing intact capsule and adrenal tumour cells, which are larger in size with nuclear pleomorphism, inconspicuous nucleoli, cytoplasm of the tumour cells are abundant, eosinophilic and vacuolated. Download : Download high-res image (593KB) Download : Download full-size image Fig. 5. Physical appearance 4 months after adrenalectomy. Post operative management: during post operative period hypokalemia and flaxuating blood sugar level was managed with oral potassium and oral glucose supplement. patient developed mild cough and respiratory distress on post op day 2, it was managed with salbutamol nebulization and respiratory physio therapy. Patient developed minor ssi and discharged on 10 th post operative day with oral prednisolone supplementation. Follow up: the patient was followed up 2week after discharge and then every monthly, the oral prednisolone was gradually tapered and completely withdrawn on 2nd month after surgery.The patient experienced no post-surgical complications. After 4 months of surgery he reduces 6 kgs of his body weight with BMI of 16.5 (between median and 1SD) & BP 100/74 mm hg (within normal range), the moon face, buffalo hump, central obesity disappeared, morning 8am serum cortisol level was found within normal range 14 μg/dl (n value 6–23 μg/dl). 3. Discussion Cushing's syndrome is caused by prolonged exposure to supraphysiological levels of circulating glucocorticoids, which may be endogenously or exogenously derived. During infancy, CS is usually associated with McCune-Albright syndrome; adrenocortical tumours most commonly occur in children under four years of age and Cushing's disease (ACTH dependent) is the commonest cause of CS after five years of age [5]. Primary adrenocortical tumours (ACTs) account for only 0.3–0.4% of all childhood neoplasms. Almost a third of these tumours manifests as Cushing syndrome and over 70% of the unilateral tumours in young children are often malignant [2,3,6,7]. There seems to be a bimodal incidence of these tumours, with one peak at under 5 years of age and the second one in the fourth or fifth decades of life. ACTs may be associated with other syndromes, such as, Li-Fraumeni syndrome, Beckwith-wiedemann syndrome, isolated hemihypertrophy, or even a germline point mutation of P53 tumour suppressor gene as reported in a series from Brazil [8]. In comparison to adult CS, growth failure with associated weight gain is one of the most reliable indicators of hypercortisolaemia in pediatric CS. The parents often fail to notice facial changes and growth failure and hence the diagnosis is often delayed. In one study, the mean time from appearing symptoms to diagnosis in 33 children with Cushing's disease was 2.5 years [5]. More recently the comparison of height and BMI SDS measurements provided a sensitive diagnostic discriminator in pediatric patients with CD and those with simple obesity [9]. In the present case, the parents observed noticeable changes in his face and presence of body hair, which made them to bring the child to medical attention. A review of 254 children on the International Pediatric Adrenocortical Tumour Registry identified virilization as the most common manifestation [10]. About 10% of the tumours can be non-functional at presentation, and approximately one third of pediatric patients present with hypertension. Majority of patients (192/254) in the Registry had localized disease and metastatic disease was found in less than 5% of cases. Older children with CS or mixed androgen and cortisol secreting adrenocortical tumours had a worse prognosis compared to younger children [10]. The present case had mild hypertension as well as dyselectrolytemia at presentation, which could be controlled with medication. He had a single adenoma confined to the adrenal gland and there was no evidence of malignancy. After surgical excision of the tumour and the right adrenal gland, the patient made rapid improvement in clinical condition and has been on follow up for last 7 months. 4. Conclusion Pediatric adrenocortical tumours (ACTs) are most commonly encountered in females and in children less than four years. But our case being an 4-year-old boy forms a rare presentation of endogenous Cushing's syndrome due to adrenal adenoma. Cushing's syndrome in this child was controlled after right adrenalectomy. Patient consent Informed written consent was taken. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for authorship. Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. References [1] M.A. Magiakou, G. Mastorakos, E.H. Oldfield, et al. Cushing's syndrome in children and adolescents. Presentation, diagnosis, and therapy N Engl J Med, 331 (10) (1994), pp. 629-636 [PubMed: 8052272] [2] C. Tsigos, G.P. Chrousos Differential diagnosis and management of Cushing's syndrome Annu Rev Med, 47 (1996), pp. 443-461 [PubMed: 8712794] [3] D.N. Orth Cushing's syndrome N Engl J Med, 332 (12) (1995), pp. 791-803 [PubMed: 7862184] [4] C.J. Migeon, R. Lanes (fifth ed.)F. Lifshitz (Ed.), “Adrenal cortex: hypo and hyper_x0002_function,” in Pediatric Endocrinology, vol. 8, Informa Healthcare, London, UK (2007), p. 214 [5] L.F. Chan, H.L. Storr, A.B. Grossman, M.O. Savage Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment Arq Bras Endocrinol Metabol, 51 (8) (2007), pp. 1261-1271, 10.1590/S0004-273 [6] C.A. Stratakis, L.S. Kirschner Clinical and genetic analysis of primary bilateral adrenal diseases(micro- and macronodular disease) leading to Cushing syndrome Horm Metab Res, 30 (6–7) (1998), pp. 456-463 [PubMed: 9694579] [7] W.L. Miller, J.J. Townsend, M.M. Grumbach, S.L. Kaplan An infant with Cushing's disease due to anadrenocorticotropin-producing pituitary adenoma J Clin Endocrinol Metabol, 48 (6) (1979), pp. 1017-1025 [8] R.C. Ribeiro, F. Sandrini, B. Figueiredo, G.P. Zambetti, E. Michalkiewicz, A.R. Lafferty, et al. An inherited P53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma Proc Natl Acad Sci U S A, 98 (16) (2001), pp. 9330-9335 [9] J.E. Greening, H.L. Storr, S.A. McKenzie, K.M. Davies, L. Martin, A.B. Grossman, et al. Linear growth and body mass index in pediatric patients with Cushing's disease or simple obesity J Endocrinol Invest, 29 (10) (2006), pp. 885-887 [10] E. Michalkiewicz, R. Sandrini, B. Figueiredo, E.C. Miranda, E. Caran, A.G. Oliveira-Filho, et al. Clinical and outcome characteristics of children with adrenocortical tumors: a report from the international pediatric adrenocortical tumor Registry J Clin Oncol, 22 (5) (2004), pp. 838-845 From https://www.sciencedirect.com/science/article/pii/S2213576620303833
  2. The treatment of adrenal insufficiency with hydrocortisone granules in children with congenital adrenal hyperplasia (CAH) was associated with an absence of adrenal crises and normal growth patterns over a 2-year period, according to study findings published in The Journal of Clinical Endocrinology and Metabolism. The study included a total of 17 children with CAH and 1 child with hypopituitarism. All included participants were <6 years old who were receiving current adrenocortical replacement therapy, including hydrocortisone with or without fludrocortisone. Hydrocortisone medications used in this population were converted from pharmacy compounded capsules to hydrocortisone granules without changing the dose. These study participants were followed by study investigators for 2 years. Glucocorticoid replacement therapy was given three times a day for a median treatment duration of 795 days. Treatment was adjusted by 3 monthly 17-hydroxyprogesterone (17-OHP) profiles in children with CAH. There were a 150 follow-up visits throughout the study. At each visit, participants underwent assessments that measured hydrocortisone dose, height, weight, pubertal status, adverse events, and incidence of adrenal crisis. A total of 40 follow-up visits had changes in hydrocortisone doses based on salivary measurements (n=32) and serum 17-OHP levels (n=8). At time of study entry, the median daily doses of hydrocortisone were 11.9 mg/m2 for children between the ages of 2 to 8 years, 9.9 mg/m2 for children between 1 month and 2 years, and 12.0 mg/m2 for children <28 days of age. At the end of the study, the respective doses for the 3 age groups were 10.2, 9.8, and 8.6. The investigators observed no trends in either accelerated growth or reduced growth; however, 1 patient with congenital renal hypoplasia and CAH did show reduced growth. While 193 treatment-emergent adverse events, including pyrexia, gastroenteritis, and viral upper respiratory tract infection, were reported in 14 patients, there were no observed adrenal crises. Limitations of this study included the small sample size as well as the relatively high drop-out rate of the initial sample. The researchers concluded that “hydrocortisone granules are an effective treatment for childhood adrenal insufficiency providing the ability to accurately prescribe pediatric appropriate doses.” Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures. Reference Neumann U, Braune K, Whitaker MJ, et al. A prospective study of children 0-7 years with CAH and adrenal insufficiency treated with hydrocortisone granules. Published online September 4, 2020. J Clin Endocrinol Metab. doi:10.1210/clinem/dgaa626
