No abstract available.
Eyelids* ; Sarcoidosis* ; Skin

Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops in the neonate within a few days and symptoms such as vomiting, lethargy, convulsion, coma follows rapidly. The diagnosis is supported by high citrulline level in serum, urine, CSF and decreased activity of argininosuccinic acid synthetase in liver biopsy. We experienced a 3-day-old male neonate with poor activity, lethargy, convulsion, and coma who was diagnosed as citrullinemia by markedly elevated plasma and urine citrulline level with hyperammonemia. We report this case with brief review of the related literature.
Argininosuccinic Acid ; Biopsy ; Citrulline ; Citrullinemia* ; Coma ; Diagnosis ; Humans ; Hyperammonemia ; Infant, Newborn ; Lethargy ; Ligases ; Liver ; Male ; Plasma ; Seizures ; Urea ; Vomiting

Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops in the neonate within a few days and symptoms such as vomiting, lethargy, convulsion, coma follows rapidly. The diagnosis is supported by high citrulline level in serum, urine, CSF and decreased activity of argininosuccinic acid synthetase in liver biopsy. We experienced a 3-day-old male neonate with poor activity, lethargy, convulsion, and coma who was diagnosed as citrullinemia by markedly elevated plasma and urine citrulline level with hyperammonemia. We report this case with brief review of the related literature.
Argininosuccinic Acid ; Biopsy ; Citrulline ; Citrullinemia* ; Coma ; Diagnosis ; Humans ; Hyperammonemia ; Infant, Newborn ; Lethargy ; Ligases ; Liver ; Male ; Plasma ; Seizures ; Urea ; Vomiting

This clinical trial was undertaken on 32 infants and chhildren who had G-I symptoms caused by a few diseases, such as meningitis, parenteral infection and intestinal infection. Out of 32 patients, six had vomiting only, caused by meingitis and habitual vomiting, eighteen had vomiting and diarrhea both which caused by parenteral infection and rest of them (8) had diarrhea only, caused by intestinal infection. Polybutine syrup, which 5.0 ml contain 24 mg of trimebutine, was given to these patients as follows; 2.5 ml twice a day at 6 months of age, 5.0 ml twice a day at 6 months to one year and 10 ml three times a day at 1-5 years of age. In ten of 24 patients, who had vomiting only (6), vomiting and diarrhea (18), the symptoms were improved within 48 hours, and rest of them (10) was also shown the improvement of their symptoms within 5 days after medication. In comparison of polybutine treated group and not treated group, the effectiveness of polybutine treatment for the symptoms was statistically meaningful.
Diarrhea ; Humans ; Infant ; Meningitis ; Trimebutine ; Vomiting

Background: The rapid evolution of artificial intelligence (AI), particularly large language models such as OpenAI’s ChatGPT, has significantly impacted various domains including healthcare. Dermatology can potentially benefit from the integration of ChatGPT. Objective: To analyze the performance of ChatGPT in dermatology, with particular focus on understanding the model’s capabilities and limitations when addressing dermatology questions. Methods: We employed 144 questions from Korean Dermatology Residency Evaluation Examinations. Questions were formatted consistently and fed to both GPT-4 and GPT-3.5 models. Performance was qualitatively assessed based on accuracy, completeness, and logical reasoning. Reasons for wrong answers were analyzed. Results: The overall correctness rate for GPT-4 was 70.8% and 59.0% for GPT-3.5. In terms of accuracy, 70.1% of the explanations were completely accurate. A total of 90.3% of responses provided by GPT-4 were complete in terms of content. Illogical reasoning was detected only in 4.9% of the answers. A total of 69.0% of wrong answers were attributed to information errors, followed by logical errors in 16.7%. There was no significant difference in the correctness rates among question types, but the correctness rate decreased significantly with question difficulty. ChatGPT showed near-perfect consistency, demonstrating a 97.9% agreement and a Cohen’s kappa of 0.958. Conclusion This study demonstrated the potential of ChatGPT in dermatology knowledge retrieval, outperforming dermatology residents in terms of correctness rate, completeness, and accuracy. There were more information errors than logical errors, and the inaccuracy caused by these errors was identified as the main limiting factor of ChatGPT.

