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Ophthalmologist (eyes)
14 years of experience
Video profile
Accepting new patients

Education ?

Medical School Score Rankings
Boston University (1996)
  • Currently 4 of 4 apples
Top 25%

Awards & Distinctions ?

American Academy of Ophthalmology
American Board of Ophthalmology
American Society of Ophthalmic Plastic and Reconstructive Surgery

Affiliations ?

Dr. Goldstein is affiliated with 12 hospitals.

Hospital Affilations



  • Doylestown Hospital
    595 W State St, Doylestown, PA 18901
    • Currently 3 of 4 crosses
    Top 50%
  • Warminster Hospital
    225 Newtown Rd, Warminster, PA 18974
    • Currently 2 of 4 crosses
  • Holy Redeemer Health System
  • Wills Eye Hospital
    840 Walnut St, Philadelphia, PA 19107
  • Hospital of the University of PA
  • Holy Redeemer Hospital and Medical Center
  • Abington Memorial Hospital
  • Abington Hospital
  • Pa Academy of Ophthalmology&
  • Pennsylvania Medical Society
  • AmAcademy of Ophthalmology
  • Scheie Eye Institute
  • Publications & Research

    Dr. Goldstein has contributed to 48 publications.
    Title Caution Cutting the Canthus: Thoughts on Microphthalmic Expansion.
    Date August 2008
    Journal Journal of Aapos : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus
    Title Erlotinib-associated Trichomegaly.
    Date March 2007
    Journal Ophthalmic Plastic and Reconstructive Surgery

    A 70-year-old woman presented with a corneal ulcer resulting from aberrant and exuberant eyelash growth. She had recently been diagnosed with recurrent non-small-cell lung cancer for which she had begun a chemotherapeutic regimen that included erlotinib HCl. After several months of treatment, she noted the onset of escalating ocular irritation, which culminated in her presentation. Examination of her ocular adnexa revealed coarse, brittle, irregular aberrant eyelash growth. She has been successfully treated with regular epilation and eyelash trimming and has remained symptom free.

    Title The Histopathologic Effects of Balloon Dacryoplasty on the Rabbit Nasolacrimal Duct.
    Date September 2006
    Journal Journal of Aapos : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus

    PURPOSE: To examine the histopathologic effects of balloon dacryoplasty on the nasolacrimal duct of the rabbit. METHODS: Sixteen nasolacrimal ducts of 8 infant white New Zealand rabbits were divided into 3 treatment groups: a control group, a 2 mm balloon dilation group, and a 3 mm balloon dilation group. The LacriCath balloon catheter (Quest, Medical, Allen, TX) was used for nasolacrimal duct dilation according to the manufacturer's recommended human protocol. The rabbits were euthanized at 1, 3, and 7 days after treatment and the nasolacrimal ducts were harvested for histological examination. RESULTS: There were no significant differences between treatment groups in the number of neutrophils in the lumen, epithelium, and stroma of the duct; the number of monocytes in the stroma of the duct; or in the number of red blood cells in the stroma of the duct. No differences were found in overall cellularity of any type in the nasolacrimal ducts of treated and untreated rabbits. Finally, no histopathologic signs of crush injury were evident. CONCLUSION: Balloon dacryoplasty does not induce significant inflammation or crush injury in the nasolacrimal duct of a rabbit model.

    Title Anatomic and Aesthetic Considerations in Midfacial Rejuvenation.
    Date September 2006
    Journal Facial Plastic Surgery : Fps

    An early sign of the aging process involves changes to the midface. The loss of the lid-cheek continuum begins with underlying laxity in the orbitomalar ligament. As the face continues to age, the malar pad drops inferomedially in position. The approach to the midface has evolved over the past 15 years with the advent of endoscopically assisted techniques of midfacial elevation. Further technology, such as thread-lifting procedures and the Endotine Midfacetrade mark, have supplemented the choices for surgical correction of this facial segment. Descriptions of the different approaches to the aging midface are reviewed.

    Title The Male Eyebrow: a Topographic Anatomic Analysis.
    Date September 2005
    Journal Ophthalmic Plastic and Reconstructive Surgery

    PURPOSE: To study and quantify the topographic anatomy of the male eyebrow and its 3-dimensional relation, based on age and location, relative to the eye and the supraorbital rim. METHODS: A prospective cohort of male patients seen for routine ophthalmic evaluation age 10 years and older had several eyebrow measurements incorporated in the examination. The presence or absence of brow ptosis was noted on the basis of observation. The location of the eyebrow relative to the center of the pupil, the lateral canthus, and the depth of the supraorbital rim was measured. The data were subjected to statistical analysis. RESULTS: The study included 444 eyebrows of 222 patients ranging in age from 10 to 86 years, with a mean age of 46.75 years. The incidence of brow ptosis was 7.4%. The mean distance between the lateral canthus and inferior brow (LIBD) was 11.23 mm. The mean distance between the pupil and the inferior brow and the superior brow border was 11.15 mm (PIBD) and 19.8 mm (PSBD), respectively. Statistical analysis demonstrated that the mean brow position did not vary with age. However, there was a significant negative correlation between the position of the eyebrow and the depth of the superior sulcus below the supraorbital rim (correlation coefficient, 0.431 for PIBD and depth and 0.381 for LIBD and depth, respectively). Deeper-set eyes had lower brow position. CONCLUSIONS: The male eyebrow sits along the supraorbital ridge and has a flat contour. In men without frank brow ptosis, the eyebrow position relative to the pupil and the lateral canthus does not significantly fall with age despite the periocular changes associated with aging. However, 3-dimensional analysis demonstrates that individuals with deep-set eyes have a lower positioned eyebrow then those with a more shallow depth below the supraorbital rim. These findings are helpful in surgical management of the eyelid-eyebrow continuum. The age-related changes in the eyebrow area should be thought of as a 3-dimensional construct with collapse of the structure and not simply descent of the eyebrow.

