Melorheostosis is a rare disease entity of bone with an etiology and pathogenesis. It cause pain and stiffness in an affected limb, and dense bone formation along the side of bone resembles the flow of candle dripping. Two cases of the disease involving left hand and wrist, and right lower leg are reported with the review of literature.
Extremities ; Hand ; Hyperostosis ; Leg ; Melorheostosis ; Osteogenesis ; Rare Diseases ; Wrist

PURPOSE: The purpose of this study was to evaluate the usefulness of intravenous lipid emulsion as well as adverse events in acute poisoning patients. METHODS: Literature was accessed through PubMed, EMBASE, Cochrane library, Web of science, and KoreaMed. All forms of literatures relevant to human use of intravenous lipid emulsion for acute poisoning were included. Cases reports or letters without description of clinical outcomes for each case were excluded. The literature search was conducted by two investigators in March, 2015, with publication language restricted to English and Korean. The effect, onset time, and adverse event of lipid emulsion and final outcome of each case were analyzed. RESULTS: Eighty-one published articles were included, excluding articles whose title and abstract were not relevant to this study. No articles were classified as high level of evidence. Sixty-eight case reports were identified, consisting of 25 local anesthetics and 43 other drugs, such as tricyclic antidepressants and calcium channel blockers. Although most cases described significant clinical improvements, some of them showed no beneficial effect or worsening of clinical course. Several adverse events including hyperamylasemia and laboratory interference were reported. CONCLUSION: Although there were many case reports illustrating successful use of lipid for various drug poisonings, the effect cannot be estimated due to significant possibility of publication bias. Therefore, lipids might be considered in severe hemodynamic instability resulting from lipophilic drug poisoning, however further studies should follow to establish the use of lipid as the standard of care.
Anesthetics, Local ; Antidepressive Agents, Tricyclic ; Calcium Channel Blockers ; Drug Overdose ; Fat Emulsions, Intravenous ; Hemodynamics ; Humans ; Hyperamylasemia ; Lipid A ; Poisoning* ; Publication Bias ; Publications ; Research Personnel ; Standard of Care

The article ‘The biofilm removal effect of MnO2-diatom microbubbler from the dental prosthetic surfaces: In vitro study’ authored by Eun-Hyuk Lee,Yongbeom Seo, Ho-Bum Kwon, Young-Jun Yim, Hyunjoon Kong, Myung-Joo Kim, published in April issue [Vol 58, No 1] of The Journal of KoreanAcademy of Prosthodontics (2020), has an erratum.The author names were mistakenly given as Ho-Bum Kwon, Young-Jun Yim. It should be corrected as Ho‐Beom Kwon, Young‐Jun Lim. The Journal ofKorean Academy of Prosthodontics apologizes to the readers for this error.

PURPOSE: The aim of this study is to evaluate the effectiveness of MnO₂-diatom microbubbler (DM) on the surface of prosthetic materials as a mouthwash by comparing the biofilm removal effect with those previously used as a mouthwash in dental clinic.MATERIALS AND METHODS: DM was fabricated by doping manganese dioxide nanosheets to the diatom cylinder surface. Scanning electron microscopy (SEM) was used to observe the morphology of DM and to analyze the composition of doped MnO₂. Stereomicroscope was used to observe the reaction of DM in 3% hydrogen peroxide. Non-precious metal alloys, zirconia and resin specimens were prepared to evaluate the effect of biofilm removal on the surface of prosthetic materials. And then Streptococcus mutans and Porphyromonas gingivalis biofilms were formed on the specimens. When 3% hydrogen peroxide solution and DM were treated on the biofilms, the decontamination effect was compared with chlorhexidine gluconate and 3% hydrogen peroxide solution by crystal violet staining.RESULTS: Manganese dioxide was found on the surface of the diatom cylinder, and it was found to produce bubble of oxygen gas when added to 3% hydrogen peroxide. For all materials used in the experiments, biofilms of the DM-treated groups got effectively removed compared to the groups used with chlorhexidine gluconate or 3% hydrogen peroxide alone.CONCLUSION: MnO₂-diatom microbubbler can remove bacterial membranes on the surface of prosthetic materials more effectively than conventional mouthwashes.
Alloys ; Biofilms ; Chlorhexidine ; Decontamination ; Dental Clinics ; Dental Plaque ; Diatoms ; Gentian Violet ; Hydrogen Peroxide ; In Vitro Techniques ; Manganese ; Membranes ; Microscopy, Electron, Scanning ; Mouthwashes ; Oral Hygiene ; Oxygen ; Porphyromonas gingivalis ; Streptococcus mutans

