OBJECTIVETo establish the quantitative diagnostic criteria for cranio-orbito-zygomatic deformity (COZD).

METHODSComputer-assisted three-dimensional (3-D) CT measurement was performed in 30 cases with unilateral COZD. The differences of the measurement data between the affected and unaffected sides were analyzed. Then the patients were diagnosed and classified according to the affected bone, soft tissue and conjunctival sac. Based on the quantitative diagnosis, 8 patients underwent surgery to test the clinical practicability of the diagnostic criteria.

RESULTSThe quantitative diagnostic criteria for COZD could reflect the affected area and the corresponding severity of deformity. It helped to preoperative design and to predict movement of osteotomy segment and. the soft tissue volume needed for augmentation. Good postoperative results were achieved.

CONCLUSIONSThe quantitative diagnostic criteria for COZD can describe the affect area and severity of deformity in detail. It is very practical in the guidance of clinical treatment.

Adolescent ; Adult ; Bone Diseases, Developmental ; diagnostic imaging ; Child ; Diagnosis, Computer-Assisted ; standards ; Humans ; Imaging, Three-Dimensional ; Male ; Orbit ; abnormalities ; Tomography, X-Ray Computed ; methods ; Young Adult ; Zygoma ; abnormalities

OBJECTIVETo summarize the methods and experiences in surgical treatment of hemifacial microsomia.

METHODSFifty-eight cases of hemifacial microsomia that have been treated by plastic and reconstructive surgery and followed-up in our department during last 20 years have been reviewed. Every patient's characteristic was assessed by physical examination, photography, craniofacial cephalometry before planned the method of surgery. Different surgical treatments were chosen according to the side and the structures involved in the abnormalities and the severity of hemifacial microsomia, and the cases were followed up since 3 months after the treatments. The follow-up lean of midline of the lower 1/3 face with that before treatment is compared. The degrees of patients' and surgeons' satisfactions with the treatments were evaluated respectively.

RESULTSThe average angle of lean of midline of skeletal and soft tissue of the lower 1/3 face decreased 4.2 degrees and 2.9 degrees respectively after treatment. Fifty (82.6%) cases satisfied with the outcome of the surgical treatment and surgeons satisfied with 84.5% of the outcome of all the cases.

CONCLUSIONSIndividual surgical treatment based on the side and the structures involved in the abnormalities can effectively correct facial asymmetry of hemifacial microsomia.

Adolescent ; Adult ; Child ; Child, Preschool ; Facial Asymmetry ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Middle Aged ; Orthopedic Procedures ; methods ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo evaluate the 3-D position changes of periorbital structures after midface distraction osteogenesis in patients with Crouzon syndrome.

METHODSThe CT data of 8 cases who had accepted the midface distraction osteogenesis following Le Fort III osteotomy were retrospectively analyzed. The patients were averagely 11.9 years old, and the CT was performed before and one year after operation. After 3-D image reconstruction, a right-hand coordinate system based on the preoperational Frankfurt Plane was then established. The pre- and post-operative positions of the superior orbit point (SOr), inferior orbit point (IOr), median orbit point (MOr), lateral orbit point (LOr), anterior ocularis point (AO), ocularis eyeball point (PO) and the four insertion ocularis rectus were documented and compared. The positions of these marked points in normal controls were also documented and compared with those in patients.

RESULTSAfter midface distraction osteogenesis, the position of AO was not changed significantly on the y-axis and z-axis, but the distance between two AO points on x-axis was reduced by 3.40 mm; IOr moved averagely 12.24 mm on y-axis and 4.25 mm on z-axis, MOr moved averagely 10.11 mm on y-axis and 2.80 mm on z-axis, LOr moved averagely 9.86 mm on y-axis and 2.31 mm on z-axis. The Inferior Rectus attachment moved averagely 3.63 mm on y-axis and 2.98 mm on z-axis. No other significant change was observed on other marked points.

