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Aim: This study aimed to determine if a new measure of sphericity of the femoral head, the Sphericity Deviation Score (SDS), could be estimated reliably at the stage of healing of Legg-Calvé-Perthes disease (LCPD) and to determine if the SDS at the healing stage reflects the outcome at skeletal maturity., Methods: The SDS was measured with image analysis software on radiographs of 120 children with unilateral LCPD at healing of the disease and at skeletal maturity. The reproducibility of measurement was assessed. SDS values for hips in different Stulberg classes were calculated. On the basis of SDS values at healing of 82 children, a logistic regression model was developed to determine the probability of a good outcome at skeletal maturity. The validity of the model was tested on another 38 children., Results: The SDS values at the stage of healing and at skeletal maturity were comparable. The SDS values were lowest for Stulberg Class I hips and highest for Class IV and V hips. The validated regression model showed a very high probability of a good outcome (Stulberg Class I or II) at skeletal maturity if the SDS at the stage of healing was below 10., Conclusions: A reliable estimate of the outcome of LCPD at skeletal maturity can be made by computing the SDS as soon as the disease heals.

A 34-year-old male, non-hypertensive, non-diabetic, had a sudden onset of cardiac arrest, he was revived and then referred to a cardiologist at an advanced center for further care. His 2D echo revealed hypertrophic obstructive cardiomyopathy, all the other investigations were normal. His father was also found to have a similar condition however the father was asymptomatic. The patient underwent an automated implantable cardioverter-defibrillator implantation and was advised the necessary precautions and care.

Osteogenesis imperfecta (OI) is a condition of decreased bone density with heterogeneous etiologies. Most of the cases are inherited in an autosomal dominant fashion and are caused by mutations in the COL1A1 or COL1A2 genes. Since these two genes are very large, there are no data about mutations in Indian patients with OI. We selected 35 Indian patients who were clinically diagnosed with OI and all exons of both the genes were sequenced. Mutations in COL1A1 (14 cases, 6 novel) and COL1A2 (11 cases, 7 novel) were identified in 25 patients. A total of 55 polymorphisms were identified in both the genes with eight novel variants in the coding region, and nine novel variants in the non-coding regions. No mutation was detected in 10 patients. Six of them were from consanguineous families, with one or two similarly affected siblings suggesting possible autosomal recessive inheritance. If we exclude families with consanguinity, mutations were identified in 25 out of 29 families giving 86% mutation detection rate. Mutations in COL1A1 accounted for 56% of the cases and COL1A2 44%, which is similar to the reported rate worldwide., (© 2014 Wiley Periodicals, Inc.)

Foreign body granulomas are common in the hand and foot. Diagnosis of the same is often delayed or missed, especially in unusual locations and in the paediatric age group. Radiographs usually reveal an osteolytic lesion with absence of periosteal reaction and non-metallic foreign bodies are not visualised in plain radiographs. An ultrasound and MRI can delineate the foreign body. Clinical and radiological presentation mimics that of a tumour and hence is rightly called pseudotumour. We present a case of thorn-induced pseudotumour of the fibula in a 5-year-old boy., (2014 BMJ Publishing Group Ltd.)

Currently employed markers for the detection of acute coronary syndrome are Troponin T, CK (Creatine Kinase) and CKMB activity. CKMB activity measured by immunoinhibition method can give falsely elevated results due to the presence of atypical CK and CKBB and at times lead to the mis-diagnosis of acute coronary syndrome. Hence, CKMB mass (CKMB) measured by electrochemiluminence sandwich principle was employed. In this cross-sectional study 183 samples of 61 patients were analyzed within 6 h of diagnosis of acute coronary syndrome and followed up to 72 h. The correlation coefficient between CKMB activity and CKMBM at 4-6 h was 0.744, while at 12-24 h it was 0.909 and at 48-72 h it was 0.337. Thus there was good association between the two methods at 12-24 h but, statistically for method comparison studies and for replacing one method by another, the two methods need to be in agreement with one another. In this study the two methods are not in agreement with one another and thus analytically not replaceable. Another finding was obtained that CKMBM reached cut off levels prior to CKMB enzyme activity and hence, CKMBM is clinically better than CKMB activity to detect reinfarction.

