Mastocytosis is a rare disease which occurs in both children and adults, and it can manifest as a solitary or multiple skin lesions. Both can cause cutaneous or systemic symptoms. Because of the heterogeneity of clinical presentation of mastocytosis and its rare prevalence, it can be hard to suspect the mastocytosis at the first time. Most solitary mastocytomas are about 1–5 cm in diameter and have features of brownish-yellow, minimally elevated plaques with a smooth shiny surface. This article presents a case of solitary mastocytoma which occurred in neonate and that we treated through surgical excision. In histopathological examination, it consisted of c-kit-positive mast cells. Although pediatric cutaneous mastocytosis might regress spontaneously, clinicians should keep in mind that it could be associated with systemic mastocytosis which involves hematopoietic system.
Adult ; Child ; Hematopoietic System ; Humans ; Infant, Newborn ; Mast Cells ; Mastocytoma* ; Mastocytosis ; Mastocytosis, Cutaneous ; Mastocytosis, Systemic ; Parturition* ; Population Characteristics ; Prevalence ; Rare Diseases ; Skin

The causes of sudden death after medullary infarction involve arrhythmia, central respiratory failure, and dysautonomia. Sudden cardiac arrest in a medullary infarction is uncommon. Most of these cases experienced sudden cardiopulmonary arrest within 2 weeks from stroke onset as the extent of lesion increased. Here, we report two cases of medullary infarction presenting as sudden cardiac arrest. These cases indicate that medullary infarction could be one of the causes of sudden cardiac arrest.
Arrhythmias, Cardiac ; Death, Sudden ; Death, Sudden, Cardiac ; Heart Arrest ; Infarction ; Medulla Oblongata ; Primary Dysautonomias ; Respiratory Insufficiency ; Stroke

BACKGROUND: The traditional approach for reduction of frontal sinus fractures is coronal incision. Inherent complications of the coronal approach include long scar, hair loss, and long operation time. We describe a simple approach for the reduction of frontal sinus anterior wall fractures using a suprabrow incision that is commonly used for brow lift. METHODS: From March 2007 to October 2016, the authors identified patients with anterior wall frontal sinus fractures treated by open reduction through a suprabrow incision. Only cases with photographic/radiographic documentation and a minimum follow-up of 6 months were included. The incision line was designed to be at the upper margin of the eyebrow. Medical records and radiographic data were retrospectively reviewed. Surgical outcomes, cosmetic results, and complication were assessed. The patient scale of the patient and observer scar assessment scale was used to assess patient satisfaction for incisional scar at the 6-month follow-up. RESULTS: Thirty-one patients underwent fracture reduction through a suprabrow approach during the study period, with a mean follow-up of 41 months. No patients showed any recurrent displacement, eyebrow asymmetry, or infection during follow-up. Thirteen patients reported their forehead paresthesia postoperatively, and 12 of them had preoperative symptom. One patient complained of incisional scar and underwent scar revision. All patients were satisfied with their eyebrow and forehead contour. CONCLUSION: The suprabrow approach allowed for an accurate reduction of the fractures in the anterior wall frontal sinus by providing direct visualization of the fracture. This transcutaneous approach can effectively restore forehead contour with acceptable postoperative complications and patient satisfaction.
Cicatrix ; Eyebrows ; Follow-Up Studies ; Forehead ; Frontal Sinus* ; Hair ; Humans ; Medical Records ; Minimally Invasive Surgical Procedures ; Paresthesia ; Patient Satisfaction ; Postoperative Complications ; Retrospective Studies ; Skull Fractures

Eradication of Helicobacter pylori using first-line therapy is becoming less effective. Subjects who had been treated for H. pylori infection were prospectively enrolled through an on-line database registry from October 2010 to December 2012. Demographic data, detection methods, treatment indication, regimens, durations, compliance, adverse events, and eradication results for H. pylori infection were collected. Data of 3,700 patients from 34 hospitals were analyzed. The overall eradication rate of the first-line therapy was 73.0%. Eradication failure was significantly associated with old age, concomitant medication, and comorbidity. Regional differences in eradication rates were observed. The most common first-line therapy was proton pump inhibitor-based triple therapy (standard triple therapy, STT) for 7 days (86.8%). The eradication rates varied with regimens, being 73% in STT, 81.8% in bismuth-based quadruple therapy, 100% in sequential therapy, and 90.3% in concomitant therapy. The eradication rate in treatment-naïve patients was higher than that in patients previously treated for H. pylori infection (73.8% vs. 58.5%, P < 0.001). The overall eradication rate for second-line therapy was 84.3%. There was no statistical difference in eradication rates among various regimens. H. pylori eradication rate using STT is decreasing in Korea and has become sub-optimal, suggesting the need for alternative regimens to improve the efficacy of first-line therapy for H. pylori infection.
Adult ; Age Factors ; Aged ; Anti-Bacterial Agents/*therapeutic use ; Databases, Factual ; Drug Therapy, Combination ; Female ; Helicobacter Infections/*drug therapy/microbiology ; Helicobacter pylori/isolation & purification ; Humans ; Internet ; Logistic Models ; Male ; Middle Aged ; Prospective Studies ; Proton Pump Inhibitors/*therapeutic use ; Registries ; Republic of Korea ; Treatment Outcome

