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    958 results match your criteria Hypopituitarism Panhypopituitarism

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    [Preoperative and postoperative endocrine disorders associated with pituitary stalk injuries caused by suprasellar growing tumors].
    Zh Vopr Neirokhir Im N N Burdenko 2018 ;82(1):13-21
    Burdenko Neurosurgery Institute, 4-ya Tverskaya-Yamskaya Str., 16, Moscow, Russia, 125047.
    The pituitary stalk (PS) is a relatively thin bundle connecting the hypophyseal stalk to the pituitary gland; it consists of both axons of the hypothalamic nuclei (terminating in the neurohypophysis) and the system of portal vessels. Compression of the PS by a space-occupying lesion or its transection (forced or intended) during surgery may lead to the development of endocrine disorders: hypopituitarism, diabetes insipidus, and hyperprolactinemia. The modern literature lacks studies evaluating the severity of endocrine disorders depending on the PS condition before and after surgery. Read More

    Hemorrhagic fever with renal syndrome accompanied by panhypopituitarism and central diabetes insipidus: a case report.
    J Neurovirol 2018 Mar 5. Epub 2018 Mar 5.
    Premedical Science, College of Medicine, Chosun University, Gwangju, Republic of Korea.
    Central diabetes insipidus (DI) was detected in a patient with hemorrhagic fever with renal syndrome (HFRS) who had been molecularly and serologically diagnosed with Hantaan virus infection. We recommend that clinicians differentiate central DI in HFRS patients with a persistent diuretic phase even when pituitary MRI findings are normal. Read More

    [Genetic factors in hypopituitarism. The role of transcription factors in pituitary hormone deficiency].
    Orv Hetil 2018 Feb;159(7):278-284
    I. Gyermekgyógyászati Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
    Developmental disorders affecting the hypothalamic-pituitary system can result in pituitary hormone deficiency showing a diverse clinical presentation. A significant majority of these disorders are closely linked to defects in transcription factor genes which play a major role in pituitary development. Those affecting the early phase of organogenesis typically lead to complex conditions affecting the pituitary as well as structures in the central nervous system. Read More

    Extended Endoscopic Endonasal Approach for Suprasellar Craniopharyngioma.
    J Neurol Surg B Skull Base 2018 Feb 18;79(2):S196-S198. Epub 2018 Jan 18.
    Department of Otorhinolaryngology, University of Insubria - Ospedale di Circolo Fondazione Macchi, Varese, Italy.
     We illustrate a suprasellar craniopharyngiomas treated with an extended endoscopic endonasal approach (EEEA).  Case report of a 43-year-old male affected by cerebral lesion located in suprasellar region involving the third ventricle and compressing the neurovascular structures, causing an anterosuperior dislocation of the chiasma. There is a complete disruption of the pituitary stalk that can explain the clinical finding of partial anterior hypopituitarism and hyperprolactinemia. Read More

    [Hypopituitarism mode in patients with craniopharyngioma in relation to tumor growth pattern].
    Zhonghua Yi Xue Za Zhi 2018 Jan;98(1):19-24
    Department of Neurosurgery, Nanfang Hospital of Southern Medical University, Guangzhou 510515, China.
    To investigate the pituitary hormone changes of patients with craniopharyngioma of different growth patterns during perioperative period and follow up time. Retrospective studies were performed on 212 cases of primary craniopharyngioma patient who received total tumor excision surgery in our hospital from January 2001 to May 2012. The characteristics of pituitary hormone and associated clinical manifestation during preoperative, perioperative and postoperative periods were analyzed according to the QST surgical classification. Read More

    Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report.
    Arch Argent Pediatr 2018 Feb;116(1):e130-e134
    Tepecik Training and Research Hospital, Department of Neonatology, Izmir,Turkey.
    Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Read More

    Congenital Hyperinsulinism and Hypopituitarism Attributable to a Mutation in FOXA2.
    J Clin Endocrinol Metab 2018 Mar;103(3):1042-1047
    Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
    Context: Persistent hypoglycemia in the newborn period most commonly occurs as a result of hyperinsulinism. The phenotype of hypoketotic hypoglycemia can also result from pituitary hormone deficiencies, including growth hormone and adrenocorticotropic hormone deficiency. Forkhead box A2 (Foxa2) is a transcription factor shown in mouse models to influence insulin secretion by pancreatic β cells. Read More