Werner mesomelia is characterized by a sequence variation in the specific region (position 404) of the enhancer ZRS of SHH. The phenotype comprises variable mesomelia, abnormalities of the thumb and great toe and supernumerary digits. We describe extensive variation in limb phenotype in a large family and report on a novel sequence variation NG_009240.1: g.106737G>T (traditional nomenclature: ZRS404G>T) in the ZRS within the LMBR1 gene. The newly recognized clinical features in this family include small thenar eminence, sandal gap, broad first metatarsals, mesoaxial polydactyly, and postaxial polydactyly. We provide information on 12 affected family members. We review the literature on how a sequence variation in ZRS may cause such diverse phenotypes., (© 2014 Wiley Periodicals, Inc.)

We report on an adolescent girl with sparse scalp hair, wide columella extending below alae nasi, webbing at elbows, broad finger tips, short distal phalanx of fingers, swan neck deformity of fingers, scoliosis, tall vertebrae, short fibulae, short fourth metatarsal bone, abnormal distal humeri, and unilateral clubfoot at birth. The combination of these features represents a novel phenotype. We sequenced the protein-coding regions of the FLNA and FLNB genes and did not observe any pathogenic sequence variation. Chromosomal microarray revealed a de novo copy number variation of uncertain clinical significance on 7p22.3., (© 2014 Wiley Periodicals, Inc.)

Perthes disease refers to self-limiting idiopathic avascular necrosis of capital femoral epiphysis in a child. There is no consensus for the optimum treatment of Perthes disease even 100 years after the first description. The prime aim of the treatment is to maintain the sphericity of the femoral head and the congruency of the femur-acetabulum relationship to prevent secondary degenerative arthritis. Early diagnosis and management can help the collapse of femoral head, progressive femoral head deformity, and impingement., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Though there is an impression that proximal femoral varus osteotomy (FVO) can result in a valgus deformity at the knee there is no agreement on this issue. This study was undertaken to ascertain whether a FVO predisposes to the development of genu valgum in children with Legg-Calvé-Perthes disease (LCPD)., Methods: One hundred and one children with unilateral LCPD who underwent a FVO during the active stage of the disease and 32 children who were treated non-operatively were followed till skeletal maturity. The FVO was performed with a 20° varus angulation in all the patients and weight-bearing was not permitted till the stage of reconstitution. The alignment of the knee was assessed clinically at skeletal maturity. A subset of 33 operated children also had full length standing radiographs of the limbs. The mechanical axis deviation, femur-tibial angle, lateral distal femoral angle and the medial proximal tibial angle of both limbs were measured on these radiographs., Results: The frequency of clinically appreciable mal-alignment of the knee was not greater on the affected side in patients who had undergone FVO when compared to the unaffected limb and also when compared to the affected limb in non-operated patients. The mechanical axis of the lower limb of operated children was relatively in more valgus than that of normal limbs but they fell within the normal range., Conclusion: This study does not support the impression that a proximal femoral osteotomy for LCPD predisposes to clinically discernable degrees of genu valgum in children who have had 20° of varus angulation at the osteotomy site and who have avoided weight-bearing for a prolonged period following surgery. Further studies are needed to clarify if genu valgum would develop if early post-operative weight-bearing is permitted., Level of Evidence: III.

Objective: This study was undertaken to identify variations in the hip joint space in patients with healed Legg-Calvé-Perthes' disease (LCPD) and to correlate these changes with the radiological outcome of treatment., Methods: The width of the joint space was measured on radiographs of 121 skeletally mature individuals with healed LCPD. The joint space width at the stage of healing in 79 patients was compared with the joint space at skeletal maturity. The hips were graded by the Stulberg classification and the femoral head radius was measured. Associations between the joint space width, the Stulberg class and the femoral head radius were tested. The reproducibility of measurement was tested by measuring 30 radiographs twice and computing the intra-class correlation coefficient (ICC). The joint space width was measured on radiographs of 25 patients taken both while recumbent and standing, to determine if the joint space alters on weight-bearing., Results: The reproducibility of measurements was satisfactory; no difference was noted in the joint space in recumbent and weight-bearing films. Increased joint space was noted both at healing of the disease and at skeletal maturity; the width was minimally increased in Stulberg Class I hips and considerably increased in Class III, IV and V hips. There was a significant correlation between the increase in joint space and enlargement of the femoral head., Conclusions: An increase in joint space width occurs quite frequently following LCPD; the increase is most evident in hips with a poor outcome. The implications of this observation need to be elucidated.