BACKGROUND/AIMS: Insertion of self-expanding metallic stents (SEMS) has become an established option for the palliation of malignant biliary obstruction (MBO). However, stent occlusion by tumor ingrowth is still an unsolved problem. To overcome this problem, membrane-covered SEMSs have been developed. The purpose of this study was to compare the effectiveness of membrane-covered SEMS versus plastic stent in the treatment of MBO. METHODS: Between Jan 2001 and Jul 2002, 57 patients with unresectable MBO who initially had plastic stents inserted were randomized to receive either plastic stent and membrane-covered SEMSs after initial plastic stent failure. RESULTS: Placement of either plastic stents or membrane-covered SEMs was successful in all patients. Mean patency duration of stents were significantly longer in membrane-covered SEMS group (189 days) than in plastic stent group (82 days) (p=0.01). The causes of stent failure were occlusion in 10 cases and migration in 1 case in membrane-covered SEMS group. Of these occluded 10 cases, tumor ingrowth was noted in 7, tumor overgrowth in 2, and biliary incrustation in 1. CONCLUSIONS: Membrane-covered SEMS has a longer patency than plastic stent. However, new membrane covering material is needed to prevent tumor ingrowth more effectively.
Humans ; Membranes ; Plastics* ; Stents*

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare autosomal dominant vascular disorder involving arteriovenous malformation. HHT is characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations. Hepatic arteriovenous malformation can lead to high output heart failure. We report a case of hereditary hemorrhagic telangiectasia patient who complained dyspnea and edema on both lower extremity.
Arteriovenous Malformations ; Dyspnea ; Edema ; Epistaxis ; Heart Failure* ; Heart* ; Humans ; Lower Extremity ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis

Adenoaquamous carcinoma of stomach is a extremely rare malignant tumor which is composed of the mixed giandular-epidermoid neoplastic comyonnts. The pathogenesis of this neoplasm remains obscure and has various different theories, such as the origin of squamous components are island of ectopic squamous epithelium in the, gastric mucosa, squamous metaplasia of gastric epithelium, squamous differentiation in a preexisting adenocarcinoma, endotheliai cell differentiated toward squamous elements, and totipotential undifferentiated cells of the gastric mucosa. Primary adenosquamous carcinoma(ASC) was classified into two types, differentiated type adenocarcinomatous component(DAC) and undifferentiated type adenocarcinomatous component(UAC), according to the degree of glandular formation of the adenocarcinomatous elements. As a consequence, with respect to biologic behavior, ASC with DAC was similar to the differentiated type adenocarinoma, and ASC with UAC to the undifferentiated type adenocarcinoma. Accodingly, the behavioristic features of ASC seemed to be governed by the adenocarcinomatous component. We reported a case of primary gastric adenosquamous carcinoma with undifferentiated type adenocarcinomatous component.
Adenocarcinoma ; Carcinoma, Adenosquamous* ; Epithelium ; Gastric Mucosa ; Metaplasia ; Stomach Neoplasms ; Stomach*

We experienced a rare case of sacral meningeal cyst so called sacral perineurial cyst(Tarlov cyst). A 15 years old girl was admitted to the hospital because of sudden severe low back pain and Lt. sciatica for about one week. We performed delayed myelography and spinal computeized tomographic myelography(C.T.M) for differential diagnosis. Delayed filling of contrast media in the cyst was significant in diagnosis of sacral meningeal cyst type II. Laminectomy of sacrum(S1) was performed for partial excision of cystic wall and plastic suture. Operative finding was typical sacral perineurial cyst(Tarlov cyst) which was recently clssified as sacral meningeal cyst type II. The classification of spinal meningeal cysts in the literature was indistinct and confused. We also agree with Nabors et. al, in current classificcation of spinal meningeal cysts, in which the spinal meningeal cysts, in which the spinal meningeal cysts were divided into 3 groups as extradural spinal Mcs Type I(Diverticulum), Type II(perineurial cyst), intradural spinal Mcs(arachnoid cyst).
Adolescent ; Classification ; Contrast Media ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Laminectomy ; Low Back Pain ; Myelography ; Plastics ; Sciatica ; Sutures