    Sheehan's Syndrome-The Most Common Cause of Panhypopituitarism at Moderate Altitude: A Sub-Himalayan Study.
    J Assoc Physicians India 2017 Dec;65(12):20-23
    Junior Resident, Deptt. of Medicine, IGMC, Shimla.
    Background: Panhypopituitarism is a rare disorder with varied clinical presentation having various etiologies. Sheehan's syndrome (SS) is decreasing in frequency worldwide and is a rare cause of panhypopituitarism in developed nations.

    Methodology: A retrospective study done between May 2011 and May 2015 in tertiary care hospital. Read More

    The Missing Link: A Case of Absent Pituitary Infundibulum and Ectopic Neurohypophysis in a Pediatric Patient with Heterotaxy Syndrome.
    J Radiol Case Rep 2017 Sep 30;11(9):28-34. Epub 2017 Sep 30.
    Department of Radiology, Harlem Hospital Center, New York, USA.
    We report a case of absent pituitary infundibulum and ectopic neurohypophysis in a 4-year-old patient presenting clinically with hypopituitarism as well as heterotaxy syndrome complicated by global developmental delay and growth retardation. The clinical and laboratory workup of our patient suggested underlying hypopituitarism related to either congenital or acquired pathology, necessitating MRI to distinguish between them. We explain the various structural causes of hypopituitarism and detail how to predict the MRI findings and treatment, based on a fundamental understanding of the anatomy and pathophysiology of the hypothalamic pituitary axis and distinguishing anterior versus posterior pituitary hormone derangements. Read More

    Identification of SLC20A1 and SLC15A4 among other genes as potential risk factors for combined pituitary hormone deficiency.
    Genet Med 2017 Oct 26. Epub 2017 Oct 26.
    Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany.
    PurposeCombined pituitary hormone deficiency (CPHD) is characterized by a malformed or underdeveloped pituitary gland resulting in an impaired pituitary hormone secretion. Several transcription factors have been described in its etiology, but defects in known genes account for only a small proportion of cases.MethodsTo identify novel genetic causes for congenital hypopituitarism, we performed exome-sequencing studies on 10 patients with CPHD and their unaffected parents. Read More

    Dual-release hydrocortisone treatment: glycometabolic profile and health-related quality of life.
    Endocr Connect 2018 Jan 12;7(1):211-219. Epub 2017 Dec 12.
    Department of Clinical and Experimental MedicineUniversity of Catania, Catania, Italy
    Objective: Adrenal insufficiency (AI) is a chronic condition associated with increased mortality and morbidity. The treatment of AI in the last years has been object of important changes due to the development of a dual-release preparation of hydrocortisone. It differs from previous therapeutic strategy as it contemplates a once-daily tablet that allows more closely mimicking the physiological circadian cortisol rhythm. Read More

    Therapy-Induced Growth and Sexual Maturation in a Developmentally Infantile Adult Patient with a PROP1 Mutation.
    Front Endocrinol (Lausanne) 2017 13;8:309. Epub 2017 Nov 13.
    Faculty of Science, Department of Anthropology and Human Genetics, Charles University, Prague, Czechia.
    Background: Hypopituitarism as a result of PROP1 (prophet of PIT1) mutation represents the most common genetic cause of combined deficiency of pituitary hormones and due to growth retardation it is typically diagnosed in childhood.

    Case Description: We present a unique case report of a prepubertal woman with growth retardation in whom combined pituitary hormone deficiency [central hypopituitarism, hypogonadism, and growth hormone (GH) deficiency] caused by homozygous mutation c.150delA in the PROP1 gene was diagnosed late in young adulthood due to unfavorable life circumstances. Read More

    A complex phenotype in a family with a pathogenic SOX3 missense variant.
    Eur J Med Genet 2018 Mar 24;61(3):168-172. Epub 2017 Nov 24.
    Department of Clinical Genetics, University Hospital of Copenhagen, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark.
    Duplications and deletions of Xq26-27 including SOX3 (Xq27.1) have been associated with X-linked mental retardation and isolated growth hormone deficiency (OMIM 300123) or X-linked panhypopituitarism (OMIM 312000). Yet, pathogenic point mutations seem to be extremely rare. Read More

