Hydrofluoric acid is a colorless gas or fuming liquid with a strong, irritating odor. Hydrofluoric acid burns are uncommon; however, if severe burn occur, they caused death from systemic effects, such as fatal cardiac arrthmia. We experienced two cases of hydrofluoric acid chemical burns on digits. These patients had typical clinical features of hydrofluoric acid chemical burns, such as pulsating pain at the burn site, as well as ischemic and necrotic skin changes. The hydrofluoric acid chemical burn was confirmed by a history of exposure. Subsequently, we made a calcium gluconate gel by mixing 20% calcium gluconate, an antagonist against hydrofluoric acid, with lubricant, and we injected 10% calcium gluconate subcutaneously when they complained of pain rated at higher than 5 on the pain scale. Simultaneously, we monitored the patients' electrocardiographs and checked their serum total calcium, ionized calcium, and magnesium levels serially. Clinical presentations and the emergency management of hydrofluoric acid chemical burns were reviewed along with the current literature. These patients were discharged without any significant complications.
Burns ; Burns, Chemical* ; Calcium ; Calcium Gluconate ; Electrocardiography ; Emergencies ; Humans ; Hydrofluoric Acid* ; Magnesium ; Odors ; Skin

Hydrofluoric acid is a colorless gas or fuming liquid with a strong, irritating odor. Hydrofluoric acid burns are uncommon; however, if severe burn occur, they caused death from systemic effects, such as fatal cardiac arrthmia. We experienced two cases of hydrofluoric acid chemical burns on digits. These patients had typical clinical features of hydrofluoric acid chemical burns, such as pulsating pain at the burn site, as well as ischemic and necrotic skin changes. The hydrofluoric acid chemical burn was confirmed by a history of exposure. Subsequently, we made a calcium gluconate gel by mixing 20% calcium gluconate, an antagonist against hydrofluoric acid, with lubricant, and we injected 10% calcium gluconate subcutaneously when they complained of pain rated at higher than 5 on the pain scale. Simultaneously, we monitored the patients' electrocardiographs and checked their serum total calcium, ionized calcium, and magnesium levels serially. Clinical presentations and the emergency management of hydrofluoric acid chemical burns were reviewed along with the current literature. These patients were discharged without any significant complications.
Burns ; Burns, Chemical* ; Calcium ; Calcium Gluconate ; Electrocardiography ; Emergencies ; Humans ; Hydrofluoric Acid* ; Magnesium ; Odors ; Skin

Dyskeratosis congenita (DC) is a rare genetic disorder encompassing abnormal skin pigmentation, dystrophic nails, leukoplakia of mucous membranes and others. Bone marrow failure is the cause of early mortality. Moreover, DC is known for its predisposition to malignancy. X-linked recessive, autosomal dominant and autosomal recessive forms of the disease are recognized. We describe here a rare case of DC in a 4-year-old girl showing dark skin, dystrophic toe nails, and mild bone marrow failure. Autosomal recessive disease was suggested as the patient is female, and tests for DKC1 and hTR mutations were negative. Intermittent treatment with oxymetholone and prednisolone for about 26 months resulted in stable hemoglobin and platelet response.
Blood Platelets ; Bone Marrow ; Child, Preschool ; Dyskeratosis Congenita* ; Female* ; Humans ; Leukoplakia ; Mortality ; Mucous Membrane ; Oxymetholone ; Prednisolone ; Skin ; Skin Pigmentation ; Toes

Veno-occlusive disease (VOD) of the liver is a life-threatening complication occurring early after blood or bone marrow transplantation (BMT). Effective treatment has not been established in case of severe forms of VOD. Defibrotide, a single-stranded polydeoxyribonucleotide, has been used on a compassionate basis in recent clinical trials with promising results. We report here with the first Korean experience of using defibrotide for the treatment of hepatic VOD occurring after unrelated umbilical cord blood transplant in a 2-year-old child with acute lymphoblastic leukemia. Defibrotide was administered for 23 days without any significant side effects with resolution of signs and symptoms of VOD.
Bone Marrow Transplantation ; Child ; Child, Preschool ; Empathy ; Fetal Blood* ; Hepatic Veno-Occlusive Disease* ; Humans ; Liver ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Umbilical Cord*