    Title Chronic Neuropathic Pain Associated with Imiquimod: Report of 2 Cases.
    Date August 2005
    Journal Journal of the American Academy of Dermatology
    Title Comparison of Monocanalicular Stenting and Balloon Dacryoplasty in Secondary Treatment of Congenital Nasolacrimal Duct Obstruction After Failed Primary Probing.
    Date November 2004
    Journal Ophthalmic Plastic and Reconstructive Surgery

    PURPOSE: To determine the success of monocanalicular stenting and balloon dacryoplasty as secondary treatment options for congenital nasolacrimal duct obstruction after failed probing surgery. METHODS: An interventional case series of consecutive secondary balloon dacryoplasty and monocanalicular stenting for congenital nasolacrimal duct obstruction was reviewed. These secondary treatments were used in cases in which a bicanalicular stent would have been used in the past. Seventy-seven nasolacrimal systems in children with epiphora after probing and irrigation surgery were treated with a monocanalicular stent or balloon dacryoplasty. The patients were then evaluated at least 3 months after surgery or after stent removal by using a dye disappearance test. Cases in which there was no significant dye at 5 minutes were considered a success. Cases with residual dye or history of persistent tearing were considered failures. RESULTS: The monocanalicular stent was used in 35 nasolacrimal systems, whereas balloon dacryoplasty was used in 42 nasolacrimal ducts. The mean age of treatment was 25.2 months for the monocanalicular stent group and 25.8 months for the balloon group. Overall, 32 of 35 (91%) nasolacrimal ducts responded to monocanalicular stenting, whereas 36 of 42 (86%) responded to balloon treatment. When the patient group was further stratified by age, the monocanalicular stenting was 94% successful in children younger than age 2 years and 89% successful for children older than 2 years. The balloon treatment had a success rate of 91% in the younger group and 79% in the older group. Chi-square statistical analysis showed no significant difference between the two treatments or on the basis of age stratification within each treatment group. CONCLUSIONS: Monocanalicular stenting and balloon dacryoplasty are excellent secondary therapies for congenital nasolacrimal duct obstruction after initial probing and irrigation surgery has failed. These two treatment options are now our procedures of choice for secondary surgery.

    Title Heterozygous Mutations of the Kinesin Kif21a in Congenital Fibrosis of the Extraocular Muscles Type 1 (cfeom1).
    Date January 2004
    Journal Nature Genetics

    Congenital fibrosis of the extraocular muscles type 1 (CFEOM1; OMIM #135700) is an autosomal dominant strabismus disorder associated with defects of the oculomotor nerve. We show that individuals with CFEOM1 harbor heterozygous missense mutations in a kinesin motor protein encoded by KIF21A. We identified six different mutations in 44 of 45 probands. The primary mutational hotspots are in the stalk domain, highlighting an important new role for KIF21A and its stalk in the formation of the oculomotor axis.

    Title Encephalocraniocutaneous Lipomatosis: a Neurocutaneous Syndrome.
    Date December 2003
    Journal Journal of Aapos : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus

    The neurocutaneous pattern syndromes are a group of disorders characterized by congenital abnormalities involving both the skin and the nervous system for which no identifiable cause has been isolated.(1) Ophthalmologic manifestations of these syndromes are common. These rare syndromes often have overlapping ophthalmic and systemic findings. Examples include encephalocraniocutaneous lipomatosis (ECCL), oculocerebrocutaneous syndrome (OCC), and linear nevus sebaceous syndrome (LNS). Clarifying the diagnostic criteria for these syndromes is a worthy goal because it will help elucidate the phenotypic spectrum of these poorly understood diseases as well as possibly facilitate genetic counseling. In this short report, a case of ECCL is used to illustrate the clinical manifestations of neurocutaneous syndromes.

    Title Cat Scratch Disease Presenting As Orbital Abscess and Osteomyelitis.
    Date October 2003
    Journal Journal of Clinical Microbiology

    Ocular manifestations of cat scratch disease are uncommon. The diagnosis is usually made on the basis of increasing Bartonella henselae serum antibody titers. We report a child presenting with orbital abscess and osteomyelitis who was diagnosed with hepatosplenic cat scratch disease by detection of B. henselae DNA in the orbital abscess fluid.