    Clinical characteristics of septo-optic dysplasia accompanied by congenital central hypothyroidism in Japan.
    Clin Pediatr Endocrinol 2017 28;26(4):207-213. Epub 2017 Sep 28.
    Department of Pediatrics, Jichi Children's Medical Center Tochigi, Tochigi, Japan.
    Septo-optic dysplasia (SOD) is a congenital anomaly in which agenesis of the septum pellucidum and optic nerve hypoplasia are accompanied by hypopituitarism. Typically, the symptoms develop in 3 organs, the brain, eyes, and pituitary, and approximately one third of the patients present with all of the three cardinal features. The diagnostic criteria for SOD were established in Japan in 2015. Read More

    A rare cause of respiratory distress and edema in neonate: Panhypopituitarism.
    North Clin Istanb 2017 26;4(2):188-191. Epub 2017 Aug 26.
    Department of Neonatology, Erdem Hospital, Istanbul, Turkey.
    Clinical presentation of hypopituitarism may be variable in the neonate. Symptoms are generally nonspecific, ranging from absent to severe, and even life-threatening, due to adrenocorticotrophic hormone deficiency. Presently described is a case of unexplained respiratory distress and edema in a neonate. Read More

    Surgical intervention for pituitary apoplexy: an analysis of functional outcomes.
    J Neurosurg 2017 Sep 15:1-8. Epub 2017 Sep 15.
    California Center for Pituitary Disorders, Department of Neurological Surgery, University of California, San Francisco, California.
    OBJECTIVE Pituitary apoplexy is a clinical syndrome consisting of neurological and endocrine abnormalities secondary to hemorrhage or ischemia of an underlying pituitary adenoma. The authors investigated whether there was a significant difference in neurological, endocrine, and nonneuroendocrine outcomes for patients with pituitary apoplexy, based on the time between symptom onset and surgical intervention. METHODS The authors retrospectively analyzed the medical records of 32 patients who had presented to their institution with acute pituitary apoplexy and subsequently undergone endonasal transsphenoidal resection in the period from 2003 to 2014. Read More

    Hypophysitis, Panhypopituitarism, and Hypothalamitis in a Scottish Terrier Dog.
    J Vet Intern Med 2017 Sep 26;31(5):1527-1532. Epub 2017 Jul 26.
    Pathology Department, Veterinary Medicine and Science, University of Nottingham, Nottingham, UK.
    A 6-year old male neutered Scottish Terrier was referred with a 1 week history of progressive lethargy and anorexia. Neurological examination localized a lesion to the forebrain and hormonal testing showed panhypopituitarism. Magnetic resonance imaging (MRI) of the brain revealed a rounded, well-defined, suprasellar central mass. Read More

    Long-Standing Isolated Autoimmune Hypothalamitis Diagnosed with Endoscopic Transventricular Biopsy.
    World Neurosurg 2017 Sep 16;105:1036.e5-1036.e9. Epub 2017 Jun 16.
    Unit of Neurosurgery, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
    Background: Autoimmune hypothalamitis, which is among the causes of acquired central diabetes insipidus, has seldom been described in the literature. This condition is probably provoked by the production of anti-vasopressin-secreting cell antibodies and antihypothalamus antibodies and is often associated with pituitary or polyendocrine autoimmunity. Correct diagnosis and immediate treatment are essential to avoid the progression of the pathologic process. Read More

    A case of acute Sheehan's syndrome and literature review: a rare but life-threatening complication of postpartum hemorrhage.
    BMC Pregnancy Childbirth 2017 Jun 14;17(1):188. Epub 2017 Jun 14.
    Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.
    Background: Sheehan's syndrome occurs because of severe postpartum hemorrhage causing ischemic pituitary necrosis. Sheehan's syndrome is a well-known condition that is generally diagnosed several years postpartum. However, acute Sheehan's syndrome is rare, and clinicians have little exposure to it. Read More

