scholarly journals SUN-LB2 Undescended Testicle and Short Stature as Manifestation of Pituitary Stalk Interruption Syndrome a Report From Saudi Arabia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Rania Ahmed ◽  
Asirvatham Alwin robert
Keyword(s):  
Growth Hormone ◽  
Saudi Arabia ◽  
Pituitary Gland ◽  
Short Stature ◽  
Sella Turcica ◽  
Pituitary Stalk ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Post Contrast ◽  
Optic Chiasma

Abstract Background Pituitary stalk interruption syndrome (PSIS) is a congenital disease with isolated growth hormone deficiency or multiple anterior pituitary hormone deficiencies. Here, the authors report a case of PSIS from Saudi Arabia. Clinical Case A 16 year old Saudi boy presented to the endocrine clinic with short stature and undescended testis, status post bilateral orchidopexy. He was delivered by caesarean section because of breech presentation and birth asphyxia. Investigation revealed underdeveloped secondary sexual characteristics with decreased facial and pubic hair growth. The patient height was 134 cm whereas the bone age was 9 - 11 years. Pelvis examination showed a scrotum with bilateral 1 mL testes and the stretch penile length was 3 cm. The patient laboratory investigations showed hemoglobin level of 13 g/dL, serum sodium 140 mmol/L, serum potassium 4.1 mmol/L, serum chloride 102 mmol/L, calcium 9.1 mg/dL, random blood sugar 110 mg/dL and albumin 3.8 mg/dL. A pituitary hormone profile showed hypopituitarism with thyroid, and adrenal sparing. The patient free T4 was 17.3 pmol/L (9-25 pmol/L) and synacthen test revealed a morning baseline cortisol level of 6.5 µg/dL (normal = 4.3-22.4 ug/dL) with adrenocorticotrophic hormone of 9.8 pmol/L (1.1 - 13.2 pmol/L). Insulin-like growth factor 1 level 50 ng/dL (normal = 193.0 - 731.0 ug/L), follicle-stimulating hormone 0.35 µIU/mL (normal, 0.0-10.0), and leutinizing hormone 0.4 µIU/mL (normal = 1.2-7.8). The patient’s morning testosterone level showed 8 ng/dL (normal = 280-800 ng/dL) and prolactin 116 mIU/L (normal = 86 - 324 mIU/L). There were no symptom suggestive of posterior pituitary involvement like polyuria and polydipsia as urine and serum osmolality. The MRI examination showed no pituitary gland identified in the sella turcica and no clear pituitary stalk. A T1 hyperintense focus with post-contrast enhancement was identified posterior to the optic chiasma representing an ectopic posterior pituitary gland. The growth hormone and testosterone therapy were added to medical therapy of the patients and no thyroid or hydrocortisone replacement therapy was given. Conclusion: Despite the fact that this is a rare disorder, it should always be kept in the differential diagnosis of a patient presenting with short stature. Patients with this disease have an excellent opportunity to reach normal height if they present before the joining of epiphyses.

scholarly journals Pituitary Hypoplasia Is the Best MRI Predictor of the Severity and Type of Growth Hormone Deficiency in Children With Congenital Growth Hormone Deficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A683-A683
Author(s):  
Himanshu Sharma ◽  
Anshul Kumar ◽  
Naincy Purwar ◽  
Nitish Mathur ◽  
Balram Sharma ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Common Finding ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Mri Findings ◽  
Pituitary Hypoplasia ◽  
Ectopic Posterior Pituitary

