Dyskeratosis congsnita is a rare congenital disorder characterized by the triad of reticular pigmentation of the skin. dystrophic naila, and leukoplakia of the mucous membrane, and is often associated with severe pancytopenia. A 9-year-old boy had reticular pigmentation of the skin, dystropbic changes of the finger and toe nails, white patches of the buccal mucosa, mild hyperkeratosia of the palms and soles, excesaive lacrimation, dysphagia and severe pancytopenia, Bone marrow showed hypoplastic anemia and decreased cell mediated immunity was noticed.
Anemia, Aplastic ; Bone Marrow ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Deglutition Disorders ; Dyskeratosis Congenita* ; Fingers ; Humans ; Immunity, Cellular ; Leukoplakia ; Male ; Mouth Mucosa ; Mucous Membrane ; Pancytopenia ; Pigmentation ; Skin ; Toes

No abstract available.
Danazol* ; Humans

Neuroleptic malignant syndrome is a rare, but potentially life-threatening adverse event associated with the use of neuroleptic agents. We describe the case of a 47-year-old schizophrenic woman who was treated with clozapine for years. The patient developed acute renal failure with pulmonary edema, and underwent mechanical ventilation and hemodialysis.
Acute Kidney Injury ; Antipsychotic Agents ; Clozapine* ; Female ; Humans ; Middle Aged ; Neuroleptic Malignant Syndrome* ; Pulmonary Edema ; Renal Dialysis ; Respiration, Artificial ; Rhabdomyolysis

A clinical observation on postoperative course in 21 cases among 35 uremic patients were performed. Seven of 36 cases had undergone kidney transplantation and were excluded from this report, And the other 7 were non-operated cases. Remaining 21 of obstructive uremic cases had surgical procedures such as urinary diversion. release of stenosis and removal of obstruction. The results of operated 21 and non-operated 7 cases were summarized as follows: 1. The incidence was the highest in the age of 31 to 40 (46.4%). 2. The most common cause of uremia was chronic glomeru1onephritis (25.7%). The next was urinary tuberculosis causing renal parenchymal damage and urinary tract obstruction (17.1%). 3. Non-operated cases showed poor prognosis in almost all cases. 4.Satisfactory results were obtained from 14 cases among 21 operated cases(66.6%).
Constriction, Pathologic ; Humans ; Incidence ; Kidney Transplantation ; Prognosis ; Tuberculosis ; Uremia ; Urinary Diversion ; Urinary Tract

No abstract available.
Enbucrilate* ; Facial Bones*

We described a 65-year-old woman with a papule on the left temple area of } year duration. The lesion showed typical findings of atypical fibroxanthoma and was located on the lesion of solar keratosis, So sunlight may be the most important one among the various insults to atypical fibroxanthoma of the skin.
Aged ; Female ; Humans ; Keratosis ; Skin ; Sunlight

A clinical observation on ureteral tailoring with anti-reflux procedure on 6 ureters of 5 patients were reported herein. Four ureters of 3 children were hydroureteronephrosis with vesicoureteral reflux due to bladder neck contracture. Two ureters of housewives were megaloureter. The results of 5 cases were summarized as follows: 1. The length of tailoring was 8 cm. in average. 2. Tailored ureters were approximated by continuous suture plus simple sutures in 2 ureters of 1 case, everting plus simple sutures in 3 cases and the other was by everting sutures alone. No difference in methods of approximation was found in result. 3. All cases had undergone antireflux procedures. Five ureters of 4 cases were done by Politano-Leadbetter technic and the other one by Paquin technic. 4. Mean duration of splinting was some 2 weeks. We believe that splinting is essential for more than 10 days. 5. One case was died of septicemia on 7th postoperative day. Another case shows only unsterile urine yet. Satisfactory results were obtained from remaining 3 cases.
Child ; Contracture ; Humans ; Neck ; Sepsis ; Splints ; Sutures ; Ureter* ; Urinary Bladder ; Vesico-Ureteral Reflux

