Blue rubber bleb nevus syndrome is a rare disease of cutaneous hemangioma of a variant of the carvenous type which is clinically characterized by multiple, protuberant, dark blue, generally soft, rubbery, and compressible cutaneous masses usually associated with similar lesions of the gastrointestinal tract. Most cases are sporadic, although autosomal dominant inheritance has been described. We report herein a cases of blue rubber bleb nevus syndrome in a 49-year-old, who had multiple, dark blue, slightly tender, easily compressible vascular tumors on the face, right postauricular area, tongue, neck, and right had and foot, measuring from 0.5 to 1.0 cm in diameter. Endoscopic examination also revealed several similar lesions on the upper third of the esophagus. Biopsy specimen from the lesion of the neck showed irregularly dilated vessels containing red blood cells and fibrinous material. The vascular space was lined by a single layer of flattened endothelial cells.
Biopsy ; Blister* ; Endothelial Cells ; Erythrocytes ; Esophagus ; Fibrin ; Foot ; Gastrointestinal Tract ; Hemangioma ; Humans ; Middle Aged ; Neck ; Nevus* ; Rare Diseases ; Rubber* ; Tongue ; Wills

We report a case of infantile acute hemorrhagic edema in a 15-month-old boy. The patient showed multiple, bean to walnut-sized, red to purple-colored, indurated purpura with a cockade pattern on the face, earlobes, and extremities. Edema of the hands, feet, and earlobes was also observed. Laboratory examinations showed thrombocytosis, decreased serum CH50, and ketone in the urine. A skin biopsy taken from indurated purpura on the lower leg exhibited findings of acute leukocytoclastic vasculitis. Direct immunofluorescence examination showed deposition of IgM along the dermo-epidermal junction and C3 in the dermal vessel walls. With-out specific management except for fluid therapy to correct dehydration, the skin lesions cleared completely in 2 weeks, and recurrence has not observed.
Biopsy ; Dehydration ; Edema* ; Extremities ; Fluid Therapy ; Fluorescent Antibody Technique, Direct ; Foot ; Hand ; Humans ; Immunoglobulin M ; Infant ; Leg ; Male ; Purpura ; Recurrence ; Skin ; Thrombocytosis ; Vasculitis

We report a case of congenital candidiasis confined to the rail plates in a 57-day-old girl whose infected nails exhibited the rough, yellow-white, thickened, opaque distal ends and pink-colored, normal healthy plates at their proximal bases. Numerous pseudohyphae and spores were demonstrated by KOH microscopic examination. Candida albicans was identified by culture on Sabourauds glucose agar and API 20C Aux system. Histopathologic findings of nail crippings also showed mutiple hyphae beneath the hyperkeratot,ic nail plate on PAS stain. Shedding of the infected distal portion of the ungual plates ws caused by growing out of the healthy nails, resulting in spontaneous healing of the disease rithin 2 months.
Agar ; Candida albicans ; Candidiasis* ; Female ; Glucose ; Humans ; Hyphae ; Spores

BACKGROUND: Several antibiotics can be used to treat ventilator-associated pneumonia caused by carbapenem-resistant A. baumannii (CRAB-VAP) including high-dose sulbactam. However, the effectiveness of high-dose sulbactam therapy is not well known. We report our experience with high-dose sulbactam for treatment of CRAB-VAP. METHODS: Medical records of patients with CRAB-VAP who were given high-dose sulbactam between May 2013 and June 2015 were reviewed. RESULTS: Fifty-eight patients with CRAB-VAP were treated with high-dose sulbactam. The mean age was 72.0 ± 15.2 years, and the acute physiology and chronic health evaluation II (APACHE II) score was 15.1 ± 5.10 at the time of CRAB-VAP diagnosis. Early clinical improvement was observed in 65.5% of patients, and 30-day mortality was 29.3%. Early clinical failure (odds ratio [OR]: 8.720, confidence interval [CI]: 1.346-56.484; p = 0.023) and APACHE II score ≥ 14 at CRAB-VAP diagnosis (OR: 10.934, CI: 1.047-114.148; p = 0.046) were associated with 30-day mortality. CONCLUSIONS: High-dose sulbactam therapy may be effective for the treatment of CRAB-VAP. However, early clinical failure was observed in 35% of patients and was associated with poor outcome.
Acinetobacter baumannii* ; Acinetobacter* ; Anti-Bacterial Agents ; APACHE ; Diagnosis ; Humans ; Medical Records ; Mortality ; Pneumonia, Ventilator-Associated* ; Sulbactam*

