Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited colon cancer-predisposing disorder, characterized by the development of innumerable adenomas in the large intestine. Polyps are present in the upper gastrointestinal tract in almost all FAP patients and there is a peculiar predilection for adenomatous change in the duodenum. Colonic adenomas in FAP are commonly observed as polyps but nonpolypoid adenomas can also be seen in the upper gastrointestinal tract. Careful endoscopic examination is needed for detecting such nonpolypoid lesions. At present, the adenoma-carcinoma sequence is thought to exist also in the duodenum, and malignancy of the duodenum represents one of the most common causes of mortality among patients with FAP. Therefore, careful periodic follow-up of upper intestinal endoscopies are recommended in patients with this disease, in consideration to the possible development of duodenal nonpolypoid adenomatous lesions. A case involving an experience of antral and duodenal nonpolypoid adenomas in a patient with FAP is herein reported.
Adenoma* ; Adenomatous Polyposis Coli* ; Colon ; Duodenum ; Follow-Up Studies ; Humans ; Intestine, Large ; Mortality ; Polyps ; Pyloric Antrum ; Upper Gastrointestinal Tract

It is very important to be aware of occurrence and distinctive histologic features of gastric glomus tumor because its clinical presentation and endoscopic ficdings are indistinguishable from other gastric tumors. A 58-year-old man was admitted because of intermittent epigastric pain and indigestion for 2 months. Physical examination and laboratory findings revealed within normal limits. Upper gastroendoscopy demonstrated a submucosal mass near the antrum with central mucosal depressionand peripheral multiple erosions. Endoscopic ultrasonography revealed a circumscribed mass that continued to the third and fourth layers which demonstrated a heterogeneous low echo pattern mixed with internal high echo spots. Histologically, the tumor cells have uniform small nuclei with inconspicuous nucleoli. Immunochemical analysis on the tumor cells expressed alpha 1 smooth muscle actin and the tumor cells are enveloped by reticulin fiber, individually or grouped. A case of glomus tumor of the stomach is herein reported with a review of literature.
Actins ; Dyspepsia ; Endosonography ; Glomus Tumor* ; Humans ; Middle Aged ; Muscle, Smooth ; Physical Examination ; Reticulin ; Stomach*

PURPOSE: Ventilator-associated pneumonia (VAP) is a serious threat in critically ill pediatric patients. Data regarding Stenotrophomonas maltophilia VAP in pediatric population is limited. We evaluated the clinical data of S. maltophilia associated VAP in critically ill pediatric patients. METHODS: A retrospective chart review was performed in pediatric patients 18 years old or younger who developed S. maltophilia associated VAP at Samsung Medical Center, Seoul Korea from January 2008 to December 2012. RESULTS: A total of 31 patients were identified S. maltophilia associated VAP. Median age was 8 months (range, 0.5 month to 16.6 years) and 13 patients were male (40.6%). Underlying illnesses were cardiologic diseases (n=11, 34.4%), hematologic oncologic malignancies (n=7, 25%), neurologic diseases (n=4, 12.5%), pulmonary diseases (n=3, 9.4%), and others (n=4, 12.5%). The median duration of ventilator use before S. maltophilia VAP diagnosis was 14 days (range, 4-256 days). Overall mortality at 30 days was 12.5% (4/32). CONCLUSIONS: S. maltophilia should be also considered as a possible pathogen for VAP in critically ill pediatric patients. Empiric antibiotic choice should include agents that are active against S. maltophilia in patients who are deteriorating on broad spectrum beta-lactam antimicrobial agents.
Anti-Infective Agents ; Child ; Critical Illness* ; Diagnosis ; Humans ; Korea ; Lung Diseases ; Male ; Mortality ; Pneumonia ; Pneumonia, Ventilator-Associated* ; Retrospective Studies* ; Seoul ; Stenotrophomonas maltophilia* ; Stenotrophomonas* ; Ventilators, Mechanical

