Diagnosis Of Hypopituitarism Research Articles

8403 A Rare Presentation: Mucosal Melanoma with Pituitary Gland Involvement

Abstract Disclosure: L.R. Sepulveda-Garcia: None. L. El Musa Penna: None. W. Medina-Torres: None. J. Segarra-Villafane: None. Z. Maisonet-Feliciano: None. I. Arroyo-Gomez: None. L.A. Gonzalez-Rodriguez: None. M. Alvarado-Santiago: None. M. Ramirez-Vick: None. Introduction: Sinonasal mucosal melanoma (SNMM) is a rare form of aggressive melanoma with poor prognosis, compromising less than 1% of all melanoma. The most frequently reported symptoms include nasal obstruction and epistaxis, but the manifestation of symptoms may occur late and be nonspecific, causing delays in diagnosis and resulting in a less favorable prognosis. Here we report a rare case of mucosal melanoma with pituitary gland involvement initially thought to be a pituitary adenoma. Clinical Case Description: A 22-year-old male with no previous medical history was found with a biopsy proven pigmented epithelioid melanocytoma (PEM) in the palate. Three weeks after the biopsy, the patient presented with a severe headache, prompting the performance of a CT scan that revealed a pituitary macroadenoma. Several days later he developed acute right eye ptosis requiring emergency evaluation. The patient reported mild epistaxis, headaches, and nausea of 2 weeks duration associated with lack of erections and diminished libido. Physical exam was remarkable for sinus bradycardia, right eye ptosis, and a palate bluish pigmentation. Hormonal work up revealed a prolactin of 23 ng/mL (3.46-19.40), TSH of 2.22 uIU/mL (0.27-4.20), fT4 of 0.37 ng/dL (0.93-1.70), total testosterone <0.007 ng/mL (1.88-6.84), free testosterone of 0.001 ng/mL (0.032-0.168), FSH of 0.41 mIU/mL (2.6-11.0), and LH of 0.27 mIU/mL (0.4-7). Serum cortisol and ACTH levels were found to be low at <1.00 µg/dL (3.7-19.40) and 5.98 pg/mL (0.00-46.00), respectively, but these were taken while patient was given dexamethasone by neurosurgery for headaches. Brain MRI was remarkable for 3.4 cm x 3.3 cm x 3.5 cm sellar mass extending inferior into the sphenoid sinus, posteriorly into the right ethmoid air cells, superiorly into the suprasellar cistern with upward displacement of the optic chiasm, as well as bilateral cavernous sinus invasion. Case outcomes: A diagnosis of hypopituitarism was made. Physiologic doses of hydrocortisone were started after being found with central adrenal insufficiency. This replacement unmasked an arginine vasopressin deficiency also requiring desmopressin therapy. Central hypothyroidism was treated with levothyroxine and treatment for the hypogonadotropic hypogonadism was deferred in the meantime. Biopsy of right sphenoid sinus revealed a mucosal melanoma. Revised pathology slides led to the conclusion that palate PEM was an independent entity to the mucosal melanoma Case discussion: Prognosis is poor in patients with SNMM since this tumor is usually diagnosed at advanced stages. In this case, the sellar mass was initially thought to be a pituitary adenoma that later was found to be an extension of a mucosal melanoma. This case serves as a reminder of the critical role that differential diagnosis plays in evaluating sellar masses. Presentation: 6/1/2024

Effect of growth hormone supplementation on liver and lung function in patients with hypopituitarism

To analyze the clinical features of patients with anterior hypopituitarism (HP) complicated with cirrhosis, and to explore the effects of growth hormone supplementation on liver and lung function. A total of 11 patients with HP complicated with cirrhosis admitted to Peking Union Medical College Hospital from January 2016 to December 2022 were included in the study, including 8 males and 3 females, aged [M(Q1, Q3)]31 (20, 37) years. There were 6 patients with pituitary stalk interruption syndrome, 4 patients after craniopharyngioma resection, and 1 patient after germinal cell tumor chemoradiotherapy. Cirrhosis appeared at [M(Q1, Q3)]7 (1, 16) years after the diagnosis of HP. There were 7 cases complicated with hepatopulmonary syndrome (HPS). The liver and lung function of 5 patients were improved significantly after the addition of growth hormone, and the arterial partial pressure of oxygen increased from (47±11) mmHg(1 mmHg=0.133 kPa) to (84±12) mmHg. Timely supplementation of growth hormone can improve the symptoms of fatty liver, cirrhosis and HPS, and postpone or even avoid the transplantation of liver and other organs.

