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Biomedical subjects

F Miller

Publications and source records attributed to F Miller.

At least 55 records · Page 3Linked to original sources
PubMed1995

Frequency of familial dilated cardiomyopathy.

Based on evaluation of 59 probands and their families, we previously demonstrated that over 20% of patients with idiopathic dilated cardiomyopathy (DCM) have familial disease. We acquired 36 additional probands and found familial disease in 24.2% of the 95 probands in this expanded consecutively ascertained cohort. The family history, as reported by the patient and relatives, is often an unreliable indicator of familial disease because patients may be unaware of the significance of a family history of sudden death, arrhythmia or stroke. We demonstrate that careful family history with review of medical records identifies more familial cases than merely asking the patient if there is a family history of DCM. However, even such a careful family history does not identify all familial cases. Some familial cases are identified only by echocardiographic investigation of asymptomatic relatives. We found no clinical attributes of probands, other than family history, which predicted familial disease.

Adolescent↗
PubMed1995

Age and migration percentage as risk factors for progression in spastic hip disease.

Age and migration percentage were evaluated as risk factors for the progression of spastic hip subluxation in patients with cerebral palsy. Three age-groups were defined: group 1 (two to eight years), group 2 (nine to 18 years), and group 3 (over 18 years). Four subluxation groups were defined by migration percentage: group A (< 30 per cent), group B (30 to 60 per cent), group C (60 to 90 per cent), and group D (> 90 per cent). The risk of progression was the same in groups 1A and 2A, and both of these were higher than the risk in group 3A. All group B hips had similar risks for progression, and all group C hips progressed to group D (> 90 per cent migration percentage). Groups 1A, 1B, 2A, 2B and 3B hips need close radiographic follow-up to detect progressive subluxation. All group C hips required surgical treatment for progression to dislocation to be avoided.

Adolescent↗
PubMed1995

Endothelin receptor mRNA expression in renal medulla identified by in situ RT-PCR.

Three subtypes of endothelin (ET) receptors have been identified by cDNA cloning, namely ET-RA, ET-RB, and ET-RC. In the current study the precise cellular distribution of the ET receptor subtypes in the renal medulla was explored by detecting the corresponding polymerase chain reaction (PCR)-amplified cDNAs by in situ reverse transcription (RT)-PCR. The PCR-amplified cDNAs were detected either by direct incorporation using digoxigenin-dUTP (dig-dUTP) as a nucleotide substrate in the PCR reaction or by in situ hybridization with the dig-dUTP-labeled probe. ET-RB mRNA was detected exclusively in the epithelial cells of the inner and outer medullary collecting duct. In contrast, ET-RA message was observed primarily in interstitial cells and pericytes of the vasae rectae in the outer and inner medulla. Southern blot analysis of PCR-amplified cDNAs reverse transcribed from extracted RNA of rat renal medulla confirmed the specificity of the RT-PCR products. ET-RC mRNA was not detected. We conclude that ET-RB is the major ET receptor found in rat renal medulla and is expressed exclusively on inner medullary collecting duct cells. The pattern of ET receptor mRNA expression described suggests different physiological actions for ET on the diverse cellular structures of the renal medulla.

Animals↗
PubMed1994

The dose-response relationship for hypoxic pulmonary vasoconstriction.

In 12 pentobarbital anesthetized dogs the lungs were independently ventilated with a double piston ventilator. The right lung was ventilated throughout with 100% oxygen. Blood was drawn from the right atrium and pumped through a bubble oxygenator to a cannula in the ligated left main pulmonary artery. The pressures in the left main pulmonary artery and the left atrium were recorded during constant flow while the oxygen tension in the left lung alveolar gas and the perfusate were varied either to match each other (Protocol 1) or differ (Protocol 2) over the range from "zero" to "100%" oxygen. From the combined data a three dimensional response surface for hypoxic pulmonary vasoconstriction was derived. The maximum increase of pulmonary vascular resistance (r%PVRmax) was defined at a stimulus oxygen tension (PSO2) of 10 mmHg amounting to a 3.15 +/- (0.18)-fold increase of the vascular resistance on "100%" oxygen. The stimulus oxygen tension was shown to be PSO2 = PVO2(0.41) x PAO2(0.59) and the dose-response sigmoid for hypoxic pulmonary vasoconstriction in canine lungs was derived as r%PVRmax = 100 (PSO2(-2.616))/(6.683 x 10(-5) + PSO2(-2.616)) These results appear to reconcile observations from a number of laboratories and to be of quite general application.

