The McCune-Albright Syndrome is characterized by polyostotic fibrous dysplasia, cafe-au-lait colored patches of the skin and endocrinological abnormalities, including precocious puberty. Affected patients progress from GnRH-independent puberty to GnRH-dependent puberty. GnRH analogues are ineffective in GnRH-independent precocious puberty. Three year and 2 month old girl with breast development(SMR B3) and irregular vaginal bleeding were seen & diagnosed as incomplete sexual precocity. Decapeptyl treatment was started for the purpose of regression of breast development & vaginal bleeding with no effect. After 10 months, cafe-au-lait skin lesion & polyostotic fibrous dysplasia were noted and diagnosed as McCune-Albright syndrome. Breast development regressed to SMR B2 and vaginal bleeding was controlled with ketoconazole. As our experience, ketoconazole treatment might be effective to delay the progression of sexual development in patients with precocious puberty in McCune-Albright Syndrome.
Adolescent ; Breast ; Female ; Fibrous Dysplasia, Polyostotic* ; Gonadotropin-Releasing Hormone ; Humans ; Infant ; Ketoconazole* ; Puberty ; Puberty, Precocious ; Sexual Development ; Skin ; Triptorelin Pamoate ; Uterine Hemorrhage

In spite of the extensive use of antibiotics over several decades, Streptococcus pneumoniae still remains as one of the most serious human bacterial pathogens. In order to clone the pneumococcal genes whose expression is induced when pneumococcus causes infection in mice, S. pneumoniae strain ATCC 6303 was subcultured on blood agar plates (BAP) ten times to reduce the virulence first, and then passaged through BALB/c mice three times to restore the virulence. Subtractive hybridization was performed using the total RNA preparations isolated from BAP-cultured and mouse-passaged strains. Complementary DNAs corresponding to any mRNA species that were differentially expressed in the mouse- passaged strain were used as the probes to screen the genomic library of pneumococcus. Positive recombinants were selected and sequenced partially to identify the genes located within the cloned DNA. GenBank search of the sequence data has identified several genes including two heat shock genes (dnaK and dnap, a transposase-encoding gene, and a sequence which is very homologous to that of the ftsH gene.
Agar ; Animals ; Anti-Bacterial Agents ; Clone Cells* ; Cloning, Organism* ; Databases, Nucleic Acid ; DNA ; DNA, Complementary ; Genomic Library ; Hot Temperature ; Humans ; Mice ; Pneumonia ; RNA ; RNA, Messenger ; Shock ; Streptococcus pneumoniae* ; Streptococcus* ; Virulence

Diarrhea is an extremely common cause of morbidity in infancy. Occasionally it becomes protracted, leading to a vicious cycle of malabsorption, malnutrition and failure to thrive. Thirty nine infants of chronic diarrhea who had been admitted to the Department of pediatrics, Yonsei University College of Medicine for 3 years between September, 1989 and December 1992, were clinically studied and analized, the results were as follows: 1) The mean age of the patiens was 7.5+/-4.9 months. Symptoms started at 4.0+/-3.4 months of age and lasted for 3.6+/-4.1 months. 2) Among 39 cases, there were 16 cases of secondary lactase deficiency (41%), 9 cases of milk allergy(23%) and 3 cases of rotaviral enteritis(8%). Three patients (8%). had hepatitis and a patient had sepsis. 3) Incidence is much more common in formula-fed infants than brast-fed infants. 4) The growth status of the patients were poor; 54% of the patients by body weight and 39% by height fell into lower 10 percentile of normal distribution. Malnutrition accompanied the majority (82%) of the patients; 46% of mild form, 23% of moderate and 13% of severe form by Gomez classification. 5) Laboratory data on admission showed anemia in 21% of patients, hypoalbuminemia in 13% and hypocholester olemia in 31%. 6) Thirty one percent of the patients were improved with conservative tratment only. 33% of the patients got improved by low lactose milk feeding and milk withdrawl was done in 23% of the patients. Total parenteral nutrition was performed in 13% of the patients. All patients were cured with the treatment. With the above results, the most common cause of chronic diarrhea in infancy is postinfectious disaccharidase deficiency. followed by milk allergy. Low lactose milk feeding and milk withdrawl can improve the infants from chronic diarrhea.
Anemia ; Body Weight ; Classification ; Diarrhea* ; Failure to Thrive ; Hepatitis ; Humans ; Hypoalbuminemia ; Incidence ; Infant ; Lactase ; Lactose ; Malnutrition ; Milk ; Milk Hypersensitivity ; Parenteral Nutrition, Total ; Pediatrics ; Sepsis

