Pseudarthrosis in fibrous dysplasia treated with bone morphogenetic protein

We present a case of pseudarthrosis in a patient suffering from polyostotic fibrous dysplasia of the right part of the body that was successfully treated with bone morphogenetic protein. Pseudarthrosis occurred after proximal femoral shaft fracture due to a motorcycle accident initially treated by intramedullary nailing. After revision, the patient was treated by pseudarthrosis debridement and grafting of bone morphogenetic protein-7/osteogenic protein-1, resulting in callus formation that allowed indolent full weight-bearing after 6 weeks. The underlying disease as well as the described treatment is discussed.     

Vitamin K status in cystic fibrosis

Appearance of PIVKA-II (protein induced by vitamin K absence-II) in serum is a biochemical sign of insufficient vitamin K-dependent carboxylation of prothrombin. Plasma concentrations of PIVKA-II and vitamin K1 were determined in 24 children with cystic fibrosis. Eight were supplemented with vitamin K1. The purpose of the study was to determine the occurrence of vitamin K deficiency in cystic fibrosis and to evaluate the effect of vitamin K supplementation. PIVKA-II was detectable in only one unsupplemented child. In this patient, the concentration of vitamin K1 was below the limit of detection of 60 ng/l. Vitamin K1 levels in the other unsupplemented children were normal (mean 476 ng/l = 1 mmol/l). The supplemented patients showed extremely high levels of vitamin K1 (mean 22445 ng/l = 50 nmol/l). In conclusion, vitamin K deficiency occurs infrequently in cystic fibrosis. Checking the coagulation system is advised, but routine vitamin K supplementation is not recommended. If additional vitamin K is needed, the starting dose should not exceed 1 mg daily.     

Osteolysis associated with a cemented modular posterior-cruciate-substituting total knee design : five to eight-year follow-up

BACKGROUND: Most intermediate and long-term studies of cemented posterior-cruciate-substituting total knee prostheses were performed with nonmodular tibial components. The purpose of this study was to evaluate the intermediate-term results of posterior-cruciate-substituting total knee arthroplasties in which a cemented modular tibial component had been used, with a particular focus on evaluating the prevalence of radiographic osteolysis. METHODS: Between 1992 and 1995, 176 consecutive primary total knee arthroplasties with use of the Insall-Burstein II system were performed in 134 patients at our institution. A modular metal-backed tibial component was inserted in 145 knees, and an all-polyethylene tibial component of the same design was inserted in thirty-one. Standard-terminology questionnaires were completed or Knee Society and The Hospital for Special Surgery scores were determined preoperatively and at the time of final follow-up, at an average of 6.4 years (range, 5.0 to 7.9 years). Initial postoperative radiographs were compared with those made at the time of final follow-up to assess component position, wear, radiolucent lines, and osteolysis. RESULTS: Ninety-two patients (128 knees) treated with the modular tibial component were alive at the time of final follow-up. No patient was lost to follow-up. Radiographs were available for 105 knees (82%). Three knees had been revised because of instability or infection; none had been revised because of loosening or osteolysis. The mean Knee Society clinical and functional scores were 85 points (range, 41 to 100 points) and 79 points (range, 30 to 100 points), respectively, at the time of final follow-up. According to The Hospital for Special Surgery score, 94% of the knees had a good or excellent result. Knee flexion averaged 113 degrees (range, 90 degrees to 130 degrees ) at the time of final follow-up. Osteolysis was present in seventeen (16%) of the knees with radiographic follow-up. Osteolysis did not develop in any knee in which an all-polyethylene tibial component had been used. Two knees (in one patient) were revised because of osteolytic lesions found at the time of follow-up for the study. Both of these knees had anterior wear of the tibial post due to impingement and backside tibial polyethylene wear. CONCLUSIONS: Modular Insall-Burstein II total knee prostheses were found to function well after five to eight years of follow-up. However, the high prevalence of osteolysis in patients who had good or excellent clinical scores is worrisome. Particular attention should be paid to preventing flexion of the femoral component, posterior slope of the tibial component, or hyperextension of the knee when posterior-cruciate-substituting total knee arthroplasty is performed. We also recommend routine follow-up radiographs after all total joint arthroplasties to detect asymptomatic osteolytic changes.     

