Chylothorax is the most common cause of pleural effusion in the neonatal period and is defined as an effusion of lymph in the pleural cavity. We report a case of chylothorax in a 13-day-old male who was admitted due to respiratory difficulty. Chest AP showed pleural effusion of the left lung and milky yellow fluid was aspirated via thoracentesis upon which laboratory and lipoprotein electrophoresis of pleural fluid revealed findings compatible with that of chylothorax. Due to reaccumulation of chyle after daily thoracentesis, chest tube was inserted at the 4th hospital day, and MCT containing diet was given. He was discharged on the 21st hospital day in good health, and revealed no recurrence for 3 months during outpatient follow up visits.
Chest Tubes ; Chyle ; Chylothorax* ; Diet* ; Electrophoresis ; Follow-Up Studies ; Humans ; Infant, Newborn ; Lipoproteins ; Lung ; Male ; Outpatients ; Pleural Cavity ; Pleural Effusion ; Recurrence ; Thoracostomy* ; Thorax ; Triglycerides*

Most of the peripheral nerve injuries from crushing or compressive forces are accompanied by surrounding soft tissue injuries. As a result, poor vascularity due to fibrosis and sacr formation compromises regeneration of the grafted nerve. Vascularized nerve graft shows superior regeneration to that of a non-vascularized one. However, the human body provides few donor sites of vascularized nerve graft clinically. We presumed that the prefabricated myoneural or adiponeural flap, which include fabricated nerves wrapped with surrounding vascularized muscle or adipose tissue flap, influences superiorly on the regeneration of grafted nerve because that surrounding vasculatity indirectly enhances the vascularity of the grafted nerve itself. Thirty adult male Sprague-Dawley rats were divided into three groups: 1) conventional reversed autogenous graft of the femoral nerve alone(n=10); 2) nerve graft entubulated with abdominal adipose tissue flap with a pedicle of inferior epigastric artery(n=10); 3) nerve graft entubulated with adductor muscle flap with a pedicle of the first muscular branch of the femoral artery(n=10). At three months postoperatively, grafted nerves were examined by electrophysiologic study to check amplitudes and motor nerve conduction velocities, as well as histopathologic study for evaluation of regenerated nerve cells, fibrosis and neo-vascularization. Consquently, nerve regeneration was found in all three groups. Both the myoneural and adiponeural flap groups had better improved results of nerve regeneration compared to that of the conventional nerve graft group. The result of myoneural flap group was superior to that of the adiponeural flap group. The myoneural flap group showed minimal fibrosis and less prominent neovascularization around moderately regenerated nerves. The adiponeural flap group showed more severe perineural and endoneural fibrosis, as well as vascular proliferation around focal regenerated nerves. The results of myoneural flap group proved to be statistically significant. We concluded that it is possible to use nerve graft entubulated with a vascularized muscle flap (myoneural flap) as a substitute for vascularized nerve graft.
Abdominal Fat ; Adipose Tissue ; Adult ; Femoral Nerve ; Fibrosis ; Human Body ; Humans ; Male ; Nerve Regeneration* ; Neural Conduction ; Neurons ; Peripheral Nerve Injuries ; Rats, Sprague-Dawley ; Regeneration ; Soft Tissue Injuries ; Tissue Donors ; Transplants*

The origin of Paget cells in extramammary Pagets disease is uncertain, however, some ultrastructural surveys revealed differentistion of Paget cells toward sweat gland apparatus. This is supported by recent immunohistochemical studies that Paget cells are irnmunohistochemically shown to react with antikeratin monoclonal antibodies specific to simple epithelia. In this siudy, seven cases of extramammary Pagets disease were immunohistochemically investigated with adenokeratin, carcinoembryonic antigen(CEA), involucrin by peroxidase-antiperoxidase(PAP) techniques. The results were as follow : l. Epidermis, hair follicies and sebaceous glands revealed negative immunoreacativity for adenokeratin in all cases. Sweat glands(6/7), Paget cells(6/7) and underlying adenocarcinoma revealed positive immunoreactivity for adenokeratin. 2. Epidermis, hair follicles and sebaceous glands(6/7) revealed negative immunoreactivity for CEA. Sweat glands, Paget cells and underlying adenocarcinoma revealed positive immunoreactivity for CEA in all cases. 3. Lower epidermis, sweat glands(6/7), Paget cells and underlying adenocarcinorna revealed negative irnmunoreactivity for involucrin. Upper epidermis, hair follicles and sebaceous glands(5/7) revealed positive immunoreactivity far involucrin. Based on these results, Paget cells of extramammary Pagets disease are considered to be derived from the sweat glands.
Adenocarcinoma ; Antibodies, Monoclonal ; Carcinoembryonic Antigen* ; Epidermis ; Hair ; Hair Follicle ; Paget Disease, Extramammary* ; Sebaceous Glands ; Sweat ; Sweat Glands