Health-related quality of life of pediatric brain tumor survivors after treatment in Jordan.

The number of cancer survivors and survivorship are increasing. Health-related quality of life (HRQOL) has not been widely studied in low-and-middle-income countries (LMICs). The aim of this study is to explore HRQOL of childhood brain tumor survivors and its determinants in Jordan. Health-related quality of life information was collected from 80 patients treated at the King Hussein Cancer Center and their parents using the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scales questionnaire in Arabic. Multivariable linear OLS regression models were used to analyze correlates of HRQOL and compare differences between child- and parent-reported responses. Health-related quality of life scores reported by survivors and by parents were positively correlated on all subscales and total PedsQL scores (r = 0.59, P = .001). Survivors reported better HRQOL in cognitive subscale (β = 0.56, P = .03) and worse HRQOL in work subscale (β = 0.43, P = .04), but no significant differences in the physical, emotional, and social subscales and total PedsQL scores. Significant predictors of HRQOL reported by parents and by children were different. Supratentorial tumor location was associated with a 10.97-unit lower physical HRQOL score, and recurrence of tumors predicted a 17.5-unit lower total HRQOL score, indicating worse quality of life. Male gender (β = 14.9, P = .002) and diagnosis of hypopituitarism (β = 16.1, P = .03) were associated with better HRQOL. Furthermore, patients that only had radiotherapy treatment had better emotional HRQOL (β = 32.9, P = .006) compared to patients that had combined radiotherapy and chemotherapy. This study provides evidence on determinants of HRQOL of pediatric brain tumor patients in Jordan. Future studies need to capitalize on the findings of this study to institute a system for regular assessment of quality of life of pediatric cancer patients in Jordan and other countries with similar health care systems and sociocultural backgrounds.

Hypopituitarism in Adults: Rational Approaches to Diagnosis and Treatment

AbstractPituitary hormones are responsible for the regulation of growth, development, metabolism, reproduction, and homeostasis. Hypopituitarism is a condition that is defined as partial or complete insufficiency of anterior pituitary hormone secretion, and rarely, posterior pituitary hormone secretion. This condition can result from diseases of the pituitary gland or the hypothalamus. The annual incidence of hypopituitarism has been estimated to be 4.2 per 100,000 yearly, and the prevalence has been estimated at 45.5 per 100,000. The symptoms of hypopituitarism vary. The onset is insidious and depends on the number of hormone deficiencies and their degree of severity. Pituitary hormone deficiency can result in substantial clinical changes that increase the risk of morbidity and mortality. People commonly report persistent symptoms and a decline in their quality of life, both of which can be explained, at least in part, by the inherent shortcomings of hormone replacement strategies in their ability to imitate the normal hormone secretion processes. The diagnosis of hypopituitarism can be straightforward by measuring the lowered basal hormone levels. In cases where the basal hormone levels are uncertain or partial hormone deficiencies have been identified, it may be necessary to perform provocative testing of the hypothalamic–pituitary axis. The hypothalamus and pituitary region can be imaged using magnetic resonance imaging, which provides useful anatomical information. When necessary, genetic studies may be added to the diagnostic approach. The treatment consists of physiological replacement of the individual end-organ hormone deficiencies, and careful monitoring is required throughout the patient's entire life. Individualized hormone replacement therapy that considers potential interactions is recommended. This article provides an overview of the pathophysiology, clinical presentation, general diagnostic guidelines, and treatment options of hypopituitarism.

Immune checkpoint inhibitor-related endocrinopathies: A nationwide population-based study.

