Osteopathia striata congenita with cranial sclerosis and intellectual disability due to contiguous gene deletions involving the WTX locus.

Osteopathia striata congenita with cranial sclerosis (OSCS) is a skeletal dysplasia caused by germline deletions of or truncating point mutations in the X-linked gene WTX (FAM123B, AMER1). Females present with longitudinal striations of sclerotic bone along the long axis of long bones and cranial sclerosis, with a high prevalence of cleft palate and hearing loss. Intellectual disability or neurodevelopmental delay is not observed in females with point mutations in WTX leading to OSCS. One female has been described with a deletion spanning multiple neighbouring genes suggesting that deletion of some neighbouring loci may result in abnormal neurodevelopment. In this cohort of 13 females with OSCS resulting from deletions of WTX, a relationship is observed where deletion of ARHGEF9 and/or MTMR8 in conjunction with WTX results in an additional neurodevelopmental phenotype whereas deletion of ASB12 along with WTX is associated with a good neurodevelopmental prognosis.

Characterization of the nitrite production of mesophilic spore-forming bacteria during the handling of reconstituted infant formula.

The microbiological safety of reconstituted infant formula (RIF) has focused on infectious pathogens, whereas the risk of spore-forming bacteria (SFB) has been limited to spoilage and toxin production. This study suggests an underrecognized niche of SFB as nitrite producers during the handling of RIF. The production of nitrite along with the bacterial growth of 133 nitrite-producing SFB isolated from infant formula processing environments and end-products (70 mesophiles and 63 thermophiles) under RIF handling conditions were analysed. Most mesophiles (68 out of 70) and two thermophiles showed nitrite production during growth at 30 °C or 40 °C. Vigorous producers of nitrite [Bacillus sp. strains (FHS-PPBM449, 481, 236, 237)] showed a rapid onset of nitrite production (within 4 h). In particular, FHS-PPBM449 (2-3 log CFU/mL) exhibited the shortest onset time (210 min) and a nitrite production level up to 521 µM in RIF with 100 ppm nitrate at 40 °C. Overall, the results of the maximum level of nitrite produced by vigorous nitrite producers indicate that infants can consume more than seven times the acceptable daily intake of nitrite (0.74 mg for 12-month-old infants with an average body weight), even via a single feeding of RIF. An analysis of the relationship of the onset time of nitrite production with the bacterial concentration based on predictive models suggests that the growth of SFB up to 5-6 log CFU/mL is regarded as a prerequisite for nitrite production. This study revealed an underreported source of nitrite from RIF handling conditions, and the rapid onset of a high level of nitrite production from SFB should be the major target in the establishment of intervention strategies against nitrite as a microbial risk.

Consumer Trend of Using Ready-to-Use Salted Napa Cabbage at Home: Current Consumer Behaviors, Beliefs, and Opinions about the Main Ingredient of Kimchi.

ABSTRACT: Salted napa cabbage is the most important ingredient of kimchi. Currently, people have started to prepare ready-to-use salted napa cabbage at home. This study focused on this trend by investigating consumers' beliefs, opinions, and actual use of the products by conducting a telephone survey (895 female consumers) and face-to-face interviews (n = 514) in 2016 and a telephone survey (n = 200) in 2021. Most respondents (93 and 91% in 2016 and 2021, respectively) answered that convenience was the main reason for using salted napa cabbage. Regarding consumption behavior, 22 and 16% of the respondents in each year used salted napa cabbage after storing it for more than 24 h. In particular, 85 and 91% of consumers stored the product at room temperature, and 60 and 58% used it without washing, which could affect the quality of the food, as microorganisms could multiply during the storage. Inappropriate handling increased by age group, especially in 2021 (P < 0.05). In the query on satisfaction after using the products, 85 and 80% of respondents were satisfied because the product was convenient (54%) and hygienic (17%). Conversely, the respondents who were not satisfied with the products did not like the degree of salting and unhygienic status of the products. The majority (93 and 80%) of consumers preferred buying salted napa cabbage again because of its convenience. Although consumers thought that hygiene and quality were important factors, many respondents (83 and 72%) were not aware of foodborne illnesses associated with kimchi. Nevertheless, consumers intended to pay more for safe, salted napa cabbage (72 and 76%). The results of this study provide useful and credible data for understanding the factors affecting consumers' consumption and general beliefs and opinions on the use of salted napa cabbage, especially for food safety management.