Background: Large sample sizes are needed if discrete outcome measures are used to perform outcome studies, whereas more practicable sample sizes are sufficient if continuous outcome measures are used. Recognizing the need to devise reliable continuous outcome measures for assessing the outcomes of treatment of Legg-Calvé-Perthes disease, we evaluated a method of quantifying the shape and size of the femoral head and the femoral-acetabular relationship from computer images of anteroposterior and lateral radiographs with the help of image-analysis software., Methods: Radiographs of 121 skeletally mature individuals with healed Legg-Calvé-Perthes disease were analyzed, and the sphericity deviation score, femoral head enlargement, femoral neck growth inhibition, and composite femoral congruency arc were calculated., Results: Each of these measurements was reproducible, with distinctly different values for hips in each of the Stulberg classes. Measures of sphericity and congruence (the sphericity deviation score and the composite femoral congruency arc) could very clearly discriminate between spherical and aspherical hips, and there was a strong correlation between these two measurements., Conclusions: The study shows that it is possible to have reliable quantitative measures of the outcome of Legg-Calvé-Perthes disease at skeletal maturity.

Congenital pseudarthrosis of the tibia (CPT) is a rare pathology, which is usually associated with neurofibromatosis type I. The natural history of the disease is extremely unfavorable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously. It is challenging to treat effectively this difficult condition and its possible complications. Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture. The key to get primary union is to excise hamartomatous tissue and pathological periosteum. Age at surgery, status of fibula, associated shortening, and deformities of leg and ankle play significant role in primary union and residual challenges after primary healing. Unfortunately, none of invasive and noninvasive methods have proven their superiority. Surgical options such as intramedullary nailing, vascularized fibula graft, and external fixator, have shown equivocal success rate in achieving primary union although they are often associated with acceptable results. Amputation must be reserved for failed reconstruction, severe limb length discrepancy and gross deformities of leg and ankle. Distinct advantages, complications, and limitation of each primary treatment as well as strategies to deal with potential complications have been described. Each child with CPT must be followed up till skeletal maturity to identify and rectify residual problems after primary healing.

We report on a 14-year-old girl with growth deficiency, microcephaly, intellectual disability, distinctive dysmorphic features (bulbous nose with wide nasal base, hypotelorism, deeply set eyes, protruding cupped ears, and thick lower lip), cataract, pigmentary retinopathy, hypoplastic thorax, kyphoscoliosis, and unusual skeletal changes but without chromosomal imbalances detected by array-CGH who probably represents a novel phenotype., (Copyright © 2012 Wiley Periodicals, Inc.)

Progressive pseudorheumatoid dysplasia (PPD) is a progressive skeletal syndrome characterized by stiffness, swelling and pain in multiple joints with associated osteoporosis in affected patients. Radiographically, the predominant features resemble a spondyloepiphyseal dysplasia. Mutations in the WISP3 gene are known to cause this autosomal recessive condition. To date, only a limited number of studies have looked into the spectrum of mutations causing PPD. We report on clinical features and WISP3 mutations in a large series of Indian patients with this rare skeletal dysplasia. Families with at least one member showing clinical and radiologic features of PPD were recruited for the study. Symptoms, signs and radiographic findings were documented in 35 patients from 25 unrelated families. Swelling of small joints of hands and contractures are the most common presenting features. Mutation analysis was carried out by bidirectional sequencing of the WISP3 gene in all 35 patients. We summarize the clinical features of 35 patients with PPD and report on 11 different homozygous mutations and one instance of compound heterozygosity. Eight (c.233G>A, c.340T>C, c.348C>A, c.433T>C, c.682T>C, c.802T>G, c.947_951delAATTT, and c.1010G>A) are novel mutations and three (c.156C>A, c.248G>A, and c.739_740delTG) have been reported previously. One missense mutation (c.1010G>A; p.Cys337Tyr) appears to be the most common in our population being seen in 10 unrelated families. This is the largest cohort of patients with PPD in the literature and the first report from India on mutation analysis of WISP3. We also review all the mutations reported in WISP3 till date., (Copyright © 2012 Wiley Periodicals, Inc.)