    Opioid-induced hyponatremia in a patient with central diabetes insipidus: independence from ADH.
    J Pediatr Endocrinol Metab 2017 May;30(6):693-696
    .
    Hyponatremia can be a complication of opioid therapy, which has been postulated to occur secondary to inappropriate antidiuretic hormone secretion (syndrome of inappropriate antidiuretic hormone secretion [SIADH]). We report severe hyponatremia following wisdom teeth extraction with opioid analgesia in a 19-year-old female with diabetes insipidus (DI) and acquired panhypopituitarism that challenges this theory. As this patient has DI, we believe opioid treatment caused severe hyponatremia by the following mechanisms: (1) Opioids have a direct antidiuretic effect independent of changes in ADH, as demonstrated in Brattleboro rats with central DI. Read More

    A case of stiff-person syndrome due to secondary adrenal insufficiency.
    Rinsho Shinkeigaku 2017 06 26;57(6):298-302. Epub 2017 May 26.
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
    We report a case of flexion contractures in a patient's legs secondary to postpartum hypopituitarism. A 56-year-old woman presented with a 3-year history of worsening flexion contractures of the hips and knees. On admission, her hips and knees could not be extended, and she had muscle stiffness and tenderness to palpation of the lower extremities. Read More

    Lymphocytic Hypophysitis Mimicking Pituitary Macroadenoma.
    J Assoc Physicians India 2017 Apr;65(4):85-86
    Residents, RNT Medical College, Udaipur, Rajasthan.
    Lymphocytic hypophysitis is an inflammatory /autoimmune disorder that primarily involves the pituitary gland and the pituitary stalk. The common clinical presentations include headache, nausea, vomiting, fatigue, features of hypopituitarism and diabetes insipidus as well as diplopia, orbital pain and bitemporal hemianopia. We report a case of lymphocytic hypophysitis which presented as hemichorea. Read More

    Combined pituitary hormone deficiency in a girl with 48, XXXX and Rathke's cleft cyst.
    Hormones (Athens) 2017 Jan;16(1):92-98
    Section on Endocrinology and Genetics, NICHD, National Institutes of Health, Pediatric endocrinology training program, NICHD, National Institutes of Health, Bethesda, MD, USA.
    Background: Tetrasomy X is a rare chromosomal aneuploidy seen in girls, associated with facial dysmorphism, premature ovarian insufficiency and intellectual disability. A Rathke's cleft cyst (RCC) is a remnant of Rathke's pouch which may cause multiple pituitary hormone deficiencies by exerting pressure on the pituitary gland in the sella.

    Methods/results: The patient was diagnosed with tetrasomy X by karyotyping during infancy. Read More

    Pituitary Hypoplasia.
    Endocrinol Metab Clin North Am 2017 Jun 22;46(2):247-257. Epub 2017 Feb 22.
    Department of Pediatrics, Child Health Institute of New Jersey, Rutgers-Robert Wood Johnson Medical School, Rutgers, The State University of New Jersey, 89 French Street, Room 4212, New Brunswick, NJ 08901, USA.
    This article summarizes pituitary development and function as well as specific mutations of genes encoding the following transcription factors: HESX1, LHX3, LHX4, POU1F1, PROP1, and OTX2. Although several additional genetic defects related to hypopituitarism have been identified, this article focuses on these selected factors, as they have been well described in the literature in terms of clinical characterization of affected patients and molecular mechanisms of action, and therefore, are very relevant to clinical practice. Read More

    gene mutations in a 36-year-old female presenting with psychosis.
    Endocrinol Diabetes Metab Case Rep 2017 23;2017. Epub 2017 Mar 23.
    RCSIBusaiteenBahrain.
    Summary: Combined pituitary hormonal deficiency (CPHD) is a rare disease that results from mutations in genes coding for transcription factors that regulate the differentiation of pituitary cells. gene mutations are one of the etiological diagnoses of congenital panhypopituitarism, however symptoms vary depending on phenotypic expression. We present a case of psychosis in a 36-year-old female with congenital panhypopituitarism who presented with paranoia, flat affect and ideas of reference without a delirious mental state, which resolved with hormone replacement and antipsychotics. Read More

