Biomedical subjects
E C Klatt
Publications and source records attributed to E C Klatt.
Calciphylaxis mimicking dermatomyositis: ischemic myopathy complicating renal failure.
BACKGROUND: Among the complications of chronic renal failure is a syndrome of medial calcification of small- to medium-sized arteries associated with ischemic necrosis of the skin and other organ systems, leading to gangrene and a poor prognosis. The syndrome has been reviewed in the renal, dermatologic, and surgical literature under the term calciphylaxis, which describes a postulated pathogenetic mechanism whereby sensitization to an endogenous or exogenous substance (such as parathyroid hormone) predisposes to calcium deposition after exposure to a challenging agent. Myopathy has rarely been reported as the presenting feature, and the syndrome has not been discussed in the neurologic literature. METHODS: We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myopathic complaints and signs suggesting dermatomyositis. We also discuss possible disease mechanisms and treatment. CONCLUSIONS: Because early treatment (including aggressively lowering the calcium and phosphate levels and parathyroidectomy) may improve the outcome, early recognition of the syndrome of calciphylaxis is essential.
HIV infection, diagnosis, and treatment: new findings and approaches.
We now understand the biology of HIV infection, and this knowledge can be applied to strategies for prevention of infection, improvement of diagnostic laboratory testing, and treatment of infection. Education about methods for preventing HIV transmission remains the primary goal for reducing the spread of AIDS. For persons already infected, laboratory testing with HIV-1 RNA levels and CD4 lymphocyte counts are useful techniques for monitoring HIV infection and prolonging survival.
Web-based pathology education.
OBJECTIVE: To develop and present computer-aided instructional resources via the Internet, intranets, and desktop computers to augment pathology education. DESIGN: World Wide Web sites available via Internet access and a CD-ROM for intranets. SETTING: The University of Utah School of Medicine, Salt Lake City. RESULTS: A comprehensive set of gross and microscopic images with text, along with interactive examination questions, has been developed and placed on World Wide Web servers. A CD-ROM has been developed for use with intranets and individual desktop computers. CONCLUSIONS: Web-based delivery of computer-aided instruction is an efficient means of enhancing courses for students studying pathology. Both Internet and CD-ROM delivery of educational resources provide flexible access to study materials. Teaching faculty can easily generate and edit web-based materials for efficient resource management. Student performance has been enhanced.
Web-based teaching in pathology.
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Evaluation of medical education.
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Postmortem fetal MR imaging: comparison with findings at autopsy.
OBJECTIVE: The purpose of this study was to prospectively compare findings from postmortem fetal MR imaging with findings at autopsy. SUBJECTS AND METHODS: Twenty-six fetuses were imaged on a 1.5-T MR scanner using two-dimensional and high-resolution three-dimensional fast spin-echo techniques immediately before autopsy. The MR images were reviewed independently by three radiologists who evaluated then for major and minor malformations. These findings were then compared with those at autopsy. RESULTS: The 26 subjects had 47 major and 11 minor malformations. All three radiologists correctly identified 37 of the major malformations on the MR images (detection rate, 79%), and at least one of the three reviewers correctly identified 43 of the abnormalities (detection rate, 91%). Only one of the 11 minor anomalies was identified by any reviewer. Reviewers made six false-positive diagnoses. In two cases, both with major CNS malformations, MR imaging was superior to autopsy in defining in situ relationships. CONCLUSION: Although autopsy remains the study of choice for evaluating causes of fetal death, MR imaging is an excellent alternative when autopsy is refused. Additionally, MR imaging may be a valuable adjunct to autopsy for fetuses with CNS anomalies.
Open your laboratory to the Internet.
The Internet can open many opportunities for laboratories. A wide variety of information resources are available via the Web, which can be researched more quickly and efficiently with a search engine. A laboratory can pursue marketing and advertising of its services on the Web as well. Consultation and collaboration can be accomplished via e-mail, listservs, and newsgroups. An Intranet can be created on a local area network to share electronic data within an organization. In addition, the rapid technological advances make the possibilities for tomorrow's communications limitless.
Correspondence re: Al-Khafaji B, Kralovic S, Smith RD. Increased hepatic iron in the acquired immunodeficiency syndrome. Mod Pathol 1997; 10:474-80.
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Aprosencephaly and cerebellar dysgenesis in sibs.
Aprosencephaly is a rare, lethal malformation sequence of the central nervous system that has been attributed to a postneuralation encephaloclastic process. We describe autopsy findings consistent with aprosencephaly in 2 fetuses conceived from a consanguineous mating (first cousins). Both showed anencephalic manifestations; however, the crania were intact, with fused sutures. The neuropathologic findings were essentially identical. Each fetus had complete absence of the telecephalon and pyramidal tracts, rudimentary diencephalic and mesencephalic structures, primitive cerebellar hemispheres, posterolateral clusters of primitive neural cells in the medullas suggesting an abnormality of neural migration, a normally-formed spinal cord, and retinal dysplasia within normally-formed globes. In addition, both fetuses manifested a peculiar perivascular mesenchymal proliferation seen only within the central nervous system. The similarity of these cases, coupled with parental consanguinity, suggests a primary malformation in brain development due to the homozygous representation of a mutant allele. We hypothesize that these patients may represent a defect in a gene important in brain development, the nature of which has yet to be elucidated.