Atrial fibrillation occurs in 10 to 25% of patients with thyrotoxicosis. Several recent studies have found a high frequency of cerebral thromboembolism in such patients. Thus anticoagulation has been recommended for patients with thyrotoxic atrial fibrillation to prevent embolic complications especially when associated with predictors of an increased risk of arterial thromboembolism in nonrheumatic atrial fibrillation such as recent congestive heart failure, history of hypertension, previous thromboembolism, left atrial enlargement, and global left ventricular dysfunction. Most reported cases of embolic events involving central nervous system in thyrotoxic atrial fibrillation affected cerebrum, and cerebellar embolic infarction from the heart was rarely seen. We herein report our experience of a case of cerebellar embolism in thyrotoxic atrial fibrillation.
Atrial Fibrillation* ; Central Nervous System ; Cerebrum ; Embolism ; Heart ; Heart Failure ; Hepatitis ; Humans ; Hypersensitivity ; Hypertension ; Infarction* ; Thromboembolism ; Thyrotoxicosis ; Ventricular Dysfunction, Left

Mycoplasma pneumoniae is responsible for approximately 20% to 30% of community-acquired pneumonia, and is well known for its diverse extrapulmonary manifestations. However, acute necrotizing pancreatits is an extremely rare extrapulmonary manifestation of M. pneumoniae infection. A 6-year-old girl was admitted due to abdominal pain, vomiting, fever, and confused mentality. Acute necrotizing pancreatitis was diagnosed according to symptoms, laboratory test results, and abdominal computed tomography scans. M. pneumoniae infection was diagnosed by a 4-fold increase in antibodies to M. pneumoniae between acute and convalescent sera by particle agglutination antibody assay. No other etiologic factors or pathogens were detected. Despite the occurrence of a large infected pseudocyst during the course, the patient was able to discharge without morbidity by early aggressive supportive care. This is the first case in Korea of a child with acute necrotizing pancreatitis associated with M. pneumoniae infection.
Abdominal Pain ; Agglutination ; Antibodies ; Child* ; Female ; Fever ; Humans ; Korea ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pancreatitis ; Pancreatitis, Acute Necrotizing* ; Pneumonia ; Pneumonia, Mycoplasma* ; Vomiting

The musculoskeletal system can be involved as an extra-intestinal manifestation of inflammatory bowel disease. Among these, myositis in ulcerative colitis (UC) is very rare. A 14-year-old girl was admitted due to severe shoulder tenderness. She had complained of left jaw pain and swelling for the past 10 days. Inflammatory markers were elevated with no evidence of infectious etiology. Myositis was suspected by shoulder magnetic resonance imaging. Three days after admission, she developed hematochezia. Muscle biopsy and colonoscopy was performed due to worsening left mandibular area pain and persistent hematochezia. Colonoscopy showed consistent findings with UC. She was finally diagnosed with UC with myositis as an extra-intestinal manifestation. She showed a dramatic response to UC treatment. Gastrointestinal symptoms were well-controlled. After 14 months, UC symptoms and muscle pain were aggravated, which were relieved after steroid and cyclosporin treatment. We report a unique case of UC initially presented with myositis, preceding gastrointestinal symptoms.

Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory bone disorder presenting with sterile osteomyelitis, most often presenting in childhood. Although the etiology is understood incompletely, its association with other auto-inflammatory diseases including inflammatory bowel disease (IBD); psoriasis; Wegener’s disease; arthritis; and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome suggests that dysregulated innate immunity may play an important role in the pathogenesis. We report a case of a 13-year-old boy with CRMO associated with Crohn’s disease (CD) successfully treated with infliximab after failure of non-steroidal anti-inflammatory drug (NSAID) treatment. He initially was diagnosed with CRMO based on symmetric and aseptic bone lesions with no fever, lack of response to antibiotic treatment, vertebral involvement, and normal blood cell counts. Despite five months of NSAID treatment, his musculoskeletal symptoms were aggravated, and he developed gastrointestinal symptoms. Finally, he was diagnosed with CRMO associated with CD. Due to the severity of symptoms, infliximab was initiated and produced symptom improvement. This case supports infliximab as another choice for treatment of bowel symptoms in addition to the bone and joint symptoms of CRMO when other first-line treatments are ineffective.

