색소실조증은 외배엽 이형성증과 같이 다수의 치아결손을 동반하는 선천성 질환으로 Bloch-Sulzberger syndrome이라고 불리며 X 연관 우성 유전질환이므로 주로 여아에서 많이 발병한다. 외배엽 및 중배엽 기원 조직인 피부, 눈, 머리카락, 치아 및 중추신경계에 증상이 나타나며 Xq28 염색체의 NEMO 유전자의 돌연변이에 의해 발병된다. 본 증례는 유치의 선천적 결손을 주소로 내원한 3명의 환아의 증례를 다루고 있으며 이들은 다수의 유치와 영구치의 선천적 결손, 전치부 원뿔형 치관, 구치부 과잉 교두 등을 보이고 있었다. 치아결손부에 공간 유지 장치를 장착하여 저작기능을 회복해 주고 심미적인 문제를 개선해주었으며 계속적인 관찰이 필요하다.
색소실조증은 외배엽 이형성증과 같이 다수의 치아결손을 동반하는 선천성 질환으로 Bloch-Sulzberger syndrome이라고 불리며 X 연관 우성 유전질환이므로 주로 여아에서 많이 발병한다. 외배엽 및 중배엽 기원 조직인 피부, 눈, 머리카락, 치아 및 중추신경계에 증상이 나타나며 Xq28 염색체의 NEMO 유전자의 돌연변이에 의해 발병된다. 본 증례는 유치의 선천적 결손을 주소로 내원한 3명의 환아의 증례를 다루고 있으며 이들은 다수의 유치와 영구치의 선천적 결손, 전치부 원뿔형 치관, 구치부 과잉 교두 등을 보이고 있었다. 치아결손부에 공간 유지 장치를 장착하여 저작기능을 회복해 주고 심미적인 문제를 개선해주었으며 계속적인 관찰이 필요하다.
Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congen...
Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.
Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.
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문제 정의
This report described the dental treatment in three patients with IP based on clinical and radiological findings. We suggest that a valid treatment plan for patients with IP should be based on precise diagnosis by evaluating clinical and radiographic symptoms.
제안 방법
4). After fitting the removable space maintainer, the patient received regular check-ups every 6 months for fluoride application and oral hygiene instruction for prevention of caries.
8). After treatment using removable space maintainers, the patient received regular check-ups every 6 months for fluoride application and oral hygiene management. Prosthetic rehabilitation was planned upon completion of growth.
13). The patient received regular check-ups every 3 months.
Patients in this study showed multiple congenitally missing teeth in both primary and permanent dentition and conically shaped supernumerary cusps in the anterior and posterior areas. The patients in this study were treated by applying a removable space-maintaining device in the vacant areas to maintain proper spacing and restore masticatory function. The patients received regular postoperative check-ups and oral hygiene management.
3). Therefore, to resolve mastication and esthetic problems caused by multiple congenitally missing teeth, we applied removable space maintainer containing pontics of the missing primary molars (Fig. 4). After fitting the removable space maintainer, the patient received regular check-ups every 6 months for fluoride application and oral hygiene instruction for prevention of caries.
대상 데이터
A 4-year-old female was seen at the pediatric dentistry clinic due to anterior cross bite and congenitally missing teeth. The patient was diagnosed with IP at the pediatric dentistry of Kyungpook National University Hospital and extraoral examination revealed features such as thin hair, scant eyebrows and mandibular prognathism (Fig.
A 5-year-old female (the sister of Case 1) visited the pediatric dentistry clinic because of unerupted teeth. Similar to Case 1, this patient was diagnosed with IP and had sparse hair and negligible eyebrows, but had no signs of systemic illness (Fig.
IP is accompanied commonly by cutaneous and dental manifestations, such as congenitally missing teeth, tooth-shape deformities, and delayed eruption. Patients in this study showed multiple congenitally missing teeth in both primary and permanent dentition and conically shaped supernumerary cusps in the anterior and posterior areas. The patients in this study were treated by applying a removable space-maintaining device in the vacant areas to maintain proper spacing and restore masticatory function.
A panoramic radiograph confirmed the absence of multiple congenital permanent teeth. The patient had a total of 17 missing teeth: the lateral incisors and second molars in all four quadrants, the right maxillary first molar and canine, the left maxillary canine and first and second premolars, the left mandibular first molar and second premolar, and the right and left mandibular first premolars (Fig. 7).
A 4-year-old female was seen at the pediatric dentistry clinic due to anterior cross bite and congenitally missing teeth. The patient was diagnosed with IP at the pediatric dentistry of Kyungpook National University Hospital and extraoral examination revealed features such as thin hair, scant eyebrows and mandibular prognathism (Fig. 9).
In addition, patients with IP exhibit thin hair and nail dystrophy22). This study described three cases of IPNwith multiple congenitally missing or deformed teeth.
참고문헌 (25)
Carney RG : Incontinentia pigmenti : a world statistical analysis. Arch Dermatol, 112:535-542, 1976.
Phan TA, Wargon O, Turner AM : Incontinentia pigmenti case series : clinical spectrum of incontinentia pigmenti in 53 female patients and their relatives. Clin Exp Dermatol, 30:474-480, 2005.
Niccoli-Filho WD : Incontinentia pigmenti : a case report. J Clin Pediatr Dent, 17:251-253, 1993.
Domingues A, Aznar T, Cabrera E : General and dental characteristics of Bloch-Sulzberger syndrome : Reviews of literature and presentation of a case report. Med Oral, 7:293-297, 2002.
Russel DL, Fin SB : Incontinentia pigmenti : a case report with emphasis on dental mianifestations. J Dent Children, 23:206-208, 1956.
Garrod AE : Peculiar pigmentation of the skin of an infant. Trans Clin Soc Lond, 39:216, 1906.
Curth HO, Warbuton D : The genetics of incontinentia pigmenti. Arch Dermatol, 92:229-235, 1965.
Smahi A, Courtois G, Vabres P : Genomic rearrangement in NEMO impairs NF-KappaB activation and is a cause of incontinentia pigmenti. Nature, 405:446-472, 2000.
Berlin AL, Paller AS, Chan LS : Incontinentia pigmenti : a review and update on the molecular basis of pathophysiology. J Am Acd Dermatol, 47:169-187, 2002.
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