  3. Childs Nerv Syst. 2018 Nov 28. doi: 10.1007/s00381-018-4013-5. [Epub ahead of print] Gazioglu N1, Canaz H2, Camlar M3, Tanrıöver N4, Kocer N5, Islak C5, Evliyaoglu O6, Ercan O6. Author information Abstract AIM: Pituitary adenomas are rare in childhood in contrast with adults. Adrenocorticotropic hormone (ACTH)-secreting adenomas account for Cushing's disease (CD) which is the most common form of ACTH-dependent Cushing's syndrome (CS). Treatment strategies are generally based on data of adult CD patients, although some difficulties and differences exist in pediatric patients. The aim of this study is to share our experience of 10 children and adolescents with CD. PATIENTS AND METHOD: Medical records, images, and operative notes of 10 consecutive children and adolescents who underwent transsphenoidal surgery for CD between 1999 and 2014 in Cerrahpasa Faculty of Medicine were retrospectively reviewed. Mean age at operation was 14.8 ± 4.2 years (range 5-18). The mean length of symptoms was 24.2 months. The mean follow-up period was 11 years (range 4 to 19 years). RESULTS: Mean preoperative cortisol level was 23.435 μg/dl (range 8.81-59.8 μg/dl). Mean preoperative ACTH level was 57.358 μg/dl (range 28.9-139.9 μg/dl). MR images localized microadenoma in three patients (30%), macroadenoma in four patients (40%) in our series. Transsphenoidal microsurgery and endoscopic transsphenoidal surgery were performed in 8 and 2 patients respectively. Remission was provided in 8 patients (80%). Five patients (50%) met remission criteria after initial operations. Three patients (30%) underwent additional operations to meet remission criteria. CONCLUSION: Transsphenoidal surgery remains the mainstay therapy for CD in pediatric patients as well as adults. It is an effective treatment option with low rate of complications. Both endoscopic and microscopic approaches provide safe access to sella and satisfactory surgical results. KEYWORDS: Cushing’s disease; Endoscopic pituitary surgery; Pediatric; Transsphenoidal microsurgery PMID: 30488233 DOI: 10.1007/s00381-018-4013-5 Full Text
  4. Children with Cushing’s syndrome are at risk of developing new autoimmune and related disorders after being cured of the disease, a new study shows. The study, “Incidence of Autoimmune and Related Disorders After Resolution of Endogenous Cushing Syndrome in Children,” was published in Hormone and Metabolic Research. Patients with Cushing’s syndrome have excess levels of the hormone cortisol, a corticosteroid that inhibits the effects of the immune system. As a result, these patients are protected from autoimmune and related diseases. But it is not known if the risk rises after their disease is resolved. To address this, researchers at the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) examined 127 children with Cushing’s syndrome at the National Institutes of Health from 1997 until 2017. Among the participants, 77.5 percent had a pituitary tumor causing the disease, 21.7 percent had ACTH-independent disease, and one patient had ectopic Cushing’s syndrome. All patients underwent surgery to treat their symptoms. After a mean follow-up of 31.2 months, 7.8 percent of patients developed a new autoimmune or related disorder. Researchers found no significant differences in age at diagnosis, gender, cortisol levels, and urinary-free cortisol at diagnosis, when comparing those who developed autoimmune disorders with those who didn’t. However, those who developed an immune disorder had a significantly shorter symptom duration of Cushing’s syndrome. This suggests that increased cortisol levels, even for a short period of time, may contribute to more reactivity of the immune system after treatment. The new disorder was diagnosed, on average, 9.8 months after Cushing’s treatment. The disorders reported were celiac disease, psoriasis, Hashimoto thyroiditis, Graves disease, optic nerve inflammation, skin hypopigmentation/vitiligo, allergic rhinitis/asthma, and nerve cell damage of unknown origin responsive to glucocorticoids. “Although the size of our cohort did not allow for comparison of the frequency with the general population, it seems that there was a higher frequency of optic neuritis than expected,” the researchers stated. It is still unclear why autoimmune disorders tend to develop after Cushing’s resolution, but the researchers hypothesized it could be a consequence of the impact of glucocorticoids on the immune system. Overall, the study shows that children with Cushing’s syndrome are at risk for autoimmune and related disorders after their condition is managed. “The presentation of new autoimmune diseases or recurrence of previously known autoimmune conditions should be considered when concerning symptoms arise,” the researchers stated. Additional studies are warranted to further explore this link and improve care of this specific population. From https://cushingsdiseasenews.com/2018/03/06/after-cushings-cured-autoimmune-disease-risk-looms-study/