PURPOSE: Tuberculosis (TB) is one of the most important diseases that cause significant mortality and morbidity in young children. Data on TB transmission from an infected child are limited. Herein, we report a case of disseminated TB in a child and conducted a contact investigation among exposed individuals. METHODS: A 4-year-old child without Bacille Calmette-Guérin vaccination was diagnosed as having culture-proven disseminated TB. The child initially presented with symptoms of inflammatory bowel disease, and nosocomial and kindergarten exposures were reported. The exposed individuals to the index case were divided into 3 groups, namely household, nosocomial, or kindergarten contacts. Evaluation was performed following the Korean guidelines for TB. Kindergarten contacts were further divided into close or casual contacts. Chest radiography and tuberculin skin test or interferon-gamma-releasing assay were performed for the contacts. RESULTS: We examined 327 individuals (3 household, 10 nosocomial, and 314 kindergarten contacts), of whom 18 (5.5%), the brother of the index patient, and 17 kindergarten children were diagnosed as having latent TB infection (LTBI). LTBI diagnosis was more frequent in the children who had close kindergarten contact with the index case (17.1% vs. 4.4%, P=0.007). None of the cases had active TB. CONCLUSION: This is the first reported case of TB transmission among young children from a pediatric patient with disseminated TB in Korea. TB should be emphasized as a possible cause of chronic diarrhea and failure to thrive in children. A national TB control policy has been actively applied to identify Korean children with LTBI.
Child* ; Child, Preschool ; Diagnosis ; Diarrhea ; Failure to Thrive ; Family Characteristics ; Humans ; Inflammatory Bowel Diseases* ; Korea ; Mortality ; Radiography ; Siblings ; Skin Tests ; Thorax ; Tuberculin ; Tuberculosis* ; Vaccination

Invasive fungal infection (IFI) is a serious threat to pediatric patients with cancer given high morbidity and mortality. We present an 18-year-old male with precursor T-cell lymphoblastic leukemia who developed Pancoast syndrome, presented with paresthesia and numbness in the right shoulder and arm during a neutropenic fever period. He was diagnosed with pneumonia in the right upper lung field. He was later found to have an invasive pulmonary fungal infection caused by multiple fungi species, including Rhizomucor, confirmed by histology and polymerase chain reaction (PCR) (proven infection), Penicillium decumbens diagnosed by PCR, and Aspergillus suspected from galactomannan assay (probable infection). Unfortunately, the patient's condition further worsened owing to the aggravation of leukemia, chemotherapy-induced neutropenia, and bacterial coinfection, leading to multiorgan failure and death. Here, we report a case of IFI caused by multiple fungal species that presented as Pancoast syndrome.

Invasive fungal infection (IFI) is a serious threat to pediatric patients with cancer given high morbidity and mortality. We present an 18-year-old male with precursor T-cell lymphoblastic leukemia who developed Pancoast syndrome, presented with paresthesia and numbness in the right shoulder and arm during a neutropenic fever period. He was diagnosed with pneumonia in the right upper lung field. He was later found to have an invasive pulmonary fungal infection caused by multiple fungi species, including Rhizomucor, confirmed by histology and polymerase chain reaction (PCR) (proven infection), Penicillium decumbens diagnosed by PCR, and Aspergillus suspected from galactomannan assay (probable infection). Unfortunately, the patient's condition further worsened owing to the aggravation of leukemia, chemotherapy-induced neutropenia, and bacterial coinfection, leading to multiorgan failure and death. Here, we report a case of IFI caused by multiple fungal species that presented as Pancoast syndrome.