    Cholestasis caused by panhypopituitarism and acquired cytomegalovirus infection in a 2-month-old male infant: A case report.
    Medicine (Baltimore) 2017 Apr;96(17):e6757
    aDivision of Gastroenterology and Nutrition, Department of Pediatrics, MacKay Children's Hospital, Taipei, Taiwan bMacKay Junior College of Medicine, Nursing and Management, Taipei, Taiwan cDivision of Endocrinology and Metabolism, Department of Pediatrics, MacKay Children's Hospital, Taipei, Taiwan dDivision of Neurology, Department of Pediatrics, MacKay Children's Hospital, Taipei, Taiwan eDivision of Hematology-Oncology, Department of Pediatrics, MacKay Children's Hospital, Taipei, Taiwan fMacKay Medical College, Taipei, Taiwan.
    Rationale: Septo-optic dysplasia (SOD) is a rare congenital disorder that may cause jaundice in infants. However, it is usually prone to neglect and misdiagnosis in infants with cholestasis because endocrine disorder such as panhypopituitarism is rare in the cause of infantile cholestasis. We report a case of SOD concurrent with acquired cytomegalovirus (CMV) infection, who presented with prolonged jaundice as the first clinical sign. Read More

    Pulsatile GnRH Therapy May Restore Hypothalamus-Pituitary-Testis Axis Function in Patients With Congenital Combined Pituitary Hormone Deficiency: A Prospective, Self-Controlled Trial.
    J Clin Endocrinol Metab 2017 Jul;102(7):2291-2300
    Department of Endocrinology, Peking Union Medical College Hospital, Key Laboratory of Endocrinology, Ministry of Health, Beijing 100730, China.
    Context: The effectiveness of pulsatile gonadotropin-releasing hormone (GnRH) therapy in patients with congenital combined pituitary hormone deficiency (CCPHD) has not been investigated because of the limited number of patients, as well as these patients' presumed pituitary hypoplasia, poor gonadotrophic cell reserve, and impaired gonadotrophic response to GnRH.

    Objective: To assess the pituitary response to pulsatile GnRH therapy in men with CCPHD.

    Design: Prospective, self-controlled, 3-month clinical trial. Read More

    Rare Frequency of Mutations in Pituitary Transcription Factor Genes in Combined Pituitary Hormone or Isolated Growth Hormone Deficiencies in Korea.
    Yonsei Med J 2017 May;58(3):527-532
    Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
    Purpose: Congenital hypopituitarism is caused by mutations in pituitary transcription factors involved in the development of the hypothalamic-pituitary axis. Mutation frequencies of genes involved in congenital hypopituitarism are extremely low and vary substantially between ethnicities. This study was undertaken to compare the clinical, endocrinological, and radiological features of patients with an isolated growth hormone deficiency (IGHD) or combined pituitary hormone deficiency (CPHD). Read More

    Very long-term sequelae of craniopharyngioma.
    Eur J Endocrinol 2017 Jun 21;176(6):755-767. Epub 2017 Mar 21.
    Section EndocrinologyDepartment of Medicine, Pituitary Centre Rotterdam, Erasmus University Medical Centre, Rotterdam, the Netherlands.
    Objective: Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation. Read More

    Two novel LHX3 mutations in patients with combined pituitary hormone deficiency including cervical rigidity and sensorineural hearing loss.
    BMC Endocr Disord 2017 Mar 16;17(1):17. Epub 2017 Mar 16.
    Department of Genetics, King Faisal Specialist Hospital and Research Centre, P.O.Box 3354, Riyadh, 11211, Saudi Arabia.
    Background: Congenital combined pituitary hormone deficiency (CPHD) is a rare heterogeneous group of conditions. CPHD-type 3 (CPHD3; MIM# 221750) is caused by recessive mutations in LHX3, a LIM-homeodomain transcription factor gene. The isoforms of LHX3 are critical for pituitary gland formation and specification of the anterior pituitary hormone-secreting cell types. Read More