No abstract available.
Rupture* ; Thorax*

BACKGROUND: Surgical correction of the full spectrum of esophageal atresia with tracheoesophageal fistula has improved over the years, but the mortality and morbidity assoiated with repair of these anomalies still remains high. MATERIAL AND METHOD: We retrospectively analyzes 27 surgically treated patients with esophageal atresia and tracheoesophageal fistula at Dong-A University Hospital between January 1992 and March 1997. RESULT: There were 21 male and 6 female patients. Mean birth weight was 2.62+/-.385 kg(2.0~3.4 kg). Twenty- four(88.9%) had esophageal atresia with distal tracheoesophageal fistula, and 3(11.1%) had pure esophageal atresia. Four(14.8%) infants were allocated to Waterston risk group A, 18(66.7%) to group B, and 5(18.5%) to group C. In eighteen(66.7%) infants with associated anomalies, cardiovascular anomalies were the most common. Three had a gap length of 3.5 cm or greater(ultra-long gap) between esophageal segments, 7 had 2.0 to 3.5 cm(long gap), 8 had 1.0 to 2.0 cm(medium gap), and 9 had 1 cm or less(short gap) gap length. Among 27 neonates, 3 cases underwent staged operation, late colon interposition was done in 2, and all other 24 cases underwent primary esophageal anastomosis. Oerative mortality was 2/27(7.4%). Causes of death included acute renal failure(n=1), empyema from anastomotic leak(n=1), necrotizing enterocolitis(n=1), sepsis(n=1), insulin-dependent diabetus mellitus(n=1 . There were 4 anastomosis- related complications including stricture in 3, leakage in 1. Mortality was related to the gap length(p<.05). CONCLUSION: Although the complication rate associated with surgical repair of these anomalies is high, this does not always implicate the operative mortality. The overall survival can be improved by effective treatment for combined anomalies and intensive postoperatve care.
Birth Weight ; Cause of Death ; Colon ; Constriction, Pathologic ; Empyema ; Esophageal Atresia* ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Mortality ; Retrospective Studies ; Tracheoesophageal Fistula

BACKGROUND: Drug-induced Parkinsonism(DIP) is the second commonest cause of Parkinsonism, after idiopathic Parkinson's disease(IPD). DIP is frequently produced by antipsychotic drugs. But the clinical characteristics of DIP did not get attention by neurologist. So we studied the clinical profiles of DIP patients. METHODS: We studied the clinical profiles of thirthone patients who showed parkinsonism after antipsychotic drug treatment. We compared the score of motor part of the Unified Parkinson's Disease Rating Scale(UPDRS) between trihexyphenidyl(n=15) & amantadine(n=16) monotherapy group(initial & 4 week after treatment). RESULTS: The mean age of patients was 45 years. Bradykinesia was the 1st symptom in 26 patients(94%), tremor in 5 patients(6%). In 25 patients(81%), the first symptom appeared within 1 week after sntipsychotic treatment. There was a statistical significant negative correlation between the dosage of antipsychotic drug and the symptom-onset interval following treatment with antipsychotic drugs(simple correlation analysis, p>0.01). Bradykinesia and rigidity were appeared in all DIP patients, symmetric distribution was more common(94%, 87%) Tremor occurred in 27 patients (87%). In patients with tremor, postural or action tremor was dominant in 15 patients(56%) asymmetric distribution was more common(16/27, 59%). There are no statistical difference in motor score of UPDRS between trihexyphenidyl & amantadine monotherapy group(student t-test, p<0.05) CONCLUSIONS: Bradykinesia was the most common 1st symptom in DIP patients. Asymmertrical postural or action tremor was relativelly common in DIP. Amantadine showed the same efficacy in the treatment of DIP compared to anticholinergics.
Amantadine ; Antipsychotic Agents ; Cholinergic Antagonists ; Humans ; Hypokinesia ; Parkinson Disease ; Parkinsonian Disorders* ; Tremor ; Trihexyphenidyl