    Title Metastatic Breast Cancer to 4 Eyelids: a Clinicopathologic Report.
    Date May 2003
    Journal Cutis; Cutaneous Medicine for the Practitioner

    A 47-year-old woman presented complaining of progressive, painless swelling of all 4 eyelids for 6 weeks. Her medical history was significant for breast cancer 14 months prior. Examination results showed a diffuse, firm thickening of all 4 eyelids with mild erythema of the overlying skin. Orbital computer tomography revealed extensive preseptal infiltration of soft tissue of all 4 eyelids, which enhanced with contrast. Results of a bilateral biopsy of the upper eyelids demonstrated extensive infiltration of the orbicularis muscle. Histologic features were consistent with metastatic breast cancer. Results of both the primary breast cancer biopsy taken 14 months previously and the eyelid biopsies were the same histologic type. Presentation of metastatic breast cancer to the eyelids is rare, but a recurrence must be considered in any patient with a history of breast cancer, despite the length of tumor-free survival. Bilateral involvement should not exclude metastases from diagnostic consideration, but rather, the diagnosis requires a high degree of clinical suspicion and recognition of the various cutaneous forms.

    Title Paediatric Periocular Granuloma Annulare.
    Date May 2003
    Journal Acta Ophthalmologica Scandinavica
    Title Acute Myelogenous Leukaemia Presenting As a Conjunctival Lesion and Red Eye.
    Date February 2003
    Journal Acta Ophthalmologica Scandinavica
    Title Orbital Presentation of Posttransplantation Lymphoproliferative Disorder: a Small Case Series.
    Date December 2002
    Journal Ophthalmology

    OBJECTIVE: To describe a small series of patients with orbital presentation of posttransplantation lymphoproliferative disorder (PTLD). DESIGN: Retrospective, interventional case series. PARTICIPANTS: Three patients with orbital presentation of histologically diagnosed PTLD. METHODS: Review of medical records. MAIN OUTCOME MEASURES: Measured parameters included vision, proptosis, and tumor extent. RESULTS: Three cases of orbital PTLD are described. In two of the cases, the tumor initially demonstrated orbital signs and symptoms, whereas in the third case, orbital and systemic signs were synchronous. Two of three patients had disseminated disease discovered at the time of presentation. One adult patient had synchronous presentation of PTLD in the orbit and prostate. One pediatric patient had tumor dissemination to the liver at the time of presentation. The PTLD tumors were classified histologically as diffuse large cell lymphoma of monomorphic or immunoblastic type in all three cases. Treatment included local irradiation, decreased immunosuppression, and antilymphocyte monoclonal antibodies. CONCLUSIONS: Orbital presentation is a rare manifestation of PTLD. However, ophthalmologists must consider this diagnosis carefully in organ transplant recipients with subtle orbital signs and symptoms at presentation. Early detection may alter prognosis. In each case presented, the diagnosis was established via lesion biopsy and subsequent histologic or flow cytometric evaluation, or both.

    Title Ptosis in the Elderly Patient.
    Date June 2002
    Journal International Ophthalmology Clinics
    Title Orbital Neural-glial Hamartoma Associated with a Congenital Tonic Pupil.
    Date May 2002
    Journal Journal of Aapos : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus

    Tonic pupils in children are rare, and only a handful of reports exist in the literature. We present the case of an orbital neural-glial hamartoma in an infant with a congenital tonic pupil and no proptosis. We have previously described this association in another child. This new case, the 6-year follow-up from the previous case, and a discussion of tonic pupils are presented.

    Title Treacher Collins Syndrome with Novel Ophthalmic Findings and Visceral Anomalies.
    Date May 2002
    Journal The British Journal of Ophthalmology
    Title Empirical Support for the Baldrige Award Framework in U.s. Hospitals.
    Date February 2002
    Journal Health Care Management Review

    Examined are relationships among constructs in the Baldrige Award Health Care Criteria framework to investigate whether quality management systems are related to organizational results and customer satisfaction in hospitals. Measures for the 19 dimensions of the Baldrige Criteria are obtained from 220 U.S. hospitals. This study provides empirical evidence that focusing on the content addressed in the Baldrige Criteria leads hospitals to improvement on some dimensions of performance.

    Title Cancer-associated Retinopathy.
    Date December 1999
    Journal Archives of Ophthalmology

    Patients with systemic cancer may have a variety of ocular complaints. Most commonly these are metastases or adverse effects of therapy. Paraneoplastic syndromes, like cancer-associated retinopathy, rarely cause ophthalmic symptoms. We describe a patient with a malignant mixed mullerian tumor and cancer-associated retinopathy who had circulating serum antibodies to recoverin and cells positive for recoverin in the tumor. We discuss the typical clinical symptoms as well as the pathophysiology of this uncommon disorder.

    Title Community Coalitions: a Self-assessment Tool.
    Date July 1997
    Journal American Journal of Health Promotion : Ajhp
    Title Advances in the Treatment of Superficial Candida Infections.
    Date March 1994
    Journal Seminars in Dermatology

    This article reviews recent controlled studies of treatment for candida infections of the skin and mucous membranes. In general, excellent agents are available for treatment, with reasonable alternatives for the uncommon emergence of resistant strains. Cutaneous infections in immunocompetent patients are effectively treated with topical agents. The major clinical problems that remain include frequently recurrent vulvovaginal candidiasis, and oroesophageal candida infections in patients with malignancies and immunosuppression. These often require systemic therapy. A number of treatment strategies have been investigated and are reviewed.