Novel and recurrent TRPV4 mutations and their association with distinct phenotypes within the TRPV4 dysplasia family.

BACKGROUND: Mutations in TRPV4, a gene that encodes a Ca(2+) permeable non-selective cation channel, have recently been found in a spectrum of skeletal dysplasias that includes brachyolmia, spondylometaphyseal dysplasia, Kozlowski type (SMDK) and metatropic dysplasia (MD). Only a total of seven missense mutations were detected, however. The full spectrum of TRPV4 mutations and their phenotypes remained unclear. OBJECTIVES AND METHODS: To examine TRPV4 mutation spectrum and phenotype-genotype association, we searched for TRPV4 mutations by PCR-direct sequencing from genomic DNA in 22 MD and 20 SMDK probands. RESULTS: TRPV4 mutations were found in all but one MD subject. In total, 19 different heterozygous mutations were identified in 41 subjects; two were recurrent and 17 were novel. In MD, a recurrent P799L mutation was identified in nine subjects, as well as 10 novel mutations including F471del, the first deletion mutation of TRPV4. In SMDK, a recurrent R594H mutation was identified in 12 subjects and seven novel mutations. An association between the position of mutations and the disease phenotype was also observed. Thus, P799 in exon 15 is a hot codon for MD mutations, as four different amino acid substitutions have been observed at this codon; while R594 in exon 11 is a hotspot for SMDK mutations. CONCLUSION: The TRPV4 mutation spectrum in MD and SMDK, which showed genotype-phenotype correlation and potential functional significance of mutations that are non-randomly distributed over the gene, was presented in this study. The results would help diagnostic laboratories establish efficient screening strategies for genetic diagnosis of the TRPV4 dysplasia family diseases.

Application of finite element analysis in pre-operative planning for deformity correction of abnormal hip joints--a case series.

In experimental and clinical research, it is difficult to directly measure responses in the human body, such as contact pressure and stress in a joint, but finite element analysis (FEA) enables the examination of in vivo responses by contact analysis. Hence, FEA is useful for pre-operative planning prior to orthopaedic surgeries, in order to gain insight into which surgical options will result in the best outcome. The present study develops a numerical simulation technique based on FEA to predict the surgical outcomes of osteotomy methods for the treatment of slipped capital femoral epiphyses. The correlation of biomechanical parameters including contact pressure and stress, for moderate and severe cases, is investigated. For severe slips, a base-of-neck osteotomy is thought to be the most reliable and effective surgical treatment, while any osteotomy may produce dramatic improvement for moderate slips. This technology of pre-operative planning using FEA can provide information regarding biomechanical parameters that might facilitate the selection of optimal osteotomy methods and corresponding surgical options.

Factors Affecting Microbiological Quality of Vegetable- and Meat-Based Meals Served at Cafeterias in the Republic of Korea.

A total of 364 samples of vegetable- and meat-based meals were collected at three processing steps: step I, preparation of raw ingredients; step II, processing and cooking; and step III, finished meals. Microbiological quality was evaluated by using data for the prevalence and concentration of the aerobic plate counts, total coliforms (TC), fecal coliforms (FC), and Escherichia coli. The data were analyzed for differences between cafeterias, seasons, raw materials, and processing steps. Fourteen (15.2%) of the 92 finished meal samples were microbiologically unsatisfactory. Neither cafeteria nor season was significantly associated with microbiological quality ( P > 0.05). However, the type of raw ingredients and processing steps were significantly associated with differences in microbiological quality. Vegetable-based meals had higher TC concentrations than meat-based meals because salad and seasoned and fermented vegetables are not cooked, unlike heat-processed meat products. Microbial counts tended to decrease through the processing steps, and E. coli, which could only be enumerated on uncooked chicken breast (1.6 log CFU/g) and sliced pork (2.6 log CFU/g), was totally eliminated by boiling and roasting. However, the presence of FC was not completely eliminated, even by cooking, and so this group of organisms should be considered as an important indicator of hygienic meal preparation in cafeterias. Although pathogenic E. coli was not isolated in this study, continuous microbiological monitoring of composite foods served in cafeterias should be performed as the presence of TC and FC in finished meals indicates the potential for contamination by pathogenic E. coli.