2653 Background: Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy. However, unleashing the body’s immune system against cancer is not without consequences and may trigger immune-related adverse events (irAEs), such as endocrinopathies. Real-world data of these endocrinopathies is lacking. This study aims to obtain the prevalence of endocrine irAEs and identify possible underlying demographic risk factors or disparities. Methods: A cross-sectional study was performed using a US-based population database (Explorys) that aggregates records from 26 healthcare systems and includes over 79 million patients. Our sample included all patients on an ICI, and we further identified patients who developed endocrinopathies while on different ICI regimens. The demographic data extracted was categorical, hence presented as counts and percentages. The odds ratio (OR), standard error, and 95% confidence interval were calculated with the use of SPSS software version 28.0 (IBM). Results: There were 20,950 patients on an ICI; 58% males, 83% Caucasians, 10% African-Americans (AA), 38% aged between 18-65, and 63% were over 65 years. The prevalence of the studied endocrinopathies is listed in the table. The most prevalent ICI-induced endocrinopathy was hypothyroidism (7.9-13%). Combination therapy with ipilimumab and nivolumab showed the highest prevalence of ICI-induced endocrinopathies, followed by durvalumab. The odds of developing ICI-induced endocrinopathy were significantly higher with combination therapy than nivolumab monotherapy (OR 1.6; 95% CI 1.4-1.9). There was no statistically significant difference in endocrinopathy prevalence when comparing Caucasians to AA and adults18-65 years to seniors over 65 years. Conclusions: This is the largest epidemiological study of ICI-related endocrinopathies. We find an intriguing divergence from endocrinopathy prevalence reported in the literature and real-world data we are presenting here. Over diagnosis of overt hypothyroidism, as opposed to subclinical hypothyroidism, is concerning as the former necessitates levothyroxine therapy. More alarming is the under diagnosis of hypopituitarism and PAI, in which delay in treatment can be fatal. The diagnosis of these conditions can be challenging. Raising awareness, setting protocols for testing, and involving endocrinologists are crucial. Further studies are needed to clarify our findings. [Table: see text]

Hypopituitarism in patients with metastatic renal cell carcinoma treated with ipilimumab and nivolumab combination therapy.

We investigated the incidence of hypopituitarism in Japanese patients with metastatic renal cell carcinoma (mRCC) who received ipilimumab and nivolumab (I-P) therapy and compared patient characteristics and survival rates between patients with hypopituitarism and those without. Twenty-two patients with mRCC who received I-P therapy as first-line treatment were the subjects of this retrospective study. The diagnosis of hypopituitarism was based on the hormone loading test. Hypopituitarism occurred in 41% (9/22) patients who received I-P therapy. Median time of diagnosis was 12weeks (IQR: 9.5-20). Clinical symptoms, such as fatigue, weakness or fever, were observed in 7 patients, while 2 patients had no clinical presentation. The following deficiency patterns were observed: isolated ACTH in 4 patients, ACTH and GH in 2 patients, ACTH and TSH in 2 patients and triple deficiency (ACTH, GH and TSH) in 1 patient. All patients with hypopituitarism were in the IMDC intermediate group, while 46% of those without hypopituitarism were in the IMDC intermediate group. Other patient characteristics were not different between the two groups. Object response rate was 33% (3/9) in patients with hypopituitarism and 23% (3/13) in those without (P=0.5954). Progression free survival (PFS) was significantly longer in those with hypopituitarism than those without (median: 24.7 vs. 4.5months, P=0.0008), while overall survival did not differ (P=0.136). Compared with the clinical trial, the incidence of hypopituitarism was higher than expected. Patients with hypopituitarism tended to have longer PFS, which may suggest that optimal management of hypopituitarism results in better prognosis.

Гіпопітуїтаризм у дітей. Сучасна лабораторна та генетична діагностика

У лекції наведені сучасні міжнародні рекомендації з діагностики гіпопітуїтаризму у дітей, зокрема правила проведення стимуляційних тестів для діагностики гіпосоматотропізму, вторинного гіпогонадизму й гіпокортицизму. Обґрунтовано використання антимюллерова гормона та інгібіну В у діагностиці різних форм гіпогонадизму. Рекомендовано впровадження генетичних методів дослідження з метою встановлення коректного діагнозу, призначення відповідного лікування й проведення медико-генетичної консультації членів сім’ї.