    Title Distinct Infrared Spectroscopic Patterns of Human Basal Cell Carcinoma of the Skin.
    Date March 1993
    Journal Cancer Research

    Infrared spectroscopy combined with high pressure (pressure-tuning infrared spectroscopy) was applied to the study of paired sections of basal cell carcinomas (BCC) and normal skin from ten patients. Atmospheric pressure IR spectra from BCC were dramatically different from those from the corresponding normal skin. Compared to their normal controls, BCCs displayed increased hydrogen bonding of the phosphodiester group of nucleic acids, decreased hydrogen bonding of the C--OH groups of proteins, increased intensity of the band at 972 cm-1, a decreased intensity ratio between the CH3 stretching and CH2 stretching bands, and accumulation of unidentified carbohydrates. Some of these changes are shared by all human epithelial malignancies studied to date, while some others appear as yet unique to basal cell carcinoma. The diagnostic value of infrared spectroscopy in BCC remains to be determined.

    Title Human Skin Mast Cell Carboxypeptidase: Functional Characterization, Cdna Cloning, and Genealogy.
    Date August 1992
    Journal The Journal of Investigative Dermatology

    We functionally characterized human skin mast cell carboxypeptidase A (MC-CPA), and explored its evolutionary relationship to other carboxypeptidases to understand further the structural basis for the substrate preferences of this enzyme. Purified human skin MC-CPA displayed more activity than did bovine pancreatic carboxypeptidase A (CPA) against carboxyl-terminal leucine residues, about equal activity with phenylalanine and tyrosine residues, and no activity with tryptophan or alanine. To correlate kinetic data with structure, we isolated and sequenced a cDNA encoding MC-CPA from human skin, and directly sequenced 30% of the purified protein. These sequences agreed with that of human lung MC-CPA, and further support the evidence for a single MC-CPA gene in humans. Four amino acid replacements, resulting in a net positive change in non-hydrogen atoms in the S1' subsite of MC-CPA, were associated with less alteration in substrate specificity, relative to bovine CPA, than might be expected from studies using rat CPA1 and CPA2. We noted two consensus N-linked glycosylation sites in human MC-CPA that are not found in rat and mouse MC-CPA, or in bovine CPA; that at least one of these sites is glycosylated in vivo was verified by N-glycosidase F treatment, lentil lectin binding, and Concanavalin A-Sepharose chromatography. Evolutionary trees constructed from the known carboxypeptidase sequences suggested that MC-CPA most likely evolved from a carboxypeptidase B-like enzyme, independent of the pancreatic CPA. Thus, in the carboxypeptidase gene family, MC-CPA displays a unique genealogy and several amino acid replacements in its S1' binding pocket that result in substrate specificity quite similar to bovine CPA.

    Title Protease Composition of Exocytosed Human Skin Mast Cell Protease-proteoglycan Complexes. Tryptase Resides in a Complex Distinct from Chymase and Carboxypeptidase.
    Date May 1992
    Journal Journal of Immunology (baltimore, Md. : 1950)

    We characterized the release and the protease composition of high m.w. complexes released from dispersed human skin mast cells, under conditions that did not disrupt the binding of proteases to proteoglycan. The net percent release ratio of tryptase to histamine, after anti-IgE and calcium ionophore A23187 stimulation was higher than those for chymase or carboxypeptidase. This was explained by the greater cell association of carboxypeptidase and chymase, compared with tryptase, after mast cell degranulation and/or differential cosedimentation of the proteases with mast cells, because treatment of activated mast cells with 1 M NaCl increased the release ratios of chymase and carboxypeptidase more than that of tryptase. Tryptase, after release, was stable in 0.12 M NaCl and had a molecular mass of approximately 200 to 250 kDa, suggesting that it was bound to proteoglycan. We demonstrated that complexes containing chymase and carboxypeptidase were separable from tryptase-containing complexes by gel filtration and by affinity chromatography. First, on fast protein liquid chromatography, released tryptase filtered at a molecular mass of approximately 200 to 250 kDa, compared with chymase and carboxypeptidase at 400 to 560 kDa. Second, by using affinity chromatography with immobilized antitryptase mAb in 0.15 M NaCl, carboxypeptidase and chymase activities were recovered primarily in the effluent and washes of an antitryptase antibody affinity column and cofiltered at 400 to 560 kDa. Tryptase was recovered only in the eluate. Finally, by using potato tuber carboxypeptidase inhibitor-Sepharose affinity chromatography, tryptase activity was found primarily in the effluent and washes, filtered at a molecular mass of 200 kDa on fast protein liquid chromatography, and was stable in 0.12 M NaCl buffer at 37 degrees C. Carboxypeptidase and chymase activities were found primarily in the eluate. These findings suggest that tryptase and carboxypeptidase/chymase reside in distinct macromolecular complexes. Separate complexes containing these proteases may help explain previous ultrastructural observations in which the distributions of chymase and tryptase within a single granule did not always coincide.