Inheritance and Organization of Glycinin Genes in Soybean.

Five genes (Gy1, through Gy5) encode most of the subunits that are assembled into glycinin, a predominant seed storage protein found in soybeans. Restriction fragment length polymorphisms are described that identify four of these five genes (Gy1/Gy2, Gy3, and Gy5). The fifth gene (Gy4) is characterized by two alleles, one of which (gy4) causes absence of the subunit. Genetic segregation studies indicate that the five genes are located at four genetic loci within the genome. Gy1 and Gy2 are in a direct tandem repeat at one locus, whereas there is a single glycinin gene at each of the other three loci. All four loci segregate independently from one another, and they also segregate independently from the genetic markers for tawny/grey pubescence (T/t), purple/white flower color (W1/w1), light/dark hilum pigmentation (l/ll), black/brown seed coat (R/r), and brown/tan pod color (I1I1L2L2/I1I1I2I2). The latter genetic markers are located on linkage groups 1 (t), 8 (w1), 7 (i), and 2 (r) in the soybean genome, respectively.

Morphometric analysis of acetabular dysplasia in cerebral palsy.

We carried out a morphometric analysis of acetabular dysplasia in patients with cerebral palsy requiring hip surgery using three-dimensional CT. We evaluated 54 hips in 27 patients. The contralateral normal hips of ten patients with unilateral Perthes' disease were used as a control group. The acetabular defects were assessed qualitatively as anterior, posterior or global. Quantitative assessments were made using three-directional acetabular indices (anterosuperior, superolateral and posterosuperior) and measured by multiplanar reformation, from which we calculated the acetabular volume. In the qualitative study, posterior defects were most common in the subluxation group whereas global defects predominated in the dislocation group. In the quantitative study, all acetabular indices in both the subluxation and dislocation groups were higher than those in the control group and the superolateral indices showed a tendency to elevation in the dislocation group. The acetabular volume was largest in the control group, smallest in the dislocation group, and intermediate between the two in the subluxation group.

Survival of foodborne pathogens (Escherichia coli O157:H7, Salmonella Typhimurium, Staphylococcus aureus, Listeria monocytogenes, and Vibrio parahaemolyticus) in raw ready-to-eat crab marinated in soy sauce.

Knowing the survival characteristics of foodborne pathogens in raw ready-to-eat (RTE) seafood is the key to predicting whether they pose a microbiological hazard. The present study examined the survival of Escherichia coli O157:H7, Salmonella Typhimurium, Vibrio parahaemoliticus, Listeria monocytogenes, and Staphylococcus aureus in raw RTE crab marinated in soy sauce. Inoculated crabs (initial bacterial population=4.1-4.4logCFU/g) were immersed in soy sauce and then stored at refrigeration (5°C) or room temperature (22°C) for up to 28days. At 5°C, all bacteria (except V. parahaemolyticus) survived in crab samples until Day 28 (counts of 1.4, 1.6, 3.1, 3.2 log CFU/g for E. coli O157:H7, S. Typhimurium, L. monocytogenes, and S. aureus, respectively). However, at 22°C, all tested bacteria were more susceptible to the antimicrobial effects of marination. Regardless of temperature, foodborne pathogens attached to crab samples were more resistant to marination than those suspended in soy sauce samples; however, the survival pattern for each species was different. Gram-positive bacteria were most resistant to marination conditions (high salinity, low pH), whereas V. parahaemolyticus was extremely susceptible. Marination is the only antibacterial step in the manufacturing processes; however, the results presented herein reveal that this is not sufficient to inactivate foodborne pathogens. In particular, the survival of pathogens on crabs at refrigeration temperature may pose a major hazard for the consumption of raw RTE seafood. Thus, appropriate decontamination methods and implementation of safety management practices are needed. This study provides predictive microbiological information of foodborne pathogens in raw RTE seafood with marination.

