scholarly journals Primary empty sella syndrome: Characteristics of the pituitary deficiency. A bicentric case series.

2020 ◽  
pp. 3-7
Keyword(s):  
Hormone Replacement ◽  
Sella Turcica ◽  
Case Series ◽  
Empty Sella ◽  
Pituitary Function ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Pituitary Deficiency ◽  
Primary Empty Sella

Background and aim Empty sella is the neuroradiological or pathological finding of an apparently empty sella turcica. The aim of the study was to analyze the clinical, hormonal and radiological characteristics of patients with empty sella and to compare anterior pituitary function in total versus partial primary empty sella. Methods The records of 36 patients with primary empty sella were retrospectively analyzed over a 24-years period. The patients were evaluated for pituitary function with basal hormone levels (FT4, TSH, IGF1, FSH, LH, cortisol, ACTH, prolactin) and dynamic testing when necessary. Results Our study included 26 women and 10 men with an average age of 47.64 ±15.47 years. Seventy-six per cent of women were multiparous. Fifteen patients were obese. The revealing symptoms were dominated by endocrine signs (52.7%). More than half of our patients complained of headache. Sixty-one of the patients had partial empty sella and the remaining 39% had total empty sella. Two or more pituitary hormone deficiency were found in 41% of cases. Secondary adrenal insufficiency was the most common pituitary hormone deficiency(41.7%).The percentage of hypopituitarism in complete primary empty sella was significantly higher than that in partial primary empty sella (P<0.05).The management was based on hormone replacement therapy in case of hypopituitarism and on analgesic therapy in case of headache. Conclusion The diagnosis of PES must be evoked in an obese, multiparous, hypertensive woman presenting with a symptomatology suggestive of a pituitary deficiency or chronic headache. The correlation between pituitary gland volume and the degree of hypopituitarism highlights the importance of the early diagnosis and hormones replacement.

scholarly journals Antibodies to pituitary surface antigens during various pituitary disease states

2002 ◽  
Vol 175 (2) ◽  
pp. 417-423 ◽  
Author(s):  
YM Keda ◽  
IV Krjukova ◽  
IA Ilovaiskaia ◽  
MS Morozova ◽  
OV Fofanova ◽  
...  
Keyword(s):  
Surface Antigens ◽  
Autoimmune Disorders ◽  
Empty Sella ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Cell Surface Antigens ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency ◽  
Pituitary Dysfunction ◽  
Significant Difference

Autoantibodies to cell surface antigens of human somatotropinoma (ASAS), human prolactinoma (ASAP) and rat adenohypophysis (ASARA) were assayed in the serum of patients with pituitary diseases associated with GH deficiency (GHD), such as pituitary dwarfism and primary empty sella syndrome (ESS), and in the serum of patients with hyperprolactinaemia of different etiologies: idiopathic hyperprolactinaemia, prolactinoma and ESS. The investigation was carried out with a cellular variant of an ELISA. Among children with GHD, the highest percentage of antibody-positive patients was found in the group with idiopathic isolated GHD (89% of ASAS(+) patients and 30% of ASARA(+) patients vs 33.3% and 0% respectively in the group with idiopathic combined pituitary hormone deficiency, and 33.3% and 9% in patients with pituitary hypoplasia associated with isolated GHD or combined pituitary hormone deficiency). Among hyperprolactinaemic patients, the highest ASAP and ASARA frequency was observed in patients with idiopathic hyperprolactinaemia (67.7% and 41.9% respectively) where it was twice as high as in the group of patients with prolactinoma. The proportion of ASAS(+) and ASARA(+) did not differ significantly between the groups of patients with ess with or without GHD. Similarly, there was no significant difference between the number of ESS ASAP(+) and ASARA(+) patients with or without hyperprolactinaemia. The data obtained suggested that autoimmune disorders may be primary, and responsible, at least in part, for pituitary dysfunction in the cases of idiopathic isolated GHD and idiopathic hyperprolactinaemia. At the same time, the autoimmune disorders in the patients with prolactinoma or ESS are probably secondary to the organic pituitary lesion and their significance in the development of the pituitary dysfunction is obscure.

scholarly journals LHX3 deficiency presenting in the United States with severe developmental delay in a child of Syrian refugee parents

2018 ◽  
Vol 2018 ◽  
Author(s):  
Susan Ahern ◽  
Mark Daniels ◽  
Amrit Bhangoo
Keyword(s):  
United States ◽  
Growth Hormone ◽  
Novel Mutation ◽  
Hormone Replacement ◽  
The United States ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Central Hypothyroidism ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency

Summary In this case report, we present a novel mutation in Lim-homeodomain (LIM-HD) transcription factor, LHX3, manifesting as combined pituitary hormone deficiency (CPHD). This female patient was originally diagnosed in Egypt during infancy with Diamond Blackfan Anemia (DBA) requiring several blood transfusions. Around 10 months of age, she was diagnosed and treated for central hypothyroidism. It was not until she came to the United States around two-and-a-half years of age that she was diagnosed and treated for growth hormone deficiency. Her response to growth hormone replacement on linear growth and muscle tone were impressive. She still suffers from severe global development delay likely due to delay in treatment of congenital central hypothyroidism followed by poor access to reliable thyroid medications. Her diagnosis of DBA was not confirmed after genetic testing in the United States and her hemoglobin normalized with hormone replacement therapies. We will review the patient’s clinical course as well as a review of LHX3 mutations and the associated phenotype. Learning points: Describe an unusual presentation of undertreated pituitary hormone deficiencies in early life Combined pituitary hormone deficiency due to a novel mutation in pituitary transcription factor, LHX3 Describe the clinical phenotype of combined pituitary hormone deficiency due to LHX3 mutations

scholarly journals Pituitary volume in patients with Primary Empty Sella and clinical relevance to pituitary hormone secretion: A retrospective single center study

2021 ◽  
Vol 17 ◽  
Author(s):  
Gamze Akkus ◽  
Sinan Sözütok ◽  
Fulya Odabaş ◽  
Bilen Onan ◽  
Mehtap Evran ◽  
...  
Keyword(s):  
Anterior Pituitary ◽  
Hormone Secretion ◽  
Sella Turcica ◽  
Volume Measurement ◽  
Pituitary Hormones ◽  
Empty Sella ◽  
Pituitary Hormone ◽  
Pituitary Dysfunction ◽  
Volume Measurements ◽  
Primary Empty Sella

Background: According to neuroradiological findings, empty sella seems to be deprived of pituitary tissue in sella turcica. Changing size of the pituitary volume is closely related to the occurrence of primary empty sella. The aim of the study is to determine pituitary dysfunction in patients with partial or total primary empty sella and the significance of pituitary volume measurements in these patients. Methods: This study was designed retrospectively. 67 patients (55 females, 12 males) diagnosed with primary empty sella syndrome between the years of 2015-2019 were included in the study. Patients were divided into two groups: partial (PES) and total (TES) empty sella by magnetic resonance imaging (MRI). Basal anterior pituitary and its hormones were assessed. We also included 26 healthy control subjects (19 females, 7 males) to compare the differences in pituitary volumes. Volumes were measured by using Osirix Dicom Viewer ( Pixmeo SARL, Geneve, Swiss) in 3.0 Tesla scanner MRI. Results: 82.1% (n=55) of all patients were PES and the others were (n=12) TES. Hypopituitarism, known as one or more pituitary hormones deficiency, was found in 12 patients (17.9%). While 9 of them had total PES, the others had partial PES. Secondary adrenal insufficiency and gonadotropin deficiency were more prevalent in patients with TES. Mean volume measurements of patients with TES, PES and healthy subjects were 0.23±0.17, 0.35±0.15, 0.54±0.17 cm3, respectively. Except for IGF1 values (p=0.026), there was not any significant correlation found between the anterior pituitary hormones and volume measurements. Conclusion: Although volume measurement has helped in the diagnosis of pituitary empty sella (partial or total), it does not seem to have any significant correlation with pituitary secretory function.

scholarly journals A Large PROP1 Gene Deletion in a Turkish Pedigree

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Suheyla Gorar ◽  
Doga Turkkahraman ◽  
Kanay Yararbas
Keyword(s):  
Genetic Analysis ◽  
Empty Sella ◽  
Hormone Deficiency ◽  
Bright Spot ◽  
Pituitary Hormone ◽  
Coding Region ◽  
Gene Encoding ◽  
Acth Deficiency ◽  
Pituitary Hormone Deficiency ◽  
Prop1 Gene

Pituitary-specific paired-like homeodomain transcription factor, PROP1, is associated with multiple pituitary hormone deficiency. Alteration of the gene encoding the PROP1 may affect somatotropes, thyrotropes, and lactotropes, as well as gonadotropes and corticotropes. We performed genetic analysis of PROP1 gene in a Turkish pedigree with three siblings who presented with short stature. Parents were first degree cousins. Index case, a boy, had somatotrope, gonadotrope, thyrotrope, and corticotrope deficiency. However, two elder sisters had somatotroph, gonadotroph, and thyrotroph deficiency and no corticotroph deficiency. On pituitary magnetic resonance, partial empty sella was detected with normal bright spot in all siblings. In genetic analysis, we found a gross deletion involving PROP1 coding region. In conclusion, we report three Turkish siblings with a gross deletion in PROP1 gene. Interestingly, although little boy with combined pituitary hormone deficiency has adrenocorticotropic hormone (ACTH) deficiency, his elder sisters with the same gross PROP1 deletion have no ACTH deficiency. This finding is in line with the fact that patients with PROP1 mutations may have different phenotype/genotype correlation.

Pituitary Function in Patients with Enlarged Sella Turcica and Primary Empty Sella Syndrome

2009 ◽  
Vol 209 (1-6) ◽  
pp. 31-35 ◽  
Author(s):  
Marja Ekblom ◽  
Leena Ketonen ◽  
Ilkka Kuuliala ◽  
Risto Pelkonen
Keyword(s):  
Sella Turcica ◽  
Empty Sella ◽  
Pituitary Function ◽  
Empty Sella Syndrome ◽  
Primary Empty Sella
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