Complete Pseudo-Anodontia in an Adult Woman with Pseudo-Hypoparathyroidism Type 1a: A New Additional Nonclassical Feature? (original) (raw)
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Dental findings in the diagnosis of idiopathic hypoparathyroidism
Annals of Saudi Medicine, 2013
Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathy, characterized by the disturbances in the calcium and phosphorous metabolism, owing to deficiency in parathyroid hormone, which leads to tetanic manifestations. Onset of the clinical features occurs early in the life and the severity depends on the extent of chemical imbalance. This article describes a case of 22-year-old male patient undiagnosed for 12 years with this endocrinopathy (IHP). Overretained deciduous teeth, delayed eruption, impacted tooth and short roots probably resulting from untreated hypocalcemia during the developmental phase of dentition enabled us to unearth this endocrinopathy through a series of investigations. Thus, the article emphasizes the importance of dental findings of this endocrinopathy.
Impacted Teeth and Early Detection of Hypoparathyroidism: A Rare Case Report with Literature Review
2018
Hypoparathyroidism is a rare endocrinological disorder accompanied by anomalies of various systems, bones and teeth. If diagnosed within first 10 years after birth and devoid of etiology, the condition is termed as idiopathic hypoparathyroidism (IHP). The dental defects due to hypoparathyroidism may present as hypocalcaemia, aplasia and/or, hypoplasia of enamel, incomplete mineralization of dentin, short and blunted roots, blunted molar apices, delayed eruptions and missing or, impacted teeth. Retention of multiple primary teeth and impacted permanent teeth can, also, be associated with various other syndromes or, hormonal and metabolic disorders. Early detection of this disorder is vital for effective management not only paving way for prevention of further dental damage progression, but, also, in improving the medical management and related outcomes. This case report elaborates a rare case where multiple impacted permanent teeth and retained primary teeth accompanied by other clin...
Prosthodontic Management of Congenital Hypothyroidism with Anodontia: A Case Report
International Journal of Clinical Pediatric Dentistry, 2021
Thyroid dysfunction has various manifestations which include orofacial abnormalities like delayed eruption, retained deciduous teeth, etc. Early detection of this deregulation of thyroid homeostasis can prevent associated complications. This report is a case of congenital hypothyroidism (CH) in a 4-year-old boy who presented with a completely edentulous maxilla and hypodontic mandible. Based on various biochemical and radiographic investigations, a diagnosis of CH was established. He was prosthodontically rehabilitated with removable dentures.
Crescent Journal of Medical and Biological Sciences, 2018
A 20-year-old Iranian male after injury in a football match with pain in the fnger was referred to the hospital. X-Ray images of his hand showed no fractures, but the shortening of third to ffth metacarpal bones was clearly observed. The short metacarpal bone is a very helpful diagnostic marker in the patients suffering from idiopathic hypoparathyroidism (IHP), pseudohypoparathyroidism type Ia (PHP-Ia) with Albright"s hereditary osteodystrophy (AHO) phenotype, pseudopseudohypoparathyroidism (PPHP), and brachydactyly type E. Briefly, in hypoparathyroidism disease, the serum calcium level decreases and the serum phosphorus level increases in blood. PHP-Ia disorder is clinically similar to hypoparathyroidism; in this disease, the level of parathyroid hormone is normal but there is resistance to it. PPHP disease is not an endocrine disorder but is related to PHP-Ia. In this disease, serum levels of calcium and phosphorus are normal, also symptoms and signs in PPHP are similar to PH...
Enamel hypoplasia associated with congenital hypoparathyroidism
Pediatric dentistry, 1981
Enamel hypoplasia is a defect in the enamel due to disturbance of ameloblastic function during amelogenesis. The etiology of such a disturbance may be either genetic or environmental in nature. This case report of undetected congenital idiopathic hypoparathyroidism emphasizes the importance of investigating the underlying cause of these enamel defects.
