We report computed tomography (CT) and magnetic resonance (MR) findings of a patient with polyostotic Paget disease and multicentric giant cell tumor (GCT). Brain CT scan showed widening of diploic space, cortical thickening and enhancing soft tissue mass in occiput with underlying calvarial destruction. Ill-defined soft tissue masses were also detected in maxillary sinus and buttock with underlying bony destruction on CT. MR image showed multifocal nodules in wide diploic space with low signal intensity on T1 -weighted image and bright signal intensity on T2-weighted image. Mass in occiput showed homogeneous hypointensity to bone marrow on T1-weighted image and homogeneous iso- intensity on T2-weighted image. Multiple nodules in diploic space and occipital mass showed contrast enhancement following administration of Gd-DTPA. Biopsy was performed at scal p, maxillary sinus and buttock, and histologic analysis revealed GCT.
Biopsy ; Bone Marrow ; Brain ; Buttocks ; Gadolinium DTPA ; Giant Cell Tumors* ; Giant Cells* ; Humans ; Maxillary Sinus ; Tomography, X-Ray Computed

Microinvasive carcinoma of the uterine cervix(Stage Ia) is the earliest stage of squamous carcinoma. The transition from preinvasive to invasive disease is a crucial juncture in the development of cervical cancer. The clinical experience that microinvasive lesions carry a better prognosis justifies a separate diagnostic category. To investigate the clinical and pathological aspects of microinvasive cervical carcinoma, a retrospective study was made on 84 cases, histologically reconfirmed surgical specimens, which had been treated during the period from January, 1985 to December, 1996 at Department of Obstetrics and Gynecology, Kyung-pook National University Hospital. We defined microinvasive carcinoma as stromal invasion not exceeding a depth of Smm from the base of the epithelium presented by FIGO in 1985. The results obtained were as follows; 1. Mean age of 84 patients was 46.9 year old, all were parous women but not 1 patients. 60 cases(71.4%) belonged to stage Ial and 24 cases(28,6%) to stage Ia2. 2. Chief complaints were postcoital spotting and leukorrhea. 3, The corresponding rate of Pap. smear to histologic diagnosis was approximately 29.8%. However correponding rate within one histologic grade was about 33,3%. 4. According to the colposcopic examination, 26.4% of cases revealed suspected abnormal findings suggesting invasive lesion. 5. As to the reports of punch biopsy, 55.1% of cases showed microinvasive and/or invasive lesion. With additional diagnostic conization of cervix, the preoperative diagnosis were correct in 72%. 6. Operation performed were simple hysterectomy, extrafascial hysterectomy or modified radical hysterectomy with both pelvic LN dissection, Simple hysterectomy was most commonly performed. 7. Post-treatment complications were developed in 73.9% of patients who were done with modified radical hysterectomy with both pelvic LN dissection and bladder dysfunction was developed in 50% of the patients.
Biopsy ; Carcinoma, Squamous Cell ; Cervix Uteri* ; Conization ; Diagnosis ; Epithelium ; Female ; Gynecology ; Humans ; Hysterectomy ; Leukorrhea ; Metrorrhagia ; Obstetrics ; Prognosis ; Retrospective Studies ; Urinary Bladder ; Uterine Cervical Neoplasms

Glycogen storage diseases are inherited disorders of carbohydrate metabolism caused by a deficiency of enzymes that are involved in degradation of glycogen in the liver. The accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form and clinically may manifest of glycogen storage disease itself rather than growth hormone deficiency. But in this case the patient showed exceptional extreme growth retardation. Growth hormone stimulation test with clonidine and L-dopa revealed that the patient had growth hormone deficiency. Therefore, we report of a case of glycogen storage disease type Ia with the presence of GH deficiency with review of literature. A 16-year-old male was admitted for the evaluation of hepatomegaly and extreme short stature. The height was 113.5cm, less than third percentile of same age group, and compatible with fiftieth percentile of height of 6 years of age. After laboratory work up including liver biopsy, he was diagnosed with type I glycogen storage disease. The patient was presented with metabolic acidosis, hyperuricemia, and hypoglycemia. Hypoglycemia was managed with frequent feeding with high starch diet and intravenous glucose infusion. Metabolic acidosis was treated with sodium bicarbonate. Secondary hyperuricemia was treated with allopurinol. The patient is being followed at out-patient clinic with clinical improvement after of GH administration.
Acidosis ; Adolescent ; Allopurinol ; Biopsy ; Carbohydrate Metabolism ; Clonidine ; Diet ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone ; Hepatomegaly ; Humans ; Hyperuricemia ; Hypoglycemia ; Levodopa ; Liver ; Male ; Outpatients ; Sodium Bicarbonate ; Starch

PURPOSE: The aim of this study was to investigate the effect of perinatal risk factors on brain maturation and the relationship of brain maturation and neurodevelopmental outcomes with brain maturation scoring system in brain MRI. METHODS: ELBWI infants born at the Seoul National University Children's Hospital from January 2006 to December 2010 were included. A retrospective analysis was performed with their medical record and brain MR images acquired at near full term. We read brain MRI and measured maturity with total maturation score (TMS). TMS is a previously developed anatomic scoring system to assess brain maturity. The total maturation score was used to evaluate the four parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. RESULTS: Images from 124 infants were evaluated. Their mean gestational age at birth was 27.1+/-2.1 weeks, and mean birth weight was 781.5+/-143.9 g. The mean TMS was 10.8+/-2.0. TMS was significantly related to the postmenstrual age (PMA) of the infant, increasing with advancing postmenstrual age (P<0.001). TMS showed no significance with neurodevelopmental delay, and with brain injury, respectively. CONCLUSION: TMS was developed for evaluating brain maturation in conventional brain MRI. The results of this study suggest that TMS was not useful for predicting neurodevelopmental delay, but further studies are needed to make standard score for each PMA and to re-evaluate the relationship between brain maturation and neurodevelopmental delay.
Birth Weight ; Brain ; Gestational Age ; Humans ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Newborn ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Magnetics ; Magnets ; Medical Records ; Myelin Sheath ; Neuroglia ; Parturition ; Premature Birth ; Retrospective Studies ; Risk Factors