Abstract Background and Objectives: Congenital idiopathic growth hormone deficiency(GHD) is associated with various MRI abnormalities, including both sellar anomalies such as pituitary hypoplasia, ectopic pituitary, empty sella and abnormalities of the pituitary stalk and extrasellar abnormalities such as Arnold Chiari malformation, corpus callosum agenesis, arachnoid cyst, septum pellucidum agenesis, enlarged ventricles, vermis dysplasia, and sphenoid cyst. However, it remains contentious whether MRI brain findings could provide an additional avenue for precisely predicting the differentiation of GHD based on severity(severe or partial) and type(isolated GHD or multiple pituitary hormone deficiency MPHD). This study aimed to ascertain the abnormality that is the best predictor of severe GHD and type of GHD amongst the different MRI findings. Methods: This was an analytical cross-sectional study conducted from 2018-2020. During the study period, we included a total of 100 subjects diagnosed to have idiopathic GHD after the exclusion of syndromic causes, system illness, presence of pituitary mass, and those with h/o cranial irradiation. Patients were divided into severe GHD and partial GHD based on peak stimulated GH of <5 ng/dl and ≥ 5 ng/dl respectively and into groups based on isolated GHD and MPHD. Patients were further divided into groups based on the presence of pituitary hypoplasia,extrasellar brain abnormalities (EBA), and presence of ectopic posterior pituitary and/or pituitary stalk abnormalities(EPP/PSA), respectively. Analyses were performed using SPSS version 24.0 software. Results: Amongst 100 subjects with idiopathic congenital GHD, 66 (66%) subjects had Isolated GHD while the remaining 34 (34%) had MPHD. 71 had severe GHD, and 29 had partial GHD. Amongst the MRI findings, pituitary hypoplasia was the most common finding observed in 53% of patients, while 23(23%) had EBA, and 25(25%) had EPP/PSA. Pituitary hypoplasia was observed to be the best predictor of severity of GHD with an odds ratio(OR) of 10.8 (95% CI 3.38-29.6) followed by ectopic posterior pituitary /pituitary stalk abnormalities (OR =2.8, 95% CI 1.5-9.5) while the presence of extrasellar abnormalities was the weakest predictor (OR =1.8, 95% CI 1.05-3.2). Pituitary hypoplasia was the only finding to significantly predict MPHD (OR=9.2). On ROC analysis, a Pituitary height SDS of -2.03 had a 73.2 % sensitivity and specificity of 79.3%(AUC =0.787,95% CI 0.7-0.873) for severe GHD and a sensitivity of 88.2 % and specificity of 66.7% (AUC =0.745, 95% CI 0.68-0.877) for MPHD. Conclusion: We observed Pituitary hypoplasia to be not only the most frequent MRI abnormality but also the best predictor of severe GHD and MPHD amongst various sellar and extrasellar abnormalities.

scholarly journals Pituitary stalk transection syndrome

2020 ◽  
Vol 7 (12) ◽  
pp. 2397
Author(s):  
Gayathri Sajeevan ◽  
Sajitha Nair ◽  
Devika Geetha ◽  
Nisha Bhavani ◽  
C. Jayakumar ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Resonance Imaging ◽  
Current Report

Growth hormone deficiency is one of the most common endocrinological causes for short stature. It can either be idiopathic or associated with organic causes like tumors or following surgery. One of the rare causes for growth hormone deficiency in children is pituitary stalk transection syndrome. It can be diagnosed by magnetic resonance imaging of the hypothalamus and pituitary gland which shows an ectopic or absent posterior pituitary, an absent or interrupted pituitary stalk, or small anterior pituitary in combination with growth hormone or other pituitary hormone deficiencies. Current report presents a child with pituitary stalk transection syndrome who was brought for evaluation of hypoglycemic seizures.

scholarly journals SAT-241 Pituitary Stalk Interruption Syndrome Presenting as Neonatal Hypotonia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Nordie Anne Bilbao
Keyword(s):  
Pituitary Gland ◽  
Neonatal Period ◽  
Hormone Replacement ◽  
Brain Mri ◽  
Rare Condition ◽  
Pituitary Stalk ◽  
Pituitary Hormone ◽  
Thyroid Function Tests ◽  
Acth Stimulation ◽  
Central Hypothyroidism