Animals↗
PubMed1994

Unilateral unitary inflatable penile prosthesis to correct impaired tumescence and severe penile deformity resulting from traumatic rupture of 1 corpus cavernosum.

Nonsurgical management of a penile fracture can result in penile deformity due to plaque, and impaired tumescence due to corporeal fibrosis and altered erectile hemodynamics. We describe the correction of penile deformity and impaired tumescence by extensive resection of corporeal fibrosis and insertion of a unilateral unitary inflatable prosthesis. A possible role is proposed for correcting penile angulation secondary to unilateral disease using a unilateral unitary inflatable penile prosthesis.

Adult↗
PubMed1994

Responses to hemorrhagic arterial pressure reduction in different ischemic renal failure models.

Renal blood flow (RBF) autoregulation has been found to be impaired in both norepinephrine (NE) and renal artery clamp (RAC) rat ischemic acute renal failure models. However, the decline in RBF at the normal lower limit of autoregulation is greater in NE-ARF. The present study was designed to determine if this difference in autoregulatory profiles has potential functional and morphologic significance. After demonstrating a fall in RBF to renal perfusion pressure reduction to 90 mm Hg that was twofold more in one week NE- than RAC-ARF (p < 0.001), separate rats of both ischemic ARF types with nearly identical levels of azotemia and glomerular filtration rate reduction and sham-ARF rats were subjected to four-hour controlled reduction in mean arterial pressure to 90 by transient phlebotomy at one week. On day 9, two days after mean arterial pressure reduction, blood urea nitrogen (BUN), serum creatinine (SCr) and creatinine clearance (CCr) showed continued improvement in RAC-ARF, but there were significant increases in BUN (46 +/- 22 to 72 +/- 10 mg/dl) and SCr (1.2 +/- 0.2 to 1.5 +/- 0.2 mg/dl) and a decline in CCr (0.434 +/- 0.127 to 0.334 +/- 0.079 ml/min) in the NE-ARF group (all P < 0.02). The mean sum of scores of morphologic indices of ARF was higher in NE- than RAC-ARF kidneys of rats sacrificed on day 9 but interstitial edema was the only individual index that was worse in NE-ARF.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Kidney Injury↗
PubMed1994

Dose response of succinylcholine at the adductor pollicis of children with cerebral palsy during propofol and nitrous oxide anesthesia.

Children with cerebral palsy may be resistant to paralysis induced by nondepolarizing neuromuscular blocking drugs. Potency of a bolus of succinylcholine in children with cerebral palsy has not been studied previously. Therefore, we measured the response of the adductor pollicis to succinylcholine in children with cerebral palsy anesthetized with propofol and nitrous oxide. Forty children between the ages of 2 and 10.2 yr with spastic quadriplegic cerebral palsy were randomly assigned to receive 100, 175, 250, or 375 micrograms/kg of succinylcholine during anesthesia with propofol and nitrous oxide. The ulnar nerve was stimulated with a train-of-four supramaximal stimulus every 10 s and the compound electromyogram of the adductor pollicis recorded by a Datex NMT monitor. Plasma cholinesterase activity was measured in all patients with three different substrates (propionylthiocholine, benzoylcholine, and succinylcholine). Dibucaine number was also determined using inhibition of benzoylcholine degradation. ED50 of succinylcholine was 146.8 micrograms/kg with 95% confidence intervals of 111.4-193.7 micrograms/kg. ED95 of succinylcholine was 360.5 micrograms with 95% confidence intervals of 273.3-475.5 micrograms/kg. We conclude that children with cerebral palsy are slightly sensitive to succinylcholine, but probably not sufficiently to be clinically important.

Anesthesia, General↗
PubMed1994

Fracture of the spine in patients with ankylosis due to diffuse skeletal hyperostosis: clinical and imaging findings.