Measurement of absolute values of hemoglobin oxygenation in the brain of small rodents by near infrared reflection spectrophotometry

Reflection near infrared spectroscopy (reNIRS) has been proposed as a novel technique for the measurement of absolute values of total hemoglobin (tHb), oxygenated hemoglobin (oxHb), hemoglobin saturation (SO2), and cytochrome aa3 oxidation status (oxCyt aa3) in living tissue. In this study, we evaluated reNIRS during physiological cerebral blood flow conditions in rats (n=6) and during the induction of global cerebral ischemia in gerbils (n=6). ReNIRS parameters were assessed over the exposed cerebral cortex and compared to regional cerebral blood flow (rCBF) data obtained by laser Doppler flowmetry. Under physiological conditions, reNIRS measurements reflected the large intra- and interindividual variability of oxHb and tHb in the brain. The absolute values obtained by reNIRS for tHb (6.3 +/- 1.7 mg/ml), oxHb (3.7 +/- 1.1 mg/ml), and SO2 (61 +/- 5%) matched expected values. In contrast, measurements of oxCyt aa3 were unstable and results unreliable. reNIRS reliably detected cerebral ischemia, verified by a reduction of rCBF to 11% of baseline. tHb dropped to 74 +/- 7% of baseline (P<0.001), reflecting ischemic microvascular vasoconstriction. oxHb and SO2 dropped to expected near-zero values (2 +/- 4 and 3 +/- 5% of baseline, respectively; P<0.001). We conclude that reNIRS provides reliable and reproducible absolute values for brain tissue tHb, oxHb, and SO2 in small rodents. Determination of physiological values requires measurements at multiple locations, while cerebral ischemia is reliably detected by continuous recordings at a single location.     

Selective neck occlusion of a large complex aneurysm of the middle cerebral artery trifurcation with the UltraSoft coil

We present a case of an unruptured, large, complex, middle cerebral artery trifurcation aneurysm that was successfully treated by selective occlusion of the neck with a single, newly available UltraSoft coil. The satisfactory initial anatomic result was stable, as demonstrated on a 3-month follow-up arteriogram that indicated complete anatomic cure. The novel UltraSoft coil offers additional possibilities in the endovascular management of difficult-to-treat vascular lesions.     

Effects of central dopaminergic stimulation by apomorphine on speech in Parkinson's disease

OBJECTIVE: To determine the effect of central dopaminergic stimulation with apomorphine on speech in PD. BACKGROUND: Most patients with PD have a speech disorder. Of those, 89% have involvement of laryngeal function, and 45% have additional articulatory dysfunction. The effect of dopaminergic medications on these two dimensions of speech impairment in PD has not been selectively studied. METHODS: In a randomized, double-blind, placebo-controlled crossover design, patients with PD and speech impairment, Hoehn and Yahr stages 2 to 4 "off," and without severe dyskinesias were given placebo or apomorphine injections 0.05 mg/kg subcutaneously during two consecutive outpatient visits. They were pretreated with domperidone for 48 hours and were tested off their parkinsonian medications for 12 hours. Laryngeal function was assessed by maximum sustained vowel phonations and comfortable vowel phonations. Articulatory function was evaluated by speech intelligibility score, speaking rate, and efficiency ratio. RESULTS: Ten patients, mean age 73.4 years (SD = 6.6), disease duration 8.7 years (SD = 6.3), were tested. The baseline motor score on the Unified Parkinson's Disease Rating Scale (UPDRSm) and all experimental speech variables were equivalent on both placebo and apomorphine days. At a dose of apomorphine that provoked improvement in UPDRSm (p = 0.0078), no index of either laryngeal or articulatory function improved significantly after apomorphine administration. CONCLUSION: Laryngeal and articulatory speech components are not under prominent dopaminergic control in PD. Treatment regimens should focus on nondopaminergic pharmacology and other therapies.     

Reliability of the life chart schedule for assessment of the long-term course of schizophrenia

We report on the inter-rater reliability of the Life Chart Schedule (LCS). The LCS is designed to assess the long-term course of schizophrenia in four key domains (symptoms, treatment, residence, and work) over two time periods (past two years, entire period of illness). The subjects were 27 consecutive admissions to a schizophrenia research unit. The LCS was filled out by pairs of raters, blinded to each others' ratings, using the same data (interview with subject and chart). Reliability was examined for 45 LCS ratings selected from all four domains and both time periods. Selected ratings pertained to the duration of specified experiences, the quality of these experiences, and the long-term time trend. The kappa statistic and the intra-class correlation coefficient (ICC) were used to determine inter-rater reliability for continuous and categorical ratings, respectively. LCS ratings proved reliable in all four key domains and both time periods. The reliability was fair to excellent for ratings of duration of experience (ICC ranged from 0.53 to 0.99), quality of experience (kappa ranged from 0.46 to 0. 92) and long-term time trends (kappa ranged from 0.66 to 0.94). The LCS can be used to obtain reliable ratings of the long-term course of schizophrenia in multiple domains.