    Title Human Mast Cell Proteases Hydrolyze Neurotensin, Kinetensin and Leu5-enkephalin.
    Date April 1992
    Journal Peptides

    Purified mast cell carboxypeptidase cleaved the C-terminal leucines from Leu5-enkephalin (Leu-ENK), neurotensin (NT), and kinetensin (KT), with Km values of 36, 16, and 15 microM, and kcat values of 44, 51, and 53 s-1, respectively. To better predict potential in vivo hydrolysis products generated by mast cell proteases, these peptides were incubated with released skin mast cell supernatants. Leu5-enkephalin was hydrolyzed only by carboxypeptidase. Kinetensin was cleaved by tryptase, chymase, and carboxypeptidase to yield KT(1-3), KT(1-7), KT(1-8), KT(4-7), and KT(4-8), the last two peptides by the concerted action of two of the proteases. NT(1-11) and NT(1-12) were generated from neurotensin by chymase and carboxypeptidase, respectively.

    Title Isolation of a Neuropeptide-degrading Carboxypeptidase from the Human Stomach.
    Date January 1992
    Journal Gastroenterology

    The aim of this investigation was to isolate and characterize a neuropeptide-degrading carboxypeptidase from the muscular and mucosal layers of the human stomach. The carboxypeptidase was solubilized from membrane preparations of gastric muscle and mucosa using Triton X-100. The detergent-solubilized enzyme was purified to apparent electrophoretic homogeneity by affinity chromatography using lisinopril or potato carboxypeptidase inhibitor as an affinity ligand. The enzyme had an apparent molecular weight of 34,300 and was bound by concanavalin A and is thus a glycoprotein. The carboxypeptidase removed C-terminal leucine, phenylalanine, or tryosine residues from peptides including angiotensin I, [Leu5]enkephalin, kinetensin, neuromedin N, neurotensin, and xenopsin. It had an alkaline pH optimum and was inhibited by lisinopril, potato carboxypeptidase inhibitor, ethylenediaminetetraacetic acid, 1,10-phenanthroline, and 8-hydroxyquinoline. Immunoblotting indicated that the gastric carboxypeptidase cross-reacted with an antibody raised against a carboxypeptidase isolated from mast cells of human skin. The gastric carboxypeptidase released from gastric mast cells upon degranulation may act to degrade and inactivate neuropeptides in the stomach wall.

    Title Human Mast Cell Carboxypeptidase. Selective Localization to Mctc Cells.
    Date July 1991
    Journal Journal of Immunology (baltimore, Md. : 1950)

    Two murine mAb were prepared against human mast cell carboxypeptidase (HMC-CP) purified from human skin, and were termed CP1 and CP2, respectively. Double immunohistochemical labeling of Carnoy's-fixed sections of human skin, lung, and gastrointestinal tissue with CP1 and CP2, respectively, and with a murine monoclonal antitryptase antibody demonstrated that HMC-CP was selectively present in a subset of human mast cells. Double labeling experiments with CP1 and CP2, respectively, and a murine anti-chymase mAb demonstrated the presence of HMC-CP in the tryptase-positive, chymase-positive mast cell type (MCTC) only. Immunohistochemical labeling of peripheral blood leukocytes resulted in staining of monocytes with CP2 but not with CP1. In addition to chymase and a cathepsin-G like proteinase, HMC-CP is another neutral protease that is selectively present in the MCTC tryptase-positive, chymase-positive mast cells type of mast cell, thus extending the biochemical definition of human mast cell heterogeneity.

    Title Mast Cell and Eosinophil Mediated Damage in Bullous Pemphigoid.
    Date May 1991
    Journal Immunology Series
    Title Human Mast Cell Carboxypeptidase.
    Date May 1991
    Journal Monographs in Allergy
    Title Human Mast Cell Tryptase: Multiple Cdnas and Genes Reveal a Multigene Serine Protease Family.
    Date June 1990
    Journal Proceedings of the National Academy of Sciences of the United States of America

    Three different cDNAs and a gene encoding human skin mast cell tryptase have been cloned and sequenced in their entirety. The deduced amino acid sequences reveal a 30-amino acid prepropeptide followed by a 245-amino acid catalytic domain. The C-terminal undecapeptide of the human preprosequence is identical in dog tryptase and appears to be part of a prosequence unique among serine proteases. The differences among the three human tryptase catalytic domains include the loss of a consensus N-glycosylation site in one cDNA, which may explain some of the heterogeneity in size and susceptibility to deglycosylation seen in tryptase preparations. All three tryptase cDNAs are distinct from a recently reported cDNA obtained from a human lung mast cell library. A skin tryptase cDNA was used to isolate a human tryptase gene, the exons of which match one of the skin-derived cDNAs. The organization of the approximately 1.8-kilobase-pair tryptase gene is unique and is not closely related to that of any other mast cell or leukocyte serine protease. The 5' regulatory regions of the gene share features with those of other serine proteases, including mast cell chymase, but are unusual in being separated from the protein-coding sequence by an intron. High-stringency hybridization of a human genomic DNA blot with a fragment of the tryptase gene confirms the presence of multiple tryptase genes. These findings provide genetic evidence that human mast cell tryptases are the products of a multigene family.