OBJECTIVES: To investigate the differences in accessing regional hospitals and the utilization rate of hospitals located in other regions for cancer patients by regions. METHODS: Data for the utilization of regional hospitals for cancer patients were obtained from the Korean National Cancer Incidence Databases in 1999 and 2002. We divided the regions into 16 provinces by administrative districts. We using the SAS 9.1.3 to analyze difference of regional self-sufficiency and the Arcview 3.2 to show in a schematize for regional variation of the regional self-sufficiency. RESULTS: There were regional variations in the regional self-sufficiency of cancer patients. Especially, Jeollanam-do(1999 13.6%, 2002 12.8%), Gyeongsangbuk-do(1999 22.0%, 2002 20.7%), and Chungcheongnam-do(1999 27.8%, 2002 27.1%) had low regional self-sufficiency. The regional self-sufficiency in Gyeonggi-do and utilization rate of cancer patients who lived in other regions were increased between 1999 and 2002(regional self-sufficiency: 1999 37.2%, 2002 48.2%). CONCLUSION: The results of this study showed that there were regional variation in utilization of regional hospitals for cancer patients. Accessibility of cancer patients in metropolitan areas was higher than in small size cities, medium size cities, and rural county areas. These results suggested that it should be considered support medical facilities for cancer patients in rural areas where have lower relevance rate.
Gyeonggi-do ; Humans ; Incidence

Richter's syndrome refers to the development of aggressive lymphoma in a patient with chronic lymphocytic leukemia (CLL). It occurs in about 2% to 10% of patients with CLL. The most frequent manifestation of Richter's syndrome is diffuse large B cell lymphoma (DLBCL). Extranodal involvement is rare but can occur. The prognosis of Richter's syndrome is very poor. We herein report a case of a rare presentation of Richter's syndrome. A 42-year-old man diagnosed with CLL 2 years previously developed nodules on the bilateral thighs and buttocks. A positron emission tomography (PET)-CT scan revealed high fluorodeoxyglucose uptake in multiple lymph nodes, skeletal muscles, and the myocardium. An ultrasonography-guided biopsy confirmed Richter's syndrome from CLL to DLBCL. The patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. After six cycles of chemotherapy, we performed a PET-CT scan that revealed a complete response. However, 3 months later, the syndrome recurred. The patient was undergoing salvage chemotherapy at the time of this writing.
Adult ; Biopsy ; Buttocks ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymph Nodes ; Lymphoma ; Lymphoma, B-Cell* ; Muscle, Skeletal ; Myocardium ; Positron-Emission Tomography ; Prednisone ; Prognosis ; Thigh ; Vincristine ; Writing ; Rituximab

BACKGROUND: JAK2 V617F is the most common mutation in myeloproliferative neoplasms (MPNs) and is a major diagnostic criterion. Mutation quantification is useful for classifying patients with MPN into subgroups and for prognostic prediction. Droplet digital PCR (ddPCR) can provide accurate and reproducible quantitative analysis of DNA. This study was designed to verify the correlation of ddPCR with pyrosequencing results in the diagnosis of MPN and to investigate clinical implications of the mutational burden. METHODS: Peripheral blood or bone marrow samples were obtained from 56 patients newly diagnosed with MPN or previously diagnosed with MPN but not yet indicated for JAK2 inhibitor treatment between 2012 and 2016. The JAK2 V617F mutation was detected by pyrosequencing as a diagnostic work-up. The same samples were used for ddPCR to determine the correlation between assays and establish a detection sensitivity cut-off. Clinical and hematologic aspects were reviewed. RESULTS: Forty-two (75%) and 46 (82.1%) patients were positive for JAK2 V617F by pyrosequencing and ddPCR, respectively. The mean mutated allele frequency at diagnosis was 37.5±30.1% and was 40.7±31.2% with ddPCR, representing a strong correlation (r=0.9712, P < 0.001). Follow-up samples were available for 12 patients, including eight that were JAK2 V617F-positive. Of these, mutational burden reduction after treatment was observed in six patients (75%), consistent with trends of hematologic improvement. CONCLUSIONS: Quantitative analysis of the JAK2 V617F mutation using ddPCR was highly correlated with pyrosequencing data and may reflect the clinical response to treatment.
Bone Marrow ; Diagnosis ; DNA ; Follow-Up Studies ; Gene Frequency ; Humans ; Polymerase Chain Reaction*