BACKGROUND: The reversal of neuromuscular blocker might be accelerated if the anticholinesterase was administered in divided doses. This study has been conducted to evaluate the correct ratio of divided doses of neostigmine for the rapid recovery in the rabbits after pancuronium when the profound relaxation(PTC=0) was confirmed. METHODS: Rabbits(n=60) were randomly allocated to 6 groups. After pancuronium 0.2 mg/kg intravenously, spontaneous recovery was evaluated in group 1. When the profound relaxation(PTC=O) was confirmed at 5 min. after pancuronium, neostigmine 50 ug/kg was injected as a bolus in group 2. At that time, neostigmine was given at 10 ug/kg followed by 40 ug/kg 3 min. later in group 3. At that time, neostigmine was given at 20 ug/kg followed by 30 ug/kg 3 min. later in group 4. At that time, neostigmine was given at 30 ug/kg followed by 20 ug/kg 3 min. later in group 5. At that time, neostigmine was given at 40 ug/kg followed by 10 ug/kg 3 min. later in group 6. RESULTS: The mean time from injection of pancuronium to 95% recovery was 99.3 min. in group 1, 59.8 min. in group 2, 53.2 min. in group 3, 51.5 min. in group 4, 50.8 min. in group 5 and 41.1 min. in group 6. The recovery index was significantly reduced in group 6(p<0.05). CONCLUSIONS: We conclude that the recovery time is reduced when neostigmine is administered in divided doses: a larger priming dose followed by a smaller bolus at profound relaxation.
Muscle Relaxation* ; Neostigmine* ; Neuromuscular Blockade ; Pancuronium* ; Pharmacokinetics ; Rabbits* ; Relaxation

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder. Over 75 pathogenic mutations have been identified in both the mitochondrial and nuclear genomes. Leigh syndrome can be diagnosed based on clinical manifestations, physical and biochemical examinations, and brain magnetic resonance imaging results. Patients with Leigh syndrome classically present in early childhood with developmental regression, ataxia, and hypotonia with subsequent respiratory and brainstem dysfunction. However, the clinical course of Leigh syndrome is heterogeneous with significant differences in age of onset, symptom severity and prognosis. Here, we report a case of Leigh syndrome with cardiac arrest as initial presentation, and identified the novel mutation of NDUFS1 gene.

BACKGROUND AND OBJECTIVES: Apoptosis may play a key role in determining the growth, aggressiveness, and therapy responsiveness of tumors. Dysregulation of apoptosis can cause the tumorigenesis of sinonasal malignant tumors. The purpose of this study was to assess apoptosis and the expression of its related proteins, bcl-2 and Fas-L, in the sinonasal malgnant tumors. MATERIALS AND METHODS: The study samples were obtained after surgical removal of 22 cases of inverted papilloma (IPs), 4 cases of IPs associated with squamous cell carcinoma (SCC), 14 cases of SCC in sinonasal cavity and 5 cases of normal inferior turbinate mucosa as a control. Apoptosis was evaluated by analysing DNA fragmentation using the TUNEL method. Bcl-2 and Fas-L expressions were assessed by immunohistochemical staining. RESULTS: Apoptotic index (Al) was decreased progressively from IPs, through IP with SCC to reach the lowest level in SCC (p<0.05). Bcl-2/Fas-L expressions were increased as tumor progressed but Fas-L expression was statistically significant only (p<0.05). An inverse relationship between the Fas-L expression and apoptosis was observed as tumor progressed (p<0.05). No significant correlation was found between AI, expressions of bcl-2/Fas-L and other clinicopathologic factors. CONCLUSIONS: These data suggest that bcl-2/Fas-L expressions are related to apoptosis and tumorigenesis of sinonasal malignant tumors. The inverse tendency between Fas-L expression and apoptosis might be an important role in evading surveillance of the immune system of sinonasal malignant tumors.
Apoptosis* ; Carcinogenesis ; Carcinoma, Squamous Cell ; DNA Fragmentation ; Fas Ligand Protein ; Immune System ; In Situ Nick-End Labeling ; Mucous Membrane ; Papilloma, Inverted ; Turbinates

