Neurohypophyseal Ectopy in Growth Hormone Insufficiency

Werder, E A; Zachmann, M.; Wichmann, W.; Valavanis, Anton

doi:10.1159/000181118

Cited by 9 publications

(6 citation statements)

References 10 publications

(12 reference statements)

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“…Also, according to recent MRI evidence of ectopic posterior pituitary, we decided to screen GHD patients with different hypothalamic-pituitary abnormalities in an attempt to analyze the role of prenatal adverse events. Several data suggest that ectopic posterior pituitary in idiopathic GHD is due to migrational arrest and nonfu sion of the neuro-and adenohypophysis in the fetal period [16][17][18]21, 24-27]; brain anomalies such as holencephaly, arrhinencephaly, agenesis of the septum pellucidum and ectopic cerebellar tonsil were described in some of these patients. Ectopic pituitary lobe has been found in Poland's syndrome [22] and in 1 patient with chromosomal abnormality and renal malformations [pers.…”

Section: Discussionmentioning

confidence: 99%

“…This was attributed to traumatic loss of the pituitary stalk at birth [14,15]. The abnormality seems, however, to be of malformative origin, due to migrational arrest and nonfusion of the neuro-and adenohy pophysis during intrauterine life [13,[16][17][18]23, 24], Congenital absence of, or hypoplastic, pituitary gland with ectopic posterior lobe associated with holencephaly and arrhinencephaly was found postmortem in the new borns many years ago [25][26][27], Such pituitary abnormal ities were recently said to be associated with brain anom alies in GHD patients as well as in one malformative syndrome; several of these cases presented no evidence of birth trauma [13,14,[16][17][18][19][20][21][22],…”

Section: Introductionmentioning

confidence: 99%

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Hypopituitarism and Stalk Agenesis: A Congenital Syndrome Worsened by Breech Delivery?

et al. 1991

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Thirty-seven patients with idiopathic hypopituitarism, of whom 12 had multiple pituitary hormone deficiencies (MPHD) and 25 isolated growth hormone deficiency (IGHD), were evaluated by magnetic resonance imaging (MRI). Twenty-two of the 37 showed congenital anterior pituitary hypoplasia, stalk agenesis and ectopic posterior pituitary gland at the infundibular recess (group A), while the remaining 15 presented isolated anterior pituitary hypoplasia (group B). Perinatal histories obtained from all patients demonstrated that 18/22 children of group A (81.81 %) had histories of adverse perinatal events, with breech presentation in 15 (68.18%). Twelve of 12 children of group A born by breech delivery developed MPHD; 3 born by cesarean section for breech presentation had only IGHD. Patients of group B had also a high incidence of perinatal insults (12/15, 80%), but breech delivery was markedly less frequent (13.33 vs. 68.18% of group A) and responsible for only IGHD. Group B had also higher percentages of maternal spontaneous abortion and low birth weight. Our study suggests that several factors may play a role in the development of growth hormone deficiency. Some patients had severe perinatal insults apparently leading to hypopituitarism. We were able to define by MRI a group of patients with congenital abnormalities, such as anterior pituitary hypoplasia, stalk agenesis and posterior pituitary ectopia, among whom breech presentation was very common. In this group, breech delivery was always followed by MPHD while cesarean or normal delivery in such patients was followed by IGHD only.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Hypopituitarism and Stalk Agenesis: A Congenital Syndrome Worsened by Breech Delivery?

et al. 1991

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“…They include 1) significant short stature (47,57,60,74); 2) microcephaly (52,57); 3) osteoporosis (49); 4) lack of eye lashes (74); 5) dysmorphic facies with long narrow face, long philtrum, down-turned mouth, and thin upper lip (48,55,61); 6) poor wound healing (2, 61); 7) cleft palate (60); 8) multiple nasal polyps (60); 9) scoliosis (60); 10) long QT syndrome (62); and 11) hyperlipoproteinemia type IIb (62). Other features.…”