Tumor necrosis factor alpha (TNF-alpha) coordinately regulates the expression of specific matrix metalloproteinases (MMPS) and angiogenic factors during fracture healing.

Recent studies from our laboratory demonstrate that TNF-alpha signaling contributes to the regulation of chondrocyte apoptosis and a lack of TNF-alpha signaling leads to a persistence of cartilaginous callus and delayed resorption of mineralized cartilage. This study examines how delays in the endochondral repair process affect the expression of specific mediators of proteolytic cartilage turnover and vascularization. Simple closed fractures were produced in wild type and TNF-alpha receptor (p55-/-/p75-/-)-deficient mice. Using ribonuclease protection assay (RPA) and microarray analysis, the expression of multiple mRNAs for various angiogenic factors and the metalloproteinase gene family were measured in fracture calluses. The direct actions of TNFalpha on the expression of specific angiogenic factors and metalloproteinases (MMPs) was examined in both cultured callus cells and articular chondrocytes to compare the effects of TNF-alpha in growth cartilage versus articular cartilage. MMPs 2, 9, 13, and 14 were quantitatively the most prevalent metalloproteases and all showed peaks in expression during the chondrogenic period. In the absence of TNF-alpha signaling, the expression of all of these mRNAs was reduced. The angiopoietin families of vascular regulators and their receptors were expressed at much higher levels than the VEGFs and their receptors and while the angiopoietins showed diminished or delayed expression in the absence of TNF-alpha signaling, VEGF and its receptors remained unaltered. The expression of vascular endothelial growth inhibitor (VEGI or TNFSF15) showed a near absence in its expression in the TNF-alpha receptor-deficient mice. In vitro assessment of cultured fracture callus cells in comparison to primary articular chondrocytes showed that TNF-alpha treatment specifically induced the expression of MMP9, MMP14, VEGI, and Angiopoietin 2. These results suggest that TNF-alpha signaling in chondrocytes controls vascularization of cartilage through the regulation of angiopoietin and VEGI factors which play counterbalancing roles in the induction of growth arrest, or apoptosis in endothelial cells. Furthermore, TNF-alpha appears to regulate, in part, the expression of two key proteolytic enzymes, MMP 9 and MMP14 that are known to be crucial to the progression of vascularization and turnover of mineralized cartilage. Thus, TNF-alpha signaling in healing fractures appears to coordinate the expression of specific regulators of endothelial cell survival and metalloproteolytic enzymes and is essential in the transition and progression of the endochondral phase of fracture repair.

The bicompartmental acetabulum in Perthes' disease: 3D-CT and MRI study.

The bicompartmental acetabulum is one of the morphological changes which may be seen in children with Legg-Calvé-Perthes' disease. Three-dimensional CT and MRI were used to analyse the detailed morphology of the acetabulum with special reference to its inner surface, in 16 patients with Perthes' disease and a bicompartmental acetabulum.The bicompartmental appearance was seen on the coronal plane image through the acetabular fossa. The lunate surface was seen to grow laterally resulting in an increased mediolateral thickness of the triradiate cartilage. On the horizontal plane images, the acetabular fossa had deepened and had a distinct prominence at its posterior border. The combination of these morphological changes resulted in a bicompartmental appearance on plain radiography. Acetabular bicompartmentalisation appears to be the result of an imbalance of growth between the cartilage-covered lunate surface and the cartilage-devoid acetabular fossa.

Changes in the Microbial Composition of Microbrewed Beer during the Process in the Actual Manufacturing Line.