Purpose: We encountered problems with the Curtis and Fisher technique of quadricepsplasty for congenital quadriceps contracture, including wound dehiscence, insufficient lengthening of the quadriceps and instability of the knee. We modified the operative technique to address these three problems. We undertook this study to evaluate the results of the modified technique of quadricepsplasty to determine if we succeeded in overcoming these limitations of the original technique., Methods: Twenty children (33 knees) underwent the modified Curtis and Fisher quadricepsplasty through a lateral incision; a long tongue of the rectus femoris was raised and the vasti mobilised without dividing the lateral retinaculae till the collateral ligaments. The children were followed up for a mean period of 63 months and evaluated. The healing of the wound, active and passive range of motion (ROM) of the knee, the stability of the knee, quadriceps power and knee function were assessed., Results: Primary wound healing occurred in 32 of 33 knees. Adequate lengthening of the quadriceps sufficient to facilitate knee flexion to 90° was possible. Considerable improvement in the ROM was noted. In non-syndromic congenital dislocation of the knee (CDK), the quadriceps power was Grade 5, but minor degrees of extensor lag was noted. In a proportion of patients, minor degrees of joint instability was present. The majority of children were community walkers. The overall results were better in non-syndromic CDK than in children with arthrogryposis, but differences of some variables were not significant., Conclusion: The modifications to the original Curtis and Fisher technique overcame the specific problems they were expected to avoid.

Background: The etiology of Legg-Calvé-Perthes disease (LCPD) remains unknown. A few studies have suggested passive smoke inhalation may be a risk factor, although the association is not confirmed and a causal relationship has not been established., Questions/purposes: We therefore undertook this study to confirm an association between environmental tobacco smoke, firewood smoke, and socioeconomic status and the risk of LCPD., Methods: We prospectively recruited 128 children with LCPD and 384 children attending the hospital for other orthopaedic complaints. The control subjects were frequency-matched with the cases by age and gender. Conditional logistic regression was used to assess the association between the exposures and risk of LCPD., Results: The main risk factors for LCPD were indoor use of a wood stove (adjusted odds ratio [OR], 2.56) and having a family member who smoked indoors (adjusted OR, 2.07). Children from the middle socioeconomic group appeared to be at a greater risk of developing LCPD (adjusted OR, 3.60)., Conclusions: This study provides further evidence that environmental tobacco smoke is associated with an increased risk of LCPD. Exposure to wood smoke also appears to be a risk factor. However, it remains unclear why there are profound differences in the incidence of the disease between regions when the prevalence of smoking is comparable and why bilateral involvement and familial disease are infrequent.

Purpose: This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD(HG))., Material and Methods: Radiographs of 271 adult patients with SEMD(HG) were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips., Results: Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores., Conclusion: Degenerative arthritis of the hip develops in the majority of patients with SEMD(HG); the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned.

Purpose: The study was undertaken to: (1) describe the characteristic radiological features and problems of management of the loss of one condyle of the femur or tibia following septic arthritis of the knee in infancy and (2) test a hypothesis of the cause of the loss of a single condyle., Methods: Radiographs of eight children with the loss of one condyle of the femur or the tibia following septic arthritis in infancy were reviewed. The course and outcome in two of these children who underwent reconstructive operations were studied. The knees of 35 stillborn foetuses were dissected to determine if the presence of synovial septae could account for the isolated loss of one condyle following infection., Results: All eight cases showed characteristic features of loss of half the epiphysis, the underlying physis and part of the adjacent metaphysis; the other condyle was totally spared. The two children who underwent elaborate reconstructive procedures had poor outcomes at skeletal maturity, despite a series of additional operations. The foetal cadaveric study showed that complete infrapatellar synovial septae are present in some foetuses approaching 40 weeks of gestation., Conclusions: The pattern of loss of a femoral or tibial condyle following septic arthritis is consistent with total preservation of the other condyle. The outcome of surgical reconstruction of the missing condyle is poor. The presence of a complete synovial septum could result in the localisation of infection to one half of the joint, with the destruction of one condyle.

PITX1 is a bicoid-related homeodomain transcription factor implicated in vertebrate hindlimb development. Recently, mutations in PITX1 have been associated with autosomal-dominant clubfoot. In addition, one affected individual showed a polydactyly and right-sided tibial hemimelia. We now report on PITX1 deletions in two fetuses with a high-degree polydactyly, that is, mirror-image polydactyly. Analysis of DNA from additional individuals with isolated lower-limb malformations and higher-degree polydactyly identified a third individual with long-bone deficiency and preaxial polydactyly harboring a heterozygous 35 bp deletion in PITX1. The findings demonstrate that mutations in PITX1 can cause a broad spectrum of isolated lower-limb malformations including clubfoot, deficiency of long bones, and mirror-image polydactyly.

Erysipelothrix rhusiopathiae is an established animal pathogen while the zoonotic infections in humans are rarely reported. Infections occur after exposure to animals or animal products that are mostly occupational in adults. Here we report in a child for the first time septic arthritis and osteomyelitis without an identifiable risk factor. A 5-year-old male child was admitted with pain in the left hip joint and inability to bear weight on the limb. Clinical examination followed by radiological and magnetic resonance imaging was suggestive of septic arthritis. Erysipelothrix rhusiopathiae grew from peroperative joint specimen. The infection was resolved following arthrotomy, joint lavage and antibiotic therapy.

Background: Split-hand/foot malformation (SHFM)-also known as ectrodactyly-is a congenital disorder characterised by severe malformations of the distal limbs affecting the central rays of hands and/or feet. A distinct entity termed SHFLD presents with SHFM and long bone deficiency. Mouse models suggest that a defect of the central apical ectodermal ridge leads to the phenotype. Although six different loci/mutations (SHFM1-6) have been associated with SHFM, the underlying cause in a large number of cases is still unresolved., Methods: High resolution array comparative genomic hybridisation (CGH) was performed in patients with SHFLD to detect copy number changes. Candidate genes were further evaluated for expression and function during limb development by whole mount in situ hybridisation and morpholino knock-down experiments., Results: Array CGH showed microduplications on chromosome 17p13.3, a locus previously associated with SHFLD. Detailed analysis of 17 families revealed that this copy number variation serves as a susceptibility factor for a highly variable phenotype with reduced penetrance, particularly in females. Compared to other known causes for SHFLD 17p duplications appear to be the most frequent cause of SHFLD. A ~11.8 kb minimal critical region was identified encompassing a single gene, BHLHA9, a putative basic loop helix transcription factor. Whole mount in situ hybridisation showed expression restricted to the limb bud mesenchyme underlying the apical ectodermal ridge in mouse and zebrafish embryos. Knock down of bhlha9 in zebrafish resulted in shortening of the pectoral fins., Conclusions: Genomic duplications encompassing BHLHA9 are associated with SHFLD and non-Mendelian inheritance characterised by a high degree of non-penetrance with sex bias. Knock-down of bhlha9 in zebrafish causes severe reduction defects of the pectoral fin, indicating a role for this gene in limb development.

Objective: To determine if the frequency of left-handedness is high in children with spastic cerebral diplegia., Design: Case-control study., Methods: One hundred and eleven children with spastic diplegic cerebral palsy and 444 age- and gender-matched controls were studied. The handedness of each child was assigned on the basis of responses to questions on the hand preference for writing and drawing, feeding and throwing a ball. The data were analysed by conditional logistic regression and computing the odds ratio and 95% confidence intervals for left handedness., Results: Of the 111 children with spastic diplegic cerebral palsy, 45 were left-handed, while 13 of 444 normal children were left-handed. The odds ratio for left-handedness in children with diplegic cerebral palsy as compared to normal children was 27.33 (95% CI = 11.63, 64.25)., Conclusion: The study shows that left-handedness is very frequently encountered in children with spastic diplegic cerebral palsy.

