scholarly journals Diagnóstico tardío de síndrome de interrupción del tallo hipofisario

2017 ◽  
Vol 4 (3) ◽  
pp. 44-46
Author(s):  
Vanessa Garzón Graciano ◽  
Santiago González Narvaez ◽  
Jhon Alexander Alzate Piedrahita ◽  
Víctor Hugo Ruiz Granada ◽  
Santiago Vallejo González
Keyword(s):  
Anterior Pituitary ◽  
Clinical Presentation ◽  
Delayed Diagnosis ◽  
Pituitary Hormones ◽  
Pituitary Stalk ◽  
Neurologic Involvement ◽  
Pituitary Hypoplasia ◽  
Laboratory Examinations ◽  
Development Laboratory ◽  
Leg Fracture

El síndrome de interrupción del tallo hipofisario (PSIS, por sus siglas en inglés) es una anomalía congénita de probable origen genético causante de hipopituitarismo, que se caracteriza por hipoplasia de la adenohipófisis, neurohipófisis ectópica e interrupción del tallo hipofisario. Suele presentarse con baja estatura y deficiencia adenohipofisaria. El retraso en el diagnóstico se relaciona con alta morbimortalidad. Se reporta el caso de un hombre de 39 años con fractura de cadera por fragilidad y posterior compromiso neurológico secundario a hiponatremia severa, hipoglucemia e hipotensión arterial. Presentaba hábito eunucoide y ausencia de caracteres sexuales secundarios, con genitales infantiles. Los paraclínicos mostraron compromiso de todas las hormonas adenohipofisarias, sin afección neurohipofisaria. Una resonancia magnética nuclear mostró hallazgos consistentes con PSIS.   Abstract The pituitary stalk interruption syndrome (PSIS) is a congenital defect with a possible genetic origin which causes hypopituitarism. It consists of anterior pituitary hypoplasia, ectopic posterior pituitary and interruption of the pituitary stalk. Its clinical presentation consists mainly in short stature and anterior pituitary hormonal deficiencies. The delayed diagnosis may cause morbidity and mortality. We report the case of a 39 year old male who presented with a frailty leg fracture and neurologic involvement due to severe hyponatremia, hypoglycemia and hypotension. He lacked secondary sexual development. Laboratory examinations showed an inadequate secretion of all anterior pituitary hormones without diabetes insipidus. A magnetic resonance image showed findings consistent with PSIS.  

Familial ROBO1 deletion associated with ectopic posterior pituitary, duplication of the pituitary stalk and anterior pituitary hypoplasia

2019 ◽  
Vol 32 (1) ◽  
pp. 95-99
Author(s):  
Marcello Scala ◽  
Andrea Accogli ◽  
Anna Maria Elsa Allegri ◽  
Elisa Tassano ◽  
Mariasavina Severino ◽  
...  
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Comparative Genomic ◽  
Posterior Pituitary ◽  
Loss Of Function ◽  
Human Pituitary ◽  
Developmental Abnormalities ◽  
Pituitary Development ◽  
Pituitary Hypoplasia ◽  
Ectopic Posterior Pituitary

Abstract Background The genetic causes of abnormal pituitary development have been extensively studied in the last few years. ROBO1 is involved in neurogenesis and axon guidance. Loss-of-function variants in ROBO1 have been associated with pituitary stalk interruption syndrome (PSIS), suggesting that its haploinsufficiency could impair the guidance of hypothalamic axons to the pituitary gland leading to developmental abnormalities. Case presentation We report a 4.5-year-old girl with anterior pituitary hypoplasia and pituitary stalk duplication in the ventral-dorsal direction. Her father had a similar pituitary phenotype, characterized by anterior pituitary hypoplasia combined with ectopic posterior pituitary. Comparative genomic hybridization (CGH) microarray analysis identified a 343.7 kb deletion of 3p12.3 encompassing ROBO1 in both individuals. Conclusions We report the first familial ROBO1 deletion in two individuals with peculiar pituitary anomalies, including the rare pituitary stalk duplication in the ventral-dorsal direction. These findings widen the spectrum of the phenotypes associated with ROBO1 haploinsufficiency and support its role in human pituitary development.

