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Biomedical subjects

M J Murphy

Publications and source records attributed to M J Murphy.

At least 199 records · Page 11Linked to original sources

Peripheral neurofibromatosis and peroneal muscular atrophy.

We studied four patients with peripheral neurofibromatosis and a neuropathy that had the clinical characteristics of peroneal muscular atrophy. Nerve conduction velocities were slowed by less than 40% of normal, and electromyography demonstrated denervation. Sural nerve biopsies from two patients, which were macroscopically free of nerve sheath tumors, were studied by recording the compound action potentials in vitro and by morphometry. These studies demonstrated a chronic axonal neuropathy with reactive Schwann cell changes. Peroneal muscular atrophy in association with neurofibromatosis may be due to progressive neuronal degeneration and may represent another, uncommon manifestation of peripheral neurofibromatosis.

Action Potentials↗

The role of erythropoietin in erythroid colony formation from Rauscher leukemia virus-infected mice.

Recent work has led to conflicting results regarding the in vitro control of erythropoiesis in mice infected with Rauscher Leukemia Virus (RLV). Some reports claimed that marrow or splenic erythropoiesis from RLV-infected mice were independent of exogenously added erythropoietin (Ep), while other reports supported the view that erythroid stem cells (CFUE) from RLV-infected mice required EP for erythroid differentiation and proliferation. Results reported here demonstrate that erythroid differentiation and proliferation. Results reported here demonstrate that CFUE cultured from RLV-inoculated mice are dependent upon Ep for erythroid differentiation. Furthermore, RLV-infected mice which had received transfusions to suppress the endogenous production of Ep not only had a reduction in spleen weight and reticulocyte percentage, but also reduced numbers of CFUE and BFUE.

Animals↗

Non-frozen preservation of committed hematopoietic stem cells from normal human bone marrow.

We examined the optimal conditions for maintaining normal human hematopoietic stem cells without cryopreservation. The effects of serum, HEPES, CPD and storage temperature were studied. Stem cell integrity (CFU-c, CFU-e, and BFU-e) was optimally maintained when the bone marrow cells were suspended in a medium containing 40% fetal calf serum, 25 mM HEPES, and 12.28% CPD and maintained at 4 degrees C. For example, under these storage conditions the recovery of functionally intact early erythroid stem cells, BFU-e, was 84% after 2 days, 44% after 4 days and 12% after 7 days. These results indicate that non-frozen short-term storage of human bone marrow may be of clinical utility in bone marrow transplantation.

Cell Survival↗

Erythropoiesis in vitro. IV. Effects of ouabain on erythroid stem cells (CFU-e and BFU-e).

Recent investigations employing various ionophores and cardiac glycosides have detected an increase in the number of late-stage erythroid progenitor cells (CFU-e) cultured from normal murine marrow. We report that the cardiac glycoside, ouabain, not only increased the number of erythroid progenitor cells, but also elevated the number of the earliest erythroid colony-forming cells (BFU-e). This rise in the number of erythroid stem cells was dose dependent with a maximum increase in erythroid colony numbers observed at 10(-15) M ouabain. Furthermore, ouabain exerted its stimulatory activity by increasing the proportion of erythroid colony-forming cells (BFU-e) in cell cycle as determined by 'thymidine suicide' experiments. The substitution of dialyzed fetal calf serum in the culture medium markedly inhibited the erythroid colony stimulation by ouabain. The deficient factor in dialyzed fetal calf serum appears to be K+, because the stimulatory influence of ouabain was restored upon reconstitution of the serum with K+. These studies suggest that ouabain-induced erythropoietic enhancement may involve mechanisms other than inhibition of membrane-bound Na+/K+ATPase.

Animals↗

Synthesis and anticancer activity of asparagine analogs.

A series of 11 asparagines substituted on N4 was prepared and evaluated for their ability to inhibit the growth of L5178Y leukemia cell cultures. These cells require an exogenous source of L-asparagine and should be sensitive to an asparagine antimetabolite. The compounds were prepared by reaction of phthalylaspartic anhydride with a primary or secondary amine, followed by removal of the phthalyl group with hydrazine. One compound, N,N-dibenzylasparagine, showed significant activity. Additional study of asparagine derivatives bearing large, lipophilic groups at N4 is warranted.

Animals↗

Spinal stabilization in acute spinal injuries.

Stabilization of acute spinal injuries is of prime importance in prevention of further neurologic compromise, prevention of late spinal deformity with associated risk of neurologic compromise, prevention of painful pseudarthrosis, and in maximizing rehabilitation. Many questions regarding the best care and stabilization of the injured spine, particularly those associated with spinal cord injury, remain unanswered. Certainly a diversity of opinion regarding these issues exists between various centers throughout the country today. Hopefully the development of the Regional Spinal Cord Injury Center concept will allow collection of clinical data sufficient to standardize care of these patients in the future.