Section: )mentioning

confidence: 99%

“…Thorough investigation of patients with triple A syndrome has ruled out the following disorders associated with or causing the disease: 1) autoimmune Addison's disease (no adrenal autoantibodies) (2, 57); 2) adrenoleukodystrophy (normal very-long-chain fatty acids) (52,57,75,77,86); 3) organic aciduria, carnitine deficiency, Refsum's disease (52); 4) thyroid dysfunction (normal thyroid hormones, no antithyroid antibodies) (47,57,62,77).…”

Section: Exclusion Of Other Disease Processesmentioning

confidence: 99%

Adrenocorticotropin Insensitivity Syndromes

1998

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“…Perinatal and postnatal complications are reported to be responsible for reduced sellar and pituitary volumes by computed tomography scan in large numbers of hypopituitaric patients (2)(3)(4)(5). Magnetic resonance (MR) imaging offers the advantage of revealing hypothalamic-pituitary abnormalities, such as pituitary hypoplasia, absence of the stalk and posterior lobe ectopia in many GH-deficient (GHD) patients (6)(7)(8)(9)(10)(11)(12); the underlying cause of these anatomic defects could be developmental in origin, dating from early intrauterine life (13)(14)(15)(16)(17).…”

mentioning

confidence: 99%

Hypothalamic-Pituitary Dysfunction in Growth Hormone-Deficient Patients with Pituitary Abnormalities*

Maghnie

Triulzi

Larizza

et al. 1991

The Journal of Clinical Endocrinology & Metabolism

118

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Hypothalamic-pituitary function was studied in 45 patients with idiopathic GH deficiency (GHD), 33 of whom had pituitary abnormalities on magnetic resonance imaging: pituitary hypoplasia, undescended stalk and ectopia of the posterior lobe in 8 patients with isolated GHD (IGHD) (group I) and in 12 patients with multiple pituitary hormone deficiency (MPHD) (group II); isolated pituitary hypoplasia in 13 patients with IGHD (group III); no evidence of pituitary abnormalities in the remaining 12 patients with IGHD (group IV). Sellar and pituitary volumes were significantly lower in groups I, II, and III than in group IV (P less than 0.001). No significant differences were observed between group I and group II in the GH response to GHRH1-44 expressed both as peak serum GH and area under the curve. Mean GH peak in group III and IV was significantly higher than that in group I (P less than 0.005) and II (P less than 0.001), as were the mean AUC (P less than 0.005), suggesting hypothalamic defect. Delayed peak serum TSH after TRH was found in all patients of group II, and overt hypothyroidism in 11 of them. Furthermore, basal hyperprolactinemia was present in 6 patients and adrenal insufficiency in 7 cases of group II. Finally, a reduced response of FSH to GnRH was observed in all these patients (P less than 0.005 vs. each of the other groups), and clinical hypogonadism was present in all of them. We suggest that: 1) A high incidence of pituitary abnormalities seems to be present in idiopathic GHD patients; 2) Pituitary hormone deficiencies are more dependent on the type of the hypothalamic-pituitary abnormality than on the size of the pituitary per se: the association of pituitary hypoplasia, undescended stalk and ectopia of the posterior lobe should possibly be considered a distinct entity reflecting an early abnormality in hypothalamic development; 3) The majority of patients with IGHD or MPHD probably have a primary hypothalamic releasing hormone deficiency even if pituitary hypoplasia is associated; 4) Magnetic resonance imaging may have a role in the diagnosis and prognosis of patients with GHD through differentiation between patients who are at risk for developing MPHD vs. those who are candidates for having a persistently isolated GHD.

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Neurohypophyseal Ectopy in Growth Hormone Insufficiency

Cited by 9 publications

References 10 publications

Hypopituitarism and Stalk Agenesis: A Congenital Syndrome Worsened by Breech Delivery?

Hypopituitarism and Stalk Agenesis: A Congenital Syndrome Worsened by Breech Delivery?

Adrenocorticotropin Insensitivity Syndromes

Hypothalamic-Pituitary Dysfunction in Growth Hormone-Deficient Patients with Pituitary Abnormalities*

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