No abstract available.
Multiple Endocrine Neoplasia Type 2a* ; Multiple Endocrine Neoplasia*

No abstract available.
Multiple Endocrine Neoplasia Type 2a* ; Multiple Endocrine Neoplasia*

We report a patient with pachydermoperiostosis accompanied by hypertrophic gastritis. A 26-year-old man showed deep folds and furrows of the face and scalp, and terminal spade-like expansion of fingers and toes. Physical examination revealed no abnormalities except a grotesque appearance. Results of routine laboratory tests were unremarkable. Mild periosteal reactoins of both femurs and humeri were noted on long bone series, and hypertrophic gastritis on fibroscopic examination drew our attention. The relationship between pachydermoperiostosis and hypertrophic gastritis is uncertain. However it is probable that hypertrophic gastritis may be the endodermal counterpart of the ectodermal manifestations of pachydermoperiostosis.
Adult ; Ectoderm ; Endoderm ; Femur ; Fingers ; Gastritis, Hypertrophic* ; Humans ; Osteoarthropathy, Primary Hypertrophic* ; Physical Examination ; Scalp ; Toes

Congenital chylothorax is a rare disorder and can be diagnosed by prenatal ultrasonography recently. Most cases of congenital chylothorax were characterized by different clinical courses of respiratory distress. We describe two female cases with congenital chylothorax observed by ultra- sonography prenatally. In the first case, left-sided pleural effusion was noted by prenatal ultrasonography taken at 34 weeks of gestation, and then pleural fluid was extracted by intrauterine thoracentesis under sonography guidance. After birth, this patient was managed by TPN(total parentral nutrition) and intermittent thoracentesis without surgical treatment. But, pleural fluid was accumulated recurrently and respiratory distress was aggravated. At 15th hospital day, shock state was developed and patient died. In the second case, bilateral pleural effusion and ascites were noted by prenatal ultrasonography, and then patient was delivered immediately without intrauterine thoracentesis. After birth, the second case received conservative therapy including mechanical ventilation, TPN, intermittent thoracentesis and paracentesis. The patient was discharged with complete regression of chylothorax. We report the two cases with brief review of related literatures.
Ascites ; Chylothorax* ; Female ; Humans ; Paracentesis ; Parturition ; Pleural Effusion ; Pregnancy ; Respiration, Artificial ; Shock ; Ultrasonography, Prenatal*

Myopathy in primary aldosteronism in relatively rare disease in Korea. A 42-year-old woman with hypokalemic periodic paralysis, proximal muscle weakness and hypertension was found to have myopathy associated with adenoma in the right adrenal gland. She showed marked elevation of muscle enzymes and myopathic pattern in EMG.
Adenoma ; Adrenal Glands ; Adult ; Female ; Humans ; Hyperaldosteronism ; Hypertension ; Hypokalemic Periodic Paralysis ; Korea ; Muscle Weakness ; Muscular Diseases* ; Rare Diseases

Scapular fractures are being seen with increasing frequency in our mechanized society, specially in patients who have multiple injuries. Most of the scapular fractures have been treated non-operatively, with early mobilization of the shoulder. The result of such functional treatment have been predictably good. However, this non-operative approach may not yield such good results in patients who have a fracture of the scapular neck in association with the ipsilateral clavicular fracture or other double disruptions of the superior shoulder suspensory complex. Ipsilateral fractures of the scapular neck and clavicle disrupt the suspensory mechanism of the shoulder resulting in fuctional loss and deformity. From January 1989 to June 1995, twelve patients were identified with this specific pattern of injury. The follow up period was 39.2 months in average. Seven patients were treated operatively using 3.5mm DCP or reconstruction plate, and five patients were performed K-wire fixation or wiring. Upon review all were healed radiographically. According to the scoring system of Rowe, five patients had an excellent fuctional result, five had a good result, and two had a fare result. The average score for the twelve patients was 85 points. The results of our retrospective study show that operative treatment for these complex fractures is safe and that functional recovery is predictably good.
Clavicle* ; Congenital Abnormalities ; Early Ambulation ; Follow-Up Studies ; Humans ; Multiple Trauma ; Neck* ; Retrospective Studies ; Shoulder

