Acromegalic Appearance Research Articles

Acromegaly increases depressive symptoms and reduces quality of life of cohabitants.

To assess how living with patients with acromegaly affects people's psychology and quality of life (QoL). Acromegaly patients and their cohabitants included in this study. Patients were administered Acromegaly Quality of Life Questionnaire (AcroQoL), Beck Anxiety Inventory (BAI), and Beck Depression Inventory (BDI). Cohabitants were administered quality of life questionnaire (SF-36), BAI, BDI, and Zarit Caregiver Burden Interview (ZBI). This study included 84 patients with acromegaly and 84 cohabitants. Sixty-nine patients (n = 84, 82.1%) had an acromegalic appearance. Cohabitants who were affected by the acromegalic appearance had higher BAI, BDI, and ZBI scores than those who were not affected (p < 0.001 for all). Cohabitants who were affected by the acromegalic appearance had a significantly lower SF-36 score (p = 0.015). The BAI (r=-0.535, p < 0.001), BDI (r=-0.592, p < 0.001), and ZBI (r=-0.465, p < 0.001) scores of the cohabitants showed a negative correlation with AcroQoL. SF-36 scores showed correlation with AcroQoL (r = 0.387, p < 0.001). The chronic process of acromegaly and the external appearance of patients with this disease can negatively affect both the patients and the people living with them. Physicians being aware of this effect and taking counteractive measures may contribute positively to the course of acromegaly.

Incidence of Pituitary Apoplexy and Its Risk Factors in Chinese People: A Database Study of Patients with Pituitary Adenoma.

BackgroundThere are few studies of the incidence and clinical characteristics of pituitary apoplexy (PA) in pituitary adenoma patients, and the findings have been inconsistent.ObjectiveThe aim of the study was to retrospectively assess the incidence, clinical presentation, surgical management and postoperative complications of PA in pituitary adenoma patients.MethodsA database was specifically designed to collect clinical, therapeutic, prognostic and histological information about pituitary adenoma patients. Using multivariate logistic regression, odds ratios (ORs) with 95% confidence intervals (CIs) were calculated to identify associated factors.ResultsA total of 2021 pituitary adenoma patients were recruited. 97 (4.8%) patients had PA. The incidence of PA was 10.11% in patients with pituitary macroadenoma, and 0.36% in patients with microadenoma. Variables for the logistic regression model independently associated with PA were sex (male vs. female, OR = 2.54, 95% CI: 1.59~4.07), tumor type (negative staining vs. positive staining, OR = 2.04, 95% CI: 1.29~3.23), and tumor size (macroadenoma vs. microadenoma, OR = 26.46, 95% CI = 9.66~72.46). Headache, visual deterioration, and vomiting were the most common symptoms in patients with pituitary adenoma. Patients with and without PA had similar frequency of visual deterioration, head trauma, acromegalic appearance, galactorrhoea, cold intolerance and Cushingoid appearance, but headache, vomiting, ptosis, diplopia, fever and blindness were significantly more common in patients with PA. Pearson Chi-Square tests revealed a significant difference in surgical approach between patients with and without PA (95.88% vs. 85.57%, P = 0.01).ConclusionOur findings suggest that PA is not a rare event. Male sex, non-functioning tumor, and macroadenoma are associated with an increased risk of PA. Compared with pituitary adenoma patients without PA, patients with PA have more severe symptoms.

A somatotropin-producing pituitary adenoma with an isolated adrenocorticotropin-producing pituitary adenoma in a female patient with acromegaly, subclinical Cushing’s disease and a left adrenal tumor

A 67-year-old female with hypertension and impaired glucose tolerance was admitted to our hospital because of a typical acromegalic appearance, including large, thickened bulky hands and feet, and a large prominent forehead and tongue. She did not have a Cushingoid appearance, such as a moon-face, buffalo hump, purple striae or central obesity. The laboratory data revealed a serum GH level of 4.6 ng/mL and serum insulin-like growth factor-1 level of 811 ng/mL. The oral glucose tolerance test showed no suppression of the GH values. An endocrine examination showed a lack of circadian rhythmicity of ACTH and cortisol. Cortisol was not suppressed by a low dose of dexamethasone during the suppression test, but was suppressed by a high dose of dexamethasone. A radiological study revealed two isolated adenomas in the pituitary and a left adrenal tumor. These findings strongly suggested a diagnosis of acromegaly with subclinical Cushing's disease and a left adrenal incidentaloma. Transsphenoidal surgery was performed. Hematoxylin and eosin staining showed that the left and right pituitary adenomas were composed of basophilic and acidophilic cells, respectively. Immunohistochemical staining showed the left adenoma to be positive for ACTH and negative for GH. In contrast, the right adenoma was GH-positive and ACTH-negative. This is a rare case of independent double pituitary adenomas with distinct hormonal features. We also provide a review of the previously reported cases of double pituitary adenomas and discuss the etiology of these tumors.

