Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review.

Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review. Int J Endocrinol. 2019;2019:5382640 Authors: Li Y, Gao H, Li Z, Zhang X, Ding Y, Li F Abstract Background: IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes. Methods: We performed PubMed search of articles using the search terms "hypophysitis [AND] IgG4." Consequently, only 54 English articles (76 cases) met Leporati's diagnostic criteria. Results: Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in ...
Source: International Journal of Endocrinology - Category: Endocrinology Tags: Int J Endocrinol Source Type: research

Related Links:

AbstractHypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients ’ clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considere d the neuroradiological investigation of choice. The features suggestiv...
Source: La Radiologia Medica - Category: Radiology Source Type: research
CONCLUSION: Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients. LEVEL OF EVIDENCE: 4. PMID: 31761518 [PubMed - as supplied by publisher]
Source: Progres en Urologie - Category: Urology & Nephrology Tags: Prog Urol Source Type: research
Discussion Holoprosencephaly (HPE) is a clefting problem of the brain. “[HPE] the result of incomplete or absent midline division of the embryonic forebrain into distinct cerebral hemispheres (prosencephalon) between the 18th and 28th day after conception.” There are four distinct subtypes: Alobar – both hemispheres are completely fused and are not separated into the left and right hemispheres. There is agenesis of the corpus callosum, arrhinencephaly and a single ventricle with fused thalami. Facial features are almost always affected. Semilobar – the cerebral hemispheres are fused anteriorly bu...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Wolfram Syndrome (WFS) is a rare autosomal recessive neurodegenerative disease which has a wide spectrum of manifestations including diabetes insipidus, diabetes mellitus, optic atrophy and deafness. WFS1 and CIS...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Wolfram syndrome is a rare disorder associated with diabetes mellitus, diabetes insipidus, optic nerve atrophy, hearing and vision loss, and neurodegeneration. Sleep complaints are common but have not been stu...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Wolfram syndrome is a rare genetic, progressive, neurodegenerative disorder characterised by childhood-onset diabetes mellitus, diabetes insipidus, optic atrophy and deafness. To date, the economic burden of W...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Langerhans cell histiocytosis (LCH) is characterized by the congregation of proliferating langerhans cells (LC). Langerhans cells are a part of dendritic cell system of primary immune response that is responsible for presenting antigen to lymphocytes. Being a rare disease, the total incidence of LCH is reported to be 1 in 2 million people. LCH mainly affects children and young adults, with a slight male predilection. LCH is clinically divided into three groups namely Letter-Siwe disease (multiple multi organ affecting LCH at very young age), Hand-Schuler-Christian disease (LCH of bone involvement exophthalmos and diabetes ...
Source: Journal of Dentistry, Shiraz University of Medical Sciences - Category: Dentistry Source Type: research
Conclusions: Definitive histopathological diagnosis and subsequent appropriate therapy, such as steroid administration, are required when LCH lesions in the hypothalamus become progressively enlarged and new lesions appear in the brain parenchyma. PMID: 30450262 [PubMed]
Source: Surgical Neurology International - Category: Neurosurgery Tags: Surg Neurol Int Source Type: research
CONCLUSION: Persistent hypernatremia with excessive diuresis should alert to CDI diagnosis. PMID: 30361085 [PubMed - as supplied by publisher]
Source: Archives de Pediatrie - Category: Pediatrics Authors: Tags: Arch Pediatr Source Type: research
Wolfram syndrome (WFS) is a rare autosomal recessive disease with clinical manifestations of diabetes mellitus (DM), diabetes insipidus (DI), optic nerve atrophy (OA) and sensorineural hearing loss (SNHL). Alt...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
More News: Diabetes | Diabetes Insipidus | Endocrinology | Hormones | Pancreas | Rare Diseases | Study | Urology & Nephrology | Women