Burst fractures of lower lumbar spine are rare and have not been well delineated in the literature. Treatment and management considerations for lower lumbar burst fractures are somewhat different from the rest of spine because this area only has the cauda equina and nerve roots. The purpose of this study was to analyse the clinical and radiological results and to determine operative approach. The authors analysed the 16 patients who had been treated with spinal instrumentation (Cotrel- Dubousset, Danek, Kaneda) from Dec. 1990 to Dec. 1994, and following results were obtained. 1. The classification of fracture by Francis-Denis method was B type in 7 cases, A type in 6 cases, E type in 2 cases, and D type in 1 case. 2. By the load-sharing classification, six points or less in 11 patients (first group) and seven or more in 5 patients (second group). In first group lordosis was corrected from 20.4degreespreoperatively to 32.6degrees postoperatively and 24.1degrees at the end of follow-up. In second group lordosis was corrected from 14.8degrees preoperatively to 21.3degrees postoperatively and 0.8degrees at the end of follow-up. 3. The short segmental fixation through posterior approach yielded good result in the first group, but poor result in the second group with complications including two cases of loss of reduction and one case of screw breakage. As a result, posterior approach might be more perferable in first group and anterior approach in the second group.
Animals ; Cauda Equina ; Classification ; Follow-Up Studies ; Humans ; Lordosis ; Spine

Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia. The pathogenic mechanisms of this endocrinopathy are not clear. We experienced a 19-year-old male with hypersecretion of GH, hyperprolactinemia and fibrous dysplasia. He was referred for evaluation of suspected acromegaly. He had no skin pigmentation. Plasma GH, PRL, somatomedin-C, LH, FSH, testosterone, estradiol, progesterone, T3, T4, TSH and cortisol were measured. Among those, the levels of plasma GH, PRL and somatomedin-C were high. Serum alkaline phosphatase was increased. OGTT did not suppress plasma OH concentration and GH showed paradoxical response to TRH and LHRH. GH was suppressed after a test-dose of somatastatin and bromocriptine. Brain MRI demonstrated a mass lesion in sella turcica and another mass lesions in nasal cavity and posterior occipital bone. Whole body bone scan revealed increased uptake in skull, nasal bone, both 9th posterior rib, both femurs, both tibias, left scapular and pelvic bone. These fmdings were consistent with bone tumor such as fibrous dysplasia. We report a case with incomplete MeCune-Albright syndrome including acromegaly, hyperprolactinemia and polyostotic fibrous dysplasia.
Acromegaly ; Alkaline Phosphatase ; Brain ; Bromocriptine ; Estradiol ; Femur ; Fibrous Dysplasia, Polyostotic ; Glucose Tolerance Test ; Gonadotropin-Releasing Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Magnetic Resonance Imaging ; Male ; Nasal Bone ; Nasal Cavity ; Occipital Bone ; Pelvic Bones ; Plasma ; Progesterone ; Ribs ; Sella Turcica ; Skin Pigmentation ; Skull ; Testosterone ; Tibia ; Young Adult

No abstract available.
Umbilical Arteries*

A 54 year old man who had taken puffer fish and noticed generalized weakness 1 hour and 30 minutes afterward was admitted to our I.C.U. due to respiratory arrest and cardiac arrhythmia. He was treated with artificial respiration via pressure cycled respirator, supportive therapy including frequent endotracheal suctioning. fluid administration, correction of acid base imbalance, change of position and antibiotics for prevention of secondary infection. 31 hours afterward, he resumed normal respiration and clear mentality. After 5 days of total admission days, he was discharged without any sequelae.
Acid-Base Imbalance ; Anti-Bacterial Agents ; Arrhythmias, Cardiac ; Coinfection ; Poisoning* ; Respiration ; Respiration, Artificial ; Respiratory Paralysis* ; Suction ; Tetraodontiformes ; Tetrodotoxin* ; Ventilators, Mechanical