Hypopituitarism: replacement of adrenal, thyroid, and gonadal axes

2011 ◽  
pp. 145-152 ◽  
Author(s):  
Trevor A. Howlett
Keyword(s):  
Anterior Pituitary ◽  
Patient Management ◽  
Hormone Replacement ◽  
Thyroid Stimulating Hormone ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Best Management ◽  
Anterior Pituitary Hormone ◽  
The Subject ◽  
Based Medicine

Hormone replacement of anterior pituitary hormone deficiency is one of the most frequent clinical interventions in pituitary disease, yet is an area which has rarely been the subject of rigorous scientific evaluation. Even in an era of ‘evidence-based’ medicine, recommendations for patient management are frequently based predominantly on clinical experience, consensus guidelines and occasional retrospective reviews rather than on controlled, prospective clinical trials. Within these limitations, this chapter will attempt to give a balanced view on current best management of adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH) and gonadotropin deficiency.

scholarly journals Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System

2021 ◽  
pp. 1-9
Author(s):  
Elisa Vaiani ◽  
Guido Felizzia ◽  
Fabiana Lubieniecki ◽  
Jorge Braier ◽  
Alicia Belgorosky
Keyword(s):  
Anterior Pituitary ◽  
Langerhans Cell Histiocytosis ◽  
Endocrine System ◽  
Langerhans Cell ◽  
Hormone Deficiency ◽  
Bone Lesions ◽  
Pituitary Hormone ◽  
Multisystem Disease ◽  
Anterior Pituitary Hormone

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.

Multiple Anterior Pituitary Hormone Deficiency

2009 ◽  
pp. 1363-1364
Author(s):  
Mark Oette ◽  
Marvin J. Stone ◽  
Hendrik P. N. Scholl ◽  
Peter Charbel Issa ◽  
Monika Fleckenstein ◽  
...  
Keyword(s):  
Anterior Pituitary ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Anterior Pituitary Hormone

The pituitary and hypopituitarism

2020 ◽  
pp. 83-120
Author(s):  
Gary Butler ◽  
Jeremy Kirk
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Hormones ◽  
Thyroid Stimulating Hormone ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Sexual Characteristics ◽  
Biochemical Assessment ◽  
Cranial Diabetes Insipidus ◽  
Clinical Problems ◽  
Stimulating Hormone

• The pituitary is formed of two anatomically and embryologically distinct lobes: ◦ anterior pituitary: which secretes growth hormone (GH), gonadotropins (luteinizing hormone (LH) and follicle-stimulating hormone (FSH)), adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH), and prolactin ◦ posterior pituitary: which secretes vasopressin and oxytocin. • Hypopituitarism is deficiency of one or more pituitary hormones. Some hormones, e.g. GH (LH/FSH), are more likely to present with isolated deficiencies, while others, e.g. TSH, ACTH, are more often found as part of multiple pituitary hormone deficiency (MPHD). • Deficiencies may be congenital (including genetic) or acquired; secondary to tumour, trauma, infiltration, infection, or irradiation. • GH deficiency: ◦ diagnosed using a combination of clinical, radiological, and biochemical assessment (including GH stimulation testing) ◦ treatment is with GH (including if necessary into adulthood). • LH/FSH deficiency: ◦ If acquired, often one of the first anterior pituitary hormones to be lost. ◦ Congenital forms: ■ present with cryptorchidism and/or micropenis in males ■ may occur in isolation, or in association with anosmia (Kallmann and CHARGE syndromes). ◦ Treatment: sex steroid therapy to induce secondary sexual characteristics, and recombinant FSH/LH to induce fertility potential. • ACTH deficiency: ◦ Unlike primary adrenal problems, hyperpigmentation does not occur. ◦ Although mineralocorticoid production is preserved, hyponatraemia may still occur. ◦ Treatment is with hydrocortisone. • TSH deficiency: ◦ TSH may be low, normal, or raised (but inappropriate for free thyroxine level). ◦ Treatment is with thyroxine. • Vasopressin deficiency: ◦ produces cranial diabetes insipidus ◦ treatment is with DDAVP (orally or nasally). • Prolactin and oxytocin deficiency rarely produce clinical problems.

scholarly journals Postpartum Cranial Diabetes Insipidus

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A603-A603
Author(s):  
Ross Andrew Cairns ◽  
Mohammed K Azharuddin
Keyword(s):  
Diabetes Insipidus ◽  
Anterior Pituitary ◽  
Urine Osmolality ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Anterior Pituitary Hormone ◽  
Patient Reported ◽  
Rare Diagnosis ◽  
Cranial Diabetes Insipidus