Fibrovascular polyp (FVP) mostly occurs in the upper cervical esophagus, hypopharynx, and occasionally in the oropharynx. However, it occurs very rarely in the nasopharynx. FVP is a slow growing tumor which often fails to produce symptoms until it has achieved massive size. Symptoms are diverse, ranging from asymptomatic to obstructive asphyxia according to the size of mass. We have recently experienced a rare case of a 14-year-old boy who complained of bilateral nasal obstruction due to a mass originating from the nasopharyngeal surface of soft palate, which is pathologically a nonnasal lesion. It was diagnosed as FVP and was successfully managed using cold instruments under endoscopy.
Adolescent ; Asphyxia ; Cold Temperature ; Endoscopy ; Esophagus ; Humans ; Hypopharynx ; Nasal Obstruction ; Nasopharynx ; Oropharynx ; Palate, Soft ; Polyps

BACKGROUND: Several antibiotics can be used to treat ventilator-associated pneumonia caused by carbapenem-resistant A. baumannii (CRAB-VAP) including high-dose sulbactam. However, the effectiveness of high-dose sulbactam therapy is not well known. We report our experience with high-dose sulbactam for treatment of CRAB-VAP. METHODS: Medical records of patients with CRAB-VAP who were given high-dose sulbactam between May 2013 and June 2015 were reviewed. RESULTS: Fifty-eight patients with CRAB-VAP were treated with high-dose sulbactam. The mean age was 72.0 ± 15.2 years, and the acute physiology and chronic health evaluation II (APACHE II) score was 15.1 ± 5.10 at the time of CRAB-VAP diagnosis. Early clinical improvement was observed in 65.5% of patients, and 30-day mortality was 29.3%. Early clinical failure (odds ratio [OR]: 8.720, confidence interval [CI]: 1.346-56.484; p = 0.023) and APACHE II score ≥ 14 at CRAB-VAP diagnosis (OR: 10.934, CI: 1.047-114.148; p = 0.046) were associated with 30-day mortality. CONCLUSIONS: High-dose sulbactam therapy may be effective for the treatment of CRAB-VAP. However, early clinical failure was observed in 35% of patients and was associated with poor outcome.
Acinetobacter baumannii ; Acinetobacter ; Anti-Bacterial Agents ; APACHE ; Diagnosis ; Humans ; Medical Records ; Mortality ; Pneumonia, Ventilator-Associated ; Sulbactam

Renal cell carcinoma (RCC) is likely to metastasize to other organs, and is often resistant to conventional chemotherapies. Thymoquinone (TQ), a phytochemical derived from the seeds of Nigella sativa, has been shown to inhibit migration and metastasis in various cancers. In this study, we assessed the effect of TQ on the migratory activity of human RCC Caki-1 cells. We found that treatment with TQ reduced the proteolytic activity of matrix metalloproteinase-9 (MMP-9) in Caki-1 cells. TQ significantly repressed prostaglandin E2(PGE2) production, its EP2 receptor expression as well as the activation of Akt and p38, the wellknown upstream signal proteins of MMP-9. In addition, treatment with butaprost, a PGE2 agonist, also induced MMP-9 activity and migration/invasion in Caki-1 cells. Moreover, pharmacological inhibitors of PI3K/Akt and p38 remarkably attenuated butaprostinduced Caki-1 cell migration and invasion, implying that activation of PI3K/Akt and p38 is a bridge between the PGE2-EP2 axis and MMP-9-dependent migration and invasion. Taken together, these data suggest that TQ is a promising anti-metastatic drug to treat advanced and metastatic RCC.