PURPOSE:Varying clinical phenotypes are associated with the chromosome 22q11.2 microdeletion syndrome. The endocrine manifestation are latent or overt hypoparathyroidism, thyroid dysfunction and short stature. This study was undertaken to investigate frequencies of endocrine abnormalities and short stature in patients with the chromosome 22q11.2 microdeletion syndrome. METHODS:Forty three unrelated patients were diagnosed having chromosome 22q11.2 microdeletion syndrome. Chromosomal microdeletion was confirmed by fluorescent in situ hybridation (FISH) with DNA probe (22q11.2 LSI TUPLE1 from Vysis). Serum total calcium and intact parathyroid hormone (PTH) were measured in all patients. Thyroid function tests including free thyroxine(T4), thyroid stimulating hormone (TSH) and thyroid autoantibodies were performed in all patients. Insulin-like growth factor-1 (IGF-1) was measured in 10 patients. Height, weight and body mass index were compared with chronological age in all patients. RESULTS:Seven patients (16%) had an overt hypoparathyroidism, presenting with hypocalcemic tetany. Thirteen patients (31%) showing hypocalcemia with normal PTH were regarded as having latent hypoparathyroidism since their PTH secretion response was blunted. Out of 2 patients with thyroid diseases, one patient had Graves disease and the other had Hashimoto thyroiditis. Five patients (12%) were below the 3rd percentile in height at evaluation. The BMI was below the 5th percentile in 23% of patients. CONCLUSION: Twenty patients (47%) presented with overt and latent hypoparathyroidism. Interestingly, autoimmune thyroid diseases such as Graves disease and Hashimoto thyroiditis were associated in patients with chromosome 22q11.2 microdeletion, indicating predisposition to autoimmune disorders. Therefore, a careful endocrine and growth evaluation is needed in these patients.
Autoantibodies ; Body Mass Index ; Calcium ; DNA ; Graves Disease ; Hashimoto Disease ; Humans ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Tetany ; Thyroid Diseases ; Thyroid Function Tests ; Thyroid Gland ; Thyrotropin

OBJECTIVES: To determine the level of mRNA expression of various members of the matrix metalloproteinase and tissue inhibitors in uterine leiomyoma compared with unaffected myometrium. Materials & Method: 30 cases of portions of leiomyoma and myometrium were collected immediately followimg hysterectomy. Thirteen cases were from proliferative phase and seventeen were from secretory phase of menstrual cycle. The mean age was 43.7years old. The level of expression of mRNAs of interstitial collagenase, gelatinase, stromelysin, TIMP-1,-2,-3 was determined by reverse transcriptase-polymerase chain reaction(RT-PCR) and normalized to GAPDH(glyceraldehyde-3-phosphate dehydrogenase) mRNA. RESULTS: Myometrium and leiomyoma expressed all the members of above mentioned matrix metalloproteinase family and tissue inhibitors. Leiomyoma expressed a significantly higher level of stromelysin-3 during secretory phase, an extremely lower level of 92kDa gelatinase and a significantly lower level of TIMP-3. The immunohistochemical localization of TIMP-3 was smooth muscle cell and arteriole wall of myometrium and leiomyoma. CONCLUSIONS: The increased expression of stromelysin-3 in uterine leiomyoma compared with myometrium suggests that this MMP may be involved in the formation of a more fibrous extracellular matrix in leiomyoma. The extremely lower expression of 92kDa gelatinase of leiomyoma means that leiomyoma do not invade myometrium and forms a separated mass. Decreased expression of TIMP-3 of leiomyoma suggests that TIMP-3 is required for differentiation and homeostasis of extracellular matrix of normal myometrium and function as a suppressive role of tumor development
Animals ; Arterioles ; Extracellular Matrix ; Female ; Gelatinases ; Homeostasis ; Humans ; Hysterectomy ; Leiomyoma* ; Matrix Metalloproteinase 1 ; Matrix Metalloproteinase 3 ; Menstrual Cycle ; Mice ; Myocytes, Smooth Muscle ; Myometrium* ; RNA, Messenger ; Tissue Inhibitor of Metalloproteinase-3

No abstract available.
Keratins*

No abstract available.
Transplantation*

Tsutsugamushi disease, an infectious disease by Rickettsia(R.) tsntsugamushi, is characterized by eschar, fever and rash. Eschar caused by chigger bite, is pre sent on 47 94% of patients with documented R. tsutsugamushi. We recently noticed one case of R. tsutsugamushi infection. Patient had fever, heaclache, lymphadenopathy, erythematous maculopapules and eschar. The R. tsu tsugamushi was isolated from serum of the patient. Histopathologic findings of eschar show epidermal ulceration overlying a zone of coagulative necrosis of upper dermis and necrotizing vasculitis. E]ectron microscopic findings of endothelial cells of capillary show rickettsia with cell division.
Capillaries ; Cell Division ; Communicable Diseases ; Dermis ; Endothelial Cells ; Exanthema ; Fever ; Humans ; Lymphatic Diseases ; Necrosis ; Rickettsia ; Scrub Typhus* ; Trombiculidae ; Ulcer ; Vasculitis