This study investigated changes in the microbial composition of microbrewed beer during the manufacturing processes and identified potential microbial hazards, effective critical quality control points, and potential contamination routes. Comprehensive quantitative (aerobic plate count, lactic acid bacteria, fungi, acetic acid bacteria, coliforms, and Bacillus cereus) and qualitative (Escherichia coli and eight foodborne pathogens) microbiological analyses were performed using samples of raw materials (malt and manufacturing water), semiprocessed products (saccharified wort, boiled wort, and samples taken during the fermentation and maturation process), and the final product obtained from three plants. The initial aerobic plate count and lactic acid bacteria counts in malt were 5.2 and 4.3 log CFU/g, respectively. These counts were reduced to undetectable levels by boiling but were present at 2.9 and 0.9 log CFU/ml in the final product. Fungi were initially present at 3.6 log CFU/g, although again, the microbes were eliminated by boiling; however, the level in the final product was 4.6 log CFU/ml. No E. coli or foodborne pathogens (except B. cereus) were detected. B. cereus was detected at all stages, although it was not present in the water or boiled wort (total detection rate » 16.4%). Results suggest that boiling of the wort is an effective microbial control measure, but careful management of raw materials and implementation of effective control measures after boiling are needed to prevent contamination of the product after the boiling step. The results of this study may constitute useful and comprehensive information regarding the microbiological quality of microbrewed beer.

Impaired fracture healing in the absence of TNF-alpha signaling: the role of TNF-alpha in endochondral cartilage resorption.

UNLABELLED: TNF-alpha is a major inflammatory factor that is induced in response to injury, and it contributes to the normal regulatory processes of bone resorption. The role of TNF-alpha during fracture healing was examined in wild-type and TNF-alpha receptor (p55(-/-)/p75(-/-))-deficient mice. The results show that TNF-alpha plays an important regulatory role in postnatal endochondral bone formation. INTRODUCTION: TNF-alpha is a major inflammatory factor that is induced as part of the innate immune response to injury, and it contributes to the normal regulatory processes of bone resorption. METHODS: The role of TNF-alpha was examined in a model of simple closed fracture repair in wild-type and TNF-alpha receptor (p55(-/-)/p75(-/-))-deficient mice. Histomorphometric measurements of the cartilage and bone and apoptotic cell counts in hypertrophic cartilage were carried out at multiple time points over 28 days of fracture healing (n = 5 animals per time point). The expression of multiple mRNAs for various cellular functions including extracellular matrix formation, bone resorption, and apoptosis were assessed (triplicate polls of mRNAs). RESULTS AND CONCLUSIONS: In the absence of TNF-alpha signaling, chondrogenic differentiation was delayed by 2-4 days but subsequently proceeded at an elevated rate. Endochondral tissue resorption was delayed 2-3 weeks in the TNF-alpha receptor (p55(-/-)/p75(-/-))-deficient mice compared with the wild-type animals. Functional studies of the mechanisms underlying the delay in endochondral resorption indicated that TNF-alpha mediated both chondrocyte apoptosis and the expression of proresorptive cytokines that control endochondral tissue remodeling by osteoclasts. While the TNF-alpha receptor ablated animals show no overt developmental alterations of their skeletons, the results illustrate the primary roles that TNF-alpha function contributes to in promoting postnatal fracture repair as well as suggest that processes of skeletal tissue development and postnatal repair are controlled in part by differing mechanisms. In summary, these results show that TNF-alpha participates at several functional levels, including the recruitment of mesenchymal stem, apoptosis of hypertrophic chondrocytes, and the recruitment of osteoclasts function during the postnatal endochondral repair of fracture healing.

Expression of osteoprotegerin, receptor activator of NF-kappaB ligand (osteoprotegerin ligand) and related proinflammatory cytokines during fracture healing.