Acromegaloid Facial Appearance: Case Report and Literature Review

Pseudoacromegaly is characterized by an acromegalic appearance without any abnormality of growth hormone function. It may be caused by several congenital and acquired conditions. One such condition is the acromegaloid facial appearance (AFA) syndrome. This condition has been described in approximately eight cases/families. It encompasses a spectrum of acromegaloid physical findings, normal growth hormone (GH) and insulin-like growth factor one (IGF-1) levels, and variable mode of inheritance. The most common physical findings are coarse facies, bulbous nose, and thickened lips. We present a case and a review of the literature on this illness. The patient is a 57-year-old woman who was referred to the endocrinology division for evaluation of suspected acromegaly. She had an acromegaloid appearance since birth as well as a terminal hypertrichosis. Her endocrine laboratory evaluation and chromosomal analyses were normal. AFA needs to be considered when evaluating any patient with pseudoacromegaly. Additional cases/families need to be identified in order to better understand the clinical spectrum, clinical implications, and mode of inheritance of AFA.

Governor Pio Pico, the monster of California…no more: lessons in neuroendocrinology

We hypothesize that Pio Pico, the last Mexican Governor of California, had acromegaly between at least ages 43 to 57, from 1844 to 1858, before Pierre Marie published the clinical description of acromegaly in 1886. Pico’s probable growth hormone-secreting pituitary tumor likely infarcted spontaneously after 1858. The tumor infarction resulted in burnt-out acromegaly and probably restored normal pituitary function. Pearce Bailey published the first account of pituitary tumor infarction only in 1898. Pico’s undiagnosed, misunderstood, profoundly acromegalic appearance was widely misinterpreted, leading to pervasive, degrading, and highly prejudicial comments. This landmark case study in neuroendocrinology provides the opportunity to re-examine elements of 19th century California and American history.

CHRONIC RENAL FAILURE IN A PATIENT WITH SOTOS SYNDROME DUE TO AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

SUMMARYSotos syndrome is characterised by accelerated growth, acromegalic appearance, mental retardation and social maladjustment. Most cases are sporadic, but familial cases have also been reported. We report a case of Sotos syndrome presenting with chronic renal failure due to autosomal dominant polycystic kidney disease (ADPKD). Ultrasonographic examination of the patient, his father and other family members revealed polycystic kidneys. Renal failure was present only in the Sotos case, who also had considerably larger cysts than other family members. We suggest that the underlying mechanism responsible from the somatic overgrowth in Sotos syndrome may also be linked with the development of larger cysts and earlier onset of renal failure in ADPKD. Although Sotos syndrome has been associated with urological abnormalities, chronic renal failure is very rare. To our knowledge, Sotos syndrome associated with ADPKD has not been reported before.

Simultaneous Occurrence of a Pituitary Adenoma and a Falcotentorial Junction Meningioma

A case of pituitary adenoma associated with a falcotentorial junction meningioma is presented. A 50-year-old female having a 12-year history of acromegalic features and a 3-year history of visual disturbance was admitted because of headache, vomiting, and a floating sensation. On admission acromegalic appearance, bitemporal hemianopsia, and truncal ataxia were noted. Computed tomography scan showed two mass lesions on the midline, one in the suprasellar region and the other in the retropineal region. With angiographical and endocrinological examinations, coexistence of a pituitary adenoma and a falcotentorial junction meningioma was diagnosed. Two operations were performed and the preoperative diagnosis was confirmed histologically. Regarding the simultaneous occurrence of pituitary adenomas and meningiomas, only 7 cases were reported previously. A falcotentorial junction meningioma itself is also rare. Only 21 such cases were found in the literature. The clinical manifestation and the management of falcotentorial junction meningiomas are discussed.