PURPOSE: We report clinical and radiologic outcomes after metacarpal extension osteotomy for mild osteoarthritis of the thumb carpometacarpal joint. MATERIALS AND METHODS: From 1999 to 2008, 11 patients were diagnosed with mild thumb carpometacarpal arthritis (Eaton stage I, II), and extension osteotomies were performed. Of these, seven patients with at least 6 years follow-up were analyzed retrospectively. Male to female ratio was 2:5, and mean age at time of surgery was 38.9 years old. Symptom onset period was a mean of 11.2 months. Two patients were I, and five patients were II in Eaton stage. Preoperative visual analogue scale (VAS) and disabilities of the arm, shoulder and hand scale (DASH) scores were 3.7 points (3-4 points), and 40.1 points (32-51 points). Radial abduction was 38.5degrees (30degrees-45degrees), and volar abduction was 42.1degrees (40degrees-45degrees). Grip strengths and pinch powers, compared with the normal contralateral side were 82% (64%-90%) and 72% (40%-100%), respectively. RESULTS: The mean follow-up period was 8.5 years, and all patients except one maintained their occupational activity during the follow-up period. Final VAS and DASH scores were 0.7 points (0-2 points) and 11.7 points (8-16 points), respectively, and were statistically significant. Volar abduction, grip strengths, and pinch power were improved to 45degrees (40degrees-50degrees), 92.3% (73%-117%), and 94.4% (75%-117%) with statistical significances. In five patients, Eaton stages did not change, and two patients advanced to the next stage (stage I to II in one patient, stage II to III in one patient). CONCLUSION: Among the various treatment options for mild thumb carpometacarpal arthritis, metacarpal extension osteotomy may be considered as an effective treatment.
Arm ; Arthritis* ; Carpometacarpal Joints ; Female ; Follow-Up Studies ; Hand ; Hand Strength ; Humans ; Male ; Osteoarthritis ; Osteotomy* ; Retrospective Studies* ; Shoulder ; Thumb*

Congenital lipoid adrenal hyperplasia is the most severe form of CAH, leading to impaired production of all steroid hormones including glucocorticoids, mineralocorticoid, and sex steroids. The affected individuals are all phenotypically female with a severe salt-losing syndrome that is fatal if steroid replacement is not begun immediately after birth. The lesion of this disorder has been suggested to be in the first step of steroidogenesis of conversion of cholesterol to pregnenolone by P450scc. Recently, molecular defect of this disease has been located in the transport of cholesterol into mitochondria due to defective regulatory protein called 'steroidogenic acute regulatory protein' while the enzyme P450scc itself is normal, differing from other types of congenital adrenal hyperplasia. We experienced 2 1/2 month old phenotypical girl who was admitted due to lethargic state and persistent vomiting with severe hyperkemia and hyponatremia. Blood levels of cortisol, aldosteron, and 17-OH progesteron were low and levels of ACTH, angiotensin, and plasma renin activity were high, urinary levels of 17-KS and 17-OHCH were low. The patient was found to have karyotype of 46, XY and has been being treated with predinisolone, fluorocortisol and sodium supplement in diet and doing well. The molecular study for P450scc gene and StAR gene of patient and family is in progress.
Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Angiotensins ; Cholesterol ; Diet ; Female ; Glucocorticoids ; Humans ; Hydrocortisone ; Hyperplasia* ; Hyponatremia ; Karyotype ; Mitochondria ; Parturition ; Plasma ; Pregnenolone ; Renin ; Sodium ; Steroids ; Vomiting

Williams syndrome(Williams-Beuren Syndrome, Williams elfin facies syndrome) is a rare syndrome and this is the first case in Korea. We experienced a case of Williams syndrome who was a 11 year old male child. He showed a characteristic facial appearance of this syndrome. A systolic thrill was palpable over the upper sternal borders and in the suprasternal notch. A Grade 3 (on the basis of 6) ejection systolic murmur was heard maximally over the right and left upper sternal borders and radiated over the entire precordium and into the neck. No manifestations of congestive cardiac failure were present. Pectus excavatum and inguinal hernia were observed. he also had mental retardation (IQ=66). Diagnosis was established by angiocardiography, and also aided by EKG and phonocardiography. A brief review of the literatures was done.
Angiocardiography ; Aortic Stenosis, Supravalvular* ; Child ; Diagnosis ; Electrocardiography ; Estrogens, Conjugated (USP) ; Facies ; Funnel Chest ; Heart Failure ; Hernia, Inguinal ; Humans ; Intellectual Disability ; Korea ; Male ; Neck ; Phonocardiography ; Systolic Murmurs ; Williams Syndrome