CONCLUSIONSMidface distraction osteogenesis following Le Fort III osteotomy can significantly move the medial, lateral and inferior peri-orbital bone structure anteriorly and inferiorly. The eyeballs have no markedly sagittal position changes after distraction except slight medial, downwards movements and anterior-upwards rotations.

Adolescent ; Child ; Facial Bones ; diagnostic imaging ; surgery ; Female ; Humans ; Imaging, Three-Dimensional ; Male ; Orbit ; diagnostic imaging ; Osteogenesis, Distraction ; methods ; Osteotomy, Le Fort ; methods ; Tomography, X-Ray Computed

OBJECTIVETo diagnose and classify the congenital craniofacial cleft with a uniform scale is helpful to evaluate the abnormality and select the repairing methods.

METHODSWe analyzed 81 cases of congenital craniofacial cleft basically using Tessier craniofacial cleft classification. Furthermore, according to the position of soft tissue or bone, the character and degree of clefts or dysplasia and the results of CT scanning, we subdivided the congenital deformities based on S (skin), T (tissue), and O (OS). Arabic numerals were used to express the degree of the abnormality.

RESULTSOf all the cases analyzed with the STO classification, No. III and IV clefts are often seen in the infraorbital region (24.70%). No. IX and X clefts are mostly seen in the supraorbital region (38.27%). The relationship between the cleft types and involved tissue has not been found.

CONCLUSIONSThe STO classification reinforces Tessier classification. It offers the basis for craniofacial cleft repair.

Adolescent ; Adult ; Child ; Child, Preschool ; Craniofacial Abnormalities ; classification ; diagnosis ; Female ; Humans ; Infant ; Male ; Young Adult

OBJECTIVETo investigate the therapeutic effects of upper airway stenosis after Le Fort III osteotomy and midfacial distraction osteogenesis (DO).

METHODSEleven cases (age, 5-16 yrs) with severe midface dysostosis complicated with exophthalmos, anterior crossbite and upper airway stenosis were treated by using Le Fort III osteotomy and midfacial DO from August 2000 to February 2007. The 3D reconstruction of the upper-airway from CT data was used to evaluate the upper airway volume before and after the operation. And meanwhile polysomnography was carried out to demonstrate the upper airway functional changes.

RESULTSThere was a 64.3% mean increase [mean, (9.13 +/- 6.94) ml, P < 0.05] in upper airway volume in the 11 cases after the operations. It showed that there was significant improvements in the indexes of polysomnography after the operations, such as apnea and hypopnea index, average SaO2, minimum oxygen saturation and snore index.

CONCLUSIONSThe Le Fort III osteotomy and midfacial distraction osteogenesis can efficiently relieve the symptoms of upper-airway stenosis in severe midfacial dysostosis.

Acrocephalosyndactylia ; complications ; Adolescent ; Airway Obstruction ; etiology ; surgery ; Child ; Child, Preschool ; Craniofacial Dysostosis ; complications ; Female ; Follow-Up Studies ; Humans ; Male ; Osteogenesis, Distraction ; methods ; Osteotomy, Le Fort ; Treatment Outcome

OBJECTIVETo observe the therapeutic effects of Le Fort III osteotomy and midface distraction osteogenesis (DO) on the upper-airway narrow.

METHODSSince 2000, 11 cases (10 cases of Crouzon syndrome and 1 case of Apert syndrome) with severe midface deficiency were treated with Le Fort III osteotomy and midface DO. The section area of different parts of upper-airway were tested by computer assistants image measurement preoperatively and postoperatively. Some patients received sleep function monitoring.

RESULTSThe face appearance and the function of upper-airway improved significantly after Le Fort III osteotomy and Midface DO. The section area at the level of posterior nasal spine and uvula increased obviously after treatment (P < 0.05), however the section area at the level of epiglottis and separation between airway and esophagus were not obviously enlarged (P > 0.05).

CONCLUSIONSMidface DO after Le Fort III osteotomy can effectively improve the upper-airway narrow, especially the upper part from uvula.

