• Investigative Magnetic Resonance Imaging
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• v.3 no.1
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• pp.60-65
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• 1999

Purpose : The purpose of this study was to evaluate the image quality, contrast characteristics, and possible clinical utility of Medium Tau Inversion Recovery(MTIR) sequence with white matter suppression in patients with brain cortical lesion. Materials and methods : Two normal volunteers and twenty-one patients with cortical lesion were scanned with MTIR as well as other MR imaging sequences. Gray-white matter contrast was evaluated objectively using region-of-interest calculations, including percent contrast and contrast-to-noise ratio(CNR). MTIR sequence was visually compared with other sequences in 21 patients with cortical lesion including conspicuity and detection rate. Results : MTIR sequence had the highest present contrast and CNR between the gray matter and white matter. In twenty-one cases of cortical lesion including cortical dysplasia, MTIR sequence improved delineation and conspicuity of lesion, but MTIR sequence could not detect new lesions. Conclusion : The MTIR sequence well delineated the cortical lesions, particularly in including cortical dysplasia. It may be used as an adjunctive imaging sequence in case of poor gray and white matter differentiation with conventional T1-weighted sequences.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.397-399
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• 2003

All complete monosomy 21 appear to be lethal early in their development in humans and only survive in mosaic forms. Complete monosomy 21 is a very rare and usually debilitating genetic disorder. Partial monosomy 21 is also rare and is thought to constitute a clinical syndrome consisting of peculiar faces, hypertonia, psychomotor retardation, and slow growth. We experienced a case of monosomy 21 mosaicism. Chromosome analysis demonstrated mosaicism for cell lines in the lymphocytes examined; 45, XX, -21/46, XX. The main clinical features were craniofacial dysmorphism including high arched palate, submucosal cleft, micrognathia and arthrogryposis-like symptoms including flexion deformity of fingers. And hematological findings were revealed dyserythropoiesis, thrombocytopenia and eosinophilia. Currently, the patient has nearly compatible growth, but a mild degree of mental retardation. We report here an 8 years old female child with apparent monosomy 21 mosaicism associated with dyserythropoiesis, thrombocytopenia and eosinophilia, with a review of the associated literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.202-207
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• 2003

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.180-187
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• 2015

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.

• Journal of the Institute of Convergence Signal Processing
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• v.21 no.4
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• pp.183-194
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• 2020

Recently, the number of hip dysplasia (DDH) that occurs during infant and child growth has been increasing. DDH should be detected and treated as early as possible because it hinders infant growth and causes many other side effects In this study, two modelling techniques were used for multiple training techniques. Based on the results after the first transformation, the training was designed to be possible even with a small amount of data. The vertical flip, rotation, width and height shift functions were used to improve the efficiency of the model. Adam optimization was applied for parameter learning with the learning parameter initially set at 2.0 x 10e-4. Training was stopped when the validation loss was at the minimum. respectively A novel image overlay system using 3D laser scanner and a non-rigid registration method is implemented and its accuracy is evaluated. By using the proposed system, we successfully related the preoperative images with an open organ in the operating room

• Journal of the korean academy of Pediatric Dentistry
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• v.50 no.4
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• pp.483-494
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• 2023

This report presents two cases concerning strategies for improving denture retention in pediatric patients with oligodontia caused by hypohidrotic ectodermal dysplasia (HED). Both patients presented with multiple missing teeth, conical canines, alveolar bone atrophy, and a skeletal Class III tendency. In the first case, a modified form of conical-crown-retained denture was used to cover the canines. This approach was carried out without tooth extraction or coping. In the second case of severe alveolar bone resorption and a distally tilted lower left canine, the tooth was restored with a hybrid ceramic crown. It was subsequently converted into a clasp-retained removable partial denture, utilizing a suction mechanism. Both patients are currently receiving regular check-ups for the maintenance of their prosthetic appliances and the evaluation of their growth patterns. This study presents innovative prosthetic treatment methods for pediatric patients with HED who have inadequate denture retention.

• The Korean Journal of Nuclear Medicine
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• v.31 no.4
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• pp.452-458
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• 1997

To evaluate the pinhole scintigraphic findings and its significance, authors retrospectively compared the pinhole bone scintigrams and corresponding radiograms of 16 lesions in 14 patients with fibrous dysplasia. They were diagnosed pathologically in 10 lesions and radiologically in 6 lesions. The mean age of patients was 41.1 years. The mean interval between two studies was 1.1 days. Locations were ribs 7, pelvic bone 4, clavicle 1, long bones 4(femur 2, tibia 1, humerus 1). The radiographic findings were as follows : the central portions were radiolucent(n=9), ground-glass opacities(n=5) or sclerotic(n=2) and the peripheral appearance were sclerotic rim(n=5), septation(n=7), cortical perforation (n=10) and invisible cortical thinning(n=9). Pinhole scintigraphic findings were as follows; Central portions showed normal 1+ uptake in 6 cases(radiolucent 5, ground-glass opacity 1), slightly increased 2+ upta- ke in 7 cases(radiolucent 4, ground-glass opacity 3), and marked 3+ uptake in 3 cases(ground-glass opacity 1, sclerotic 2). The 15 of 16 lesions showed more intense uptake in the peripheral portion: slightly increased 2+ uptake corresponding to the sclerotic rim(5/5) and unvisible cortical thinning(1/9), and irregular foci of marked 3 + uptake corresponding to septation(7/7), cortical perforation(10/10) and invisible cortical thinning (8/9). One of 16 lesions showed homogeneous 2+ uptake. In conclusion, pinhole scintigram provides information on regional activity of the fibrous dysplasia, which would be helpful in diagnosis, prediction of prognosis and determination of treatment plan.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.74-79
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• 2014

