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The first Japanese case of Hb Santa Ana, an unstable abnormal hemoglobin, identified rapidly by electrospray ionization mass spectrometry.

Two members of a family had chronic hemolytic anemia due to unstable hemoglobin. The abnormal beta-chain with a molecular weight of 16 u smaller than the normal beta-chain was found within 5 minutes by analysis using electrospray ionization mass spectrometry. The substitution of amino acid was also determined rapidly by this new strategy. The leucine at the 88th position of the normal beta-chain was substituted by proline in the hemoglobin as in Hb Santa Ana. This is the first report of a Japanese case of Hb Santa Ana; the clinical course was similar to that in the previously reported cases.

Adolescent↗

Bronchoscopic therapy with neodymium-yttrium-aluminum-garnet laser during intravenous anesthesia. Effect on arterial blood gas levels, pH, hemoglobin saturation, and production of abnormal hemoglobin.

Photoresection with the neodymium-yttrium-aluminum-garnet (Nd-YAG) laser has been shown to be an effective bronchoscopic treatment for patients with obstructive lesions of the airway. We monitored levels of arterial blood gases, carbon monoxide, methemoglobin, and sulfmethemoglobin, and the hemoglobin oxygen saturation by ear oximeter in patients during intravenous anesthesia. The measurements were made after induction but before treatment with the Nd-YAG laser, during treatment, and immediately after treatment. Thirteen patients were entered into the study, ten of whom were used for statistical analysis. Partial arterial oxygen pressure decreased significantly during treatment (p less than 0.001). The alveolar-arterial oxygen pressure difference increased significantly during treatment (p less than 0.0025). The partial arterial carbon dioxide pressure increased significantly (p less than 0.005). The pH decreased significantly (p less than 0.02). No statistically significant change was found in the levels of carbon monoxide, methemoglobin, or sulfmethemoglobin. Comparison of arterial hemoglobin oxygen saturation and hemoglobin oxygen saturation by ear oximeter revealed no statistically significant difference in the two methods. During the procedure, careful attention must be given to the patient's oxygenation and ventilation. These variables can be monitored satisfactorily during general anesthesia by the use of an ear oximeter and end tidal carbon dioxide analyzer.

Adult↗

Two cases in which an abnormal hemoglobin (Hb Kamakura) was identified on the basis of an abnormally low glycohemoglobin levels.

We encountered two patients with abnormally low glycohemoglobin levels in spite of normal plasma glucose levels. Since the presence of an abnormal hemoglobin was suggested by high-performance liquid chromatography (HPLC), gene analysis of these two patients was conducted by single-strand conformation polymorphism (SSCP) analysis followed by direct sequencing, and Hb Kamakura with one base substitution (beta3 Leu --> Val) in the beta globin gene was detected in both patients. There was been only one report of Hb Kamakura in Kanagawa Prefecture, and our patients represent the second and third cases, respectively.

Adult↗