  5. ORLANDO – Cushing’s disease may begin to exert its harmful cardiovascular effects quite early, a small pediatric study has found. Children as young as 6 years old with the disorder already may show signs of cardiovascular remodeling, with stiffer aortas and higher aortic pulse-wave velocity than do age-matched controls, Hailey Blain and Maya Lodish, MD, said at the annual meeting of the Endocrine Society. “The study, which included 10 patients, is small, but we continue to add new patients,” said Dr. Lodish, director of the pediatric endocrinology fellowship program at the Eunice Kennedy Shriver National Institute of Child Health and Human Development. Ten more children are being added to the cohort now, and she and Ms. Blain, a former research fellow at NIH, intend to grow the group and follow patients longitudinally. Cushing’s disease has long been linked with increased cardiovascular risk in adults, but the study by Dr. Lodish and Ms. Blain is one of the first to examine the link in children. Their findings suggest that early cardiovascular risk factor management should be a routine part of these patients’ care, Dr. Lodish said in an interview. “It’s very important to make sure that there is recognition of the cardiovascular risk factors that go along with this disease. Elevated levels of cholesterol, hypertension, and other risk factors that are in these individuals should be ameliorated as soon as possible from an early age and, most importantly, physicians should be diagnosing and treating children early, once they are identified as having Cushing’s disease. And, given that we are not sure whether these changes are reversible, we need to make sure these children are followed very closely.” Indeed, Dr. Lodish has reason to believe that the changes may be long lasting or even permanent. “We are looking at these children longitudinally and have 3-year data on some patients already. We want to see if they return to normal pulse wave velocity after surgical cure, or whether this is permanent remodeling. There is an implication already that it may be in a subset of individuals,” she said, citing her own 2009 study on hypertension in pediatric Cushing’s patients. “We looked at blood pressure at presentation, after surgical cure, and 1 year later. A significant portion of the kids still had hypertension at 1 year. This leads us to wonder if they will continue to be at risk for cardiovascular morbidity as adults.” Ms. Blaine, an undergraduate at Bowdoin College, Brunswick, Maine, worked on the study during a summer internship with Dr. Lodish and presented its results in a poster forum during meeting. She examined two indicators of cardiovascular remodeling – aortic pulse wave velocity and aortic distensibility – in 10 patients who were a mean of 13 years old. All of the children came to NIH for diagnosis and treatment of Cushing’s; as part of that, all underwent a cardiac MRI. The patients had a mean 2.5-year history of Cushing’s disease Their mean midnight cortisol level was 18.8 mcg/dL and mean plasma adrenocorticotropic hormone level, 77.3 pg/mL. Five patients were taking antihypertensive medications. Low- and high-density lipoprotein levels were acceptable in all patients. The cardiovascular measures were compared to an age-matched historical control group. In this comparison, patients had significantly higher pulse wave velocity compared with controls (mean 4 vs. 3.4 m/s). Pulse wave velocity positively correlated with both midnight plasma cortisol and 24-hour urinary free cortisol collections. In the three patients with long-term follow-up after surgical cure of Cushing’s, the pulse wave velocity did not improve, either at 6 months or 1 year after surgery. This finding echoes those of Dr. Lodish’s 2009 paper, suggesting that once cardiovascular remodeling sets in, the changes may be long lasting. “The link between Cushing’s and cardiovascular remodeling is related to the other things that go along with the disease,” Dr. Lodish said. “The hypertension, the adiposity, and the high cholesterol all may contribute to arterial rigidity. It’s also thought to be due to an increase in connective tissue. The bioelastic function of the aorta may be affected by having Cushing’s.” That connection also suggests that certain antihypertensives may be more beneficial to patients with Cushing’s disease, she added. “It might have an implication in what blood pressure drug you use. Angiotensin-converting enzyme inhibitors increase vascular distensibility and inhibit collagen formation and fibrosis. It is a pilot study and needs longitudinal follow up and additional patient accrual, however, finding signs of cardiovascular remodeling in young children with Cushing’s is intriguing and deserves further study.” Neither Ms. Blain nor Dr. Lodish had any financial disclosures. msullivan@frontlinemedcom.com
×
×
  • Create New...