Fracture healing is a unique biological process regulated by a complex array of signaling molecules and proinflammatory cytokines. Recent evidence for the role of tumor necrosis family members in the coupling of cellular functions during skeletal homeostasis suggests that they also may be involved in the regulation of skeletal repair. The expression of a number of cytokines and receptors that are of functional importance to bone remodeling (osteoprotegerin [OPG], macrophage colony-stimulating factor [M-CSF], and osteoprotegerin ligand [receptor activator of NF-kappaB ligand (RANKL)]), as well as inflammation (tumor necrosis factor alpha [TNF-alpha] and its receptors, and interleukin-1alpha [IL-1alpha] and -beta and their receptors) were analyzed over a 28-day period after the generation of simple transverse fractures in mouse tibias. OPG was expressed constitutively in unfractured bones and elevated levels of expression were detected throughout the repair process. It showed two distinct peaks of expression: the first occurring within 24 h after fracture and the second at the time of peak cartilage formation on day 7. In contrast, the expression of RANKL was nearly undetectable in unfractured bones but strongly induced throughout the period of fracture healing. The peak in expression of RANKL did not correlate with that of OPG, because maximal levels of expression were seen on day 3 and day 14, when OPG levels were decreasing. M-CSF expression followed the temporal profile of RANKL but was expressed at relatively high basal levels in unfractured bones. TNF-alpha, lymphotoxin-beta (LT-beta), IL-1alpha, and IL-1beta showed peaks in expression within the first 24 h after fracture, depressed levels during the period of cartilage formation, and increased levels of expression on day 21 and day 28 when bone remodeling was initiated. Both TNF-alpha receptors (p55 and p75) and the IL-1RII receptor showed identical patterns of expression to their ligands, while the IL-1R1 was expressed only during the initial period of inflammation on day 1 and day 3 postfracture. Both TNF-alpha and IL-1alpha expression were localized primarily in macrophages and inflammatory cells during the early periods of inflammation and seen in mesenchymal and osteoblastic cells later during healing. TNF-alpha expression also was detected at very high levels in hypertrophic chondrocytes. These data imply that the expression profiles for OPG, RANKL, and M-CSF are tightly coupled during fracture healing and involved in the regulation of both endochondral resorption and bone remodeling. TNF-alpha and IL-1 are expressed at both very early and late phases in the repair process, which suggests that these cytokines are important in the initiation of the repair process and play important functional roles in intramembraneous bone formation and trabecular bone remodeling.

Vascular proliferation and blood supply during distraction osteogenesis: a scanning electron microscopic observation.

This scanning electron microscopic study examined the spatial and temporal features of proliferating vessels of regenerating bone tissue and blood supply during distraction osteogenesis. A rat model of tibial lengthening was used with a protocol divided into a latency period of 7 days, a distraction period that lasted 14 days with a daily distraction rate of 0.5 mm in two steps, and a consolidation period of 21 days. Vascular casting was done on the hindlimbs before osteotomy and on postoperative days 7, 14, 21, 28, and 42. Scanning electron microscopic findings were correlated with radiological and histological observations. On days 7 and 14, the proliferation of periosteal vessels was pronounced and there was distinct subperiosteal bone formation on the osteotomized surfaces. On day 21, vascular branches from the medullary canal of the host bone formed a vascular network, which gave rise to multiple axial, straight vascular branches, running parallel to the direction of distraction, toward the interzone, in accordance with the progress of mineralization. On day 28, the periosteum provided vascularization to the peripheral side of the interzone whereas the center of the interzone was still relatively avascular. On day 42, the periosteal and medullary vascular channels were completely connected at the distraction site including the interzone, which was occupied by developing and mature bone trabeculae. These results suggest that vascular proliferation occurs actively during the latency and distraction periods and then gradually decreases over time. A close temporal and spatial relationship exists between formation of regenerated bone and vascular proliferation of the periosteum and medullary canal.

Large-scale analysis of expressed genes from the leaf of oilseed rape (Brassica napus L.).

While the number of leaf-specific expressed genes is estimated to be approximately 6,000, an overview of gene diversity and expression patterns in the leaf of oilseed rape (Brassica napus L.) has not yet been reported. In an effort to understand gene expression patterns and to identify new genes, we generated 754 expressed sequence tags (ESTs) from the leaf of B. napus. By comparing them to public databases, we showed that 204 of the ESTs (27.1%) have sequence homology to known genes, with 52 of them (6.9%) matching to genes not previously studied in B. napus. The most abundant transcripts were found to be involved in photosynthesis and energy metabolism. When compared with maize leaf ESTs and rice leaf ESTs, the pattern of gene expression was different depending on the developmental stages of the leaf.