A Clinical Audit on Diagnosis and Management of Hypopituitarism in Scarborough General Hospital

Objectives: The main objective was to evaluate the use and compliance to the guidelines issued by the Endocrine Society in diagnosis and management of hypopituitarism and their impact on the clinical practice in Scarborough General Hospital (SGH). Design: A retrospective review of secondary data of patients with a diagnosis of hypopituitarism in SGH. Methodology: The data was collected from both patients’ case notes and electronic medical records. Data analysis was performed using Microsoft excel. Results: 18 patients with the diagnosis of Hypopituitarism were identified. 5 patients were females and 13 were males. 11 patients aged between 60-80 years, 4 patients were between 51-60 years and 3 patients were between 31-50 years. All the patients had low cortisol levels; however, cortisol was checked at 9AM in only 4 patients. All the patients had their thyroid stimulating hormone levels checked, which were low in all of them. Free T4 levels were normal in 2 patients, and low in 16 patients. 17 patients had low follicular stimulating hormone levels, whereas 15 had low luteinising hormone levels. Testosterone levels were checked in 9 male patients out of which 7 had low levels. Insulin-like growth factor-1(IGF1) was done in all patients and levels were low in 10 patients. All those with low IGF1 had glucagon stimulation test. Serum sodium levels were low in 3 patients and high in 5 patients. Plasma osmolality was only checked in 8 patients (was high in 5 patients). Urine osmolality was checked in 10 patients, and it was low in 5 patients. Brain MRI was performed in 16 patients, and 2 patients had brain CT. The most common cause of hypopituitarism was non-functioning pituitary adenoma (44.4%), and the least common causes were empty Sella syndrome (5.6%) and craniopharyngioma (5.6%). All patients received glucocorticoid replacement therapy, 17 received thyroid hormone replacement therapy, 8 received testosterone replacement therapy, 3 received desmopressin (DDAVP) treatment, and one patient received growth hormone replacement therapy. Conclusion: This Audit shows that our practice in diagnosis and management of hypopituitarism is mostly in line with the recommendation of the Endocrine Society. However, cortisol levels were not always measured at 9:00 am and we are not compliant with performing Growth Hormone stimulation test in every patient.

A Case Report on Hyponatremia: Leading Sign of Hypopituitarism [Secondary to Adrenal Insufficiency

Severe hyponatremia due to hypopituitarism and adrenal insufficiency can be life threatening and treatment with glucocorticoids is very effective once the diagnosis of the underlying disorder has been made. In our experience, the diagnosis of hypopituitarism in hyponatremic patients is often overlooked. We report the case of a 54 year old female admitted to our hospital in an obtunded state with altered sensorium. We found that a severe hyponatremia was the cause of patient condition . Previously she was diagnosed with recurrent episodes of hyponatremia leading to encephalopathy. The diagnosis of hypopituitarism is often overlooked in hyponatremic patients. Hyponatremia should always be ruled out for endocrinological cause Our study concludes that hyponatremia is due to hypopituitarism that is secondary to adrenal insufficiency leading to encephalopathy.

Russell's viper bite and the empty sella syndrome.

Snake bites are very common in India especially in rural areas. Empty sella syndrome is a relatively uncommon sequela of Russell's viper bite. Here, we describe a case of 22-year-old female who was seriously envenomed by Russell's viper bite at the age of 14 years. Diagnosis of hypopituitarism following Russell's viper bite was significantly delayed. We attribute her empty sella syndrome and hypopituitarism to Russell's viper envenoming.