The time when the insult/triggering event occurs in Legg-Calvé-Perthes' (LCPD) is unknown. the purpose of this study was to determine, using the mathematical tool of incubation period modeling, the time of such event and the incubation period for LCPD. We reviewed 2,911 children with LCPD from 10 different centers around the world. They were divided into two groups: those from India (505 children, mean age 8.1 ± 2.3 years) and those from other than India (2,406 children, mean age 5.8 ± 2.2 years). A simple distribution with an excellent fit to the data was ln(y) = a + bx + cxln(x), where y is the proportion of children with LCPD at age of diagnosis x (r(2) = 0.994 for non-Indian and 0.959 for Indian children). The age of the triggering event was 1.32 years for non-Indian and 2.77 years for Indian children; the median incubation period was 4.30 years non-Indian and 5.33 years for Indian patients. Knowing the incubation period and age of triggering event narrows the number of potential etiologies in LCPD. this study does not support a prenatal triggering event as postulated in the past. similar incubation periods with different ages at diagnosis supports a common insult which occurs at different ages in different populations dependent upon local factors such as geographic location and ethnicity.

Background: The problems of managing congenital pseudarthrosis of the tibia include difficulty in obtaining union, refractures, limb-length inequality, and deformities of the ankle and leg. As the problems may develop at different stages of growth, these children must be followed till skeletal maturity. This study evaluates the results of the treatment of congenital pseudarthrosis of the tibia by excision of the pseudarthrosis, transarticular intramedullary rodding, and onlay autogenous cortical bone grafting in a cohort of children followed till skeletal maturity., Methods: Eleven of 38 children treated with this technique for more than 20 years reached skeletal maturity. Their case records and radiographs were reviewed and each child was assessed clinically and radiographically at final follow-up (mean age, 18.4 y). The time to union and details of additional operations during the period of follow-up were noted. At final follow-up the status of union of the pseudarthrosis, deformities of the tibia and ankle, and the limb lengths were documented. The function of the ankle was assessed by applying the American Orthopaedic Foot and Ankle Society ankle-hindfoot Score., Results: Union of the pseudarthrosis was achieved in 9 of 11 children after the index operation, and in 2 cases after further surgery. At final follow-up, all 11 patients had a soundly united tibia, although persistent fibular pseudarthrosis was present in 10 patients. Ten children underwent 21 secondary operations for various indications. At final follow-up anterior or medial bowing of the tibia, ranging from 5 to 28 degrees, were noted in 7 patients and ankle valgus was present in 5 patients. The mean shortening was 2.6 cm. Only 1 patient with 8 cm shortening used a brace with a sole raise. Only 3 patients who had their transarticular rods removed had satisfactory ankle motion. The American Orthopaedic Foot and Ankle Society ankle-hindfoot scores ranged between 70 and 98. No patient experienced pain. There was no donor site morbidity., Conclusion: Excision of pseudarthrosis, intramedullary rodding, and cortical bone grafting is very effective in achieving union of congenital pseudarthrosis of the tibia but a proportion of children will require additional operations to deal with refractures and other complications., Level of Evidence: Level IV.

Background: Crouch gait in cerebral palsy is associated with spasticity and contracture of the hamstrings and weakness of the extensors of the hip and knee and ankle plantar flexors. Different treatment options have been described in the literature to deal with this difficult problem. We devised a different protocol of treatment aimed at correction of the flexion deformity of the knee, weakening of the hamstrings, and augmenting the power of the knee and hip extension, which we used on 17 children with severe crouch., Methods: This surgery, performed in 2 stages, entailed shortening of the femur, plication of the patellar tendon, transfer of the semitendinous to the back of the femur, and fractional lengthening of the other hamstrings. The degree of fixed deformity, the popliteal angle, quadriceps power, range of knee motion, ambulatory status and the efficiency of gait, and the position of the patella were evaluated before surgery and again after a minimum 2-year follow-up., Results: The gait improved and the power of the quadriceps and the range of knee motion increased. The flexion deformity and popliteal angle decreased significantly. Patella alta was corrected and all fragmentation of the tibial tuberosity and fractures of the patella healed. The Functional Mobility Scores and the ambulatory capacity increased in all the children. There was no evidence of damage to the sciatic nerve in any patient., Conclusions: The method of treatment of severe crouch gait outlined in this study seems to be an effective and safe method of dealing with this difficult problem., Level of Evidence: IV.

