Incontinentia pigmenti(IP), so called Bloch-Sulzberger syndrome is a rare hereditary neurocutaneous syndrome and was described first by Bardach in l925. This disorder is known as a systemic disease caused by a defect at the developmental stage of organs originated from ectoderm or mesoderm. Incontinentia pigmenti is presented by characteristic linear hyperpigmented skin lesions, often associated with central nervous system involvement, dysplasia in the dental system, and ocular abnormalities. This disorder occurs almost in female infants, usually lethal in males, inherited as X-linked dominantly. Thirty percent of the patients suffer from central nervous system complications such as mental retardation, encephalopathy, delayed development, seizure, spastic paralysis and microcephaly. We report a case of a 12 month-old female with incontinentia pigmenti who was hospitalized with linear hyperpigmented skin lesions and delayed development.
Central Nervous System ; Ectoderm ; Female ; Humans ; Incontinentia Pigmenti ; Infant ; Intellectual Disability ; Male ; Mesoderm ; Microcephaly ; Muscle Spasticity ; Neurocutaneous Syndromes ; Paralysis ; Seizures ; Skin

The principal objective of this study was to determine whether visceral fat or liver fat is a more relevant risk factor for metabolic syndrome. A total of 98 subjects aged 18-65 yr, who visited a health promotion center in a university hospital, were enrolled in this study. Metabolic syndrome was diagnosed based on the modified National Cholesterol Education Program's Adult Treatment Panel III report (NCEP-ATPIII) criteria. We defined the visceral obesity as a visceral fat area of > or = 100 cm2 which was acquired by CT at the L4-5 level. To evaluate fatty liver, we applied a liver-to-spleen attenuation ratio < or = 1.1 as measured by CT at the T12 level. We employed binary logistic regression models that used the presence or absence of metabolic syndrome as a dependent variable and age, sex, and the presence or absence of visceral obesity and fatty liver as independent variables. Visceral obesity was not found to be an independent variable as a risk factor of metabolic syndrome (odds ratio 2.7; 95% confidence interval 0.55-13.30), but fatty liver was found to be significant in this model (odds ratio 71.3; 95% CI 13.04-389.53). Our study suggests that liver fat may be a more important risk factor than visceral fat in terms of its association with metabolic syndrome.
Adolescent ; Adult ; Aged ; Blood Pressure ; Body Composition ; Demography ; Fatty Liver/*complications ; Female ; Humans ; Intra-Abdominal Fat/*anatomy & histology/radiography ; Liver/anatomy & histology/radiography ; Logistic Models ; Male ; Metabolic Syndrome X/diagnosis/epidemiology/*etiology ; Middle Aged ; Odds Ratio ; Risk Factors ; Sex Factors ; Spleen/anatomy & histology/radiography ; Tomography, X-Ray Computed ; Young Adult

No abstract available.

BACKGROUND: Millard’s rotation-advancement repair, which is used by many surgeons, can make a natural philtral column, but most surgeons use a modification of the rotation-advancement flap. The purpose of this article is to introduce a modification utilized by the authors and to provide detailed surgical procedure. METHODS: We retrospectively reviewed 82 patients’ medical records and presented surgical technique and outcomes. The main features of the authors’ strategy are emphasizing horizontal length of the lip, orbicularis oris muscle duplication for improving the definition of the philtral column, overcorrection of domal portion than the non-cleft side in order to compensate for the recurrence during growth. Two judges rated two times the appearance of the patients’ nose and lip using Asher-McDade aesthetic index. Intra- and interobserver reliabilities were determined using Cohen’s kappa statistics. RESULTS: All patients recovered eventually after surgery; however, two patients have a minor complications (wound infection in one patient, wound disruption due to trauma in the other patient). The improvement of the aesthetic results can be achieved with this modified Millard technique. Total mean scores of the Asher-McDade index was 2.08, fair to good appearance. The intraobserver reliabilities were substantial to almost perfect agreement and the interobserver reliabilities were moderate to almost perfect agreement. CONCLUSION: We modified Millard method for repair of complete unilateral cleft lip. The surgical outcomes were favorable in long-term follow-up. We hope our technique will serve as a guide for those new to the procedure.
Cleft Lip* ; Follow-Up Studies ; Hope ; Humans ; Lip ; Medical Records ; Methods ; Nose ; Recurrence ; Retrospective Studies ; Rhinoplasty ; Surgeons ; Wounds and Injuries