Ultrastructural morphology of nontumorous lactotrophs in human pituitaries exposed to high prolactin levels

1987 ◽  
Vol 45 ◽  
pp. 896-897
Author(s):  
S. Jalalah ◽  
K. Kovacs ◽  
E. Horvath
Keyword(s):  
Anterior Pituitary ◽  
Secretory Activity ◽  
Pituitary Hormones ◽  
Feedback Mechanism ◽  
Endocrine Activity ◽  
Hormone Synthesis ◽  
Anterior Pituitary Hormones ◽  
Negative Feedback Mechanism ◽  
Ultrastructural Morphology ◽  
Transmission Electron

Lactotrophs, as many other endocrine cells, change their morphology in response to factors influencing their secretory activity. Secretion of prolactin (PRL) from lactotrophs, like that of other anterior pituitary hormones, is under the control of the hypothalamus. Unlike most anterior pituitary hormones, PRL has no apparent target gland which could modulate the endocrine activity of lactotrophs. It is generally agreed that PRL regulates its own release from lactotrophs via the short loop negative feedback mechanism exerted at the level of the hypothalamus or the pituitary. Accordingly, ultrastructural morphology of lactotrophs is not constant; it is changing in response to high PRL levels showing signs of suppressed hormone synthesis and secretion.By transmission electron microscopy and morphometry, we have studied the morphology of lactotrophs in nontumorous (NT) portions of 7 human pituitaries containing PRL-secreting adenoma; these lactotrophs were exposed to abnormally high PRL levels.

scholarly journals Estrogen mediates sex differences in preoptic neuropeptide and pituitary hormone production in medaka

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Junpei Yamashita ◽  
Yuji Nishiike ◽  
Thomas Fleming ◽  
Daichi Kayo ◽  
Kataaki Okubo
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Hormones ◽  
Pituitary Hormone ◽  
Sexually Dimorphic ◽  
Hormone Production ◽  
Anterior Pituitary Hormones ◽  
Vertebrate Brain ◽  
Pituitary Adenylate Cyclase ◽  
Evolutionarily Conserved ◽  
Sexually Dimorphic Expression

AbstractThe preoptic area (POA) is one of the most evolutionarily conserved regions of the vertebrate brain and contains subsets of neuropeptide-expressing neurons. Here we found in the teleost medaka that two neuropeptides belonging to the secretin family, pituitary adenylate cyclase-activating polypeptide (Pacap) and vasoactive intestinal peptide (Vip), exhibit opposite patterns of sexually dimorphic expression in the same population of POA neurons that project to the anterior pituitary: Pacap is male-biased, whereas Vip is female-biased. Estrogen secreted by the ovary in adulthood was found to attenuate Pacap expression and, conversely, stimulate Vip expression in the female POA, thereby establishing and maintaining their opposite sexual dimorphism. Pituitary organ culture experiments demonstrated that both Pacap and Vip can markedly alter the expression of various anterior pituitary hormones. Collectively, these findings show that males and females use alternative preoptic neuropeptides to regulate anterior pituitary hormones as a result of their different estrogen milieu.

scholarly journals Delayed diagnosis of Peutz–Jeghers syndrome due to pathological information loss or mistake in family/personal history

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yu-Liang Jiang ◽  
Xiao-Dong Xu ◽  
Bai-Rong Li ◽  
En-Da Yu ◽  
Zi-Ye Zhao ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Novel Mutation ◽  
Delayed Diagnosis ◽  
Personal History ◽  
Pathological Examination ◽  
The Novel ◽  
Novel Mutations ◽  
The Family ◽  
Genetic Level ◽  
Peutz Jeghers Syndrome

Abstract Objective To report Peutz–Jeghers syndrome (PJS) cases with non-definitive clues in the family or personal history and finally diagnosed through pathological examination and STK11 gene mutation test. Clinical presentation and intervention PJS was suspected in 3 families with tortuous medical courses. Two of them had relatives departed due to polyposis or colon cancer without pathological results, and the other one had been diagnosed as hyperplastic polyposis before. Diagnosis of PJS was confirmed by endoscopy and repeated pathological examinations, and the STK11 mutation test finally confirmed the diagnosis at genetic level, during which 3 novel mutation were detected (536C > A, 373_374insA, 454_455insGGAGAAGCGTTTCCCAGTGTGCC). Conclusion Early diagnosis of PJS is important and may be based on a family history with selective features among family members, and the pathological information is the key. The novel mutations also expand the STK11 variant spectrum.