MON-330 Cancer Incidence in 1,296 Patients with Acromegaly Is Not Increased: A Nationwide Population-Based Study

Background: Single- and multi-center studies have shown an increased incidence of malignancies in patients with acromegaly. These findings may be affected by selection bias. Our aim was therefore to investigate the incidence of malignancies in a nationwide unselected cohort of patients with acromegaly. Methods: Adult patients diagnosed with acromegaly due to a pituitary tumor between 1987 and 2017 were identified in the Swedish National Patient Registry. All malignancies following the diagnosis of acromegaly were identified in the Swedish Cancer Registry that has a coverage of over 96%. Standardized incidence ratios (SIRs) for malignancies, with 95% confidence intervals (CI), were calculated by using the Swedish general population as a reference. Incidence of malignancies was also analyzed in sub-groups of patients treated with radiotherapy and in those having diabetes mellitus and hypopituitarism. Results: A total of 1,296 patients with acromegaly were included (621 men, 675 women). The mean age (±SD) at diagnosis was 51.6±14.7 years. The mean follow-up was 12.7±8.3 years, with a total of 16,395 person years at risk. Pituitary surgery was performed in 842 (65%) patients and radiation therapy in 152 (12%) patients. The diagnosis of hypopituitarism and diabetes mellitus was recorded in 29% and 16% of patients, respectively. Overall, 186 malignancies were identified in patients with acromegaly as compared to 179 expected malignancies in the general population (SIR 1.04; 95% CI 0.90-1.20). Incidence of malignancies was similar in men and women [SIR 1.08 (95% CI 0.88-1.32) vs 1.00 (95% CI 0.80-1.23)]. Incidence of colorectal cancer (SIR 1.12; 95% CI 0.75-1.62) or malignancies of the respiratory system (SIR 1.22; 95% CI 0.76-1.84) was not increased. Incidence of kidney and ureter cancer (n=17) was, however, increased (SIR 3.81; 95% CI 2.22-6.11). In the entire study cohort, only three cases of thyroid cancer were recorded. SIR for malignancies in patients treated with radiotherapy (1.12; 95% CI 0.56-2.01) and in patients with hypopituitarism (SIR0.91; 95% CI 0.68-1.18) or diabetes (SIR 1.08; 95% CI 0.78-1.45) did not differ from the general population. Conclusions: This large nationwide population-based study showed that the overall incidence of malignancies in patients with acromegaly was not different from the general population. In particular, incidence of colorectal and thyroid cancer was not increased. Incidence of malignancies of the urinary tract was, however, increased.

Clinical picture and the treatment of TBI-induced hypopituitarism.

Traumatic brain injury (TBI) is an important public health problem with an increasing incidence in the last years. Relatively few cases are fatal; most individuals will survive and, in the long-term, the sequalae of TBI will include neuroendocrine dysfunctions with a much higher frequency than previously suspected. Patients who develop hypopituitarism after TBI present manifestations due to the number of deficient hormones, severity of hormonal deficiency, and the duration of hypopituitarism without diagnosis and treatment. The clinical spectrum of hypopituitarism is very large and many signs and symptoms of TBI survivors such as fatigue, concentration difficulties, depressive symptoms are nonspecific and overlap with symptoms of post-traumatic stress disorder and variably severe hypopituitarism related to brain damage remaining undiagnosed. This can explain why the diagnosis of hypopituitarism is often missed or delayed after this condition with potentially serious and hazardous consequences for the affected patients. Moreover, clinical experience cumulatively suggests that TBI-associated hypopituitarism is associated with poor recovery and worse outcome, since post-traumatic hypopituitarism is independently associated with cognitive impairment, poor quality of life, abnormal body composition, and adverse metabolic profile. In the present review, the current data related to clinical consequences of pituitary dysfunction after TBI in adult patients and therapeutic approaches are reported.

Congenital Hypopituitarismin Saudi Arabia: Is it That Rare?

