Renal thromboembolism during treatment with recombinant activated factor VII (rFVIIa) in a child with hemophilia B with factor IX inhibitors.

BACKGROUND: Serious thromboembolic events connected with rFVIIa therapy in hemophilia patients are rare. Only three cases are reported in children, all of them with hemophilia A. CASE PRESENTATION: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies. CONCLUSION: Caution is necessary if hematuria B requires administration of rFVIIa. US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

Evaluation of cytogenetic damage in nuclear medicine personnel occupationally exposed to low-level ionising radiation.

The aim of this study was to provide data on genetic hazards associated with occupational exposure to low doses of ionising radiation in nuclear medicine departments. The DNA damage in peripheral blood lymphocytes of medical staff was assessed using the chromosome aberration test. Altogether 120 subjects (60 exposed and 60 controls) participated in the study. The exposed subjects showed significantly higher frequencies of chromosome aberrations than controls. Significant inter-individual differences in DNA damage within the exposed population indicate different genome sensitivity. Age and sex were not confounding factors, while smoking increased DNA damage only in control subjects. This study suggests that chronic exposure to low doses of ionising radiation in nuclear medicine departments causes cytogenetic damage. For this reason, exposed medical personnel should minimise radiation exposure wherever possible. Our results also point to the significance of biological indicators, which provide information about the actual risk for the radiation-exposed individuals.

Successful early pyeloplasty in infants.

BACKGROUND: Early pyeloplasty for the treatment of congenital ureteropelvic junction obstruction to maximize nephron salvage is justified only if potential hazards of operating on small infants are avoided. METHODS: The records were analyzed of all infants who underwent pyeloplasty over a 5-year period. Open pyeloplasty was performed if collecting systems had deteriorated or were demonstrated to be obstructed; it was also performed for severe cases of hydronephrosis. Outcome of surgery in the younger infant (patients <2 months of age) was compared with the older infant group (patients >2 months of age). Preoperative evaluation in case of mild or moderate hydronephrosis was directed toward ruling out a non-obstructed collection system and included voiding cystourethrography, and serial ultrasonography and/or dual isotope diuretic renography. Postoperative assessment consisted of serial ultrasonography and/or nuclear imaging to confirm decompression and relief of obstruction. RESULTS: A total of 24 pyeloplasties were performed on 22 patients in the younger infant group (two bilateral) and 30 were performed on 27 infants in the older infant group (three bilateral). The only significant differences between the groups were as follows: patients in the younger infant group were likely to present in utero (75%, p = 2.69), whereas those in the older infant group were more likely to present with a urinary tract infection (48%, p = 4.12). During follow-up examination, 23 renal units in the younger infant group and 24 in the older infant group were judged to be stable or improved. Four kidneys were not salvaged after pyeloplasty, one in the younger infant group and three in the older infant group. CONCLUSIONS: Good results of pyeloplasties performed in the infants in this series support early correction of ureteropelvic junction obstruction in infants.