We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.

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This study was undertaken to characterize the clearance of circulating rat glandular kallikrein and to determine the contribution of various organs and the urinary excretion to the removal of glandular kallikrein from the bloodstream. We injected either active 125I-kallikrein or kallikrein inactivated with phenylmethylsulfonyl fluoride (125I-PMSF-kallikrein) intravenously into intact or nephrectomized rats and then studied the disappearance rate of trichloroacetic acid (TCA)-precipitable radioactivity from the circulation. Inactivation by PMSF markedly reduced the binding of kallikrein to plasma protease inhibitors. The removal rate of the acid-precipitable radioactivity fit a biexponential curve for both active and inactive kallikrein. In the intact rats approximately 50% of the radioactivity was removed from the circulation 30 min after the injection of active 125I-kallikrein. Removal of the kidneys did not significantly affect the clearance of active kallikrein. On the other hand, inactive 125I-PMSF-kallikrein was removed from blood faster than active 125I-kallikrein in normal animals. Approximately 50% of the radioactivity was removed from the circulation 8 min after the injection, and the half-life of inactive 125I-PMSF-kallikrein was markedly prolonged by bilateral nephrectomy. Active 125I-kallikrein was taken up by tissues, particularly the liver and the kidney. In urine, less than 2% of the radioactivity was excreted in 60 min as TCA-precipitable material. We concluded that glandular kallikrein is cleared rapidly from the circulation of the rat, probably in the form of a complex with a plasma protease inhibitor.(ABSTRACT TRUNCATED AT 250 WORDS)

A five-year old girl, diagnosed with fever of unknown origin (FUO), was transferred to our hospital due to a deterioration in her general condition. Pseudomonas cepacia (P. cepacia) was detected in her blood culture and she soon recovered after imipenem cilastatin (IPM/CS) administration. When the former hospital was informed of the results of her blood cultures, we learned that five other cases of FUO had occurred there within the previous two weeks in noncompromised children. Except for one case, Pseudomonas species was detected in their blood cultures. P. cepacia was found in one case, Pseudomonas aeruginosa (P. aeruginosa) was detected in two other cases, and both P. cepacia and P. aeruginosa were demonstrated in the other patient. Although the origin of the bacteria is unknown, it may have spread to the children through a contaminated liquid reservoir or medical devices.

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