    Advances in understanding hypopituitarism.
    F1000Res 2017 22;6:178. Epub 2017 Feb 22.
    Max Planck Institute of Psychiatry, Clinical Neuroendocrinology, Kraepelinstr. 2-10, D-80804 Munich, Germany.
    The understanding of hypopituitarism has increased over the last three years. This review provides an overview of the most important recent findings. Most of the recent research in hypopituitarism has focused on genetics. Read More

    Rapid exacerbation of lymphocytic infundibuloneurohypophysitis.
    Medicine (Baltimore) 2017 Mar;96(9):e6034
    aDepartment of Diabetes, Endocrinology and Nutrition, Graduate School of Medicine, Kyoto University, Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, Japan bDepartment of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan. cDepartment of Neurosurgery, Graduate School of Medicine, Kyoto University, Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, Japan.
    Rationale: Lymphocytic hypophysitis is a relatively rare autoimmune disease defined by lymphocytic infiltration to the pituitary. Its rarity and wide spectrum of clinical manifestations make clarification of the pathology difficult. Here, we describe a case we examined from the primary diagnosis to final discharge, showing the serial progression of lymphocytic infundibuloneurohypophysitis (LINH) to panhypopituitarism with extrapituitary inflammatory invasion in a short period, and responding favorably to high-dose glucocorticoid treatment. Read More

    Craniopharyngioma presenting with severe hyponatremia, hyponatremia-induced myopathy, and panhypopituitarism: a case report.
    J Med Case Rep 2017 Feb 5;11(1):31. Epub 2017 Feb 5.
    University Medical Unit, National Hospital of Sri Lanka, Colombo 10, Sri Lanka.
    Background: Craniopharyngiomas are rare intracranial tumors commonly presenting with neurological symptoms. Reports of severe hyponatremia as a presenting manifestation of a craniopharyngioma and hyponatremia-induced myopathy are rare. We report the case of a patient with craniopharyngioma presenting with severe hyponatremia, panhypopituitarism, and hyponatremia-induced myopathy. Read More

    Risk of cumulative toxicity after complete melanoma response with pembrolizumab.
    BMJ Case Rep 2017 Feb 1;2017. Epub 2017 Feb 1.
    Department of Medical Oncology, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
    Pembrolizumab is an approved first-line systemic therapy for unresectable metastatic melanoma. Despite the achievement of complete and durable responses in a small subgroup of patients, it is standard practice that pembrolizumab therapy continues beyond complete response. Nevertheless, the incidence of immune-related toxicities gradually increases with continuing pembrolizumab therapy. Read More

    Mikulicz's Disease with hypophysitis - a new IgG4-mediated disorder.
    Endokrynol Pol 2016 ;67(6):622-626
    Department of Endocrinology, B.Y.L Nair Charitable Hospital & Topiwala National Medical College, Mumbai-400008, India.
    Introduction: We present a case of Mikulicz's Disease with hypophysitis. This is a rare clinical association as part of the group of IgG4- related diseases, a group of disorders which can have multiorgan involvement.

    Methods: A 55-year-old male patient was diagnosed with Mikulicz's disease. Read More

    Infundibulo-hypophysitis-like radiological image in a patient with pituitary infiltration of a diffuse large B-cell non-Hodgkin lymphoma.
    Endocrinol Diabetes Metab Case Rep 2016 17;2016. Epub 2016 Dec 17.
    Department of Endocrinology and Metabolism , Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan , Mexico.
    Non-Hodgkin lymphoma (NHL) is a hematological tumor caused by abnormal lymphoid proliferation. NHL can arise in any part of the body, including central nervous system (CNS). However, pituitary involvement is a quite rare presentation. Read More

    Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients.
    Case Rep Pediatr 2016 28;2016:4328492. Epub 2016 Nov 28.
    Departments of Medical Genetics and Pediatrics, University of Calgary, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada.
    Hypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Read More