Abstract Pituitary stalk interruption syndrome (PSIS) is a rare condition that include congenital anatomic abnormalities of the pituitary gland and hypopituitarism. There is a wide variety of clinical presentation, with the age at presentation encompassing from neonatal period to adulthood and including one or more pituitary hormone deficiencies. In recent literature there is increasing recognition of PSIS presenting in the neonatal period, mostly involving hypoglycemia. Our patient is a full-term male infant who presented in the newborn period with hypotonia and hypothermia. He also had hypoglycemia, which was initially thought to be associated to hyperinsulinism in the context of gestational diabetes. Micropenis was noted on physical exam. As part of the study for hypotonia, serial thyroid function tests were obtained revealing central hypothyroidism. A low dose ACTH stimulation test was performed which revealed adrenal insufficiency. The patient was started on cortisol and thyroid hormone replacement. Brain MRI showed an ectopic neurohypophysis located along the floor of the hypothalamus, a small anterior pituitary gland, and a partially absent infundibulum, findings consistent with pituitary stalk interruption syndrome. The patient received testosterone injections for micropenis and is being followed for development of other pituitary hormone deficiencies. PSIS is a rare congenital condition that is increasingly recognized in neonates manifesting with signs of hypopituitarism.

Extra pituitary midline structural abnormalities associated with ectopic posterior pituitary detected on a new rapid MR protocol (FAST1.2)

2021 ◽  
Author(s):  
Arthur Lyra ◽  
Daniel de Faria Guimarães ◽  
Altino Sá Meira ◽  
Arthur Castello Berchielli Nunes ◽  
Guilherme Vieira Peixoto ◽  
...  
Keyword(s):  
Sella Turcica ◽  
Pituitary Stalk ◽  
Control Group ◽  
Hypothalamic Hamartoma ◽  
Pars Intermedia ◽  
Posterior Pituitary ◽  
Pineal Cyst ◽  
Imaging Results ◽  
Structural Abnormalities ◽  
Ectopic Posterior Pituitary

Abstract Background Ectopic posterior pituitary (EPP) is a malformation of the hypothalamic-pituitary region. Our goal was to describe midline structural brain abnormalities in patients with EPP using a dedicated protocol (FAST1.2 protocol) of magnetic resonance imaging (MRI) to evaluate the hypothalamus and pituitary gland, highlighting their clinical-laboratory correlations. Methods A cross-sectional study of patients diagnosed with EPP, and a control group. All individuals were submitted to a dedicated MRI protocol called FAST1.2, which combines the FAST1 protocol developed by our group with 3D T2DRIVE imaging. Results We evaluated 36 individuals with EPP and 78 individuals as a control group. One patient had two posterior pituitary lobes, one inside the sella turcica and the other along the pituitary stalk; in five patients, the EPP was along the pituitary stalk; in 28 the EPP was at the infundibular recess of the third ventricle, and in two the EPP was hypothalamic. In the EPP group, eleven individuals had interhypothalamic adhesion (IHA), three septo-optic dysplasia, one a cerebellar malformation, and one a pineal cyst. We did not observe a higher frequency of severe hormonal deficiency or developmental delay in patients with IHA. In the control group, eleven patients had a pineal cyst, three pars intermedia cysts, one hydrocephalus, and one hypothalamic hamartoma. Conclusion FAST1.2 acquisition allows confident recognition of regional anatomy and recognition of midline structural abnormalities on T2DRIVE, particularly including the pituitary stalk and IHA, thereby making MRI acquisition faster with no need for intravenous contrast administration. We suggest that IHA could be associated with defects in neuronal migration, as might occur in patients with EPP.

EFFECT OF SOMATOSTATIN ON THE CONCENTRATION OF GROWTH HORMONE IN THE PLASMA OF FOETAL SHEEP

1978 ◽  
Vol 78 (3) ◽  
pp. 453-454 ◽  
Author(s):  
I. C. McMILLEN ◽  
G. JENKIN ◽  
G. D. THORBURN ◽  
J. S. ROBINSON
Keyword(s):  
Growth Hormone ◽  
Pituitary Gland ◽  
Medical Research ◽  
Pituitary Stalk ◽  
Obstetrics And Gynaecology ◽  
Nuffield Department ◽  
John Radcliffe Hospital ◽  
Ovine Placenta