Eradication of Helicobacter pylori using first-line therapy is becoming less effective. Subjects who had been treated for H. pylori infection were prospectively enrolled through an on-line database registry from October 2010 to December 2012. Demographic data, detection methods, treatment indication, regimens, durations, compliance, adverse events, and eradication results for H. pylori infection were collected. Data of 3,700 patients from 34 hospitals were analyzed. The overall eradication rate of the first-line therapy was 73.0%. Eradication failure was significantly associated with old age, concomitant medication, and comorbidity. Regional differences in eradication rates were observed. The most common first-line therapy was proton pump inhibitor-based triple therapy (standard triple therapy, STT) for 7 days (86.8%). The eradication rates varied with regimens, being 73% in STT, 81.8% in bismuth-based quadruple therapy, 100% in sequential therapy, and 90.3% in concomitant therapy. The eradication rate in treatment-naïve patients was higher than that in patients previously treated for H. pylori infection (73.8% vs. 58.5%, P < 0.001). The overall eradication rate for second-line therapy was 84.3%. There was no statistical difference in eradication rates among various regimens. H. pylori eradication rate using STT is decreasing in Korea and has become sub-optimal, suggesting the need for alternative regimens to improve the efficacy of first-line therapy for H. pylori infection.
Adult ; Age Factors ; Aged ; Anti-Bacterial Agents/*therapeutic use ; Databases, Factual ; Drug Therapy, Combination ; Female ; Helicobacter Infections/*drug therapy/microbiology ; Helicobacter pylori/isolation & purification ; Humans ; Internet ; Logistic Models ; Male ; Middle Aged ; Prospective Studies ; Proton Pump Inhibitors/*therapeutic use ; Registries ; Republic of Korea ; Treatment Outcome

PURPOSE: This is a retrospective descriptive study to determine the incidence of congenital heart disease(CHD) in Jeju and to estimate the suitability of the Jeju population as the subject for a regional birth cohort study. METHODS: All patients with CHD diagnosed by two dimensional echocardiography in Cheju National University Hospital and Halla General Hospital from January 1999 to March 2003 were included in this study. RESULTS: From April 1999 to March 2002, the crude incidence rate was 10.06 per 1,000 live births (236 cases/23,464 live births). Among the 236 cases, the proportion of each type was as follows: Ventricular septal defect(44.9%), atrial septal defect(21.2%), pulmonary stenosis(11.0%), patent ductus arterios us(9.3%), tetralogy of Fallot(3.4%), transposition of great arteries(2.1%), aortic stenosis(1.3%), hypoplastic left heart syndrome(1.3%), coarctation of aorta(0.8%), total anormalous pulmonary venous return(0.8%), tricuspid atresia(0.8%), heterotaxia(0.4%), single ventricle(0.4%), atrioventricular septal defect(0.4%), Ebstein anomaly(0.4%). The male to female ratio was 1:1.380(P=0.011). For unknown reasons, females showed significantly higher incidence in VSD(P=0.005) and PDA(P=0.019). Mortality rate was 0.34 per 1,000 live births. The types of VSD were 72.1% of perimembranous, 16.4% of trabecular muscular, 5.7% of subarterial, and 5.7% of unknown. CONCLUSION: This is the first regional study on the incidence of CHD in Korea. The incidence of CHD in Jeju was 10.06 per 1,000 live births. Most of our cases were VSDs and ASDs. Jeju would be a good subject for a regional cohort study in the future.
Cohort Studies ; Echocardiography ; Female ; Heart ; Heart Defects, Congenital* ; Hospitals, General ; Humans ; Incidence* ; Jeju-do ; Korea ; Live Birth ; Male ; Mortality ; Parturition ; Retrospective Studies

Granular cell tumor is mostly benign and thought to be of Schwann cell origin. The head and neck, particularly tongue, breast, and upper respiratory tract are frequently involved. Recently, we have experienced a case of granular cell tumor of the right thigh in a 30-year old male, diagnosed by fine needle aspiration cytology which revealed distinct cytologic features. The smear revealed cellular aspirates with clear background. The tumor cells showed uniform small nuclei and abundant eosinophilic, granular cytoplasm with hazy cell border. Mitoses were not found.
Adult ; Biopsy, Fine-Needle ; Breast ; Child* ; Cytoplasm ; Eosinophils ; Granular Cell Tumor ; Head ; Humans ; Lupus Nephritis* ; Male ; Mitosis ; Neck ; Paraganglioma ; Respiratory System ; Statistics as Topic* ; Thigh ; Tongue

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.
Adolescent ; Child* ; Cyclophosphamide ; Hemorrhage* ; Humans ; Lupus Erythematosus, Systemic* ; Male ; Methylprednisolone ; Plasmapheresis*

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.
Adolescent ; Child* ; Cyclophosphamide ; Hemorrhage* ; Humans ; Lupus Erythematosus, Systemic* ; Male ; Methylprednisolone ; Plasmapheresis*

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 7:4, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.
Abdominal Pain ; Age of Onset ; Child ; Cystoscopy ; Diagnosis ; Diagnosis, Differential ; Flank Pain ; Hematuria* ; Hemorrhage ; Humans ; Medical Records ; Proteinuria ; Renal Veins ; Retrospective Studies ; Sex Ratio ; Ultrasonography ; Ultrasonography, Doppler ; Ureter ; Varicocele ; Vena Cava, Inferior