We managed 118 patients with benign prostatic hyperplasia by transurethral resection during January 1994 and December 1995 in Eul Ji Medical Center and all patients were operated upon by 2 staff doctors. Among 118 cases, 7 cases with intraoperative complications such as bladder injury or severe prostatic capsule injury and 8 cases with combined neuropathic bladder or chronic urinary retention and 2 cases with urethral stricture were excluded. The average age of 101 cases were 66.4 years old (50-92), and average weight of resected adenoma was 10.0 gm (2-32). We categorized these patients randomly into 2 groups. In group I (n=48), we removed the urethral catheter within 2 days after TURP and in group II (n=53), we removed it after 3 days (mean 4.2 days) following surgery. The average hospital stay postoperatively was4.1 days in group I and 7.4 days in group II. Recatheterization rate was 8.3% (4/48) in group I and 5.6% (3/53) in group II (p>0.05). There was no statistical difference in other complication including failure to void, intraoperative bleeding, urethral stricture, incontinence and TUR syndrome. This study supports early catheter removal after TURP would become an accepted and routine postsurgical practice following uncomplicated TURP.
Adenoma ; Catheters* ; Hemorrhage ; Humans ; Intraoperative Complications ; Length of Stay ; Prospective Studies* ; Prostatic Hyperplasia ; Transurethral Resection of Prostate* ; Urethral Stricture ; Urinary Bladder ; Urinary Bladder, Neurogenic ; Urinary Catheters ; Urinary Retention

We experienced one case of severe pituitary dwarfism in a 10 years old female girl. Magnetic resonance image (MRI) revealed transection of the pituitary stalk stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland. The serum growth Hormone (GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH. The morning cortisol concentration and serum T4 concentration were decreased below the normal range. These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI. Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary stalk. We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature.
Child ; Clonidine ; Craniocerebral Trauma ; Dwarfism, Pituitary* ; Female ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Hypothalamus ; Levodopa ; Magnetic Resonance Imaging ; Median Eminence ; Pituitary Gland* ; Pituitary Gland, Anterior* ; Prolactin ; Reference Values

PURPOSE: This study was conducted to investigate the relationship between axial length(AL) and proliferative diabetic retinopathy(PDR). METHODS: We reviewed the medical charts of the nondiabetic patients who had undergone cataract surgery and diabetic patients who had undergone vitrectomy or cataract surgery at Korea University Kuro Hospital between January, 1998 and July, 2000. Eyes with previous ocular surgery or other severe ocular disease were excluded from the analysis. Eyes with axial length greater than 24 mm were also excluded. The eyes were divided into 4 groups: Non-DM group, nondiabetic; Non-DR group, diabetic without retinopathy; NPDR group, with nonproliferative diabetic retinopathy; and PDR group, with proliferative diabetic retinopathy. Each group contained 42 eyes which were matched for age and sex. RESULTS: The AL values of each group were compared. The mean AL of the PDR group(22.2+/-0.8 mm) was significantly shorter than each of the other groups: Non-DM group(23.1+/-0.7 mm, p<0.05); Non-DR group(22.9+/-0.9 mm, p<0.05); NPDR group(22.8+/-0.8 mm, p<0.05). Within the PDR group, the eyes that had received preoperative panretinal photocoagulation(27 eyes, mean: 22.0+/-1.1 mm) showed a shorter AL than those that had not(15 eyes, mean: 22.7+/-0.8 mm), which was not statistically significant(P=0.08, t-test). CONCLUSION: The results suggest that a shorter axial length may be associated with severe proliferative diabetic retinopathy.
Cataract ; Diabetic Retinopathy* ; Humans ; Korea ; Vitrectomy

BACKGROUND: Human hair usually emerges from the scalp in groups of 2 or 3-hair units. Hair densities and grouping patterns in androgenetic alopecia (AGA) patients are known to be different from those in normal adults, but no comparative study has ever before been made in Korea. Therefore we have developed some technical improvements on phototrichogram methodology to evaluate the differences between these two groups. BACKGROUND: Objectives: This study was performed to quantify Koreans' hair characteristics at each site (vertex, occipital, temporal) of the scalp according to the age and to compare the differences of hair characteristics of AGA and normal adults using phototrichogram. METHODS: Hairs in the precisely defined circle at each (vertex, temporal, occipital) site of the scalp in 37 AGA male patients and 15 healthy male volunteers were evaluated according to age. Each circumscribed area of the scalp, centered with a dot tattoo to ensure reproducibility, was photographed just after shaving, and named 'primary image'. Two days later, the same area was again photographed, and named 'secondary image', from which the hair density and the grouping pattern of hair follicular units were determined by the image analyzer program. RESULTS: The results can be summarized as follows: 1. In normal adult males, the range of hair density was 117~13/cm2~140~16/cm2, with the hair density of the occiput being significantly higher than that of the temple. No significant differences in hair density were shown between vertex and occiput. 2. In AGA male patients, the range of hair density was 102~23/cm2-137~17/cm2, and the differences of the hair density between vertex and occiput were statistically significant. 3. The numbers of grouped hair units per square centimeter in the non-bald adult male group ranged from 59~11/cm2 to 73~14/cm2. The most common grouped hair unit was a 2-hair unit, followed by a 1-hair unit and a 3-hair unit group. 4. In the AGA group, the numbers of grouped hair units per square centimeter ranged from 57~6/cm2 to 72~12/cm2, showing no significance with the control group. The most common grouped hair unit was a 1-hair unit, followed by a 2-hair unit and a 3-hair unit group. CONCLUSIONS: The AGA group compared remarkably with the normal adult male group, showing a lower hair density with a larger proportion of 1-grouped hair units. Based on the data about hair density and hair grouping patterns in AGA patients, surgeons can estimate the area of donor scalp with the most probable number of follicular units in hair trans-plantation procedures.
Adult ; Alopecia* ; Hair* ; Humans ; Korea ; Male ; Scalp* ; Tissue Donors ; Volunteers

No abstract available.
Neck*