Background: Congenital hypopituitarism is a clinical syndrome of deficiency in pituitary hormones production. Panhypopituitarism refers to involvement of more than one pituitary hormone while involvement of one hormone refers to partial hypopituitarism It is an uncommon disorder of the hypophyseal system but could be life threatening, however, it is treatable if the diagnosis is made early. Design and setting: A retrospective hospital based study was conducted at Pediatric endocrine service, King Khalid University Hospital ( KKUH ) Riyadh, Saudi Arabia during the period of January 1990 and December 2017. Material and Methods: The medical records of patients with the diagnosis of hypopituitarism were retrospectively reviewed. Data included age, sex, clinical presentation, and results of relevant laboratory investigations and radiological imaging. Results: During the period under review, a total of 177 patients were diagnosed with possible congenital hypopituitarism. The mean age was 6.5 years range 0-18 years. Seventy-five percent were having isolated hormone deficiency; Growth hormone 117 (87.9%), gonadotrophic hormone 8 (6.0%), central hypothyroidism 5 (3.8%) and adrenocorticotrophic hormone 3 (2.3%). In twenty- five percent of patients the diagnosis was multiple pituitary hormone deficiency (MPHD), in which septo-optic dysplasia and other midline defects, constitute the majority (56.8%). Diabetes Insipidus was found in association in five (2.5%) patients. Conclusion: Congenital hypopituitarism in not that rare in Saudi Arabia. An early diagnosis can be obtained with high accuracy based on a high clinical suspicion index. Imaging abnormalities are frequent and associated with the clinical and biochemical phenotypes. It had variable presentations, such as hypoglycemia, micro phallus in boys and neonatal cholestasis, or in association with midline defects. There is a need to compliment hormonal and radiological investigations with gene study.

Evaluation of ICD-10 algorithms to identify hypopituitary patients in the Danish National Patient Registry.

ObjectiveRoutinely collected health data may be valuable sources for conducting research. This study aimed to evaluate the validity of algorithms detecting hypopituitary patients in the Danish National Patient Registry (DNPR) using medical records as reference standard.Study design and settingPatients with International Classification of Diseases (10th edition [ICD-10]) diagnoses of hypopituitarism, or other diagnoses of pituitary disorders assumed to be associated with an increased risk of hypopituitarism, recorded in the DNPR during 2000–2012 were identified. Medical records were reviewed to confirm or disprove hypopituitarism.ResultsHypopituitarism was confirmed in 911 patients. In a candidate population of 1,661, this yielded an overall positive predictive value (PPV) of 54.8% (95% confidence interval [CI]: 52.4–57.3). Using algorithms searching for patients recorded at least one, three or five times with a diagnosis of hypopituitarism (E23.0x) and/or at least once with a diagnosis of postprocedural hypopituitarism (E89.3x), PPVs gradually increased from 73.3% (95% CI: 70.6–75.8) to 83.3% (95% CI: 80.7–85.7). Completeness for the same algorithms, however, decreased from 90.8% (95% CI: 88.7–92.6) to 82.9% (95% CI: 80.3–85.3) respectively. Including data of hormone replacement in the same algorithms PPVs increased from 73.2% (95% CI: 70.6–75.7) to 82.6% (95% CI: 80.1–84.9) and completeness decreased from 94.3% (95% CI: 92.6–95.7) to 89.7% (95% CI: 87.5–91.6) with increasing records of E23.0x.ConclusionThe DNPR is a valuable data source to identify hypopituitary patients using a search criteria of at least five records of E23.0x and/or at least one record of E89.3x. Completeness is increased when including hormone replacement data in the algorithm. The consequences of misclassification must, however, always be considered.

Relationship of each anterior pituitary hormone deficiency to the size of non-functioning pituitary adenoma in the hospitalized patients.

Non-functioning pituitary adenoma (NFPA) is often associated with hypopituitarism. Diagnosis of hypopituitarism is important because of its poor prognosis and low quality of life. Among hypopituitarism, it is difficult to diagnose secondary adrenocortical insufficiency and GH deficiency without hormone stimulation test. Therefore, the aim of our study was to identify patients with NFPA who require more careful endocrinological examination. We examined the relationship between NFPA size and the prevalence of each hypopituitarism or the response of each anterior pituitary hormone by insulin tolerance test, LHRH test and TRH test. We studied 63 patients with NFPA admitted for evaluation of pituitary function and surgical indication. They were classified three groups by tumor diameter. The prevalence of GH deficiency, male secondary hypogonadism, secondary hypothyroidism and PRL deficiency were higher in the group of larger tumor diameter (p<0.0001, p<0.05, p<0.05 and p<0.05, respectively). However, that of secondary adrenocortical insufficiency only tended to be higher (p=0.07). In the group with small NFPA (less than 20 mm), the prevalence of secondary adrenocortical insufficiency was 38% although those of GH deficiency, male secondary hypogonadism, secondary hypothyroidism and PRL deficiency were 0%, 0% and 8% and 9%, respectively. Anterior pituitary hormone responses except TSH had significantly negative correlation with tumor diameter (ACTH: r=-0.40, GH: r=-0.57, LH: r=-0.69, FSH: r=-0.46, PRL: r=-0.36). The results suggested physicians should proactively suspect GH deficiency, male secondary hypogonadism and secondary hypothyroidism in patients with larger NFPA. On the other hand, adrenocortical function should be examined even in patients with small NFPA.