    Title Mast Cell Carboxypeptidase: Biochemistry and Biology.
    Date December 1989
    Journal Progress in Clinical and Biological Research
    Title A New Sign of Neurofibromatosis on Magnetic Resonance Imaging of Children.
    Date December 1989
    Journal Archives of Neurology

    Ten pediatric patients with clinically proved neurofibromatosis underwent magnetic resonance imaging of the brain. Seven of these patients had lesions of increased signal intensity on T2-weighted images in the globus pallidus, brain stem, or cerebellar white matter. The lesions did not correlate with results of the neurologic examination or with developmental status. These abnormalities most likely represent hamartomas and should be recognized as part of the diagnostic spectrum of neurofibromatosis.

    Title Human Mast Cell Carboxypeptidase. Purification and Characterization.
    Date June 1989
    Journal The Journal of Clinical Investigation

    A carboxypeptidase activity was recently identified in highly purified human lung mast cells and dispersed mast cells from skin. Using affinity chromatography with potato-tuber carboxypeptidase inhibitor as ligand, mast cell carboxypeptidase was purified to homogeneity from whole skin extracts. The purified enzyme yielded a single staining band of approximately 34,500 D on SDS-PAGE. Carboxypeptidase enzyme content estimated by determination of specific activity, was 0.5, 5, and 16 micrograms/10(6) mast cells from neonatal foreskin, adult facial skin, and adult foreskin, respectively. Human mast cell carboxypeptidase resembled bovine carboxypeptidase A with respect to hydrolysis of synthetic dipeptides and angiotensin I, but was distinguished from carboxypeptidase A in its inability to hydrolyze des-Arg9 bradykinin. The amino acid composition of human mast cell carboxypeptidase was similar to the composition of rat mast cell carboxypeptidase. The amino-terminal amino acid sequence of mast cell carboxypeptidase demonstrated 65% positional identity with human pancreatic carboxypeptidase B, but only 19% with human carboxypeptidase A. Thus, human mast cell carboxypeptidase is a novel member of the protein family of zinc-containing carboxypeptidases, in that it is functionally similar but not identical to bovine carboxypeptidase A, but has structural similarity to bovine and human pancreatic carboxypeptidase B.

    Title Detection and Partial Characterization of a Human Mast Cell Carboxypeptidase.
    Date November 1987
    Journal Journal of Immunology (baltimore, Md. : 1950)

    Using a high performance liquid chromatography assay that detects the cleavage of the C-terminal leucine from angiotensin I, we have identified a carboxypeptidase activity in mast cells from human lung and in dispersed mast cell preparations from human skin. The enzyme activity was detected in a preparation of dispersed human mast cells from lung of greater than 99% purity and was released with histamine after stimulation with goat anti-human IgE. In nine preparations of dispersed human mast cells from lung of 10 to 99% purity, net percentage of release of carboxypeptidase correlated with the release of histamine, localizing carboxypeptidase to mast cell secretory granules. The enzyme activity was also detected in preparations of dispersed human mast cells from skin and in extracts of whole skin. The inhibitor profile and m.w. of carboxypeptidase activity from preparations of dispersed mast cells from skin was similar to that from dispersed mast cells from lung. Mast cell carboxypeptidase had a m.w. on gel filtration of 30,000 to 35,000. The enzyme in crude lysates of dispersed mast cell preparations had optimal activity between pH 8.5 and 9.5 and was inhibited by potato inhibitor, which distinguished it from carboxypeptidase in cultured human foreskin keratinocytes and adult fibroblasts, and from other proteolytic mast cell enzymes. The enzyme activity was also inhibited by EDTA, o-phenanthroline, and, to a small extent, by 8-OH quinoline, but not by Captopril, soybean trypsin inhibitor, or pepstatin. These findings demonstrate that human mast cell secretory granules contain carboxypeptidase in addition to tryptase and chymase. It appears that mast cells from skin may have a higher content of carboxypeptidase than do mast cells from lung.

    Title Toxic Epidermal Necrolysis. Unmuddying the Waters.
    Date September 1987
    Journal Archives of Dermatology
    Title Inhibition of Stimulated Prostaglandin Biosynthesis by Retinoic Acid in Smooth Muscle Cells.
    Date February 1984
    Journal Cancer Research

    Bovine aorta smooth muscle cells (SMC) incubated with a tumor-promoting phorbol ester, 12-O-tetradecanoylphorbol-13-acetate (TPA), released increased levels of prostaglandin I2 [measured as its stable hydrolytic product, 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha)], and this response was inhibited by all-trans-retinoic acid (RA) at concentrations as low as 17 nM. Retinol and retinyl acetate, at concentrations as high as 1.7 and 1.5 microM, respectively, did not inhibit the TPA-stimulated 6-keto-PGF1 alpha production. RA was not cytotoxic at 1.7 microM, as assayed by exclusion of trypan blue dye. Inhibition by RA was increased after preincubation of the SMC with RA prior to TPA stimulation. The inhibition of arachidonic acid (AA) metabolism by RA was not specific for TPA stimulation; RA inhibited prostaglandin production after SMCs were stimulated by serotonin; melittin; the Ca2+ ionophore, A23187; and fetal calf serum. RA had no effect on phorbol ester binding to SMC, nor did it inhibit increased 6-keto-PGF1 alpha production in SMC treated with exogenous AA. While RA inhibited TPA-stimulated production of 14C-labeled 6-keto-PGF1 alpha from [14C]AA-labeled cells, it did not inhibit the accumulation of [14C]AA in the culture medium. The data suggest that RA inhibits stimulated, rather than basal, levels of prostaglandin production. RA does not seem to act by inhibiting the deacylation of AA from cellular phospholipid pools, insofar as this is reflected in the accumulation of AA in the media, but may inhibit reactions at, or after, the generation of endoperoxides by cyclooxygenase.

