Aplastic Anemia in a Patient with Cronkhite-Canada Syndrome

Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Paroxysmal nocturnal hemoglobinuria (PNH)-type cells were detected in a peripheral blood specimen. In addition, human leukocyte antigen (HLA) included DRB1*15:01 and DRB1*15:02. Mesalazine was discontinued in consideration of possible drug-induced pancytopenia. Immunosuppressive therapy ameliorated both the gastrointestinal symptoms of CCS and pancytopenia. A common autoimmune abnormality might underlie both CCS and AA.     

Analysis of nocturnal desaturation waveforms using algorithms in patients with idiopathic pulmonary fibrosis

Purpose

Sleep-disordered breathing is recognized as a comorbidity in patients with idiopathic pulmonary fibrosis (IPF). Among them, nocturnal hypoxemia has been reported to be associated with poor prognosis and disease progression. We developed a diagnostic algorithm to classify nocturnal desaturation from percutaneous oxygen saturation (SpO₂) waveform patterns: sustained pattern, periodic pattern, and intermittent pattern. We then investigated the prevalence of nocturnal desaturation and the association between the waveform patterns of nocturnal desaturation and clinical findings of patients with IPF.

Methods

We prospectively enrolled patients with IPF from seven general hospitals between April 2017 and March 2020 and measured nocturnal SpO₂ and nasal airflow by using a home sleep apnea test. An algorithm was used to classify the types of nocturnal desaturation. We evaluated the association between sleep or clinical parameters and each waveform pattern of nocturnal desaturation.

Results

Among 60 patients (47 men) who met the eligibility criteria, there were 3 cases with the sustained pattern, 49 cases with the periodic pattern, and 41 cases with the intermittent pattern. Lowest SpO₂ during sleep and total sleep time spent with SpO₂?<?90% were associated with the sustained pattern, and apnea–hypopnea index was associated with the intermittent pattern.

Conclusion

We demonstrated the prevalence of each waveform and association between each waveform and sleep parameters in patients with IPF. This classification algorithm may be useful to predict the degree of hypoxemia or the complication of obstructive sleep apnea.

     

Carcinoma of the fallopian tube: Results of a multi-institutional retrospective analysis of 127 patients with evaluation of staging and prognostic factors

The aim of this study was to determine the impact of prognostic factors in primary fallopian tube carcinoma (PFTC). All cases of PFTC diagnosed between 1990 and 2010 were retrieved from the files of 6 academic centers. The cases were staged according to a modification of the International Federation of Obstetrics and Gynecology staging system proposed by Alvarado-Cabrero et al (Gynecol Oncol 1999; 72: 367–379). One hundred twenty-seven PFTC cases were identified. The mean age of the patients was 64.2 years. Stage distribution was as follows: 72 (57%), stage I; 19 (15%), stage II; 28 (22%), stage III; and 8 (6.2%), stage IV. Depth of infiltration of the tubal wall was an independent prognostic factor in stage I cases (P < .001). Carcinomas located in the fimbriated end even without invasion had a worse prognosis than did carcinomas involving the tubal portion of the organ. The presence of vascular space invasion correlated with the depth of tubal wall invasion (P = .001) and the presence of lymph node metastases (P = .003). Tumor grade significantly correlated with survival (P < .0001), but histologic type was of marginal significance and only if it was grouped as nonserous/non–clear cell vs serous/clear cell (P = .04). The depth of invasion of the tubal wall and the presence of carcinoma in the fimbriated end even without invasion are important prognostic indicators. The modified International Federation of Obstetrics and Gynecology staging system should be used on a routine basis in all carcinomas of the fallopian tube.     

Evaluation of condyle-fossa relationships in adolescents with various skeletal patterns using cone-beam computed tomography

Objectives:To evaluate the condyle-fossa relationship in adolescents with various skeletal patterns using cone-beam computed tomography (CBCT).Materials and Methods:CBCT images obtained in 120 adolescent patients were used for this study. The patients were divided into 3 groups according to 3 criteria: (1) age (early, middle, and late adolescence); (2) facial height ratio or Jarabak quotient (hyperdivergent, normodivergent, and hypodivergent); and (3) ANB classification (Class I, Class II, and Class III). Temporomandibular joint space (TMJS: AS, anterior space; SS, superior space; PS, posterior space; MS, medial space; LS, lateral space), width and depth of the condyle (MLT, mediolateral thickness; APT, anteroposterior thickness), articular slope (ArS) and vertical height of the fossa (VHF) were measured and compared using CBCT.Results:Differences in condyle-fossa relationships were not significantly different between male and female adolescents, but were significantly different (P < .05) between left and right sides. The mean values showed no statistical differences according to age and skeletal pattern. Most measurements in the sagittal view showed that SS was the greatest, and the mean ratio of AS to SS to PS was 1.00 to 1.27 to 1.19, respectively. The mean values of coronal MS and LS were not significantly different.Conclusions:There were almost no statistical differences in the TMJS in adolescents across various factors except between left and right sides.     