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism.

Carotid-cavernous fistulas (CCFs) are rare, pathologic communications of the carotid artery and the venous plexus of the cavernous sinus. They can develop spontaneously in certain at risk individuals or following traumatic head injury. Typical clinical manifestations include headache, proptosis, orbital pain, and diplopia. We report a case of bilateral carotid-cavernous fistulas associated with these symptoms and also with pituitary enlargement and hypopituitarism, which improved following surgical intervention. Arterialization of the cavernous sinus and elevated portal pressure may interfere with normal venous drainage and the conveyance of inhibiting and releasing hormones from the hypothalamus, resulting in pituitary enlargement and hypopituitarism. This condition should be considered in the differential diagnosis of hypopituitarism associated with anterior pituitary enlargement.

Excess Mortality in Women and Young Adults With Nonfunctioning Pituitary Adenoma: A Swedish Nationwide Study.

Patients with hypopituitarism of various etiologies have excess mortality. The mortality in patients with nonfunctioning pituitary adenoma (NFPA), regardless of pituitary function, is less well studied. Our aim was to investigate mortality in patients with NFPA and to examine whether age at diagnosis, gender, tumor treatments, or hormonal deficiencies influence the outcome. NFPA patients were identified and followed up in nationwide health registries in Sweden, 1987-2011. The criteria for identification were tested and validated in a subpopulation of the patients. This was a nationwide, population-based study. A total of 2795 unique patients with NFPA (1502 men, 1293 women) were identified and included in the study. Mean age at diagnosis was 58 years (men, 60 y; women, 56 y) and mean follow-up time was 7 years (range 0-25 y). There were no interventions. Standardized mortality ratios (SMRs) and annual incidence rates were calculated using the Swedish population as reference and presented with 95% confidence intervals. Annual incidence of NFPA was 20.3 (18.8-21.9) cases per 1 million inhabitants. During the observation period, 473 patients died against an expected 431, resulting in an SMR of 1.10 (1.00-1.20). Patients diagnosed at younger than 40 years of age had an increased SMR of 2.68 (1.23-5.09). The SMR for patients with hypopituitarism (n = 1500) was 1.06 (0.94-1.19), and for patients with diabetes insipidus (n = 145), it was 1.71 (1.07-2.58). The SMR was increased in women with NFPA (1.29; 1.11-1.48) but not in men (1.00; 0.88-1.12). Women, but not men, with a diagnosis of hypopituitarism and/or diabetes insipidus also had an increased mortality ratio. SMRs due to cerebrovascular (1.73; 1.34-2.19) and infectious diseases (2.08; 1.17-3.44) were increased, whereas the SMR for malignant tumors was decreased (0.76; 0.61-0.94). This nationwide study of patients with NFPA showed an overall excess mortality in women and in patients with a young age at diagnosis. Increased mortality was seen for cerebrovascular and infectious diseases.