    Title Inhibition of Plasminogen Activator Associated with Chronic Granulomatous Inflammation.
    Date March 1980
    Journal Thrombosis Research
    Title Fibrin Deposition and Clearance in Chronic Granulomatous Inflammation: Correlation with T-cell Function and Proteinase Inhibitor Activity in Tissue.
    Date February 1980
    Journal The Journal of Investigative Dermatology

    Patterns of fibrin deposition were investigated by immunofluorescence microscopy in livers of thymus intact (TI) and athymic (AT) mice infected with Schistosoma mansoni. Thrombin and fibrinolysis inhibitor activity in tissue extracts also were measured. In TI mice fibrin was detected perivascularly by 6 weeks after infection and at 8 weeks it was found over the granulomas as they developed. Fibrin was cleared from the center of granulomas by 10 weeks. Thrombin inhibitor activity increased at 4 to 6 weeks but declined below control levels later as granulomas formed. Fibronolysis inhibitor activity, on the other hand, peaked at 9 to 12 weeks after infection. In AT mice extensive fibrin deposition was detected in the liver throughout the period when smaller and incomplete granulomas developed. Central clearing did not occur. Thrombin inhibitor activity greatly increased by 8 weeks after infection but fibrinolysis inhibitor activity remained unchanged. These findings suggest that fibrin deposition and firbinolysis are orderly events regulated in the lesions by proteinases and their inhibitors and this seems to be a general tissue reaction in the early stage of chronic granuloma formation. Since local clearance of fibrin in vivo and fibrinolysis inhibitor activity from tissue extracts studied in vitro are more evident in TI mice than in AT mice, it appears that T cell fu;ction is important in modulating the tissue response during granulomatous inflammation.

    Title Effects of Conventional Hypothermic Ischemic Arrest and Pharmacological Arrest on Myocardial Supply/demand Balance During Aortic Cross-clamping.
    Date July 1977
    Journal The Annals of Thoracic Surgery

    Aortic cross-clamping may produce ischemic damage due to a discrepancy between supply and demand. Supply is determined by noncoronary collateral flow and substrate stores, and demand by electromechanical activity, wall tension, and temperature. The effects of 60 minutes of conventional hypothermic ischemic arrest were compared to those of pharmacological arrest. Noncoronary collateral blood supply was comparable in both groups during cross-clamping. With ischemic arest, mechanical activity and endocardial electrical activity persisted and wall tension fell progressively. With pharmacological arrest, electromechanical activity stopped in less than 1 minute but returned (with increased wall tension) nearly 1 hour. Thirty minutes following reperfusion, coronary flow was redistributed away from subendocardium after ischemic arrest and toward endocardium after pharmacological arrest. Myocardial performance was depressed severely after conventional arrest and only mildy after pharmacological arrest. We conclude that aortic cross-clamping is safer with pharmacological arrest than with ischemic arrest. The cardioplegic solution modifies the supply/demand balance favorably, but it is washed out by noncoronary collateral blood supply.

    Title Studies of the Effects of Hypothermia on Regional Myocardial Blood Flow and Metabolism During Cardiopulmonary Bypass. V. Profound Topical Hypothermia During Ischemia in Arrested Hearts.
    Date March 1977
    Journal The Journal of Thoracic and Cardiovascular Surgery

    This study compares the effects of 60 minutes of ischemic arrest with profound topical hypothermia (10 dogs) on myocardial (1) blood flow and distribution (microspheres), (2) metabolism (oxygen and lactate), (3) water content (wet to dry weights), (4) compliance (intraventricular balloon), and (5) performance (isovolumetric function curves) with 180 minutes of cardiopulmonary bypass with the heart in the beating empty state (seven dogs). Studies performed before and 30 minutes after 1 hour of ischemic arrest with profound topical hypothermia showed: (1) total left ventricular blood flow increased 50 per cent but became redistributed away from the subendocardium (endocardial/epicardial flow ratio fell from 1.13 to 0.77,(2) left ventricular oxygen consumption fell 30 per cent while left ventricular oxygen extraction fell from 51 to 29 per cent; (3) lactate extraction fell from 15 to 4 per cent (two dogs produced lactate); (4) left ventricular endocardial (papillary muscle) water content rose 2.4 per cent; (5) left ventricular compliance decreased from 1.68 to 1.01 ml. H2O/mm. Hg (at 25 ml.); (6) left ventricular performance was depressed 49 per cent below control values. In contrast, 3 hours of cardiopulmonary bypass in the beating empty heart produced only minimal changes in these variables.