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder characterized by the proliferation of activated Langerhans cells. Although current therapies are very effective at inducing remission, multiple recurrences and long-term sequelae are common for young patients. For this reason, more effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2-chlorodeoxyadenosine (2-CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or refractory LCH. METHODS: Four children with recurrent or refractory LCH received 2-CdA (5~7 mg/m2/day for 5 days, given as a 24-hr continuous infusion and repeated every 21~28 days for 5~7 courses). RESULTS: All four patients had multiorgan involvement, and were heavily pretreated. Of the two children with recurrent diseases, one had complete response and the other showed no active disease except for the remaining diabetes insipidus. Two infants who showed poor early response to previous combination chemotherapy also responded poorly: partial response in one, and progressive disease resulting in death in the other. Toxicity consisted mainly of myelosuppression, but significant infections did not occur. The peripheral neuropathy was not seen. CONCLUSION: 2-CdA, tolerable in children without significant side effects, might be effective for the treatment of recurrent LCH in children. However, the efficacy in infants with multi-system, refractory diseases needs further study. The feasibility of 2-CdA treatment as the first-line therapy for high-risk diseases, and the possibility of combination with other agents needs to be addressed in the future.
Child* ; Cladribine* ; Diabetes Insipidus ; Drug Therapy, Combination ; Histiocytes ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Langerhans Cells ; Lymphocytes ; Peripheral Nervous System Diseases ; Recurrence

Tufted angioma is a rare slowly progressive vascular tumor, characterized by histopathologic findings of numerous angiomatous lobules of "cannonball" appearance within the dermis. It affects children but occurs infrequently at birth. It appears as a clinical imitator of hemangioma of infancy (HOI), so it needs to be differentiated from HOI. Clinically, lesions most often present as red to purple, indurated or nodular plaque, and are usually located on the neck, upper trunk or proximal limb. We report six cases of tufted angioma of congenital onset. We undertook immunohistochemical study using CD31, D2-40 and GLUT1 to rule out other vascular tumors or malformations, particularly HOI. These cases may demonstrate the keys of differential diagnosis between HOI and tufted angioma, which share common features clinically and rarely histopathologically.
Child ; Dermis ; Diagnosis, Differential ; Extremities ; Hemangioma ; Humans ; Iodine Compounds ; Neck ; Parturition ; Skin Neoplasms

PURPOSE: To determine the sonographic findings of degenerative change in femoral articular cartilage of the knee by comparative study of specimen sonography and pathology. MATERIALS AND METHODS: We obtained 40 specimens of cartilage of the femur (20 medial and 20 lateral condylar) from 20 patients with osteoarthritis of the knee who had undergone total knee replacement. The specimens were placed in a saline-filled container and sonography was performed using a 10MHz linear transducer. Sonographic abnormalities were evaluated at the cartilage surface, within the cartilage, and at the bone-cartilage interface, and were compared with the corresponding pathologic findings. In addition, cartilage thickness was measured at a representative portion of each femoral cartilage specimen and was compared with the thickness determined by sonography. RESULTS: 'Dot 'lesions, irregularity or loss of the hyperechoic line, were demonstrated by sonography at the saline-cartilage interface of 14 cartilages. Pathologic examination showed that these findings corresponded to cleft, detachment, erosion, and degeneration. Irregularities in the hyperechoic line at the bone-cartilage interface were revealed by sonography in eight cartilages and were related to irregularity or loss of tidemark, downward displacement of the cartilage, and subchondral callus formation. Dot lesions, corresponding to cleft and degeneration, were noted within one cartilage. Cartilage thickness measured on specimen and by sonography showed no significant difference (p=0.446). CONCLUSION: Specimen sonography suggested that articular cartilage underwent degenerative histopathological change. Cartilage thickness measured by sonography exactly reflected real thickness.
Arthroplasty, Replacement, Knee ; Bony Callus ; Cartilage ; Cartilage, Articular* ; Femur ; Humans ; Knee* ; Osteoarthritis ; Pathology* ; Transducers ; Ultrasonography

