Pituitary Stalk Interruption Syndrome

Fatima, Tehreem; Chandio, Sajjad Hussain; Muzaffar, Kainat; Mumtaz, Hassan; Jahan, Nusrat

doi:10.7759/cureus.10518

Cited by 5 publications

(9 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1 Epidemiologically, it incidence represents 0.5/100,000 births. 2 Genetic factors have been suggested, mainly those involved in pituitary embryogenesis in less than 5% of cases. These genes are PROP1, LHX3, HEXSX1, PROKR2, and GPR161.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Pituitary stalk interruption syndrome on MRI: Case report

Yehouenou Tessi,

Adeyemi,

El Msaadi

et al. 2023

Clinical Case Reports

View full text Add to dashboard Cite

Key clinical messagePituitary stalk interruption syndrome (PSIS) is an antenatal anatomical defect characterized by pituitary insufficiency with symptomatology depending on associated hormonal deficits. Diagnosis is often delayed because many clinical findings. The gold standard for detection is pituitary MRI showing absence of pituitary stalk, anterior pituitary hypoplasia, and postpituitary ectopy. The treatment remains polyhormonal substitution.AbstractPituitary stalk interruption syndrome (PSIS) is an antenatal anatomical defect. It is characterized by pituitary insufficiency with symptomatology depending on associated hormonal deficits. Diagnosis of PSIS is often delayed probably because of various clinical characteristics findings. Pituitary imaging abnormality is a specific indicator of hypopituitarism. The symptomatological triad associates a very thin or interrupted pituitary stalk, an ectopic or absent pituitary gland and hypoplasia of the anterior pituitary gland. The gold standard for detection is pituitary MRI. Some genetic factors are associated with the disease. The treatment remains polyhormonal substitution depending on the associated deficits. We reported the case of a 14‐year‐old child with growth retardation in whom the biological work‐up and pituitary MRI concluded that the diagnosis was PSIS with growth hormone deficiency. The treatment implemented was a recombinant growth hormone treatment. The immediate outcome was marked by a regression of symptoms.

show abstract

Section: Discussionmentioning

confidence: 99%

“…Diagnosis is most often early, during childhood when there is a delay in weight and height or when there is isolated hypopituitarism, but also when there are hormonal disorders (growth hormone, thyroid hormones). 1,2 MRI remains the gold standard imaging to confirm the diagnosis.…”

Section: Abstract Hypopituitarism Interruption Mri Pituitary Stalk Sy...mentioning

confidence: 99%

Pituitary stalk interruption syndrome on MRI: Case report

Yehouenou Tessi,

Adeyemi,

El Msaadi

et al. 2023

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

“…The work position is relatively fixed, and the consistency test between multiple people will be carried out regularly to ensure the accuracy of the examination. Secondary sexual characteristics are highly correlated with bone age, and the examination is simple and feasible, with a unified international standard that has been implemented in China for several years (13,14). Therefore, it is a better alternative to use the second sexual characteristics to predict bone age and BDS.…”

Section: Discussionmentioning

confidence: 99%

“…Body weight and fat percentage were measured with a body composition instrument (Inbody 720, Inbody Corp., Korea). The secondary sexual characteristics were evaluated with Tanner stages method (13,14). The BDS was determined by the difference between chronological age and bone age.…”

Section: Data Collectionmentioning

confidence: 99%

Nomogram for Predicting Bone Development State of Female Children and Adolescents–A Fast Screening Approach Based on Pubes Stages for Growth and Development

Yang

Wang

Wu³

et al. 2021

Front. Pediatr.

View full text Add to dashboard Cite

Objective: To develop a nomogram for predicting bone development state (BDS) of female children and adolescents in a large scale.Methods: Four hundred forty-seven female students were designated as the training cohort to develop the predictive model, whereas 196 female students were used as the validation cohort to verify the established model. Bone age, height, body mass, body fat percentage, and secondary sexual characteristics were recorded, and BDS was determined with the chronological age and bone age. Multivariate logistic regression was conducted to determine the factors, and nomogram was developed and validated with the training and validation cohorts, respectively.Results: One hundred forty-seven female students were identified as BDS abnormal in the training cohort (32.9%), and 104 were determined in the validation cohort (53.1%). Age, height, weight, and pubes stage were selected for the predictive model. A nomogram was developed and showed a good estimation, with a C-index of 0.78 and a good calibration in the training cohort. Application of the nomogram to the validation cohort showed a similar C-index of 0.75 and a good calibration.Conclusion: A nomogram for predicting bone development was developed, which can provide a relatively good estimation of BDS for female children and adolescents in Chinese metropolis.

show abstract

“…Pituitary stalk interruption syndrome (PSIS) is a rare disease which often relates to anterior pituitary hormone deficiency with prevalence of 0.5 in 100,000 births [ 1 – 3 ]. Its distinct radiological manifestations in magnetic resonance imaging in aid to make the specific diagnosis included the triad of thin or absent pituitary stalk, hypoplastic anterior pituitary and ectopic posterior pituitary [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Evaluations of exercise intolerance with cardiopulmonary exercise tests in an 18-year-old adolescent with pituitary stalk interruption syndrome: a case report

et al. 2022

View full text Add to dashboard Cite

Background Pituitary stalk interruption syndrome (PSIS) is a rare disease associated with different level of anterior pituitary hormone deficiency resulting with a variety of clinical manifestations which could limit exercise capacity. Cardiopulmonary exercise test (CPET) is valuable in differential diagnosis of exercise intolerance and exercise prescription. Case presentation An 18-year-old male adolescent was diagnosed with PSIS at 4 years old, had undergone growth hormone supplement until puberty, and was referred to rehabilitation department due to exercise intolerance. We arranged pulmonary function test (PFT) and CPET to clarify the cause of limited capacity. The test result provided evidence of moderate functional impairment (54% of predicted maximal oxygen uptake) mainly affected by physical unfitness without significant cardiovascular or pulmonary limitations. Conclusion CPET serves as a valuable tool for diagnostic purpose. Aerobic and resistance exercise training for the patient should be conducted promptly for better prognosis but under safe circumstances, with criteria which could be provided by CPET results.

show abstract

Pituitary Stalk Interruption Syndrome

Cited by 5 publications

References 6 publications

Pituitary stalk interruption syndrome on MRI: Case report

Pituitary stalk interruption syndrome on MRI: Case report

Nomogram for Predicting Bone Development State of Female Children and Adolescents–A Fast Screening Approach Based on Pubes Stages for Growth and Development

Evaluations of exercise intolerance with cardiopulmonary exercise tests in an 18-year-old adolescent with pituitary stalk interruption syndrome: a case report

Contact Info

Product

Resources

About