Spontaneous pneumomediastinum is rare in children, mainly affecting male adolescents. It usually occurs secondary to alveloar rupture in the pulmonary interstitium, followed by dissection of gas towards the hilum and mediastinum. Many pathological and physiological events can lead to alveolar rupture, but the most common cause in children is asthma. The most frequent triggers in the pediatric age group are asthma, vomiting of any cause, situations reproducing the Valsalva maneuver (e.g., shouting, coughing), and intense sport activities. We report two cases of pneumomediastinum, occuring in a patient with vigorous coughing and pneumonia and in a patient with dyspnea and pneumonia. We describe the clinical and radiologic findings of two cases. In addition, we have made a comprehensive review of the literature on spontaneous pneumomediastinum in children.
Adolescent ; Asthma ; Child ; Cough ; Dyspnea ; Humans ; Mediastinal Emphysema* ; Mediastinum ; Pneumonia ; Rupture ; Sports ; Subcutaneous Emphysema ; Valsalva Maneuver ; Vomiting

Despite their existence all around, primary cutaneous infections caused by Serratia marcescens are still fairly rare. In many cases, symptoms caused by S. marcescens usually manifest as opportunistic infections in pulmonary, urinary and digestive organs of immunocompromised patients. It is unusual to find manifestations as "primary" cutaneous infection. A 56-year-old woman presented to our hospital with ulcerative skin lesions. She had a medical history of poorly controlled diabetes mellitus for 5 years. Multiple nodules and ulcerative lesions of various sizes were distributed on the abdomen, both buttocks and thighs. Two biopsy samples revealed deep dermal and subcutaneous suppurative inflammation. Two culture samples from the skin biopsy sites demonstrated S. marcescens. Small-to-medium sized lesions showed improvements with antibiotic treatment for 14 days. However, for large sized lesions, partial-thickness skin grafts were done.
Abdomen ; Biopsy ; Buttocks ; Diabetes Mellitus ; Ecthyma ; Female ; Humans ; Immunocompromised Host ; Inflammation ; Middle Aged ; Opportunistic Infections ; Serratia ; Serratia marcescens ; Skin ; Thigh ; Transplants ; Ulcer

Exraadrenal paragangliomas are rare tumors of a neural crest origin. Their preoperative diagnosis can be suspected from the clinical history and biochemical examination of the catecholamines or their metabolic by-products. Surgery is the mainstay of therapy, but surgical removal makes an additional problem for the repair of the ureteral defect if a paraganglioma is located near the ureter or if it encloses the ureter. The ureteral repair often poses a therapeutic dilemma, and especially when the ureteral defect is too extensive to be repaired with traditional methods. We report here on a case of successful replacement with using appendix for the repair of a long ureteral defect that was caused by a retroperitoneal paraganglioma.
Appendix* ; Catecholamines ; Diagnosis ; Neural Crest ; Paraganglioma* ; Ureter*

The clinical studies were performed on 757 patients who had been admitted to Severance Hospital from May, 1964 to June, 1975 with the diagnosis of congenital heart disease. The following results were obtained; 1. Among 757 patients, cardiac catheterization was performed on 367 patients. 2. Four hundred and thirty eight patients were male and 319 were female. Over half of them were below two years of age, but among 357 cardiac catheterized patients, 137 (37.3%) patients were ranged between 6 to 10 years. 3. V.S.D., Tetralogy of Fallot, P.D.A., A.S.D. were found in order of frequency. 4. Among 757 patients, combined extra-cardiac anomalies were found in 42 patients, and 14 patients were with Downanjx syndrome. 5. Among 367 cardiac catheterized patients, 164 (44.7%) patients had cardiac operations, and among these, 18 (11.0%) patients died including 7 patients of Tetralogy of Fallot.
Cardiac Catheterization ; Cardiac Catheters ; Diagnosis ; Female ; Heart Defects, Congenital* ; Humans ; Male ; Tetralogy of Fallot