Purpose: Fibrous dysplasia is related to the mutation of gene encoding the alpha-subunit of a signal-transducing G-protein and has variable clinical course. Operation can be performed to prevent functional disorder or structural deformity. After curettage, autologous bone graft were used to fill the defects after curettage. The aim of this study is to compare the result of autogenous cancellous bone grafting and allogenic bone grafting for fibrous dysplasia. Materials and Methods: Among the patients who visit our hospital during the period of April, 1997 to October, 2013, we selected 34 patients who diagnosed fibrous dysplasia and visited our clinic over 1 year. There were 13 males and 21 females. Average age was 26.4 (range 2 to 57) years old. Autogenous bone graft (group I) in 5 cases, Non-autogenous bone graft (group II) in 30 cases. Iliac bone is used in all cases of autogenous bone graft. There were no significant difference in age, follow-up period, preoperational laboratory finding between two groups. Radiographic image was done to evaluate the recurrence of fibrous dysplasia or secondary degeneration. Results: There were four cases in recurrence (group I: 1 case, group II: 3 cases, p=0.554). In all recurrent cases, reoperations were done using curettage and autogenous iliac bone graft. There was no re-recurrence after reoperation. One case of secondary aneurysmal bone cyst was confirmed (group II) and 1 cases of pathologic fractures had developed (group I: 0 case, group II: 1 cases, p=0.559). No malignant change occurred. Conclusion: There were no significant difference between autogenous bone graft group and non-autogenous bone graft group. Our result suggested that autogenous bone graft seems to be good method to treat fibrous dysplasia, in the case of small volume of tumor lesion or non-weight bearing portion.

• Journal of the Korean Orthopaedic Association
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• v.54 no.6
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• pp.519-527
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• 2019

Purpose: To evaluate the treatment result in polyostotic fibrous dysplasia classified according to the involvement of the femoral head. Materials and Methods: Twenty-three patients from March 1987 to March 2014 were reviewed retrospectively. Patients with no involvement of the physeal scar in the femoral head were classified as Type I, and those with involvement of the physeal scar were classified as Type II. A plain radiograph was used to measure the femoral neck shaft angle, articulo-trochanteric distance (ATD), and anterior bowing through the lateral view. A teleoroentgenogram of the lower limb was used to measure the leg length discrepancy and lower extremity mechanical axis. The pre- and postoperative femoral neck-shaft angle and ATD were compared to assess the degree of correction of the deformity. Results: Among a total of 46 cases (23 patients), 28 cases (23 patients) had lesions in the proximal femur. Type I were 16/28 cases (15/23 patients) and Type II were 12/28 cases (9/23 patients). The preoperative proximal femoral neck-shaft angle was 116.8° in Type I and 95.3° in Type II. The ATD was 12.08 mm in Type I and -5.54 mm in Type II. The deformity correction showed significant improvement immediately after surgery, the deformity correction was lost in Type II (neck shaft angle Type I: 133.8°-130.8°, Type II: 128.6°-116.9°, and ATD Type I: 17.66-15.72 mm, Type II: 7.44-4.16 mm). The extent of anterior bowing was 12.74° in Type I and 20.19° in Type II. The mean differences of 12 mm between the 9 patients who showed a leg length discrepancy and the lower extremity mechanical axis showed 4 cases of lateral deviation and 7 cases of medial deviation. Conclusion: In polyostotic fibrous dysplasia, when the femur head is involved, the femur neck shaft angle, ATD, and anterior bowing of the femur had more deformity, and the postoperative correction of deformity was lost, suggesting that the involvement of the femoral head was an important factor in the prognosis of the disease.

• Investigative Magnetic Resonance Imaging
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• v.7 no.1
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• pp.47-55
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• 2003

Purpose : Cortical dysplasia is known to be of variety of MR imaging findings. We attempted to classify MR imaging findings of cortical dysplasia into several types and to correlate those with histopathologic grades and subtypes. Materials and Methods : Preoperative MR images of 97 patients with pathologically-proven cortical dysplasia were retrospectively reviewed with knowledge of the diagnosis and operative sites. The patients were divided into MR-positive and MR-negative groups based on the presence or absence of MR imaging abnormalities. In MR-positive group, MR imaging features were arbitrarily classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types) on the basis of size of the gyrus and adjacent CSF space, cortical thickness, signal intensity of the subcortical white matter, and blurring of the gray-white matter junction. The pathologic findings were also retrospectively reviewed without knowledge of MR imaging findings and divided into three grades (mild, moderate, and severe) and two subtypes (nonballoon-cell and balloon-cell). Pathologic grades and subtypes we re compared between MR-positive and MR-negative groups. Four MR types of the MR-positive group were correlated with the pathologic grades and subtypes. Results : MR-positive and MR-negative groups consisted of 39 (40%) and 58 (60%) patients, respectively. Of the MR-positive group, atrophic type was seen in 13 patients (33 %), cortical-band type in 9 (23%), inward-rounding type in 9 (23%), and nonspecific type in 8 (21%). There was no significant difference in the pathologic grades between MR-positive and MR-negative groups, although MR-positive group tended to have higher pathologic grades than MR-negative group did. Balloon-cell subtype was found significantly higher in MR-positive group than in MR-negative group (p<0 .05): 21% (8/39) versus 5% (3/58). The inward-rounding type corresponded to the pathologically severe grade and balloon-cell subtype in 78% (7/9) and 56% (5/9) of the patients, respectively, while the atrophic type to the mild grade and nonballoon-cell subtype in 77% (10/13) and 100% (13/13), respectively. Conclusion : A variety of MR imaging abnormalities were found in 40% of the patients with cortical dysplasia and those were classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types), of which the inward-rounding type correlated well with the pathologically severe grade and balloon-cell subtype, whereas the atrophic type with the mild grade and nonballoon-cell subtype.