Nuffield Institute for Medical Research and Nuffield Department of Obstetrics and Gynaecology, John Radcliffe Hospital, Headington, Oxford, 0X3 9DU (Received 6 April 1978) Growth hormone (GH) has been located in the ovine foetal pituitary gland by day 50 of gestation (Stokes & Boda, 1968). The concentration of GH in the plasma of foetal sheep is ten times higher than the postnatal value, increasing from 40 ng/ml on day 100 of gestation to 100–120 ng/ml on day 140 (Bassett, Thorburn & Wallace, 1970). After foetal hypophysectomy, the concentration of GH falls to < 2 ng/ml, indicating that it originates in the foetal pituitary gland (Wallace, Stacey & Thorburn, 1973). Labelled GH does not cross the ovine placenta (Wallace et al. 1973). After sectioning the foetal pituitary stalk, the concentration of GH in the foetal plasma drops to approximately 5 ng/ml (Wallace et al. 1973), which implies that the secretion of GH

Growth hormone and insulin-like growth factor regulate insulin-like growth factor-binding protein-1 in Laron type dwarfism, growth hormone deficiency and constitutional short stature

1992 ◽  
Vol 127 (4) ◽  
pp. 351-358 ◽  
Author(s):  
Zvi Laron ◽  
Anne-Maria Suikkari ◽  
Beatrice Klinger ◽  
Aviva Silbergeld ◽  
Athalia Pertzelan ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Factor ◽  
Growth Hormone Deficiency ◽  
Short Stature ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Healthy Children ◽  
Insulin Like Growth Factor ◽  
Factor Binding ◽  
Igf I

Insulin-like growth factors (IGFs) mediate the effects of growth hormone (GH), and the insulin-like growth factor-binding proteins (IGFBPs) modulate the actions of IGFs in tissues. We studied the circulating levels of IGFBP-1 in 6 children and 9 adults with Laron type dwarfism (LTD), in 11 children and 21 adults with growth hormone deficiency (GHD), and in 8 children with constitutional short stature. Compared with the situation in healthy children, the basal serum IGFBP-1 concentration was 5.4-fold higher in LTD children, 4.1-fold higher in GHD children, and 3.8-fold higher in children with short stature (p<0.02 vs controls in all groups). In adult patients with multiple pituitary hormone deficiency (MPHD), the IGFBP-1 concentration was 2-fold elevated, but it was normal in adult LTD patients. Intravenous (N= 10) or subcutaneous (N=9) administration ofIGF-I (75 μg·kg−1 and 150 μg·kg−1, respectively) in LTD children resulted in a rapid 50–60% fall in serum insulin (p<0.02), a decline in blood glucose and a concomitant 40–60% rise of IGFBP-1 levels (p<0.05). Treatment for seven days with IGF-I (150 μg·kg−1·d−1) resulted in a decrease by 34% and 44% of serum IGFBP-1 level in two out of three children with LTD. After prolonged GH therapy, the IGFBP-1 level fell in GHD children by 29% (p<0.05), in GHD adults by 52% (p<0.02) and in children with constitutional short stature by 17% (p<0.02). IGFBP-1 and insulin concentrations were inversely related in patients with GHD (r= −0.66, p<0.001) or with LTD (r= −0.57, p<0.05). Our data suggest that: (a) increased IGFBP-1 concentration in LTD, GHD and constitutional short children may, at least in part, be accounted for by an IGF-I deficiency; (b) both the rise in IGF-I and a fall in insulin contributed to the rise in IGFBP-1 after acute IGF-I administration; (c) prolonged IGF-I or GH treatment causes a persistent decline in IGFBP-1 concentration. In conclusion, IGF-I and GH may regulate IGFBP-1 secretion either directly or via insulin.

Suckling-Induced Rise in Prolactin: Mediation by Prolactin-Releasing Factor From Posterior Pituitary

Physiology ◽  
1988 ◽  
Vol 3 (4) ◽  
pp. 172-175
Author(s):  
N Ben-Jonathan ◽  
JF Hyde ◽  
I Murai
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Small Peptide ◽  
Anterior Pituitary Hormone

The suckling-induced rise in prolactin, an anterior pituitary hormone that is essential for the maintenance of lactation, is mediated by the posterior pituitary. The posterior pituitary contains prolactin-releasing factor (PRF), a small peptide that is distinct from known prolactin secretagogues. The hypothalamus, the site of all known releasing hormones, has only little PRF activity. Pituitary stalk secretion abolishes the PRF activity in the posterior pituitary, indicating that it originates in the hypothalamus, perhaps as a biologically inactive precursor. The chemical identity of PRF is unknown but it appears to play an important role during lactation and might also be involved in the pathology of hyperprolactinea.