OBJECTIVE: Only 12 patients with a fracture through a portion of the spine ankylosed by diffuse idiopathic skeletal hyperostosis have been reported. The purpose of this study was to determine the types of causative trauma, spinal sites at risk for fracture, complications mortality, diagnostic difficulties, and abnormalities identified only with special imaging studies in a group of 15 patients with this complication seen at our institution. MATERIALS AND METHODS: The study included 15 patients with diffuse idiopathic skeletal hyperostosis who had a spinal fracture through an area of ankylosed spine. The criteria for diffuse idiopathic skeletal hyperostosis include flowing calcification or ossification along the anterolateral margin of at least four contiguous vertebral bodies; preservation of disk height in the involved areas; and absence of bony ankylosis of the apophyseal joint and erosion, sclerosis, or bony fusion of the sacroiliac joints. All spinal radiographs, tomograms, CT scans, and MR images obtained in these patients were reviewed to determine diagnostic difficulties, site and level of fracture, displacement of fractures, and extent of anatomic injury. The hospital charts of all patients were reviewed for history, physical examination, clinical status, treatment, and outcome. RESULTS: Fourteen fractures of the cervical spine, one of the thoracic spine, and one of the lumbar spine occurred in 15 patients. Five fractures were caused by high-energy trauma and 11 fractures by low-energy, seemingly trivial injuries. The spinal fracture caused complete quadriplegia in seven patients, incomplete quadriplegia in one patient, complete paraplegia in two patients, a central cord syndrome in two patients, and no neurologic deficit in three patients. In two of three patients in whom fracture diagnosis was delayed, paraplegia developed during the delay. Three patients died within 1 week and three more died within 6 months after injury. CT and conventional tomography enabled diagnosis of posterior element fractures not seen on plain radiographs in five patients. All fractures healed, except those in the three patients who died within a week of injury. CONCLUSION: Trivial trauma was the most common cause of fracture in the spine ankylosed by diffuse idiopathic skeletal hyperostosis. The severity of spinal cord injury in our patients was greater than in previous reports. We suggest that this may be a function of the relatively long segments of ankylosed spine (average, 16 vertebrae) in our patients providing a long lever arm for any traumatic force to act on. Patients with shorter ankylosed segments had less severe cord injuries. Delay in diagnosis of a fracture through an area of ankylosed spine associated with diffuse idiopathic skeletal hyperostosis was common if no neurologic deficit was present, and led to permanent paraplegia in two of three patients. CT and MR studies were useful in determining the anatomic abnormalities present, but were performed only in a limited number of patients.

Aged↗
PubMed1994

Loin pain-hematuria syndrome with a distinctive vascular lesion and alternative pathway complement activation.

We describe a 48-year-old woman with loin pain-hematuria syndrome. Her renal abnormalities included conspicuous microaneurysmal and glomeruloid (plexiform) angiomatous changes. The deposition of both properdin and the C5b-9 complex, as well as the usual C3, in arterioles argues for complement activation. To our knowledge, neither of these features has been previously described. We speculate about the cause of loin pain-hematuria syndrome and note the uncommonness of this entity in the United States as opposed to Great Britain.

Complement Pathway, Alternative↗
PubMed1994

Detailed immunohistologic evaluation of a methotrexate-induced nodule.

Accelerated nodulosis in patients with rheumatoid arthritis who are receiving methotrexate has become a well-recognized phenomenon, and it appears to occur in both individuals with seropositive and seronegative findings. No histologic differences permit a distinction between seronegative and seropositive nodulosis--either occurring during the natural history of rheumatoid arthritis or when "induced" by methotrexate. We examined such a nodule in a man with seronegative rheumatoid arthritis. Results of immunofluorescence examination were not contributory, but studies of the inflammatory cell populations within the nodule showed 5% of the cells to be lymphocytes with a CD4/CD8 ratio of 25:1. Of the total mononuclear cells, 85% were reactive for Leu-10 (HLA-DQ) and 95% were reactive for HLA-DR, and about 75% of the macrophages were reactive for CD4+. The strong expression of CD4, in conjunction with macrophage markers, and the high T-helper/suppression ratio are of note. We discuss the implications of these findings and the role that they play in the pathogenesis of methotrexate-induced nodules.

Arthritis, Rheumatoid↗
PubMed1994

Fibrogenic growth factors in the eosinophilia-myalgia syndrome and the toxic oil syndrome.