Dental anomalies and orthodontic characteristics in patients with pseudohypoparathyroidism
BMC Oral Health
Background Pseudohypoparathyroidism (PHP) is a rare and inherited disease caused by mutations in the GNAS-gene or upstream of the GNAS complex locus. It is characterized by end-organ resistance to PTH, resulting in hypocalcemia and hyperphosphatemia. We aimed to investigate the dental anomalies according to tooth types and the orthodontic characteristics of patients with PHP. Methods Using a cross-sectional design, 29 patients (23 females) with PHP, living in Denmark, were included, and their clinical intraoral photos and radiographs were examined. Results Pulp calcification was found in 76% of the patients. Blunting of root apex was present in 55% and shortening of root in 48% of the examined patients. Blunting and shortening of roots were seen more often in premolars than in other tooth types (pboth
Symptomatic hypoparathyroidism based on a 22qII deletion first diagnosed in a 43-year-old woman
The Netherlands Journal of Medicine
Congenital hypoparathyroidism usually manifests in early childhood with hypocalcaemia with or without clinical characteristics. This report describes a Caucasian woman who, at the age of 43 years, was diagnosed with dysgenesis of the parathyroid glands due to a de novo microdeletion in chromosome 22q11 or diGeorge syndrome. This syndrome is characterised by a considerable variability in clinical symptoms, including heart defects, thymic hypoplasia and mental retardation. our patient presented with generalised convulsions due to extreme, symptomatic hypocalcaemia. The convulsions had been apparent for 18 months at the time of the diagnosis. remarkably, whereas parathyroid hormone levels were undetectable, the 1,25-dihydroxy vitamin d level was normal. Chromosome 22q11 deletion was confirmed by fluorescence in situ hybridisation analysis.
Congenital hypothyrodism and its oral manifestations
Revista Odontologica Mexicana, 2014
Hypothyroidism is one of the most common thyroid disorders. Hypothyroidism can be congenital in cases when the thyroid gland does not develop normally. Female predominance is a characteristic of congenital hypothyroidism. Dental characteristics of hypothyroidism are thick lips, a large-sized tongue which, due to its position, can elicit anterior open bite as well as fanned-out anterior teeth. In these cases, delayed eruption of primary and permanent dentitions can be observed, and teeth, even though normal-sized, are crowded due to the small-sized jaws. This study presents clinical cases of female patients diagnosed with congenital hypothyroidism who sought treatment at the Dental Pediatrics Unit of the Autonomous University of the State of Mexico. RESUMEN El hipotiroidismo es el más común de los trastornos de la tiroides, puede ser congénito si la glándula tiroides no se desarrolla correctamente (hipotiroidismo congénito). La predominancia femenina es una característica. Entre las características odontológicas del hipotiroidismo se observan labios gruesos, lengua de gran tamaño, que debido a su posición suele producir mordida abierta anterior y dientes anteriores en abanico, destaca que la dentición temporal y permanente presentan un retardo eruptivo característico y, aunque los dientes son de tamaño normal, suelen estar apiñados por el tamaño pequeño de los maxilares. Se presentan dos casos clínicos de pacientes de sexo femenino que acuden a la
Oral and systemic manifestations of congenital hypothyroidism in children. A case report
Journal Oral Of Research, 2015
Hypothyroidism is the most common thyroid disorder. It may be congenital if the thyroid gland does not develop properly. A female predominance is characteristic. Hypothyroidism is the most common congenital pediatric disease and its first signs and early symptoms can be detected with neonatal screening. Some of the oral manifestations of hypothyroidism are known to be: glossitis, micrognathia, macroglossia, macroquelia, anterior open bite, enamel hypoplasia, delayed tooth eruption, and crowding. This paper briefly describes the systemic and oral characteristics of congenital hypothyroidism in a patient being treated at a dental practice. The patient had early childhood caries and delayed tooth eruption. There are no cases of craniosynostosis related to the primary pathology, which if left untreated, increases the cranial defect. Early diagnosis reduces the clinical manifestations of the disease. Delayed tooth eruption will become a growing problem if the patient does not receive timely treatment and monitoring.