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

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: 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

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: 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

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

Primary adrenocortical carcinoma (ACC) is a rare tumor and its usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%). However, intracaval invasion extending into the right atrium is very rare and spontaneous regression of tumor burden in adrenal carcinoma is also rare. We report a case of ACC with direct invasion of the inferior vena cava and right atrium. A 34-yr-old male patient presented with progressive dyspnea, weight loss, and poor oral intake over 3 months. Non-functioning ACC with direct invasion of the inferior vena cava and right atrium was confirmed by imaging, pathologic, and hormonal study. Chemo-radiotherapy was attempted. However, tumor burden was not changed, but rather toxic hepatitis and thrombocytopenia were developed. His subjective symptoms and general conditions were improved after 1 month of conservative management and the patient was discharged. During clinical follow-up, this tumor showed spontaneous regression.
Vena Cava, Inferior/*pathology ; Tomography, X-Ray Computed ; Remission Induction ; Neoplasm Metastasis ; Male ; Humans ; Heart Neoplasms/pathology/*secondary ; Heart Atria/*pathology ; Follow-Up Studies ; Echocardiography ; Biopsy ; Adult ; Adrenocortical Carcinoma/*diagnosis/*pathology ; Adrenal Cortex Neoplasms/*diagnosis/*pathology

Mesenteric pseudocyst is rare. This term is used to describe the abdominal cystic mass, without the origin of abdominal organ. We presented a case of mesenteric pseudocyst of the small bowel in a 70-year-old man. Esophago-gastro-duodenoscopy showed a 3.5 cm sized excavated lesion on the posterior wall of angle. Endocopic biopsy confirmed a histologic diagnosis of the poorly differentiated adenocarcinoma, which includes the signet ring cell component. Abdominal computed tomography scan showed a focal mucosal enhancement in the posterior wall of angle of the stomach, a 2.4 cm sized enhancing mass on the distal small bowel loop, without distant metastases or ascites in rectal shelf, and multiple gallbladder stones. The patient underwent subtotal gastrectomy with gastroduodenostomy, segmental resection of the small bowel, and cholecystectomy. The final pathological diagnosis was mesenteric pseudocyst. This is the first case report describing incidentally detected mesenteric pseudocyst of the small bowel in gastric cancer patients.
Adenocarcinoma ; Aged ; Ascites ; Biopsy ; Cellular Structures ; Cholecystectomy ; Gallbladder ; Gastrectomy ; Humans ; Mesenteric Cyst ; Neoplasm Metastasis ; Stomach ; Stomach Neoplasms

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