Poland's syndrome is congenital anomaly which was described first by Alfred Poland in 1841. The clinical features are variable but always include congenital aplasia of fingers and syndactyly. We have experienced a case of Poland's syndrome. This case was male children and revealed thoracic anomaly of right side (absence of pectoral muscles and anterior axillary fold), atrophy of forearm muscles, ipsilateral syndactyly with aplasia of thumb and middle phalanges of 2, 3, 4, 5,th fingers, and ipsilateral congenital radio-ulnar synostosis.
Atrophy ; Child ; Fingers ; Forearm ; Humans ; Male ; Muscles ; Poland ; Syndactyly ; Synostosis ; Thumb

No abstract available.

No abstract available.

DiGeorge syndrome, a developmental defect of the third and fourth pharyngeal pouches, is characterized by aplasia or hypoplasia of the thymus and parathyroid glands and by conotruncal cardiac malformation. This syndrome is usually associated with deletion of long arm in chromosome 22 (22q11-). We experienced a case of partial DiGeorge syndrome in a 2-month-old male who had hospitalized because of recurrent hypocalcemic tetany and tetralogy of Fallot. Immunologic studies revealed the decreased percentage of T lymphocyte and increased percentage of B lymphocyte. Chromosomal study with high resolution banding, showed 46, XY, 22q13 deletion. We report a case of partial Digeorge syndrome with a brief review of literatures.
Arm ; Chromosomes, Human, Pair 22 ; DiGeorge Syndrome* ; Humans ; Infant ; Lymphocytes ; Male ; Parathyroid Glands ; Tetany ; Tetralogy of Fallot ; Thymus Gland

A total of 48 cases of tuberculous lesion in the lymph nodes (43 cases), lung (3 cases) and soft tissue (2 cases), was subjected to fine needle aspiration cytology(FNAC). The age of the patients ranged from 19 to 77 year-old (average 33.6 years in age) and the male to female ratio was 1: 4. Thirty-four cases (70.8%) demonstrated distinct granulomatous reaction with or without caseation necrosis, nine cases (18.8%) showed no granulomas, but large amount of necrotic debris with numerous polymorphonuclear cells and histiocytes, and five cases (10.4%) revealed acellular material only. The overall AFB positivity in smears was 62.5%. In areas associated with granulomatous reaction and necrosis, AFB positivity was 55.8%, while it was 80.0% in cases with acellular necrotic material. There were 2 cases of parasitic infestation which could not be easily differentiated from tuberculosis based on aspiration smears only.
Aged ; Biopsy, Fine-Needle ; Child* ; Female ; Granuloma ; Histiocytes ; Humans ; Lung ; Lymph Nodes ; Male ; Necrosis ; Tuberculosis ; Vesico-Ureteral Reflux*

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

Intraventricular hemorrhage is a common hemorrhagic cerebral disorder in premature, but occurs much less frequently in the full-term newborn. In order to obtain documentation of clinical and ultrasonographic characteristics of intraventricular hemorrhage in full-term, we performed cerebral ultrasonography on 602 newborn infants who had been admitted to neonatal intensive care unit at the Deppartment of Pediatrics, Yonsei University College of Medicine between December 1989 and June 1991. 15 cases (2.5%) of intraventricular hemorrhage were analysed. The results were as follows: 1) Among 15 cases with intraventricular hemorrhage, there was no obstetrical and perinatal complication in 8 cases (53%) and 13 cases (87%) were neurologically normal at birth. 2) Sudden onset of dramatic neurologic abnormalitis inclued seizures, fever, bulging fontanel, irritability, lethargy, vomiting in cases (73%) and the remainder (27%) had no clinical symptoms and signs. 3) The age at diangosis of hemorrhage was before 72 hours in 5 cases (33%), between 4 and 7 days in 2 cases (13%), at 8 to 28 days in 8 cases (53%), 8 of 15 cases (53%) had Grade 1 IVH, four with Grade 2, cases with Grade 3, and 1 case of these infant demonstrated Grade IV IVH. The source of hemorrhage was subependymal germinal matrix in 12 cases (80%) and choroid plaxus in 3 cases (20%). 4) Precipitating factors were cerebral venous infarction in 7 cases, hypoxic injury in 5, and 3 other infants had no identifiable medical risk factors. 5) Among all 15 survivors, 2 of 3 infants with choroid plexus hemorrhage required placement of a ventriculo-peritoneal shunt, in the other case, ventriculomegaly decreased spontaneously.
Choroid ; Choroid Plexus ; Hemorrhage* ; Humans ; Infant ; Infant, Newborn* ; Infarction ; Intensive Care, Neonatal ; Lethargy ; Parturition ; Pediatrics ; Precipitating Factors ; Risk Factors ; Seizures, Febrile ; Survivors ; Ultrasonography ; Ventriculoperitoneal Shunt ; Vomiting

Malignant transformation of fibrous dysplasia to osteosarcoma is rare. We report a case in which monostotic fibrous dysplasia of the proximal femur treated with curettage, Ender nailing and bone grafting was differentiated into the osteosarcoma in a 58-year-old female.
Bone Transplantation ; Curettage ; Female ; Femur ; Fibrous Dysplasia, Monostotic ; Humans ; Middle Aged ; Osteosarcoma

The incidence of diabetes mellitus is in increasing tendency because of expension of medical care, economic development and increasing of the population of the aged person recently. The diabetic foot is one of serious complication of diabetes. With the advancement of the method of treatment of diabetes the other acute metabolic complications are decreased, but the development of diabetic gangrene encounteres at any age of adult life with increasing of incidence according to the duration of diabetes. The most of them requires operation, and its treatment is difficult frequently because of high incidence of wound problem in healing. So it is important to educate and prevent for lowering its incidence. From January 1978 to December 1984, 38 patients with diabetic foot were treated and analysed at Department of Orthopaedic Surgery of Presbyterian Medical Center. The results obtained are as follow. 1. The average of annual incidence of diabetic gangrene was 2.85%. There was increasing tendency of incidence to 1981, thereafter decreasing tendency. 2. 71% of patients with gangrene were in over-50 year age group. 3.72% of patients were in duration of diabetes more than 5 years. 4.79% of patiens were in inadequate treatment before admisson. 5. There was no relationship between fasting blood sugar level and development of gangrence. 6. The most common site of the lesion was big toe. 7. The most common predisposing fector was local pressure (39%), then minor trauma, and burn injury. 8. The primary wound healing rate was 53% after operation. 9. There was no relationship between the lowest palpable artery and primary wound healing. 10. The most common combined complication of diabetes was retinopathy (47%), then neuropathy and hypertension. 11. The rate of bacterial infection was 66%, and the most common organism was staphylococcus aureus (36%).
Adult ; Arteries ; Bacterial Infections ; Blood Glucose ; Burns ; Clinical Study ; Diabetes Mellitus ; Diabetic Foot ; Economic Development ; Fasting ; Gangrene ; Hallux ; Humans ; Hypertension ; Incidence ; Methods ; Protestantism ; Staphylococcus aureus ; Wound Healing ; Wounds and Injuries

No abstract available.
Humans ; Liver Cirrhosis* ; Liver* ; Sepsis* ; Vibrio cholerae* ; Vibrio*