Louis Edouard Octave Crouzon, a French neurologist, in 1912, described the hereditary syndrome of craniofacial synostosis in a mother and son. He described the triad as skull deformities, facial anamolies and exopthalmos now known as Crouzon syndrome (CS). CS accounts for about 4.8% of all cases of craniosynostosis. We report a case of CS in 4 year old girl with characteristic features of cranial deformity, maxillary hypoplasia, cleft palate and exopthalmos.
We report a case where life threatening gram negative sepsis developed in a patient with CLL in association with post chemotherapy neutropenia on three occasions. Bacterial typhlitis or neutropenic enterocolitis, which is a well described entity of bowel necrosis seen in immunosuppressed patients, was demonstrated at colonoscopy and was the probable portal of entry of micro-organisms. After spontaneous resolution of the typhlitis, further chemotherapy has been given without recurrent sepsis. Typhlitis should be considered as a cause of recurrent septicaemia in neutropenic patients.
Using a limiting dilution assay the frequency of long‐term culture‐initiating cells (LTC‐IC) in the apheresis products following mobilization by granulocyte‐colony stimulating factor (G‐CSF) with or without chemotherapy from 14 normal donors (ND) for allogeneic bone marrow transplantation, 16 patients with multiple myeloma (MM) and 15 patients with acute myeloid leukaemia (AML), where the aphereses were intended for autologous transplantation, were compared. The estimated median incidences of LTC‐IC in the first apheresis products from ND, MM and AML were 1/3289, 1/1775 and 1/13 075 mononuclear cells (MNC) respectively. The patients with AML had a significantly lower incidence compared with the other two groups (P < 0.0001). There was a positive correlation between the incidence of LTC‐IC and the number of CD34+ cells, the number of GM‐CFC, and the number of BFU‐E. The positive association with GM‐CFC or BFU‐E was weaker. In these experiments the percentage of CD34+ cells was the best predictor for the frequency of LTC‐IC in the peripheral blood progenitor cells (PBPC). In eight cases of MM the LTC‐IC assay was performed for both the first and second harvest. All cases had a lower LTC‐IC frequency in the second harvest compared with the first, an average of 23% (13–42%, 95% confidence interval) and this reduction was statistically significant (P < 0.001); CD34+ cells were also lower (P < 0.001).
Carcinogenesis is a multistep process and individual risk to development of cancer depends not only on environmental factors or extrinsic exposure to carcinogens but also on genetic susceptibility of an individual. In head and neck cancer, tobacco exposure and alcohol consumption are predominantly the most significant external factors for tumor formation. Individual’s susceptibility to cancer may be partly explained by variability in enzymatic activities of metabolic genes. Mutations in genes concerned with production of enzymes for metabolism of tobacco products may lead to increased risk of carcinogenesis with respect to oral mucosa. Therefore variations in the expression of these genes due to heritable genetic polymorphisms might modulate the process of carcinogenesis by altering the exposure levels of tobacco derived carcinogens. Objective: This non systematic review summarizes current data available on the role of environment gene interaction in form of GSTM1 null polymorphism and oral carcinogenesis. Literature review: Relevant data was selected in order to summarize the studies conducted on GSTM1 null polymorphism and oral cancer. Conclusion: Relationship between GSTM1 null polymorphism in oral cancer needs to be established to confirm the role of environment gene interaction in oral carcinogenesis.
The pattern of emergence of multipotential (CFU-A) and committed (CFU-GM and BFU-E) progenitor cells in peripheral blood has been examined in patients with Hodgkin's disease and non-Hodgkin's lymphoma. Mobilization protocols used chemotherapy with or without granulocyte colony-stimulating factor (n=8 and n=5, respectively). In all patients, the numbers of CFU-A, CFU-GM and BFU-E peaked simultaneously, rather than sequentially, suggesting that marrow regeneration after these mobilization protocols occurred from progenitors at all stages of differentiation. We conclude that peripheral blood stem cell harvest strategies based on peak values for total progenitor numbers will also capture maximum numbers of multipotential progenitors. However, the variable relationship between CFU-A and CFU-GM numbers suggests that overall progenitor cell numbers can give only a broad estimate of the absolute numbers of multipotential progenitors in an individual harvest.
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