To evaluate the effect of prophylactic epiphyseodesis of the greater trochanter in Perthes' disease, 62 children with unilateral Perthes' disease who underwent trochanteric epiphyseodesis combined with varus osteotomy of the femur during the active stage of the disease (mean age at surgery: 8.4 y) and 20 controls were followed up until skeletal maturity. On radiographs taken at skeletal maturity, the articulo-trochanteric distance, the center-trochanteric distance, the length of the abductor lever arm, the neck-shaft angle, the radius of the femoral head, and the Reimer's migration index of normal and affected hips were measured. The shape of the femoral head was assessed according to the criteria of Mose. The range of hip motion, the strength of hip abduction, and limb lengths were measured and the Trendelenburg sign was elicited. The mean values of articulo-trochanteric distance and center-trochanteric distance were greater and the frequency of a positive Trendelenburg sign was less in children who had undergone trochanteric epiphyseodesis than in children who had no surgery (P<0.01). Trochanteric epiphyseodesis achieved optimal trochanteric growth arrest in 60% of operated children; the procedure was not effective in 30%, and in 10% of children there was overcorrection. Logistic regression analysis showed that the size of the femoral head at healing and the age at surgery were variables that significantly influenced the effectiveness of trochanteric growth arrest. At skeletal maturity, the mean shortening of the affected limb in operated children was 0.44 cm (SD 0.68 cm), whereas that of non-operated children was 0.86 cm (SD 0.78 cm) (P<0.05). The range of motion of the hip was excellent and there were no significant differences in the range of motion among children with optimal correction, under-correction, and overcorrection. A probability curve plotted on the basis of a logistic regression model suggests that effective trochanteric arrest may be achieved in a high proportion of children operated at or before 8.5 years of age and in half the children operated between the age of 8.5 years and 10 years. On the basis of this study, we recommend prophylactic epiphyseodesis of the greater trochanter as a means of minimizing trochanteric overgrowth and resultant Trendelenburg gait in older child with Perthes' disease.

Radiographs of 75 children with unilateral disease were studied to quantify the extent of remodeling of the proximal femur and the acetabulum between healing and skeletal maturity in children with Perthes disease. The mean ages at disease onset, healing, and skeletal maturity were 8.3, 11.9, and 15.5 years, respectively. Fifty-seven children were treated surgically by subtrochanteric varus osteotomy with trochanteric epiphyseodesis; the remaining 18 children were treated nonoperatively. Assessment of the Mose's index and Stulberg grading and measurements of the proximal femur and the acetabulum were made at healing and at skeletal maturity. The Stulberg grade and the Mose's index did not change between healing and skeletal maturity in 88% and 95% of children, respectively. In operated children, there was significant improvement of the articulotrochanteric distance, acetabular depth, neck-shaft angle, and Sharp's angle between disease healing and skeletal maturity, but no such changes occurred in children who were not operated on. We conclude that because little change occurs in the shape of the femoral head and congruity of the hip between healing and skeletal maturity, the outcome of treatment based on these 2 variables can be assessed once the disease heals in children with late-onset Perthes disease.

Purpose: The study was undertaken to identify the patterns and spectrum of aberrant development of the skeletal elements of the leg and foot in children with pre-axial mirror polydactyly of the foot., Methods: Case records and radiographs of eight children (ten feet) with pre-axial mirror polydactyly were studied. Four feet were seen in association with fibular dimelia, three feet with dysplastic trapezoid shaped tibia, two with tibial aplasia and one with tibial hypoplasia., Results: Distinct patterns of anomalies of the hindfoot, midfoot and forefoot were noted, with different patterns of leg malformation. Fibular dimelia was associated with duplication of the calcaneum, cuboid and the lateral cuneiform. Talar duplication occurred in children with a trapezoidal tibia. No duplication of tarsal bones was seen in association with tibial aplasia or hypoplasia. The first metatarsal was hypoplastic wherever the tibia failed to develop. Mere resection of the supernumerary rays resulted in a near normal looking foot in feet without calcaneal duplication. The cosmetic appearance of the foot was poorest in children with fibular dimelia.