Adolescent ; Airway Obstruction ; etiology ; surgery ; Child ; Child, Preschool ; Craniofacial Dysostosis ; complications ; surgery ; Epiglottis ; pathology ; Female ; Humans ; Male ; Nasopharynx ; pathology ; Osteotomy, Le Fort ; methods ; Palate, Soft ; pathology ; Postoperative Period ; Sleep Apnea, Obstructive ; etiology ; surgery ; Treatment Outcome

A 26-year-old male presented with tremor of bilateral shoulders and hands as the major symptom and also had cognitive and emotional abnormalities for more than 1 year, who was diagnosed as cerebral hepatolenticular degeneration (HLD) in Tongji Hospital of Huazhong University of Science and Technology in October 2021. The serum ceruloplasmin and urine copper levels of the patient were 0.023 g/L and 3760.00 μg/24 h, respectively, and the Kayser-Fleischer (K-F) ring was seen in the cornea. Genetic testing revealed a homozygous mutation of ATP7B gene c.2975C>T (p.Pro992Leu), while transcranial sonography (TCS) showed lenticular nucleus hyper-echogenicity. The literature was searched using hepatolenticular degeneration and transcranial sonography as key words; and 9 articles involving 150 HLD cases were obtained. The lenticular nucleus hyper-echogenicity was presented in 76.9% HLD patients (150/195), while only in 12.7% healthy subjects (17/134) ( P<0.001), suggesting that advanced transcranial sonography can detect the metal deposition and may be used for diagnosis of cerebral HLD.

Purpose: This study investigated the value of synchronous tele-ultrasonography (TUS) for naive operators in thyroid ultrasonography (US) examinations. Methods: Ninety-seven patients were included in this prospective, parallel-controlled trial. Thyroid scanning and diagnosis were completed by resident A independently, resident B with guidance from a US expert through synchronous TUS, and an on-site US expert. The on-site expert’s findings constituted the reference standard. Two other off-site US experts analyzed all data in a blind manner. Inter-operator consistency between the two residents and the on-site US expert for thyroid size measurements, nodule measurements, nodule features, American College of Radiology (ACR) Thyroid Imaging Reporting and Data System (TI-RADS) categories, and image quality was compared. Two questionnaires were completed to evaluate the clinical benefit. Results: Resident B detected more nodules consistent with the on-site expert than resident A did (89.4% vs. 56.5%, P<0.001). Resident B achieved excellent consistency with the on-site expert in terms of ACR TI-RADS categories, nodule composition, shape, echogenic foci, and vascularity (all intra-class correlation coefficients [ICCs] >0.75), while resident A achieved lower consistency in ACR TI-RADS categories, composition, echogenicity, margin, echogenic foci, and vascularity (all ICCs 0.40-0.75). Residents A and B had excellent consistency in target nodule measurements (all ICCs >0.75). Resident B achieved better performance than resident A for gray values, time gain compensation, depth, color Doppler adjustment, and the visibility of key information (all P<0.05). Furthermore, 61.9% (60/97) of patients accepted synchronous TUS, and 59.8% (58/97) patients were willing to pay for it. Conclusion Synchronous TUS can help inexperienced residents achieve comparable thyroid diagnostic capability to a US expert.

Purpose To explore the prognostic value of deletion of the TNFAIP3 gene in natural killer/T-cell lymphoma,nasal type detected with fluorescence immunophenotyping and interphase cytogenetics as a tool for the investigation of neoplasm (FICTION).Methods FICTION was performed to detect the abnormalities of TNFAIP3 gene in 109 cases of natural killer/T-cell lymphoma,nasal type.Results TNFAIP3 deletion was found in 25/79 detectable cases.The deletion of TNFAIP3 was positively correlated with the International Prognosis Index (IPI) (P =0.019).Conclusion Frequent deletion of TNFAIP3 was associated with IPI in natural killer/T-cell lymphoma,nasal type,suggesting the important prognostic value of TNFAIP3 in the natural killer/T-cell lymphoma,nasal type.