Anterior Pituitary Hormones

Hormones ◽  
1997 ◽  
pp. 133-168
Author(s):  
Anthony W. Norman ◽  
Gerald Litwack
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Hormones ◽  
Anterior Pituitary Hormones

Neurosarcoidosis Presenting as Schizophreniform Disorder

1992 ◽  
Vol 22 (3) ◽  
pp. 269-274 ◽  
Author(s):  
Mohamed Sabaawi ◽  
Jose Gutierrez-Nunez ◽  
M. Richard Fragala
Keyword(s):  
Cognitive Decline ◽  
Mental Status ◽  
Clinical Presentation ◽  
Psychiatric Symptoms ◽  
Steroid Treatment ◽  
Schizophreniform Disorder ◽  
Schizophrenic Psychosis ◽  
Neurologic Involvement ◽  
Mental Status Examination ◽  
Psychiatric Manifestations

A patient whose clinical presentation met criteria for schizophreniform disorder was ultimately found to have neurosarcoidosis, and the psychiatric symptoms responded to steroid treatment. The ongoing search for organic etiology was prompted by the presence of cognitive decline, perseveration and rare bizarre automatisms. This is virtually the first reported association between schizophreniform disorder and sarcoidosis. We reviewed the literature on neurologic involvement and psychiatric manifestations in sarcoidosis as well as the concurrence between organicity and schizophrenic psychosis. The importance of attending to all elements of the mental status examination in a patient with complex atypical findings is underscored.

Vasopressin as a neuroendocrine regulator of anterior pituitary hormone secretion

1993 ◽  
Vol 129 (6) ◽  
pp. 489-496 ◽  
Author(s):  
Andreas Kjær
Keyword(s):  
Arginine Vasopressin ◽  
Mode Of Action ◽  
Anterior Pituitary ◽  
Hormone Secretion ◽  
Pituitary Hormones ◽  
Pituitary Hormone ◽  
Anterior Pituitary Hormones ◽  
Anterior Pituitary Hormone

Secretion of the anterior pituitary hormones adrenocorticotropin (ACTH), β-endorphin and prolactin (PRL) is complex and involves a variety of factors. This review focuses on the involvement of arginine-vasopressin (AVP) in neuroendocrine regulation of these anterior pituitary hormones with special reference to receptor involvement, mode of action and origin of AVP. Arginine-vasopressin may act via at least two types of receptors: V1− and V2−receptors, where the pituitary V1−receptor is designated V1b. The mode of action of AVP may be mediating, i.e. anterior pituitary hormone secretion is transmitted via release of AVP, or the mode of action may be permissive, i.e. the presence of AVP at a low and constant level is required for anterior pituitary hormones to be stimulated. Under in vivo conditions, the AVP-induced release of ACTH and β-endorphin is mainly mediated via activation of hypothalamic V1− receptors, which subsequently leads to the release of corticotropin-releasing hormone. Under in vitro conditions, the AVP-stimulated release of ACTH and β-endorphin is mediated via pituitary V1b− receptors. The mode of action of AVP in the ACTH and β-endorphin response to stress and to histamine, which is involved in stress-induced secretion of anterior pituitary hormones, is mediating (utilizing V1− receptors) as well as permissive (utilizing mainly V1− but also V2−receptors). The AVP-induced release of PRL under in vivo conditions is conveyed mainly via activation of V1−receptors but V2−receptors and probably additional receptor(s) may also play a role. In stress- and histamine induced PRL secretion the role of AVP is both mediating (utilizing V1 −receptors) and permissive (utilizing both V1− and V2− receptors). Arginine-vasopressin may be a candidate for the PRL-releasing factor recently identified in the posterior pituitary gland. Arginine-vasopressin of both magno- and parvocellular origin may be involved in the regulation of anterior pituitary hormone secretion and may reach the corticotrophs and the lactotrophs via three main routes: the peripheral circulation, the long pituitary portal vessels or the short pituitary portal vessels.

Effect of the Dopamine Agonist, Lergotrile Mesylate, on Circulating Anterior Pituitary Hormones in Man*

1978 ◽  
Vol 47 (2) ◽  
pp. 372-378 ◽  
Author(s):  
M. O. THORNER ◽  
SUSAN M. RYAN ◽  
J. A. H. WASS ◽  
ANN JONES ◽  
P. BOULOUX ◽  
...  
Keyword(s):  
Dopamine Agonist ◽  
Anterior Pituitary ◽  
Pituitary Hormones ◽  
Anterior Pituitary Hormones
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