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Blood pressure in pediatric patients with Cushing syndrome
Maya B Lodish1, Ninet Sinaii, Nicholas Patronas
1Section on Endocrinology Genetics, Program on Developmental Endocrinology Genetics, National Institute of Child Health and Human Development, NIH, Building 10, CRC, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA. lodishma@mail.nih.gov
Insights
Pediatric Cushing syndrome (CS) patients often have hypertension (HTN) that improves after surgery but can persist. High cortisol levels correlate with blood pressure, and rare serious side effects may occur.
Area of Science:
- Pediatric Endocrinology
- Cardiovascular Health in Children
- Surgical Outcomes
Background:
- Hypertension (HTN) affects up to 60% of children with Cushing syndrome (CS).
- The long-term course, side effects, and differences in HTN based on CS cause are understudied.
- Understanding HTN in pediatric CS is crucial for managing patient outcomes.
Purpose of the Study:
- To assess blood pressure changes in pediatric CS patients pre- and post-surgery (transsphenoidal surgery or adrenalectomy).
- To identify the incidence of residual HTN and associated side effects.
- To compare HTN characteristics between Cushing disease (CD) and ACTH-independent CS (AICS).
Main Methods:
- Analysis of data from 86 children with CD and 27 children with AICS.
- Measurement of blood pressure before and after surgical intervention.
- Evaluation of side effects and rates of residual HTN.
Main Results:
- Significant preoperative HTN was observed in both CD and AICS groups.
- AICS patients had a higher rate of systolic HTN (SHTN) preoperatively compared to CD patients (74% vs. 44%).
- Postoperative HTN significantly decreased, but residual SHTN and diastolic HTN (DHTN) persisted in 16% and 4% (CD) and 21% and 5% (AICS) at one year, respectively. Preoperative blood pressure correlated with cortisol levels. Two patients experienced severe side effects, including hypertensive encephalopathy.
Conclusions:
- Children with CS remain at risk for residual HTN even after successful surgical treatment.
- The degree of hypercortisolemia is linked to the severity of HTN.
- While rare, serious HTN-related complications can occur perioperatively.
Context:
Hypertension (HTN) has been reported in up to 60% of children with Cushing syndrome (CS), but its course, side effects, and potential differences among various causes of CS have not been adequately studied.
Objective:
The objective of the study was to measure blood pressure in pediatric patients with CS before and after transphenoidal surgery or adrenalectomy and identify side effects and rates of residual HTN.
Design:
Data from 86 children with corticotropinomas [Cushing disease (CD)] and 27 children with ACTH-independent CS (AICS) were analyzed.
Results:
Patients with CD and AICS had significant HTN before surgery; more patients with AICS had systolic HTN (SHTN) than with CD (74 vs. 44%, P = 0.0077), but the rate of diastolic HTN (DHTN) was similar. Both groups experienced significant decreases in SHTN immediately after transphenoidal surgery and adrenalectomy. One year postoperatively, both SHTN and DHTN were lower than the preoperative values in all patients, but as many as 16 and 4% of the patients with CD and 21 and 5% of the patients with AICS still had SHTN and DHTN, respectively. Higher blood pressure preoperatively correlated with cortisol levels. Two patients suffered serious side effects: one with multiple infarcts and another with hypertensive encephalopathy.
Conclusions:
Children with CS are at risk for residual HTN despite a significant improvement after surgical cure. HTN appears to correlate with the degree of hypercortisolemia. Serious HTN-related side effects, although rare, may occur during the perioperative period.
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