Age-related changes and sex differences in chondroitin sulfate isomers and hyaluronic acid in normal synovial fluid

The effects of factors such as age and sex on the metabolism of chondroitin sulfate (CS) and hyaluronic acid (HA) in knee joint tissues are believed to be profoundly important in the onset of joint diseases including osteoarthritis. To test whether age and sex influence CS isomers and HA in normal synovial fluid, we determined concentrations of chondroitin 6-sulfate (C6S), chondroitin 4-sulfate (C4S), and HA in healthy subjects of different ages. Synovial fluids were obtained from 187 healthy volunteers, 14–89 years of age. Chondroitin 6-sulfate, C4S, HA concentrations, and C6S : C4S ratio showed a significant negative correlation with age. There were no sex-related differences in HA concentration, but the concentrations of C6S and C4S and the C6S : C4S ratio were significantly lower in women than in men in most age groups.     

Carboxyl-terminal sequence variation of latent membrane protein 1 gene in Epstein-Barr virus-associated gastric carcinomas from Eastern China and Japan

OBJECTIVES: To elucidate variations of latent membrane protein 1 (LMP1) in Epstein-Barr virus (EBV)-associated gastric carcinoma (EBVaGC) and explore the LMP1 variations of neighboring countries, China and Japan. METHODS: In 12 and 8 EBVaGCs from eastern China and Japan, respectively, the C-termini of LMP1 were analyzed using PCR and sequencing. The sequences were compared with previously published strains and were characterized on a phylogenetic tree. The difference between Chinese and Japanese isolates was characterized. RESULTS: Ten of 12 Chinese GC isolates (83.3%) and all of the 8 (100%) Japanese GC isolates belonged to the China 1 strain. Also, B95-8 type isolates were found in 2 of 12 Chinese GC. In the 18 China 1 type isolates, additional mutations outside the signature sequence changes were found. All Japanese isolates (100%) had two or more additional mutations, whereas only 5 of 10 (50%) Chinese isolates had two or more additional mutations. The difference was statistically significant (p = 0.0359). CONCLUSIONS: China 1 is the dominant strain in GC from eastern China and Japan. The similarity to that of nasopharyngeal carcinoma (NPC) from China supports the view that China 1 strain represents a geographic-associated polymorphism rather than an NPC-associated polymorphism. Japanese isolates show more mutations than Chinese isolates, suggesting a geographic difference between Chinese and Japanese isolates in GC.     

Adhesion and bactericidal properties of nanostructured surfaces dependent on bacterial motility

Different nanostructured surfaces have bactericidal properties that arise from the interaction between the bacteria and the nanostructured surface. In this study, we focused on the relationship between bacterial motility and bactericidal properties. The motility of Escherichia coli (E. coli) was tuned by genetic engineering, and four types of E. coli (wild type (WT), lacking flagella, and flagellated with deficient motility or deficient chemotaxis) were used to evaluate the adhesion and bactericidal properties of nanostructured surfaces. Cicada (Cryptotympana facialis) wings and Si nano-pillar array substrates were used as natural and artificial nanostructured surfaces, respectively. Differences in motility and chemotaxis strongly influenced the adhesion behavior and to some extent, the damage to the cell membrane. These results suggest that the bactericidal properties of nanostructured surfaces depend on bacterial motility.

Bactericidal effect derived from nanostructured surface was evaluated in the point of view of the motility of E. coli. The results suggest that the properties strongly depend on bacterial motility.     

The evolution of Epstein-Barr virus inferred from the conservation and mutation of the virus glycoprotein gp350/220 gene

To study variations of Epstein-Barr virus (EBV), we analyzed the gp350/220 gene for several cell lines and Japanese wild isolates using direct sequencing. The N-terminal region was highly conserved in all EBVs except for Jijoye/P3HR-1 and a few isolates. The variation of the region coincided with EBV types A and B (also referred to as types 1 and 2) and were, respectively, designated as the types a and b. The type A/a was detected in most Japanese cell lines and wild isolates, and was classified as China1 type with latent membrane protein (LMP) 1 gene. The type B/b was detected in only a few wild isolates with the Med and China2 types. The C-terminus had more diversity than the N-terminus and lacked the divergence between types A/a and B/b. The phylogenetic analyses of the gp350/220 and LMP1 genes may suggest a mode of EBV evolution into types A/a and B/b and then to LMP1 subtypes.