    Title Studies of the Effects of Hypothermia on Regional Myocardial Blood Flow and Metabolism During Cardiopulmonary Bypass. I. The Adequately Perfused Beating, Fibrillating, and Arrested Heart.
    Date February 1977
    Journal The Journal of Thoracic and Cardiovascular Surgery

    The effects of hypothermia (32 degrees, 28 degrees, and 22 degrees C.) on left ventricular flow distribution (microspheres) and oxygen uptake in adequately perfused, beating, empty, fibrillating, and arrested hearts were studied. Minute left ventricular oxygen uptake fell progressively as myocardial temperature was reduced under all conditions. In beating hearts, however, left ventricular oxygen uptake per beat increased significantly due to the inotropic effect of hypothermia and diastolic compliance fell. Cold fibrillating hearts consumed slightly less oxygen per minute than beating hearts at comparable temperatures as fibrillation became less forceful with hypothermia. Myocardial wall tension, however, was always higher in fibrillating than beating hearts at each level of hypothermia. The lowest myocardial oxygen requirements were always found in arrested hearts (70 to 80 per cent less than either beating empty or fibrillating hearts) at any myocardial temperature. Left ventricular coronary flow remained distributed evenly across the beating heart at all myocardial temperatures and in fibrillating hearts at 28 degrees, and 22 degrees C. Left ventricular flow became redistributed toward the subendocardium in fibrillating hearts at 37 degreegs and 32 degrees C. and in arrested hearts at all myocardial temperatures.

    Title Improved Myocardial Performance After Aortic Cross Clamping by Combining Pharmacologic Arrest with Topical Hypothermia.
    Date January 1977
    Journal Circulation

    In 22 dogs, we compared the protective effects of combining topical hypothermia and cardioplegia to those of either technique alone on myocardial flow, distribution, metabolism, compliance, and performance after 1 hour of aortic cross clamping. Topical hypothermia alone caused redistribution of coronary flow away from the subendocardium (endocardial/epicardial flow fell from 1.13 to 0.77) and reduced left ventricular compliance and performance severely (48% depression of ventricular function). Cardioplegia alone caused redistribution of flow toward the subendocardium (endocardial/epicardial flow increased to 1.4) and decreased compliance and function moderately (31% depression). In contrast, combined topical hypothermia and cardioplegia caused no significant change in left ventricular flow distribution, compliance, or performance. We concluded that the normal heart can be cross clamped safely for up to 60 minutes (no myocardial depression) only when cardioplegia and topical hypothermia are used in combination (P less than 0.01).

    Title Importance of Alkalosis in Maintenance of "ideal" Blood Ph During Hypothermia.
    Date April 1976
    Journal Surgical Forum
    Title Effects of Profound Topical Cardiac Hypothermia on Myocardial Blood Flow, Metabolism, Compliance, and Function.
    Date April 1976
    Journal Surgical Forum
    Title Effect of Mannitol on Coronary Flow in Ventricles Made Ischemic by Fibrillation or Arrest During Bypass.
    Date April 1976
    Journal Surgical Forum
    Title Cardiac Arrest After Aortic Cross-clamping: Effects of Conventional Vs Pharmacologic Arrest on Myocardial Supply/demand Balance.
    Date April 1976
    Journal Surgical Forum
    Title Ringer's Lactate Infusion with Severe Hepatic Damage: Effect on Arterial Lactate Level.
    Date October 1972
    Journal Canadian Journal of Surgery. Journal Canadien De Chirurgie
    Title Pediatric Thyroid-associated Orbitopathy: the Children's Hospital of Philadelphia Experience and Literature Review.
    Journal Thyroid : Official Journal of the American Thyroid Association

    BACKGROUND: As Graves' disease is uncommon in children, Graves' eye disease should be even more unusual. Here we report our experience with Graves' eye disease at the Children's Hospital of Philadelphia and review the literature on ophthalmic findings in children with Graves' disease. SUMMARY: A retrospective review identified 152 children with Graves' disease seen in the endocrinology clinic of the Children's Hospital of Philadelphia over a 3-year period. Of this cohort, only 26 (17%) were referred to ophthalmology because of prominent ophthalmic manifestations. The ages of the patients ranged from 4 months to 17 years. Sixteen of 26 patients were female. Most patients had mild findings consistent with Graves' disease. Proptosis was noted in 10 of 26 (38%). Lid retraction was present in 6 of 26 (23%). Mild corneal punctuate staining was identified in only 3 of the 26 patients (12%). No patients had strabismus or optic neuropathy. Three newly diagnosed Graves' patients who were seen as the retrospective review was being completed were all girls. All three had normal vision, motility, and fundus exams. Two had mild proptosis, lid retraction, and lid lag on down gaze. None had corneal, motility, or optic nerve pathology. These findings are consistent with previous studies in the literature. CONCLUSIONS: Eye findings in pediatric Graves' disease are usually mild and typically respond to local measures and control of disturbed thyroid function. Surgery is indicated in a small number of patients for cornea exposure or appearance issues. Graves' disease-associated optic neuropathy has never been reported in the pediatric population.

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