A case of congenital combined pituitary hormone deficiency who showed respiratory distress and hypoglycemia soon after birth

Congenital combined pituitary hormone deficiency (CCPHD) is a rare disease, presenting with respiratory distress, hypoglycemia and jaundice soon after birth. It is caused by .pituitary aplasia or hypoplasia or abnormality of transcription factor which related to pituitary development. We describe a Japanese patient with CCPHD, who presented with respiratory distress, hypoglycemia and jaundice soon after birth. The patient is now 13 months old Japanese girl. She was born by cesarean section because of fetal distress with 39 weeks and 4 days gestation after an uneventful pregnancy. Her parents were unrelated. Birth length and weight were 43.8cm (-2.2SD) and 2,100g and Apgar scores were 8 at both of 1 and 5 min. She had showed tachypnea and cyanosis since soon after birth and taken nasal-CPAP. However respiratory distress had not improved. She had also showed jaundice and hypoglycemia and was treated with phototherapy and infusion therapy. At 6 days old, we found her FT4 level was 0.4 pg/ml and TSH level was below 0.01 μIU/ml. She was suspected CPHD and anterior pituitary hormone levels were assessed. All of anterior pituitary hormones were undetectable. Anterior pituitary was not visible, posterior pituitary was detected at normal position and there were no other abnormality on brain magnetic resonance imaging. We diagnosed her as CCPHD and hydrocortisone was started since 6 days old and levothyroxine since 8 days old. After that respiratory distress and jaundice had improved rapidly. But hypoglycemia had appeared again after interrupting infusion therapy. GH level after arginine stimulation was also undetectable. She had also started growth hormone therapy since 25 days old and she has had no hypoglycemic episodes after that. Her length and weight were 70.3cm (-1.2SD) and 8,485g at 1 year old. GH replacement therapy that was started soon after diagnosis was able to prevent the severe growth retardation. She has no gene abnormalities of transcription factors, HESX1, LHX4 and OTX2, that involves pituitary development. Further studies are needed for investigating genetic basis of this patient. Careful and prompt clinical, endocrinological and neuroradiological assessment are needed for diagnosis of hypopituitarism in patients who show prolonged respiratory distress, hypoglycemia or jaundice soon after birth. Written informed consent was obtained from the patient's parent or guardian for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Can a Diagnosis be Ruled Out Based on One Cortisol Result? A Case Report on Delayed Diagnosis of Hypopituitarism

There are numerous potential causes for hyponatraemia including hypovolaemia; oedema; syndrome of inappropriate ADH secretion; and acute episodes of Addison’s disease (hypopituitarism and adrenal insufficiency) [1]. Hyponatraemia can have serious consequences for patients and there are many ways to investigate the cause. Here we present a case of an elderly patient presenting with hyponatraemia and highlight the limitations of the standard dose (250 mcg) short synacthen test (SST) for the investigation of adrenal insufficiency as a potential cause of hyponatraemia.

PO-0592 Distinguishing Congenital Combined Pituitary Hormone Deficiency From Biliary Atresia As A Cause Of Cholestasis In Infants

<h3>Background and aims</h3> Neonatal cholestasis is caused by either biliary atresia or intrahepatic cholestasis. Congenital combined pituitary hormone deficiency (CPHD) is a rare disease and a recognised cause of intrahepatic cholestasis. It is important to differentiate cholestasis due to this entity from cholestasis due to biliary atresia, since both diseases can cause jaundice at about 1 month of age. However, doing so in a timely fashion remains a diagnostic dilemma. This retrospective study was performed to clarify differences between cholestasis due to congenital CPHD and cholestasis due to biliary atresia. <h3>Methods</h3> From 2004 to 2010, 4 infants (2 boys and 2 girls) with cholestasis due to congenital CPHD were admitted to Nagoya City University Hospital. Head magnetic resonance imaging of the 4 infants revealed an invisible pituitary stalk; 3 of these 4 infants had an ectopic posterior pituitary. Liver biopsy was performed in 3 of the 4 infants, and histological findings included giant cell hepatitis. Findings from these 4 infants were then compared with those from55 infants treated in our hospital for cholestasis due to biliary atresia. <h3>Results</h3> The results showed a significant difference in mean gamma-glutamyl transpeptidase levels between the two groups of infants (115.0 IU/l vs. 553.0 IU/l, respectively). <h3>Conclusions</h3> The gamma-glutamyl transpeptidase level was found to be useful for distinguishing congenital CPHD from biliary atresia as the cause of cholestasis. The diagnosis of hypopituitarism should always be considered in infants with unexplained neonatal hepatitis.