1. 39 cases of fractures of the distal femur treated at the Gwang Ju Triservice Armed Hospital during period from January, 1968 to May, 1973 were subjected to analysis. 2. Most common cause of fracture was traffic accident and supracondylar fractures were most common. 3. Of 39 cases, 22 cases were treated by closed method with or without skeletal traction and 17 cases were treated by open reduction and internal fixation. 4. Closed methods resulted in better funtional recovery after union of fracture. 5. The healing period was 14 weeks in average.
Accidents, Traffic ; Adult ; Arm ; Clinical Study ; Femur ; Gwangju ; Humans ; Methods ; Traction

PURPOSE: Prader-Willi Syndrome(PWS) is caused by absence of paternal contributions of the chromosome region 15q11-q13. To detact this region, high resolutional cytogenetic analysis, FISH with probe at PWS critical region or microsatellite polymorphism can be used. The gene for the small nuclear ribonucleoprotein polypeptide N(SNRPN) is not expressed in patients with PWS. We conducted molecular analysis with RT-PCR with SNRPN primers to find out more useful diagnostic tool in PWS. METHODS: Four patients with obesity and other characteristics of PWS were studied. The exprssion of SNRPN and control gene were studed by RT-PCR from peripheral lymphocytes. RESULTS :The SNRPN expression in reverse transcribed RNA from blood were easily detected in normal control but not in patients with suspected Parder-Willi Syndrome. CONCLUSION: We conclude that SNRPN expression study is a useful diagnostic method for detection of Prader-Willi Syndrome.
Cytogenetic Analysis ; Humans ; Lymphocytes ; Microsatellite Repeats ; Obesity ; Pathology, Molecular* ; Prader-Willi Syndrome* ; Ribonucleoproteins, Small Nuclear ; RNA ; snRNP Core Proteins*

We analysed 26 cases of pyogenic osteomyelitis of the spine who were treaed at Younsei University Hospital during 25 years from January 1952 to August 1977 by operative or conaervative treatment. The diagnosis of pyogenic osteomyelitis of the spine is not easy and very confused. The cause and entry of the offending organism is mainly by venous drainage from the genitourinary and lower extremity circulation and the next is direct spreading from the adjacent focus. The typical clinical symptom is severe pain and tenderness over the lesion accompanied by febril history and the clinical course is relatively rapid than tuberculosis of the spine. In blood examination leukocytosis is uncommon but it showed relative increase of neutrophils in 62%, and erythrocyte sedimentation rate in 89%. Radiological findings are narrowing of joint space, erosion and/or destuctive change of vertebral body, vertebral marginal sclerotic change, and new bone formation and less frequently abscess shadow. The results obtained from this study were as follows, 1) The incidence of pyogenic osteomyelitis of the spine among total cases of the pyogenic osteomyelitis was 2.5% and the ratio of the pyogenic osteomyelitis of the spine to the spine tuberculosis was 2.8%. 2) The sexual incidence was in 11 and 15 each male and female. and the avrage, prevalent age was 34 years of age. 3) The pathogenic organism was isolated in 20 cases from the diseased focus during surgical procedure; stapyhloccus in 16 cases, pseudomonas in two and in a case of E.coli and proteus each. 4) The lumber vertebrae was mostly involved, i.e.lumbar in 11 cases, cervical in four, thoracic in nine, lumbosacral and sacrum in one each. 5) The operative treatment was performed in 23 cases and conservative in 3 cases with the satisfatory result in all cases. 6) We conclude that the focal curettage with anterior interbody fusion through anterior approach was better than the conservative treatment.
Abscess ; Blood Sedimentation ; Curettage ; Diagnosis ; Drainage ; Female ; Humans ; Incidence ; Joints ; Leukocytosis ; Lower Extremity ; Male ; Neutrophils ; Osteogenesis ; Osteomyelitis ; Proteus ; Pseudomonas ; Sacrum ; Spine ; Tuberculosis