BACKGROUND/AIMS: Few studies have assessed the prognostic value of the primary tumor maximum standardized uptake value (SUVmax) measured by 2-[18F]-fluoro-2-deoxy-D-glucose PET-CT for patients with bile duct and gallbladder cancer. METHODS: A retrospective analysis of 61 patients with confirmed bile duct and gallbladder cancer who underwent FDG PET-CT in Kangbuk Samsung Medical Center (Seoul, Korea) from April 2008 to April 2011. Prognostic significance of SUVmax and other clinicopathological variables was assessed. RESULTS: Twenty-three patients were diagnosed as common bile duct cancer, 17 as hilar bile duct cancer, 12 as intrahepatic bile duct cancer, and nine as gallbladder cancer. In univariate analysis, diagnosis of intrahepatic cholangiocarcinoma and gallbladder cancer, mass forming type, poorly differentiated cell type, nonsurgical treatment, advanced American Joint Committee on Cancer (AJCC) staging and primary tumor SUVmax were significant predictors of poor overall survival. In multivariate analysis adjusted for age and sex, primary tumor SUVmax (hazard ratio [HR], 4.526; 95% CI, 1.813-11.299), advanced AJCC staging (HR, 4.843; 95% CI, 1.760-13.328), and nonsurgical treatment (HR, 6.029; 95% CI, 1.989-18.271) were independently associated with poor overall survival. CONCLUSIONS: Primary tumor SUVmax measured by FDG PET-CT is an independent and significant prognostic factor for overall survival in bile duct and gallbladder cancer.
Aged ; Bile Duct Neoplasms/*diagnosis/mortality/radionuclide imaging ; Cholangiocarcinoma/diagnosis/mortality/radionuclide imaging ; Female ; Fluorodeoxyglucose F18/diagnostic use/metabolism/standards ; Gallbladder Neoplasms/*diagnosis/mortality/radionuclide imaging ; Humans ; Kaplan-Meier Estimate ; Liver Neoplasms/diagnosis/mortality/radionuclide imaging ; Male ; Middle Aged ; Neoplasm Staging ; Positron-Emission Tomography/standards ; Prognosis ; Proportional Hazards Models ; Radiopharmaceuticals/diagnostic use/metabolism/standards ; Retrospective Studies ; Tomography, X-Ray Computed/standards

BACKGROUND/AIMS: In clinical practice, among the technique to detected Helicobacter pylori (H. pylori) infection, IgG serological test is noninvasive, safe, quick, widely available, and inexpensive. We studied that whether the titers of anti-H. pylori IgG antibody were correlated with endoscopic finding, and the degree of microscopic gastric damage and H. pylori density in dyspeptic patients. METHODS: Gastric biopsy specimens were obtained in 109 patients with H. pylori infection undergoing upper gastric endoscopy. The titers of serum IgG antibodies to H. pylori were measured by enzyme immunoassay. Macroscopic gastric damages and histologic grades were scored by the Sydney system. RESULTS: Endoscopic findings showed no significant association with H. pylori antibody titers (p=0.111). There was significant correlation between H. pylori antibody titers and lymphocyte infiltration (p=0.002), neutrophil infiltration (p=0.002), H. pylori density (p=0.0001), respectively. There was no significant correlation between H. pylori antibody titers and atropy (p=0.142), intestinal metaplasia (p=0.368), respectively. CONCLUSIONS: H. pylori antibody titer has significant association with the H. pylori density, neutrophil and lymphocyte infiltration. The serological test using EIA method is a useful in detecting H. pylori infection and it may be used as a predictor for the H. pylori density and degree of inflammation.
Antibodies ; Biopsy ; Endoscopy ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoenzyme Techniques ; Immunoglobulin G ; Inflammation ; Lymphocytes ; Metaplasia ; Neutrophil Infiltration ; Neutrophils ; Serologic Tests

BACKGROUND: Iron is essential for life, but iron overload state cause potentially fatal health risk. There is growing evidence that only mildly increased amounts of hepatic iron can be damaging, particulary if combined with other hepatotoxic factors such as alcoholic or chronic viral hepatits B,C. The aim of this study was to assess the serum iron status of patients with various forms of hepatitis and cirrhosis of liver and to determine the correlation between the degree of hepatocyte damage (expressed as ALT activity) and status of serum iron parameters. METHODS: Our research involved 107 patients (69 male ranging in age from 27-67 and 38 female ranging in age from 32-62) diagnosed with chronic viral hepatitis B or type C, alcoholic hepatitis or cirrhosis of the liver. Serum iron parameters such as serum iron, ferritin, TIBC, and aminotransferase measured as necroinflammatory activity in Chronic hepatitis. RESULTS: There was no difference s-iron level between chronic hepatitis and cirrhosis but, significantly higher in alcoholic hepatitis and cirrhosis than viral hepatitis and cirrhosis respectively. s-Ferritin level was significantly higher in cirrhosis than hepatits group, and more higher in alcoholic hepatitis and cirrhosis than viral hepatitis and cirrhosis respectively. In chronic hepatitis groups, there are significant correlation between ALT and s-ferritin level regardness of etiology. CONCLUSION: Serum iron overload state was prominent in alcoholic hepatitis and cirrhosis than viral hepatitis and cirrhosis. High serum ferritin level can predict hepatocyte damage in chronic hepatitis.
Alcoholics ; Female ; Ferritins ; Fibrosis ; Hepatitis ; Hepatitis B ; Hepatitis, Alcoholic ; Hepatitis, Chronic ; Hepatocytes ; Humans ; Iron Overload ; Iron* ; Liver Diseases* ; Liver* ; Male