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Fabry disease (FD) is an X-linked metabolic disease caused by a deficiency in α-galactosidase A (α-Gal A) activity. This causes accumulation of glycosphingolipids, especially globotriaosylceramide (Gb3), in different cells and organs. Neuropathic pain and gastrointestinal (GI) symptoms, such as abdominal pain, nausea, diarrhea, constipation, and early satiety, are the most frequent symptoms reported by FD patients and severely affect their quality of life. It is generally accepted that Gb3 and lyso-Gb3 are involved in the symptoms; nevertheless, the origin of these symptoms is complex and multifactorial, and the exact mechanisms of pathogenesis are still poorly understood. Here, we used a murine model of FD, the male α-Gal A (-/0) mouse, to characterize functionality, behavior, and microbiota in an attempt to elucidate the microbiota-gut-brain axis at three different ages. We provided evidence of a diarrhea-like phenotype and visceral hypersensitivity in our FD model together with reduced locomotor activity and anxiety-like behavior. We also showed for the first time that symptomology was associated with early compositional and functional dysbiosis of the gut microbiota, paralleled by alterations in fecal short-chain fatty acid levels, which partly persisted with advancing age. Interestingly, most of the dysbiotic features suggested a disruption of gut homeostasis, possibly contributing to accelerated intestinal transit, visceral hypersensitivity, and impaired communication along the gut-brain axis.

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The number of partial-foot amputations performed is increasing, and many recommendations have been made regarding the use of prostheses and footwear designed to prevent higher-level amputations in this population. The present study investigated the use of prostheses and shoe inserts and the types of footwear worn by partial-foot amputees in the inner city to determine whether previous recommendations are being followed as well as whether new prosthetic styles are being used. The study surveyed 110 patients (73 men and 37 women) with a mean age of 58.6 years (range, 21 to 86 years) with partial-foot amputations of all levels. The results showed that about one-half of all patients wore a shoe-insert orthosis. Although 54% wore some form of special footwear to accommodate and protect the residual foot, no patient in this study wore a shoe with a rocker-bottom sole. Only one patient with a transmetatarsal amputation used a brace and only one patient in the entire study wore a modern cosmetic foot prosthesis.

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Surgical treatment for clubfoot has been largely directed at finding the best one-stage operation for the resistant clubfoot. Eighteen patients with 27 clubfeet (average follow-up 11 years since first surgery; range, 3.5-24 years) were reviewed. More than one clubfoot operation was required in 56% of cases. Forty-six percent were corrected after one surgery; 33% required a second surgery and 14% required a third operation. One patient with particularly severe feet required a fourth operation on each foot. The mean age at the time of surgery was 1.26 years, 5.12 years, and 8 years for the first, second, and third operations, respectively. The first operation consisted of a soft-tissue release. The second and third operations consisted of more extensive soft-tissue release and various rearfoot and forefoot procedures. Radiographic values revealed an AP talocalcaneal angle of 18 degrees, AP talo-first metatarsal angle of 6 degrees, lateral talocalcaneal angle of 29.6 degrees, lateral talo-first metatarsal angle of 15 degrees, and calcaneo-first metatarsal angle of 143 degrees. At follow-up all patients had adequate function as determined by personal interview and clinical examination. We conclude that correction of resistant congenital clubfoot often requires more than one surgery, not because of a "failed first operation," but due to dynamic muscle imbalances that may not be fully recognized in infancy and early childhood. Thus, the need for a second operation should not be perceived as a failure of the first, but as part of the natural history of congenital clubfoot.

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Macrodactyly can affect the fingers and/or toes1. Histopathologic examination will distinguish macrodactylia fibrolipomatosis or neural fibrolipoma with macrodactyly, from macrodactylia as a part of neurofibromatosis. Surgical repair is aimed at decreasing the size of the affected foot so it is as near in size and shape to the normal foot as possible. Surgical approaches have included reconstructive surgery (usually staged debulking procedures), epiphyseal plate arrest and amputation. Repeated reconstructive surgical procedures, as illustrated in this report covering patient care over a 15 year period, are usually necessary due to recurring soft tissue and boney enlargement.

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A comparative retrospective study of 48 open heel spur surgeries and 20 endoscopic plantar fasciotomies was conducted involving 59 patients over a 10-year period. There was a significant reduction in heel pain at the time of follow-up (average, 3 years) for both groups. Overall, 85% of procedures were associated with patient satisfaction with the results, and patients said that they would recommend heel spur surgery for relief of severe heel pain in 94% of cases. Factors influencing the postoperative outcome, such as duration of preoperative symptoms, extent of conservative care, and obesity, are discussed.

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Neuromuscular disease commonly affects the rearfoot as equinus, equinovarus, and equinovalgus deformity. Spastic hemiplegia caused by stroke, head injury, and cerebral palsy results in equinovarus deformity of the rearfoot. Spastic diplegia, most frequently caused by cerebral palsy, results in equinovalgus rearfoot deformity. Problems in ambulation, footwear, and bracing, as well as their orthopedic management, in patients with neuromuscular disease are discussed in a case-report format.