PURPOSE: To evaluate the effectiveness and the most accurate element of the diagnostic criteria of the Japan Society of Uitrasonics in medicine(JSUM, Nov. 1989) for distinguishing between benign and malignant solid breast masses on the US. METHODS AND MATERIALS: We analyzed the ultrasonic findings of histopathologically proved 51 fibroadenomas, 12 fibrocystic disease, and 39 breast cancers in relation to the diagnostic criteria of the JSUM (shape, border, boundary echo, internal echo, posterior echo, lateral echo, and depth/width ratio). RESULTS: The number of cases of fibroadenoma, fibrocystic disease, and breast cancer corresponding to the diagnostic criteria was in the shape(26/51, 5/12, 33/39), border(41/51, 9/12, 29/39), boundary echo(48/51, 12/12, 27/39), internal echo(43/51, 9/12, 24/39), posterior echo(32/51, 3/12, 21/39), and lateral shadowing(15/51, 1/12, 35/39). All diagnostic criterias showed statistical significance for differentiation of benign/malignant breast mass on the US(Chi-square test: p<0.05). The order of accuracy was boundary echo, internal echo, and border. The mean of depth/width ratio was 0.54 +/- 0.15, 0.52 +/- 0.12, and 0.69 +/- 0.21 in fibroadenoma, fibrocystic disease, and breast cancer respectively and it had statistical significance for differentiation of benign/malignant breast mass on the US(ANOVA test: p=0.0002). CONCLUSION: The diagnostic criteria of JSUM is effective for differentiation of benign/malignant breast solid masses on the US and has accuracy in the order of boundary echo, internal echo, and border. Depth/width ratio also has statistical significance.
Breast Neoplasms ; Breast* ; Fibroadenoma ; Japan* ; Ultrasonics*

Splenic artery pseudoaneurysm is a relatively rare and potentially life-threatening complication of chronic pancreatitis. The authors present a case of splenic artery pseudoaneurysm complicating ,chronic pancreatitis. It was converting into a pseudoaneurysm by vessel rupturs. In this case report, color doppler US, CT, and MRI made the definite diagnosis.
Aneurysm, False* ; Diagnosis ; Magnetic Resonance Imaging ; Pancreatitis, Chronic* ; Splenic Artery*

The authors observed a case of typical pemphigus foliaceus who was 26 year old male. The pemphigus foliaceus is a rare chronic vesicobullous skin disease which is a type of pemphigus, which has been reported rarely in Korea. This patient has been compained of formication of the face, trunk and upper arms since 7 monthes ago. The skin lesion revealed pea sized, flaccid bullar formation and dark bownish colored hyperpigmented macular eruptions with scattering distribution on the face, trunk, back and upper arms. This patient has experienced localized and generalized exfoliation during 7 rnonthes after onset, but he has not affected in general health condition. Biopsies were performed from the skin of the back, left wrist, and histopathologically the skin showed typical pemphigus foliaceus. So, diagnosis was confirmed by clinical appearance, histopathological finding and others. This patient was markedly imporved by prednisolone without immunesuppressant drugs, and antibiotics and antihistaminics etc.
Adult ; Anti-Bacterial Agents ; Arm ; Biopsy ; Diagnosis ; Humans ; Korea ; Male ; Paresthesia ; Peas ; Pemphigus* ; Prednisolone ; Skin ; Skin Diseases ; Wrist

Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis