Biomedical subjects

E B Hoffman

Publications and source records attributed to E B Hoffman.

At least 19 recordsLinked to original sources

PubMed2006

Four decades of conjoined twins at Red Cross Children's Hospital--lessons learned.

Conjoined twins represent a rare but fascinating congenital condition, the aetiology of which remains obscure. Over the past four decades, the paediatric surgeons at Red Cross Children's Hospital have been involved in the management of 46 pairs of conjoined twins, of which 33 have been symmetrical and 12 asymmetrical. Seventeen symmetrical twins have undergone separation with 22 children (65%) surviving; all of the live asymmetrical twins survived separation. We describe the important features of this unique cohort, outline our approach to management and present the results of this approach. We consider some of the ethical and moral dilemmas we have confronted, and discuss the prenatal diagnosis, obstetric implications and postnatal care of these children, including the relevant investigations and anaesthetic and surgical management. Specific aspects related to the cardiovascular system, hepatobiliary and gastrointestinal tracts, urogenital tract, central nervous system and musculoskeletal system are highlighted.

Adolescent↗

PubMed2005

Double-elevating osteotomy for late-presenting infantile Blount's disease: the importance of concomitant lateral epiphysiodesis.

We reviewed 34 knees in 24 children after a double-elevating osteotomy for late-presenting infantile Blount's disease. The mean age of patients was 9.1 years (7 to 13.5). All knees were in Langenskiöld stages IV to VI. The operative technique corrected the depression of the medial joint line by an elevating osteotomy, and the remaining tibial varus and internal torsion by an osteotomy just below the apophysis. In the more recent patients (19 knees), a proximal lateral tibial epiphysiodesis was performed at the same time. The mean pre-operative angle of depression of the medial tibial plateau of 49 degrees (40 degrees to 60 degrees ) was corrected to a mean of 26 degrees (20 degrees to 30 degrees ), which was maintained at follow-up. The femoral deformity was too small to warrant femoral osteotomy in any of our patients. The mean pre-operative mechanical varus of 30.6 degrees (14 degrees to 66 degrees ) was corrected to 0 degrees to 5 degrees of mechanical valgus in 29 knees. In five knees, there was an undercorrection of 2 degrees to 5 degrees of mechanical varus. At follow-up a further eight knees, in which lateral epiphysiodesis was delayed beyond five months, developed recurrent tibial varus associated with fusion of the medial proximal tibial physis.

Adolescent↗

PubMed1997

A phase I/II study of polymerized bovine hemoglobin in adult patients with sickle cell disease not in crisis at the time of study.

BACKGROUND: The painful episodes of sickle cell disease (SCD) involve vaso-occlusion and impaired oxygen delivery. HBOC-201, a hemoglobin-based oxygen carrier, has been shown to support oxygen delivery in animal studies and to be safe and well tolerated in normal human volunteers. Therefore, we speculated that it might have a therapeutic role in SCD. METHODS: Eighteen adults with SCD who were asymptomatic at the time of study were enrolled in a Phase I/II single-blind, placebo-controlled, dose-escalation study of HBOC-201. The primary purpose was to assess the safety of the material in this patient population. In addition, as a surrogate marker of efficacy, each subject underwent a variety of exercise tests before and after HBOC-201 was given. RESULTS: All HBOC-201 infusions were well tolerated by the study subjects and no evidence of toxicity was noted. In addition, there was a significant difference in heart rate response to the identical aerobic exercise workload when the study subjects who received HBOC-201 were compared to the subjects who received placebo (p = 0.0061). CONCLUSIONS: HBOC-201 was safely administered to patients with SCD who were not in crisis at the time of study. Furthermore, following infusion of the study material, subjects with SCD performed the identical aerobic exercise-induced workload with an increase in heart rate that was significantly less than the increase observed in the subjects who received an infusion of the saline placebo. These safety and surrogate efficacy data support the notion that HBOC-201 could have efficacy as a treatment for the vasoocclusive episodes of SCD.

Adult↗

PubMed1996

Immunoglobulin allotypes and genetic susceptibility to invasive Haemophilus influenzae type b and Staphylococcus aureus infections in South African children.

OBJECTIVE: The purpose of this study was to determine whether the G2m(n), G1m(f) and Km(3) immunoglobulin allotypes have any association with susceptibility to invasive Haemophilus influenzae type b (Hib) and Staphylococcus aureus (S. aureus) infections in children. METHODS: Direct enzyme-linked immunosorbent assays with commercially available monoclonal antibodies were established to quantitate G2m(n) and G1m(f) allotypes. A qualitative enzyme-linked immunosorbent assay with polyclonal rabbit anti-Km(3) antibody was established for Km(3) determination. RESULTS: The G2m(n) marker occurred in 34.4% of the mixed ancestry population and 2.9% of the Black population. There was a significantly decreased frequency of the G2m(n) allotype in mixed ancestry children with Hib meningitis (8.5%) and Hib osteomyelitis/septic arthritis and a decreased frequency of Km(3) in black and mixed ancestry children with Hib meningitis. The frequency of G2m(n), G1m(f) and Km(3) allotypes in patients with S. aureus osteomyelitis/septic arthritis were not significantly different from normal population frequency. CONCLUSIONS: This study shows a clear association between the absence of the G2m(n) allotype in mixed ancestry children and susceptibility to invasive infections caused by H. influenzae and an association between the absence of Km(3) and Hib meningitis in both black and mixed ancestry children.

Arthritis, Infectious↗

PubMed1995

Tuberculosis of the knee in children.

We reviewed 33 children with tuberculosis of the knee treated during the period from 1979 to 1991. All were treated with triple chemotherapy, using rifampicin, isoniazid and pyrazinamide for nine months. No patient had a synovectomy; surgery was limited to open biopsy or salvage procedures such as posterior release and arthrodesis for late stages of the disease. The radiological appearance of the knee at presentation predicted the outcome. The 30 patients with stage-1 (normal) or stage-2 (osteomyelitic) disease had excellent or good results; the three with narrowed joint spaces in stage 3 or stage 4 (arthritic) had fair or poor results. Early active mobilisation, as against long-term immobilisation, seemed to have no effect on the outcome of stage-1 or stage-2 disease.

Antitubercular Agents↗

PubMed1995

Tuberculosis of the hip in children.

We have reviewed 74 tuberculous hips in 73 children treated from 1950 to 1991. From 1979 to 1991 we treated 28 patients with rifampicin, isoniazid and pyrazinamide given for nine months (series A), using active mobilisation for the more recent cases. Before this, 46 hips had been treated with streptomycin and isoniazid with or without para-aminosalicyclic acid given for a mean of 18 months (series B), and all these patients were immobilised for a mean of 2.2 years. The radiological appearances at presentation as classified by Shanmugasundaram (1983) predicted the outcome. Most hips were of the 'normal' type (50% and 59% of series A and B respectively) followed by the dislocating type (25% and 13%) and the atrophic type (8% and 9%). There were good or excellent results in 93% of the 'normal' type. All the atrophic type had poor results. The dislocating type had a poor result if the joint space was narrow after reduction of the hip. Early mobilisation had no effect on the outcome of the 'normal' type of disease. The newer drug regimens allowed for shorter courses of treatment, but did not necessarily give a better outcome.

Adolescent↗

PubMed1994

Isotope bone scanning for acute osteomyelitis and septic arthritis in children.

We prospectively studied 86 children to assess the value and accuracy of isotope bone scanning in the diagnosis of suspected acute haematogenous osteomyelitis and septic arthritis. The patients were scanned because of difficulty in localising the exact site of the pathology. Characteristic scan appearances were found. In osteomyelitis there was increased or decreased uptake extending beyond the confines of the joint capsule; in septic arthritis there was increased or decreased uptake on either side of the joint line, but largely limited to and uniform within the joint capsule. The bone-scan images were correlated with the final diagnosis in 34 sites of septic arthritis and in 62 sites of osteomyelitis. The overall accuracy of the bone scans was 81%. The predictive value for a positive scan to be correct was 100% for a cold scan and 82% for a hot scan. The main reason for a false-positive scan was contiguous soft-tissue infection. The predictive value for a negative scan to be correct was 63%. One reason for a false-negative scan was that the patient was in the transitional phase from cold to hot.

Adolescent↗

PubMed1993

Spondylolysis and spondylolisthesis after five-level lumbosacral laminectomy for selective posterior rhizotomy in cerebral palsy.

Of 163 children who have undergone five-level lumbosacral laminectomies for selective posterior rhizotomy, 20% of those re-X-rayed post-operatively (19/99) have developed incidental isthmic spondylolysis or grade I spondylolisthesis. The majority of patients were ambulatory, active, spastic diplegics. There were five children with isthmic defects at L3-4, three at L4-5 and 11 at L5-S1. Six of the 19 children had grade I spondylolisthesis, but none were symptomatic nor has there been any evidence of further slipping to date. It is postulated that the laminectomy, associated lordosis and increased mobility after rhizotomy may be factors in the causation.

Adolescent↗

PubMed1993

CT and MR in the management of advanced spinal tuberculosis.

In regions where tuberculosis is endemic, CT and MR are of limited use in making the initial diagnosis of spinal tuberculosis. Their optimum use is for monitoring the management of cases involving paralysis. CT best evaluates the integrity of posterior bony structures which provide spinal stability and MR best demonstrates the content and extent of the extradural mass that is causing the paralysis. Their use is illustrated by three recent cases.

Child, Preschool↗

PubMed1993

Imaging in children with spinal tuberculosis. A comparison of radiography, computed tomography and magnetic resonance imaging.

We compared the usefulness of radiography, CT and MRI in 25 children with spinal tuberculosis. Radiography provided most of the information necessary for diagnosis and treatment. Axial CT was the most accurate method for visualising the posterior bony elements. Sagittal MRI best showed the severity and content of extradural compression and helped to differentiate between an abscess and fibrous tissue. The main value of CT and MRI is in the preoperative evaluation of the small proportion of patients who require surgical treatment for paraplegia.

Anti-Bacterial Agents↗

PubMed1993

Chondrolysis in slipped upper femoral epiphysis. Long-term study of the aetiology and natural history.

Of 44 patients (55 hips) with slipped upper femoral epiphysis treated from 1963 to 1989, 13 (14 hips) developed chondrolysis. Eight hips had chondrolysis at the time of presentation, all in female patients who were either coloured or black and who had moderate or severe slips. The other six hips had persistent pin penetration of the joint; in five of these the pin penetrated the anterosuperior quadrant of the head. Removal of penetrating pins resulted in improvement in pain in all six hips and in the range of movement in four. Chondrolysis did not develop in any of 11 hips with transient intraoperative pin penetration. In hips with chondrolysis maximum joint-space narrowing developed within the first year; improvement in joint space and range of movement continued for up to three years after maximal involvement. At an average follow-up of 13.3 years no patient had pain but five hips were stiff.

Adolescent↗

PubMed1992

Treatment of chronic granulomatous disease with recombinant gamma interferon.

Chronic granulomatous disease is a rare, primary immunodeficiency associated with serious bacterial and fungal infections caused by phagocytic defects of oxidative metabolism. To date the mainstay of management has been aggressive treatment of infections and the use of prophylactic antibiotics. Two patients, who showed remarkable clinical improvement when treated with recombinant gamma interferon, are reported. Both have been on treatment for at least 18 months and have continued to thrive and remain free of infections.

Child, Preschool↗

PubMed1992

The unstable hip and mid-lumbar myelomeningocele.

We reviewed 55 patients with mid-lumbar myelomeningocele (L3 and L4) first seen over a 17-year period from 1970 to 1986 and followed up for an average of ten years. We assessed a number of factors which might affect hip stability and ability to walk, recording the natural history of clinical and radiological hip deformity. Two-thirds of the hips had become dislocated or subluxed by the end of the first year of life, involving 86% of hips in patients with an L3 level and 45% of those with an L4 level. All the hips that developed instability secondary to muscle imbalance did so within the first year. The neurological level was the most significant determinant of walking ability: all patients with L4 neurological levels could walk but only one-third of those with L3 lesions could do so. Hip stability, intelligence quotient and fixed deformity did not influence walking ability.

Follow-Up Studies↗

PubMed1992

Remodelling of angular deformity after femoral shaft fractures in children.

We reviewed 28 children with unilateral middle-third fractures of the femoral shaft who had an angular deformity after union of 10 degrees to 26 degrees. At an average follow-up of 45 months (20 to 66), we measured remodelling of the proximal physis, the distal physis and the femoral shaft. The average correction was 85% of the initial deformity. We found that 74% of correction occurred at the physes and only 26% at the fracture site. Neither the direction nor the magnitude of the angulation much influenced the degree of remodelling. Younger children remodelled only a little better than older children. We conclude that in children under 13 years of age, malunion of as much as 25 degrees in any plane will remodel enough to give normal alignment of the joint surfaces.

Bone Remodeling↗

PubMed1992

[Grice subtalar arthrodesis: a long-term follow-up in poliomyelitis sequelae].

A retrospective review was done of 19 poliomyelitis feet on which the standard Grice subtalar arthrodesis was performed for correction of valgus feet deformity. The average time of follow-up was 12.1 years. Seventy-nine per cent of the feet had satisfactory results, and all the failures were due to technical errors of graft placement. The calcaneo-first-metatarsal angle on the standing lateral radiograph was found to be the most objective angle and correlated best with the clinical results. The long term effects on the rest of the foot was negligible, with only 3 of the 10 feet, with a follow-up longer than 12 years showing minimal talonavicular arthritis and only 1 ankle developing a ball and socket joint. Ankle valgus was not a significant problem.

Adolescent↗

PubMed1991

Fracture-separation of the distal humeral epiphysis.

We have reviewed 12 cases of fracture-separation of the distal humeral epiphysis, three of which were initially misdiagnosed as fractures of the lateral condyle and one as an elbow dislocation. Cubitus varus deformity is as common after this fracture-separation as it is following supracondylar fracture, and is most common in children under two years of age. Closed reduction and simple immobilisation is adequate for the older child, but we recommend for those under two years of age that closed reduction should be followed by percutaneous pinning, so that the carrying angle can be assessed immediately after reduction. If the elbow is then in varus the wires should be removed, reduction repeated and treatment by straight lateral traction used to maintain a valgus carrying angle.

Arthrography↗

PubMed1991

Calcaneus deformity in the ambulant patient with myelomeningocele.

We reviewed our experience of tibialis anterior transfer and anterior release for calcaneus deformity in 46 feet of 26 ambulant patients with myelomeningocele. At an average follow-up of 8.4 years (2 to 17.6) there were 89% who had satisfactory results; 64% of the patients having tibialis anterior transfers were able to stand on their toes. Hip abductor power was a good predictor of a functional transfer. Pre-operative trophic ulceration of the heel increased from 3.2% to 33% if surgery was delayed. Secondary deformities, two-thirds of them into valgus, developed in 76% of feet.

Calcaneus↗

PubMed1990

Incidence of spinal deformity in children after multiple level laminectomy for selective posterior rhizotomy.

Fifty-five children with cerebral palsy had multiple-level laminectomies for selective posterior rhizotomies for the relief of spasticity. They were followed up clinically and radiologically to assess their spinal stability and the possible development of post-laminectomy deformity of the spine. The majority of the deformities found were related to cerebral palsy and did not appear to be due to the laminectomy: 16% had scoliosis, 5% kyphosis, 7% lordosis, and 9% spondylolysis/spondylolisthesis. Spondylolysis is the only abnormality that appeared to be more common in this group than in children with cerebral palsy.

Adolescent↗