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A retrospective analysis was conducted of 32 patients (33 ankles) who had undergone surgical ankle arthroscopy for chronic ankle pain that was recalcitrant to conservative treatment. All patients were examined clinically and completed a written questionnaire. Intraoperative ankle arthroscopy showed hypertrophic synovitis, adhesive bands, chondral bands with synovitis, osteophytes, and abnormalities in the talar dome. Results of treatment after an average follow-up time of 1.4 years (range: 0.33 to 12.5 years) showed ankle scores of 15 excellent, 11 good, 5 fair, and 2 poor. Obesity was significantly related to the outcomes of arthroscopy procedures. Obese patients were more likely to be rated as fair or poor, while nonobese patients were significantly more likely to be rated excellent or good. Those patients who received physical therapy postoperatively for one or more months had significantly better ankle ratings than those who did not elect to have physical therapy.

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The longitudinal epiphyseal bracket is a rare ossification defect of bones of the hands and feet and results in an abnormal epiphysis that is C-shaped, longitudinally oriented, and vertically extends into the diaphysis. The longitudinal epiphyseal bracket may exist as an isolated deformity, but is commonly associated with digital deformities such as polydactyly, duplicated digits, congenital hallux varus, and clinodactyly. Five case histories of children with the longitudinal epiphyseal bracket in different bones of the foot are described with three distinct clinical presentations. The development of the longitudinal epiphyseal bracket and implications for treatment are considered.

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Surgical correction of the neglected, and severely deformed clubfoot in the adult is necessary to achieve plantigrade, pain free, and cosmetically acceptable feet. Also, it is important that the patient is able to wear shoes and participate in a more active and normal life. Three adult immigrants with neglected clubfoot underwent surgical correction for the first time. Posterior medial plantar soft tissue release and a double arthrodesis involving calcaneal cuboid wedge resection with talonavicular fusion have been effective in removing rearfoot varus, providing minimal shortening, and restoring the foot to a normal plantigrade position.

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Ten patients with 12 symptomatic severe flatfeet were treated with a combined surgical technique. Patients 10 years or older were treated with Evan's calcaneal osteotomy, Young tenosuspension, and tendo achillis lengthening. Patients under age 10 were treated with modified Young tenosuspension, talonavicular desmoplasty and subtalar arthroereisis. Eight of the 10 patients had marked relief in pain and fatigue and were able to perform all physical activities. Radiographic criteria showed improvement for all patients in all categories.

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The purpose of this study was to evaluate the long term results of clubfoot surgery. Forty-four patients were evaluated for periods from one to 41 years. The evaluation was based upon clinical, radiological and functional criteria. Results revealed that in spite of anatomically and radiologically imperfect feet, most patients demonstrated excellent levels of function. The results of other follow-up clubfoot studies were analyzed and compared to the present study.

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The goal of podiatric surgery in the older patient should be pain management with the highest degree of functional restoration. Thorough preoperative analysis, meticulous surgical technique, and careful postoperative planning are necessary to ensure a favorable surgical result. Each planned procedure must coincide with the specific needs of the individual patient while conforming to the body's physical limitations. A complete medical examination with laboratory studies is indicated due to the elderly patient's inherent diminished physical status. When the patient is deemed a proper candidate for surgery, the medical team, family, and friends should be consulted regarding immediate and long-term postoperative care. Strict adherence to these principles is in the best interest of both the practitioner and the older patient.

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A case of dislocated first and second metatarsophalangeal joints was reported along with the mechanics and mechanism of injury. The practitioner must be familiar with the mechanism of injury and radiographic classification to determine the proper indications for closed versus open reduction. The authors' review of the literature did not reveal a similar case involving a dislocation of the first and second metatarsophalangeal joints.

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The literature reports that 70% of the cases of sinus tarsi syndrome are post-traumatic, following an inversion sprain, and that 30% result from inflammatory disorders, such as rheumatoid arthritis, ankylosing spondylitis, and gouty arthritis. However, in the case presented, talipes equinovarus deformity and sinus tarsi syndrome coexisted. One of the corrective goals in the management of the talipes equinovarus deformity is the realignment of the articulation between the medial plantarly deviated talar head and the anteromedial segment of the calcaneus. The calcaneus must be rotated from a plantarflexed position into a dorsiflexed position. The posterior tubercle will be moved down and in, with the anterior process moved up and out away from the talar head. By correcting the plantarflexed varus attitude of the calcaneus, it is put in a valgus position that often closes down the sinus tarsi upon weightbearing. This compression may result in pain over the lateral aspect of the midfoot with hindfoot instability, as seen in the case presented. As a result of the abnormal anatomical relationship of the talus and calcaneus, the patient developed severe pain in the sinus tarsi. Based on the medical history and present postoperative results, the authors find a long-term sequela of talipes equinovarus deformity to be sinus tarsi syndrome.

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The authors review four cases of Kaposi's sarcoma that were presented to the Foot Clinics of New York and affiliated North General Hospital during a 1-year period from the fall of 1987 to the fall of 1988. The authors conclude that it is sometimes difficult to diagnose Kaposi's sarcoma and to differentiate between the acquired immunodeficiency (AIDS) form and the classic form. Guidelines for diagnosis and a profile of the AIDS-related and non-AIDS-related Kaposi's sarcoma patient are discussed.

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This study reports the evolution and results of an arthroereisis procedure utilizing a talonavicular desmoplasty, tibial tendon suspension (posterior and anterior) and implantation of a Silastic interpositional sphere in the sinus tarsi. Four cases were reviewed with a maximum 6-year follow-up. This study should be considered when presented with a severely hypermobile and symptomatic flatfoot in the child. The procedure is efficacious in combination with a strict postoperative regimen and appropriate biomechanical management.

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