OBJECTIVE: To determine the pharmacokinetics of erythromycin stinoprate capsules and to provide guidance for clinical research. METHODS: Thirty healthy volunteers (15 men and 15 women) were divided into 3 groups randomly, each including 5 men and 5 women. Single oral doses of 250, 500 and 750 mg were given to each volunteer. The concentrations of erythromycin propionate and erythromycin base in the plasma were determined by HPLC-MS. RESULTS All 30 volunteers completed the experiment without adverse reactions. Using 3P87 we analyzed the model and calculated the pharmacokinetic parameters. Three dose groups taking high, middle and low dose were all single compartment model. The pharmacokinetic parameters of erythromycin propionate after taking erythromycin stinoprate capsules were as follows: Low dose group: Ka (2.007 +/- 1.281 )/h, tmax ( actual value) (1.9 +/- 0.6) h, Cmax (437.0 +/- 295.0) microg/L, AUC0-14 (trapezoid area) (1840.2 +/- 1476.87) microg x h/L, Ke (0.329 +/- 0.119)/h, T1/2 (2.45 +/- 0.9) h. Middle dose group: Ka (1.451 +/- 0.380)/h, tmax (1.7 +/- 0.3) h, Cmax (923.1 +/- 217.5) microg/L, AUC0-14 (4542.44 +/- 1579.4) microg x h/L,Ke (0.237 +/- 0.057)/h, T1/2 (3.1 +/- 1.1) h; High dose group: Ka (2.076 +/- 1.559)/h, tmax (1.7 +/- 0.3) h, Cmax (1336.5 +/- 366.0) microg/L, AUC0-14 (7481.5 +/- 2496.2) microg x h/L, Ke (0.266 +/- 0.051)/h, T1/2 (2.7 +/- 0.5) h. The pharmacokinetic parameters of erythromycin were as follows: Low dose group: Ka (1.410 +/- 0.626)/h, tmax (1.8 +/- 0.5) h, Cmax (197.5 +/- 227.6) microLg/L, AUC0-14 (766.4 +/- 981.0) microg x h/L, Ke (0.519 +/- 0.240)/ h, T1/2 (1.6 +/- 0.8) h. Middle dose group: Ka (1.900 +/- 1.049)/h, tmax (1.6 +/- 0.2) h,Cmax (488.3 +/- 216.7) microg/L, AUC0-14( 488.3 +/- 216.7) microg/L, Ke (0.329 +/- 0.057)/h, T1/2(2.2 +/- 0.4) h; High dose group: Ka (1.934 +/- 0.794)/h, tmax (1.7 +/- 0.3) h, Cmax (749.3 +/- 387.2) microg/L, AUC0-14(3820.1 +/- 1966.4) microg x h/L, Ke (0.373 +/- 0.174)/h, T1/2( 2.2 +/- 0.7) h. AUC of both erythromycin propionate and erythromycin base was linearly correlated to the doses; T1/2 was not correlated to the doses, so they followed the first order processes. The pharmacokinetic parameters of erythromycin The erythromycin stinoprate propionate and erythromycin base had no gender differences. Conclusion was absorbed as erythromycin propionate. Cmax reached at about 1.6 h. T1/2 of elimination was 2.4-3.1 h. The active component of erythromycin propionate was erythromycin. Cmax of erythromycin is 1.8, T1/2 is 2.4-3.1 h. In the range of oral dose of 250 to 750 mg, both erythromycin propionate and erythromycin base accorded the first order processes. The pharmacokinetic parameters were different with those reported in foreign documents while the gender difference did not exist in Chinese adults.
Adult ; Area Under Curve ; Biological Availability ; Capsules ; Chromatography, High Pressure Liquid ; methods ; Erythromycin ; administration & dosage ; analogs & derivatives ; pharmacokinetics ; Female ; Humans ; Male