BACKGROUND AND DESIGN: We sought to determine if growth factors of potential pathogenetic significance are deposited in the skin, muscle, and peripheral nerve lesions of eosinophilia-myalgia (EMS) and toxic oil syndrome. Immunohistochemical studies using affinity-purified peroxidase-conjugated antibodies to detect transforming growth factor-beta, platelet-derived growth factorAA and growth factorBB, fibroblast growth factor, epidermal growth factor, and interleukin 4 were performed on formalin-fixed, paraffin-embedded specimens. Seven skin biopsy specimens from EMS, six skin biopsy specimens from toxic oil syndrome, nine muscle biopsy specimens from EMS, and one sural nerve biopsy specimen from EMS were studied. RESULTS: Growth factor staining was noted primarily in the epidermis and periappendageal locations of the dermis. The presence of TGF-beta and platelet-derived growth factorAA in the periappendageal dermis was significantly more prevalent in EMS than toxic oil syndrome (57% vs 0%). Prominent staining of transforming growth factor-beta was also present in the perimysial connective tissue of five (63%) of eight EMS muscle biopsy specimens and one sural nerve biopsy specimen. CONCLUSIONS: These studies implicate transforming growth factor-beta and platelet-derived growth factorAA as potentially important cytokines in EMS and suggest that the pathogenesis of tissue fibrosis in EMS and toxic oil syndrome may be dependent on different growth factors.

Biopsy↗
PubMed1993

A 5' splice junction mutation leading to exon deletion in an Ashkenazic Jewish family with phosphofructokinase deficiency (Tarui disease).

A deficiency of the muscle isoform of the enzyme, phosphofructokinase (PFK, EC 2.7.1.11), leads to an illness (glycogenosis, Type VII) characterized by myopathy and hemolysis. A patient with this disease and an affected sister were found to have a G to A substitution at the 5' donor site of intron 5 of the PFK-M gene. This mutation led to a splicing defect: a complete deletion of the preceding exon in the patient's mRNA. The patient, an affected sister, and related and unrelated family members, who were of Ashkenazic Jewish background, were screened for the mutation by denaturing gradient gel electrophoresis and by allele specific hybridization of genomic DNA. The affected sisters are homozygous for the mutation, and their children, who are unaffected, are heterozygous. The only previously characterized genetic defect in this disease, found in a Japanese patient, was a G to T mutation at the beginning of intron 15 with splicing to a cryptic site within exon 15 (1). Both mutations lead to inframe deletions, but of different parts of the protein. The differences between the two aberrant proteins may account for clinical differences between our patients and the Japanese patient.

Base Sequence↗
PubMed1993

Proximal femoral resection for older children with spastic hip disease.

Thirteen patients (19 hips) who underwent proximal femoral resection arthroplasty for symptomatic subluxation or dislocation of the hip were reviewed retrospectively. All had severe neurological involvement, and when older were non-ambulatory with spastic quadriplegia or myelodysplasia. Resections were either at the level of the neck (nine hips) or in the subtrochanteric region (10 hips). The latter provided a better operative result than did the former. Poor clinical results noted were continued pre-operative pelvic obliquity, postoperative adduction contracture, and bony contact between the pelvis and the remaining proximal femur or its heterotopic ossification.

Adolescent↗
PubMed1993

Reversible renal failure in the primary antiphospholipid syndrome--a report of two cases.

The primary antiphospholipid/anticardiolipin syndrome is a recently described entity wherein multiorgan thrombotic events occur in the absence of objective evidence of systemic lupus erythematosus. The spectrum of renal involvement remains poorly described. Two patients with coagulation abnormalities consistent with the primary antiphospholipid/anticardiolipin syndrome who developed profound renal insufficiency are reported. Striking microangiopathic lesions were documented on renal biopsy. Renal function improved concomitant to the institution of steroid therapy. Reversible renal failure should be added to the spectrum of clinical manifestations of this entity. The diagnosis of the primary antiphospholipid/anticardiolipin syndrome should be contemplated in individuals with unexplained acute renal failure.

Adult↗
PubMed1993

Isometric exercise increases strength and does not produce sustained creatinine phosphokinase increases in a patient with polymyositis.

A patient with active stable idiopathic polymyositis received a 4-week supervised right quadriceps and biceps isometric strengthening program. He demonstrated a significant increase in isometric peak torque without a sustained rise in creatinine phosphokinase (CPK). A significant decrease in postexercise CPK occurred at the end of the exercise program. We conclude a randomized clinical trial using a nonexercised patient group vs an exercised patient group may be useful.

Creatine Kinase↗