The Sprengel deformity. Morphometric analysis using 3D-CT and its clinical relevance.

We evaluated scapular dysplasia and malposition in 15 patients with the Sprengel deformity using three-dimensional CT (3D-CT). The shape, height-to-width ratio, the areas of both scapulae, the anterior curvature of the supraspinous portion and glenoid version were assessed on scapular posterior, medial and inferior views. The degree of rotation and superior displacement were measured on the trunk posterior view. The omovertebral connection was also assessed and correlated with the operative findings. Most of the affected scapulae had a characteristic shape with a decrease in the height-to-width ratio and were larger than the contralateral scapulae. There was an inverse relationship between scapular rotation and superior displacement. The typical curve of the supraspinous portion of the scapula was seen in only three cases. There was no significant difference in glenoid version. The point of tethering of the omovertebral connection may determine the shape, rotation and superior displacement of the scapula. 3D-CT was helpful in delineating the deformity in detail, and in planning scapuloplasty.

Valgus femoral osteotomy for hinge abduction in Perthes' disease. Decision-making and outcomes.

We studied, clinically and radiologically, the growth and remodelling of 21 hips after valgus femoral osteotomy with both rotational and sagittal correction for hinge abduction in 21 patients (mean age, 9.7 years) with Perthes' disease. The exact type of osteotomy performed was based on the pre-operative clinical and radiological assessment and the results of intra-operative dynamic arthrography. The mean IOWA hip score was 66 (34 to 76) before surgery and 92 (80 to 100) at a mean follow-up of 7.1 years (3.0 to 15.0). Radiological measurements revealed favourable remodelling of the femoral head and improved hip joint mechanics. Valgus osteotomy, with both rotational and sagittal correction, can improve symptoms, function and remodelling of the hip in patients with Perthes' disease.

Correction of genu recurvatum by the Ilizarov method.

The Ilizarov apparatus was used to carry out opening-wedge callotasis of the proximal tibia in ten patients who had suffered premature asymmetrical closure of the proximal tibial physis and subsequent genu recurvatum. In four knees, the genu recurvatum was entirely due to osseous deformity, whereas in six it was associated with capsuloligamentous abnormality. Preoperatively, the angle of recurvatum averaged 19.6 degrees (15 to 26), the angle of tilt of the tibial plateau, 76.6 degrees (62 to 90), and the ipsilateral limb shortening, 2.7 cm (0.5 to 8.7). The average time for correction was 49 days (23 to 85). The average duration of external fixation was 150 days (88 to 210). Three patients suffered complications including patella infera, pin-track infection and transient peroneal nerve palsy. At a mean follow-up of 4.4 years, all patients, except one, had achieved an excellent or good radiological and functional outcome.

The treatment of recurrent arthrogrypotic club foot in children by the Ilizarov method. A preliminary report.

Between 1994 and 1997 we used the Ilizarov apparatus to treat 12 recurrent arthrogrypotic club feet in nine patients with a mean age of 5.3 years (3.2 to 7). After a mean of three weeks (two to seven) for correction of the deformity and 1.5 weeks (one to four) for stabilisation in the apparatus, immobilisation in a cast was carried out for a mean of 14 weeks (7 to 24). The mean follow-up period was 35 months (27 to 57). Before operation there were one grade-II (moderate), eight grade-III (severe) and three grade-IV (very severe) club feet, according to the rating system of Dimeglio et al. After operation, all the club feet except one were grade I (benign) with a painless, plantigrade platform. Radiological assessment and functional evaluation confirmed significant improvement. Two complications occurred in one patient, namely, epiphysiolysis of the distal tibia and recurrence of the foot deformity. These results suggest that our proposed modification of the Ilizarov technique is effective in the management of recurrent arthrogrypotic club foot in young children.