scholarly journals SAT-105 The Natural History of Pituitary Cysts in Patients with Growth Hormone Deficiency and Idiopathic Short Stature

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Zeyad El-Naghy ◽  
Nicholas Andrew Krasnow ◽  
James Haigney ◽  
Tara Patale ◽  
Liam McGuirk ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Idiopathic Short Stature ◽  
Hormone Deficiency ◽  
Pediatric Endocrinology ◽  
Post Contrast ◽  
Pituitary Cyst ◽  
Pituitary Cysts ◽  
Over Time

Abstract Background: The sequential follow-up of simple fluid-filled pituitary cysts (PC) has not been fully elucidated. In this study, we further report our follow up of PCs in a cohort of pediatric patients (PTs). Objective: To further analyze the sequential cyst volume (CV) change in short children. Patients and Methods: A pediatric endocrinology and neuroradiology center was queried for the presence of PCs. PTs who underwent multiple high resolution post-contrast MRIs (1mm slices) were subjects of this study. PTs with additional MRI abnormalities were excluded. Pituitary volumes (PV) and CVs were measured using the ellipsoid formula (LxWxH/2). The percentage of the gland occupied by the cyst (POGO) was measured and calculated. A cyst with a POGO ≤15% was defined as a small pituitary cyst (SPC), and a POGO &gt;15% was defined as a large pituitary cyst (LPC). 34 PTs met inclusion criteria, all of whom were diagnosed with short stature (23 growth hormone deficient (GHD) PTs and 11 idiopathic short stature (ISS) PTs). All PTs were receiving GH during data collection. Results: The mean (MN) and median (MD) ages for these subjects were 10.7 yrs ±3.5 and 11.1 yrs, respectively (RSP). Of the 34 PTs, 24 PTs’ (71%) initial MRI demonstrated a SPC and 10 PTs’ (29%) initial MRI demonstrated a LPC. The MN and MD times between first and second MRIs were 1.23 yrs and 0.83 yrs RSP, with a range (RG) of 0.14 to 4.08 yrs. The MN and MD ΔCV for all PTs was 23.33% ±179.17% and -25.94% RSP, with a RG of -100.00% to 763.94%. The MN and MD ΔPOGO by the cyst for all PTs was 48.59% ±313.26% and -36.84% RSP, with a RG of -100.00% to 1734.79%. The MN and MD ΔCV for PTs with a SPC was 10.68% ±2.65% and 11.09% RSP, with a RG of -100.00% to 763.94%. The MN and MD ΔPOGO by the cyst for PTs with a SPC was 78.33% ±369.96% and -31.34% RSP, with a RG of -100.00% to 1734.79%. The MN and MD ΔCV for PTs with a LPC was -24.60% ±51.89% and -26.57% RSP, with a RG of -88.57% to 91.38%. The MN and MD ΔPOGO by the cyst for PTs with a LPC was -22.79% ±44.90% and -40.46% RSP, with a RG of -80.95% to 47.11%. Statistical analysis showed no significant %ΔCV or %ΔPOGO when comparing male vs. female, SPC vs. LPC, GHD vs. ISS, or pre-pubertal vs. pubertal PTs. Analysis of ΔPOGO of the 24 SPC PTs demonstrated that 4 (17%) of them developed into LPCs. Analysis of the 10 LPC PTs showed that 6 (60%) of them shrunk into SPCs, one of which re-enlarged into a LPC, and another of which fluctuated between LPC and SPC over a period of 7.34 yrs and 9 sequential MRIs. None of the PTs experienced significant sequelae related to their PCs. Conclusion: CV can change greatly over time, however few sequelae should be expected. LPCs tend to demonstrate major changes in size and should be tracked for CV change. A minority of SPCs will develop into LPCs. Prediction of change in CV over time requires more sequential data. Change in CV did not appear to be influenced by GH therapy.

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