Hurler syndrome: a case report

Thomas, Susan; Tandon, Sidharth

doi:10.17796/jcpd.24.4.ku653u75nv5vt735

Cited by 19 publications

(14 citation statements)

References 2 publications

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“…This spacing has been attributed to both a relatively posterior position of the molars and to distoangular tipping of these teeth [10]; it is of note that these features are absent in this case. In Hurler's syndrome the teeth have often been described as peg‐shaped and poorly formed [11] and may be hypoplastic [7]. In this case the incisors were slightly conical and the permanent canine teeth extremely pointed.…”

Section: Discussionmentioning

confidence: 89%

“…The dental findings of the syndrome include spaced, hypoplastic, peg‐shaped teeth with retarded eruption, flattened alveolar ridges with hyperplastic gingivae, macroglossia, and a high‐arched palate. The mandible is short and broad, with abnormal condyles; localized dentigerous cyst‐like radiolucencies are also often present [5–11].…”

Section: Introductionmentioning

confidence: 99%

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Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy

Hingston¹,

Hunter²,

Hunter³

et al. 2006

Int J Paed Dentistry

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Hurler's syndrome, also known as mucopolysaccharidosis I (MPS I-H), is a rare condition inherited as an autosomal recessive trait. It is caused by a deficiency in alpha-L-iduronidase, an enzyme that participates in the degradation of the glycosaminoglycans (GAGs) heparin sulphate and dermatan sulphate. Children with Hurler's syndrome appear nearly normal at birth but, left untreated, show a progressive mental and physical deterioration caused by a build-up of GAGs in all organs of the body. Death is often caused by cardiac or respiratory failure and usually occurs before the second decade of life. In recent years, bone marrow transplantation (BMT) has been employed in the management of patients with Hurler's syndrome. However, the dental findings observed in these cases have not previously been reported in the dental literature. Here we report a patient aged 11 years and 6 months, presented to a Specialist Paediatric Dentistry Unit, who was successfully treated by BMT at 18 months of age.

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Section: Discussionmentioning

confidence: 89%

Section: Introductionmentioning

confidence: 99%

Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy

Hingston¹,

Hunter²,

Hunter³

et al. 2006

Int J Paed Dentistry

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“…Hurler syndrome, also known as mucopolysaccharidosis 1 (MPHS 1‐H), is a rare yet severe form of a group of syndromes characterized by inadequate metabolism of mucopolysaccharides 1 . Inherited as an autosomal recessive trait caused by mutations in the alpha‐L‐iduronidase gene, the frequency of this disorder is low and occurs in approximately 1 in 100,000 live births 2 .…”

Section: Introductionmentioning

confidence: 99%

Hurler syndrome: a case report of a 5-year follow-up of dental findings after bone marrow transplantation

Wadenya

Stout²,

Gupta³

et al. 2010

Special Care in Dentistry

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Hurler syndrome is a rare autosomal recessive disorder of mucopolysaccharide metabolism. It results from a deficiency in lysosomal enzymes responsible for the breakdown of glycosaminoglycans. Affected individuals may show progressive physical and mental deterioration as glycosaminoglycans are deposited in the organs of the body. Bone marrow transplantation (BMT) is effective in improving some of the clinical manifestations of Hurler syndrome. Death is caused by cardiorespiratory failure and usually occurs before the second decade of life. In this case report, the course of dental development was followed over 5 years, from the primary dentition into the permanent dentition, of a child who was successfully treated with a bone marrow transplant in infancy. The timing of bone marrow therapy has significant and variable effect on the stages of tooth development with implications for the long-term maintenance of the dentition.

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“…Radiological finding of hypoplastic condyles[6] appears to be the cause of partial trismus in this case and may pose difficulties in dental treatments and delivery of general anesthesia. Wide mandibular and maxillary arches with interdental spacing results from the pressure of the tongue with macroglossia due to deposition of GAGs within tongue[378] Dentigerous cyst-like lesions observed in current case resulting from progressive destruction of the bone are common in other cases as well and generally affect the mandible bilaterally. [36–8] The remaining root stumps of 85 75 were extracted and oral hygiene instructions were given.…”

Section: Discussionmentioning

confidence: 99%

Clinical manifestation of Hurler syndrome in a 7 year old child

Sharma

Sabharwal²,

Datta

et al. 2012

Contemp Clin Dent

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Mucopolysaccharidosis type I (MPS I H, Hurler syndrome) is a rare autosomal recessive inborn deficiency in the metabolism of glycosaminoglycans (GAGs) heparan sulfate and dermatan sulfate, resulting from deficiency of Alpha-L-iduronidase enzyme. This condition is characterized by accumulation of incompletely degraded glycosaminoglycans into various organs of body, which leads to impairment of organs and body functions. Such children appear nearly normal at birth; however, if left untreated, show a progressive mental and physical deterioration leading to death due to cardiorespiratory failure before the second decade of life. Pedodontists have a role for early diagnosis, rendering corrective and preventive treatment to the developing dentition, and referring the patient to the concerned specialities. An interesting case of a seven year old boy with a combination of skeletal, neurological, ophthalmologic, oro-dental and radiological findings of this diverse and devastating clinical entity with MPS I-(Hurler syndrome) has been presented here in this case report.

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Hurler syndrome: a case report

Cited by 19 publications

References 2 publications

Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy

Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy

Hurler syndrome: a case report of a 5-year follow-up of dental findings after bone marrow transplantation

Clinical manifestation of Hurler syndrome in a 7 year old child

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