Improved hematopoietic stem cell transplantation upon inhibition of natural killer cell-derived interferon-gamma.

Hematopoietic stem cell transplantation (HSCT) is a frequent therapeutic approach to restore hematopoiesis in patients with hematologic diseases. Patients receive a hematopoietic stem cell (HSC)-enriched donor cell infusion also containing immune cells, which may have a beneficial effect by eliminating residual neoplastic cells. However, the effect that donor innate immune cells may have on the donor HSCs has not been deeply explored. Here, we evaluate the influence of donor natural killer (NK) cells on HSC fate, concluded that NK cells negatively affect HSC frequency and function, and identified interferon-gamma (IFNγ) as a potential mediator. Interestingly, improved HSC fitness was achieved by NK cell depletion from murine and human donor infusions or by blocking IFNγ activity. Thus, our data suggest that suppression of inflammatory signals generated by donor innate immune cells can enhance engraftment and hematopoietic reconstitution during HSCT, which is particularly critical when limited HSC numbers are available and the risk of engraftment failure is high.

Pavlik harness and Frejka pillow: compliance affects results of outpatient treatment.

PURPOSE OF THE STUDY: Outpatient treatment of hip dysplasia in newborns has excellent results. A combination of general screening with early treatment with a functional abduction device works well. Treatment with the Frejka pillow and the Pavlik harness is frequently used in our region. The aim of the study is to compare efficiency and treatment duration, related to the brace used, and to prove that the choice of an abduction device implies parental compliance with the treatment protocol. MATERIALS AND METHODS: Data of 286 treated children were analyzed. The diagnosis was made in the first weeks of life by clinical and sonographic examinations during general screening. The choice of treatment device was expert dependent and was involved by many variables. The experience, type of clinical finding and sonographic pathology according to Graf, availability of a treating facility, and the potential cooperation of individual parents were major parameters. The Frejka pillow was used to treat 145 children and the Pavlik harness was used in 137 children. The treatment duration and percentage of infants lost from follow-up in relation to the device used was documented. RESULTS: The success rate of outpatient treatment was 98.6%. In six patients, the type of device had to be changed during the treatment period. Physiological sonographic findings were achieved in all hips by the end of the treatment. The Frejka pillow was used as the preferred device in milder stable dysplastic hips, while unstable and decentered hips were treated more frequently with the Pavlik harness. Treatment lasted, on average, 95 days and 119 days in the Frejka and in the Pavlik group, respectively; there was no statistical significance in treatment duration of comparable sonographic pathologies. We observed statistically greater parental non-compliance with the treatment protocol in the Pavlik harness group (p = 0.0279; OR 2.7; 95% CI 1.07; 8.5). CONCLUSIONS: Neither of the abduction devices was inferior with regard to treatment efficiency. We found that parental cooperation was an important factor during screening and treatment. The treatment decision and the choice of the brace must be made with full consent of the parents, keeping in mind that comfort during the nursing care may have a significant influence on compliance with the treatment protocol.

Management of ischemic deformity after the treatment of developmental dysplasia of the hip.

Long-term results of 69 hip operations for deformities due to avascular necrosis following the conservative treatment of the hip dysplasia were evaluated. The authors' technique of intertrochanteric valgus osteotomy allows for simultaneously handling of varus deformity and shortening of the femoral neck, fusion of the greater trochanter, and correction of increased femoral anteversion, if necessary. This type of surgery was used in children with Buchholz-Ogden type III deformity starting from 3 years of age until adolescence. Patients were followed for an average of 19.3 years. Eighty-seven percent had no marked limitation in range of motion and 57% were free of complaints. Trendelenburg gait was present in only six patients. Radiographs of all patients showed improvement of coxometric values. Acetabular dysplasia was handled in 29 patients with a subsequent shelf arthroplasty. Total hip arthroplasty for hip degeneration was not indicated for any of the patients to date.

Fracture of the clavicle after surgical treatment for congenital pseudarthrosis.

BACKGROUND: Congenital pseudarthrosis of the clavicle is a rare disease. There are about 200 individual cases reported so far in world literature, mainly in the form of case reports. The majority of authors recommend surgical. An analysis of 103 cases published since 1980 shows only a few post-surgical complications. CASE REPORT: The authors present a female patient operated at the age of 4.5 years. During surgery both cartilaginous ends were resected and internal fixation was performed by a one-third semitubular plate fixed by 3.5 mm cortical screws. Ten days after hardware removal, a fracture occurred in the screw-hole following a minor impact. Surgery to heal the congenital pseudarthrosis was successful, but a new fracture occurred, resulting from bone weakening. This simple fracture was treated conservatively. At present (5 years after the first surgery) the patient's parents are completely satisfied with both the functional result and the cosmetic appearance. CONCLUSIONS: The authors have not found in the literature any reports of similar complications after surgical treatment of this condition. The use of more delicate osteosynthetic material might prevent the complication described here.