Abstract Cranial Diabetes Insipidus is a rare diagnosis and rarer still postpartum. We present the case of 24-year-old woman who developed CDI following pregnancy. The patient had developed persistent polydipsia and polyuria 5 months following her first pregnancy. The pregnancy had been complicated by Gestational Diabetes Mellitus, Obstetric Cholestasis and a Postpartum Haemorrhage which had required a 3 unit transfusion of blood. The patient reported feeling fatigued and lightheaded and stated that she had needed to drink water frequently: around 8 litres throughout the day and 4 litres overnight. She reported that she had been unable to breastfeed but the rest of her systemic enquiry was unremarkable. The patient had attributed her symptoms of lethargy to sleepless nights with her new born baby and the polyuria as a consequence of labour and as such had presented for review at her primary care Physician 18 months following delivery. Initial investigations revealed a fasting blood glucose of 4.9 mmol/l, an Adj. Calcium of 2.23 mmol/l and a fasting urine osmolality of 85 mmol/kg. A diagnosis of DI was suspected and was confirmed by water deprivation test: the patient had an inappropriately dilute urine osmolality of 111 mmol/kg when compared to the serum osmolality of 301 mOsm/Kg at the start of the test and her urine failed to concentrate as water was withheld. Administration of DDAVP resulted in appropriate concentration of urine and therefore confirmed the diagnosis specifically as Cranial Diabetes Insipidus. Blood tests revealed normal anterior pituitary function: TSH was 2.78 mU/L, Prolactin was 361 mU/l, LH and FSH were 23.6 U/L and 5.3 U/L, IGF and GH were 197 ug/L and 0.1 ug/l and ACTH was 10 mU/L. Her basal cortisol was 392 nmol/l and was stimulated to 593 nmol/l by SST. MRI Pituitary revealed an unusually flat and broad pituitary gland with a possible tiny lesion in the posterior pituitary suggestive of an adenoma. The patient was established on DDAVP replacement therapy and her quality of life improved: she enjoyed restful sleep and reported less exhaustion. There was no anterior pituitary hormone deficiency 28 months following delivery. DI is a rare diagnosis with an estimated prevalence of 1 in 25000 people. CDI has been commonly reported as being caused by infiltrative or inflammatory pituitary disease, as an iatrogenic sequelae of pituitary surgery or as a result of a congenital defect in the production of vasopressin. We suspect that in this case the patient’s PPH may have resulted in isolated cranial diabetes insipidus though the significance of the MRI scan findings remains unclear. The learning points highlighted by this case are that CDI can occur following pregnancy in an isolated form without anterior pituitary hormone deficiency. We also highlight that patients may misattribute significant symptoms and signs of DI as being a normal part of the postpartum period resulting in a delayed diagnosis.

Pituitary Stalk Interruption Syndrome: Presentation of a Rare Case

2018 ◽  
Vol 17 (05) ◽  
pp. 176-179
Author(s):  
Burcin Agridag Ucpinar ◽  
Ahmet Ucar ◽  
Evrim Ozmen
Keyword(s):  
Mental Retardation ◽  
Incidence Rate ◽  
Anterior Pituitary ◽  
Hormone Replacement ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Magnetic Resonance Imaging Scan ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Mild Mental Retardation

AbstractPituitary stalk interruption syndrome is a congenital anomaly characterized by interrupted or thin pituitary stalk, hypoplastic or absent anterior pituitary, and an absent or ectopic posterior pituitary gland. The exact incidence rate of this syndrome is not known. However, the estimated incidence rate is 0.5/1,000,000 births. In this case report, we wanted to present a case of interrupted pituitary stalk syndrome, which presented with seizures and thyroid hormone deficiency. A 5-year-old female patient was admitted to our emergency department with vomiting, fever, and seizures with new onset. She had a twin who was ex-utero intrapartum in taken history. She was diagnosed with hypothyroidism and started on levothyroxine. Her height was in 25 to 50th percentile and her weight was in 10 to 25th percentile. She had mild mental retardation. On contrast-enhanced cranial magnetic resonance imaging scan, the pituitary stalk was absent, posterior pituitary was ectopic, and anterior pituitary was hypoplastic. The patient was diagnosed with interrupted pituitary stalk syndrome. After the symptoms were relieved, patient started on carbamazepine for epileptic seizures and hormone replacement therapy with levothyroxine and hydrocortisone. She was routinely followed up after the proper diagnosis. Leuprolide (gonadotropin-releasing hormone) and Norditropin (biosynthetic growth hormone) were added to medical therapy. Her height and weight were in 25th percentile after the long-term follow-up of approximately 10 years. On neurological examination, situation of mild mental retardation persisted. Pituitary stalk interruption syndrome is a very rare entity. However, radiologists should keep this syndrome in mind for patients who present with hypoglycemia, seizures, jaundice, cryptorchidism, and hypothyroidism in neonatal period and growth retardation with pituitary hormone deficiencies in childhood.

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