    Classical and non-classical causes of GH deficiency in the paediatric age.
    Best Pract Res Clin Endocrinol Metab 2016 Dec 24;30(6):705-736. Epub 2016 Nov 24.
    Department of Pediatrics, Istituto Giannina Gaslini, University of Genova, Genova, Italy; Department of Endocrine Unit, Istituto Giannina Gaslini, University of Genova, Genova, Italy. Electronic address:
    Growth hormone deficiency (GHD) may result from a failure of hypothalamic GHRH production or release, from congenital disorders of pituitary development, or from central nervous system insults including tumors, surgery, trauma, radiation or infiltration from inflammatory diseases. Idiopathic, isolated GHD is the most common sporadic form of hypopituitarism. GHD may also occur in combination with other pituitary hormone deficiencies, and is often referred to as hypopituitarism, combined pituitary hormone deficiency (CPHD), multiple pituitary hormone deficiency (MPHD) or panhypopituitarism. Read More

    Genetic causes of isolated and combined pituitary hormone deficiency.
    Best Pract Res Clin Endocrinol Metab 2016 Dec 15;30(6):679-691. Epub 2016 Oct 15.
    Department of Health Sciences, Laboratory of Human Genetics, University of Eastern Piedmont, Novara, Italy. Electronic address:
    Research over the last 20 years has led to the elucidation of the genetic aetiologies of Isolated Growth Hormone Deficiency (IGHD) and Combined Pituitary Hormone Deficiency (CPHD). The pituitary plays a central role in growth regulation, coordinating the multitude of central and peripheral signals to maintain the body's internal balance. Naturally occurring mutation in humans and in mice have demonstrated a role for several factors in the aetiology of IGHD/CPHD. Read More

    A novel heterozygous intronic mutation in POU1F1 is associated with combined pituitary hormone deficiency.
    Endocr J 2017 Feb 22;64(2):229-234. Epub 2016 Nov 22.
    Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
    POU class 1 homeobox 1 (POU1F1) regulates pituitary cell-specific gene expression of somatotropes, lactotropes, and thyrotropes. In humans, two POU1F1 isoforms (long and short isoform), which are generated by the alternative use of the splice acceptor site for exon 2, have been identified. To date, more than 30 POU1F1 mutations in patients with combined pituitary hormone deficiency (CPHD) have been described. Read More

    Administration of anti-receptor activator of nuclear factor-kappa B ligand (RANKL) antibody for the treatment of osteoporosis was associated with amelioration of hepatitis in a female patient with growth hormone deficiency: a case report.
    BMC Endocr Disord 2016 Nov 24;16(1):66. Epub 2016 Nov 24.
    Internal Medicine 1, Shimane University Faculty of Medicine, 89-1, Enya-cho, Izumo, Shimane, 693-8501, Japan.
    Background: Growth hormone deficiency (GHD) is associated with non-alcoholic fatty liver disease (NAFLD). A recent animal study showed that hepatocyte-specific receptor activator of nuclear factor-kappa B (RANK) knockout mice had significantly lower liver fat content compared with control mice concomitant with a decrease in production of inflammatory cytokines such as tumor necrosis factor-α (TNF-α) from hepatocytes and kupffer cells. The role of anti-RANK ligand (RANKL) antibody for osteoporosis on hepatitis in patients with aGHD is still unknown. Read More

    IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis.
    Brain Pathol 2017 11 11;27(6):839-845. Epub 2017 Jan 11.
    Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
    IgG4-related disease is an immune-mediated disease with manifestations in most organ systems among them the pituitary gland. To date, few cases of histologically confirmed cases of IgG-related hypophysitis have been reported. The aim of this study was to retrospectively determine the prevalence of IgG4-related hypophysitis among cases previously diagnosed as primary hypophysitis (lymphocytic hypophysitis, granulomatous hypophysitis and hypophysitis not otherwise specified). Read More

    Cholesterol granulomas presenting as sellar masses: a similar, but clinically distinct entity from craniopharyngioma and Rathke's cleft cyst.
    Pituitary 2017 Jun;20(3):325-332
    Department of Neurosurgery, Thomas Jefferson University, 909 Walnut Street, 3rd Floor, Philadelphia, PA, 19107, USA.
    Purpose: Cholesterol granulomas in the sella are rare and can mimic the appearance of craniopharyngioma or Rathke's cleft cysts. Information regarding the clinical presentation, imaging characteristics, and clinical course of sellar cholesterol granulomas can help clinicians to differentiate these lesions from other sellar cystic lesions.

    Methods: We present three cases of sellar cholesterol granulomas. Read More

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