PURPOSE: There are several well-established prognostic factors to predict the course of breast cancer. Recently, a new category of genes that control the process of programmed cell death, also called apoptosis, has been identified. It includes the bcl-2 proto-oncogene which actively blocks apoptosis. The purpose of this retrospective study is to investigate the relationship between Bcl-2 protein expression in primary breast cancers and other markers of prognostic value. MATERIALS AND METHODS: We analyzed 43 cases of invasive ductal carcinoma of breast cancer, which were consecutively operated in the period from January 1990 to December 1994. Analysis of Bcl-2 protein, ER and PgR expression was carried out using immunohistochemistry on the paraffin-embedded tissue section. The following indices were measured.; size of the tumor, number of axillary metastasis, histological grade, menopausal status, ER, PgR, and Bcl-2 status. RESULTS: Expression of the bcl-2 proto-oncogene was found in 28 cases of 43 patients (65.1%). No relationship could be observed between Bcl-2 status and tumor grade, TNM staging and menopausal status. A strong positive relationship was demonstrated between Bcl-2 immunoreactivity and ER status (P 0.001) and PgR status (P=0.014). A favorable prognostic value was demonstrated for Bcl-2 expression on overall survival (P=0.0427), but no prognostic value was demonstrated on disease-free survival (P=0.1587). CONCLUSION: Our results suggest that the Bcl-2 expression may be a favorable prognostic marker and its important role may be a modulator of response to adjuvant therapy in breast cancer.
Apoptosis ; Breast Neoplasms* ; Breast* ; Carcinoma, Ductal ; Cell Death ; Disease-Free Survival ; Humans ; Immunohistochemistry ; Neoplasm Metastasis ; Neoplasm Staging ; Prognosis ; Proto-Oncogenes ; Retrospective Studies

PURPOSE: This study assessed the feasibility and compliance of induction chemotherapy with gemcitabine and cisplatin followed by simultaneous integrated boost–intensity modulated radiotherapy (SIB-IMRT) with concurrent gemcitabine in patients with locally advanced unresectable pancreatic cancer. MATERIALS AND METHODS: In this trial, patients received induction chemotherapy consisting of gemcitabine (1,000 mg/m²) and cisplatin (25 mg/m²) on days 1, 8, and 15 of each treatment cycle. Patients were subsequently treated with gemcitabine (300 mg/m²/wk) during SIB-IMRT. The patients received total doses of 55 and 44 Gy in 22 fractions to planning target volume 1 and 2, respectively. As an ancillary study, digital polymerase chain reaction was performed to screen for the seven most common mutations in codons 12 and 13 of the KRAS oncogene of circulating cell free DNA (cfDNA). RESULTS: Forty-four patients were enrolled between 2012 and 2015. Of these, 33 (75%) completed the treatment. The most common toxicities during induction chemotherapy were grades 3 and 4 neutropenia (18.2%), grade 3 nausea (6.8%) and vomiting (6.8%). The most common toxicities during SIB-IMRT were grade 3 neutropenia (24.2%) and grade 3 anemia (12.1%). Ten patients (23%) underwent a curative resection after therapy. Median overall survival was significantly longer in patients who underwent curative resection (16.8 months vs. 11 months, p < 0.01). The median cfDNA concentration was significantly lower after treatment (108.5 ng/mL vs. 18.4 ng/mL, p < 0.001). CONCLUSION: Induction chemotherapy with gemcitabine and cisplatin followed by concurrent SIB-IMRT was well tolerated and active.
Anemia ; Cisplatin* ; Codon ; Compliance ; DNA ; Feasibility Studies* ; Humans ; Induction Chemotherapy* ; Nausea ; Neutropenia ; Oncogenes ; Pancreatic Neoplasms* ; Polymerase Chain Reaction ; Radiotherapy* ; Vomiting

L-ascorbic acid (vitamin C) is one of the well-known anti-viral agents, especially to influenza virus. Since the in vivo anti-viral effect is still controversial, we investigated whether vitamin C could regulate influenza virus infection in vivo by using Gulo (-/-) mice, which cannot synthesize vitamin C like humans. First, we found that vitamin C-insufficient Gulo (-/-) mice expired within 1 week after intranasal inoculation of influenza virus (H3N2/Hongkong). Viral titers in the lung of vitamin C-insufficient Gulo (-/-) mice were definitely increased but production of anti-viral cytokine, interferon (IFN)-alpha/beta, was decreased. On the contrary, the infiltration of inflammatory cells into the lung and production of pro-inflammatory cytokines, tumor necrosis factor (TNF)-alpha and interleukin (IL)-alpha/beta, were increased in the lung. Taken together, vitamin C shows in vivo anti-viral immune responses at the early time of infection, especially against influenza virus, through increased production of IFN-alpha/beta.
Animals ; Ascorbic Acid ; Cytokines ; Humans ; Influenza A virus ; Influenza, Human ; Interferons ; Interleukins ; Lung ; Mice ; Mustelidae ; Orthomyxoviridae ; Tumor Necrosis Factor-alpha ; Vitamins