Laboratory infarction diagnostics are based on the detection of elevated serum activities of creatine kinase (CK) Creatine kinase Isoenzyme MB (CKMB) and Transaminases. Determination of these cardiac marker enzymes permits the diagnosis of transmural myocardial infarction. However in such patients the diagnosis of acute myocardial infarction can be confirmed by the clinical symptoms and changes in the ECG, in addition to the enzyme assays. The 50 AMI patients selected in the present study were those admitted to the ICCU of Shri Krishna Hospital, Karamsad. The blood samples were taken at Zero hours (i.e. at the time of admission of the patient). Within 6 hrs of the starting of chest pain, 1.5 million units of streptokinase were mixed with 100 to 150ml of normal saline and administered by infusion over a period of one hour. The blood samples were further collected at intervals of 6 hrs, 14hrs, 32hrs, 48hrs, 5(th) day and 7(th) day. The blood samples were analyzed for CK, CKMB, SGOT, α HBDH and Cardiac specific Troponin T. By 6hrs the CK and CKMB values had started rising, the rise continuing at 14hrs with peak values at 32hrs. The CK showed a slight decrease by 48 hrs. The cardiac Troponin T showed wide time window from 4 hrs to 7(th) day for detecting myocardial damage. The maximum cardiac Troponin T values were during the first 24hrs. Cardiac Troponin T in serum appears to be a more sensitive and early indicator of myocardial cell injury in comparison to CKMB.

It is well established that smoking increases the risk for cardiovascular disease. From the studies in diabetic subjects it has been shown that smoking induces microalbuminuria and accelerates the progression to end stage renal disease. Little is known whether smoking is also related to microalbuminuria and renal end organ damage in non diabetic subjects. The hypothesis which was put forward that tobacco chewing is related to microalbuminuria and renal functional changes in non diabetic subjects. We therefore performed a population based study in the Anand city of Gujarat in which we studied the relation between tobacco chewing and urinary albumin excretion. Tobacco chewers had a higher urinary albumin excretion (Albumin excretion 373±13.9 mg/day: P<0.01) than those who do not consume tobacco. In conclusion tobacco chewing is associated with albuminuria.

Laboratory infarction diagnostics are based on the detection of elevated serum activities of total Creatine Kinase (CK), Creatine Kinase isoensyme MB, (CKMB), Lactate dehydrogenase (LDH), isoenzyme forms of LDH and transaminases. Determination of these cardiac marker enzymes permits a highly sensitive diagnosis of transmural myocardial infarction. In such patients the diagnosis of acute myocardial infarction can be confirmed by the clinical, symptoms, and changes in the ECG in addition to the enzyme assays. The 50 AMI patients selected in the present study were those admitted to the ICCU of Shri Krishna Hospital, Karamsad. The blood samples were taken at the time of admission (ie. within four hours of the start of chest pain). The samples were analyzed for CK, CKMB, SGOT, (Serum glutamate oxaloactate transaminase) αHBDH α-hydroxybutyrate dehydrogenase and troponin T. The serum CKMB activity in AMI showed an increase only 5-6 hours after the commencement of chest pain. The elevation in SGOT and αHBDH was still delayed. At the same time we could observe that the cardiac Troponin T (cTnT) was elevated at the time of admission of the patient itself. This increase of cTnT in AMI patients was 20 times higher than the normal blood donors. The controls included 25 normal blood donors and 25 patients with polytraumatic injuries with no chest contusion. The study shows that cTnT estimation could serve in the early diagnosis of AMI. The increase of cardiac troponin T in AMI patients was 20 times higher than the normal blood donors in AMI patients at the time of admission. Cardiac troponin T in serum appears to be a more sensitive indicator of myocardial cell injury than CKMB activity and its detection in the circulation may be a useful prognostic indicator in patients with unstable angina as well. When the blood of normal blood donors or that of patients with polytraumatic injury was analysed the troponin T values were well within the normal range in both the above categories showing that cardiac troponin T is highly specific for heart tissue. Although CKMB and cardiac troponin T are released soon after the myocardial injury, the release of cardiac troponin T is much earlier than CKMB thereby invalidating the important role of cardiac troponin T in diagnosing AMI. Cardiac troponin T has been shown to be highly sensitive for cardiac injury and not elevated in any other trauma, heavy exercise or skeletal muscle injury. Cardiac troponin T is ordinarily undetectable in healthy individuals, and so its measurement can serve as a powerful tool in the diagnosis of AMI.