Cervical Vertebrae↗

Intracranial dermoid cyst in Goldenhar's syndrome. Case report.

A patient with Goldenhar's syndrome and an ipsilateral intracranial dermoid cyst is reported. It is suggested that the combination of both entities may not be coincidental but due to their related embryological origin. This report also reemphasizes the value of computerized tomography in the preoperative diagnosis of intracranial dermoid cysts.

Adult↗

Fractures of the tibial tuberosity in adolescents.

The cases of fourteen adolescents with fifteen physeal fractures of the tibial tuberosity were reviewed to more accurately define specific fracture patterns, to establish treatment for the different types, and to determine the incidence of complications. A modified classification scheme with a greater emphasis on intra-articular extension of the fracture and communution of the tuberosity was devised. Closed or open reduction, as necessary, gave satisfactory results. The primary indications for surgery were: (1) displacement of one or more fragments of the tuberosity anterosuperiorly, and (2) extension of the fracture through the proximal tibial ossification center into the knee joint, with disruption of the joint surface. There appears to be an increased incidence of pre-existing Osgood-Schlatter disease (ipsilateral and contralateral) in patients who have an acute tuberosity injury. Complications are rare. The theoretical possibility of the subsequent development of genu recurvatum appears unlikely, since most of these injuries occur when the physis of the tuberosity is undergoing normal closure.

Adolescent↗

Impact of EFNEP on some nutrition-related practices. Developing a tool to record changes.

Described are the design and preliminary administration of an instrument to measure the impact of the Pennsylvania Expanded Food and Nutrition Education Program (EFNEP) on food storage and safety, kitchen sanitation, and food money management practices of program participants. No significant differences among these practices were found when each was correlated with duration of program participation. Overall improvement, however, was revealed, particularly in the first six months in the program.

Agriculture↗

Clinical correlations of CT scan-detected calcifications of the basal ganglia.

A review of CT scans of 7,081 patients demonstrated calcifications of the basal ganglia in 53. The calcifications were evident in the skull roentgenograms of only 4 patients out of 40 in whom both CT scans and plain roentgenograms were available, demonstrating the superior resolution of this new method. Seventy-five percent of the patients were older than 50 years of age. Of the younger patients, 5 had had prior cranial irradiation; 1 had received cranial irradiation and intrathecal methotrexate therapy for meningeal leukemia; and 2 others had deep-seated arteriovenous malformations. Serum concentrations of calcium and phosphorus were normal in all 46 patients in whom they were measured. We conclude that the detection of small calcifications of the basal ganglia in persons above 50 years of age is infrequently associated with either clinical signs of basal ganglia dysfunction or calcium and phosphorus abnormalities. Calcium deposition in these patients may be related to vascular changes associated with aging. In younger patients a specific pathogenetic factor or underlying process is infrequently found.

Adolescent↗

A case of poisoning with mercuric chloride.

A male patient was admitted to hospital 12 h after self-poisoning with mercuric chloride. He suffered multiple complications including acute renal failure, ulcerative colitis, anaemia, disseminated intravascular coagulation, chronic sepsis and severe weight loss. Initially he responded well to resuscitative measures and intensive supportive therapy, which included ventilation of the lungs, haemodialysis, dimercaprol, antibiotics, parenteral feeding and gastrointestinal surgery. Unfortunately the sepsis was never satisfactorily eradicated despite satisfactory serum concentrations of the appropriate antibiotics. On day 43 after poisoning he had a grand mal fit; after this there were focal neurological signs and on lumbar puncture he was found to have a raised protein concentration and raised pressure in his cerebrospinal fluid. The condition of the patient rapidly deteriorated and on day 47 he died. Post-mortem examination revealed a large cerebellar abscess. The literature on mercury poisoning is reviewed.

Acute Kidney Injury↗

Erythroid progenitors (BFU-e and CFU-e) in acute leukaemia.

Bone marrow erythroid progenitor cells were examined from 50 cases of acute leukaemia and from 20 normal subjects using an in vitro semisolid culture method. Numbers of both primitive erythroid progenitor cells (BFU-e) and later-stage erythroid progenitor cells (CFU-e) were remarkably depressed in patient with acute leukaemia in active phase. However, both BFU-e and CFU-e recovered to within normal range when the patients achieved remission. Peripheral blood BFU-e of children with acute lymphocytic leukaemia in remission were also examined and found to have values not significantly different from those of control subjects. There was no distinct correlation between the numbers of erythroid bursts or colonies and the duration of remission in patients with acute leukaemia in remission. The reduction of BFU-e and CFU-e in active acute leukaemia suggests the involvement of erythropoietic progenitors in the pathophysiology of this type of leukaemia.

Acute Disease↗