Pathologic examination of fetal specimens from dilation and evacuation procedures.
The systematic examination of fetal specimens obtained from dilation and evacuation (D & E) procedures with termination of pregnancy for fetal malformations and chromosomal abnormalities is described. Although a D & E procedure does not yield an intact fetus, pathologic examination of fetal parts by use of radiography, gross dissection, microscopic examination, and/or cell culture for karyotyping or biochemical analysis can aid in confirming most prenatal clinical diagnoses or provide a specific diagnosis. In this study, a major abnormality could be detected in 92% of 37 D & E cases examined, and in 46% a specific diagnosis was obtained only from pathologic examination. This information is useful for genetic counselling and future family planning.
Safety in bullet recovery procedures: a study of the Black Talon bullet.
Bullets or bullet fragments that are recovered during forensic autopsy and examined later as evidence may present hazards to both pathologists and forensic scientists due to sharp edges or jagged projections created by the deformation or fragmentation of a bullet upon impact with its target. The recent introduction of the Black Talon bullet has increased this hazard by its design, which produces a controlled expansion upon target impact that raises six harp edges from its copper jacket. We describe the appearance of the bullet along with methods for detection and recovery that can be applied as universal precautions of safe bullet handling.
Accessory brains (extracerebral heterotopias): unusual prenatal intracranial mass lesions.
Prenatal ultrasonographic evidence of intracranial mass lesions generally results in a diagnosis of primary glial or primitive neuroectodermal neoplasm. We describe two infants, one who was stillborn at 25 weeks' estimated gestational age and one term infant who was born live and died shortly after birth with large intracranial space-occupying lesions that exerted significant mass effect. At autopsy, large soft-tissue spheres of partially organized brain tissue containing neurons, astrocytes, oligodendroglia, ependyma, and choroid plexus were found adjacent to intact, fully formed cerebral hemispheres with normal brain stems and cerebelli within the cranial cavity. We have termed these extracerebral heterotopias "accessory brains." The telencephalic vesicles arise as lateral outpouchings at the rostral end of the developing embryo during the 5th week of embryogenesis. These accessory brains may arise embryologically from an accessory third evagination inferior to the telencephalic vesicles.
Atelencephalic aprosencephaly.
Absence of the telencephalon and diencephalon characterizes the syndrome of aprosencephaly, while in atelencephaly, only the telencephalon is absent. Atelencephalic aprosencephaly is characterized by the presence of at least a rudimentary diencephalon. Embryologically, aprosencephaly is thought to occur after the optic vesicles form but before the cerebral vesicles appear. The syndrome is quite rare, with only 10 cases previously reported. We describe two fetuses with atelencephalic aprosencephaly. A 25-week estimated gestational age fetus was born to first-cousin parents and had a prenatal ultrasonographic diagnosis of anencephaly. The second, a 19-week estimated gestational age fetus, was thought to have semilobar holoprosencephaly by prenatal ultrasound. At autopsy, neuropathologic examination in both cases showed virtual absence of the cerebral hemispheres with an incomplete diencephalon. Microscopic examination in one case revealed disorganized neuropil with a proliferative vasculopathy. The optic globes were completely formed and attached to hypoplastic optic nerves, but retinal dysplasia was apparent histologically in both cases, and bilateral colobomata were present in one case. The findings in these cases demonstrate a spectrum of congenital variations that lie between the syndromes of atelencephaly and aprosencephaly, underscoring the complexity of the congenital anomalies.
Evolving trends revealed by autopsies of patients with the acquired immunodeficiency syndrome. 565 autopsies in adults with the acquired immunodeficiency syndrome, Los Angeles, Calif, 1982-1993 [corrected].
OBJECTIVE: To determine changes in causes of death, survival, and organ system distribution of major opportunistic infections and neoplasms in adults dying with the acquired immunodeficiency syndrome (AIDS) following the widespread use of antiretroviral therapy and prophylaxis for opportunistic infections since 1988. DESIGN: A retrospective review of autopsy records with gross and microscopic pathologic findings, laboratory data, and clinical histories in cases of AIDS, comparing findings from 1982 through 1988 with those from 1989 through May 1993. SETTING: All autopsies were performed on persons dying in the metropolitan Los Angeles, Calif, area from January 1982 through May 1993. RESULTS: In 565 adult cases of AIDS at autopsy, Pneumocystis carinii pneumonia (PCP) remained the most common cause of death, but both the frequency of and number of deaths of PCP declined over time. Deaths from bacterial sepsis, cytomegalovirus infection, Mycobacterium avium complex infection, and toxoplasmosis also declined during this period, but mortality from fungal infections, tuberculosis, encephalopathy, and causes unrelated to AIDS increased. The death rate from malignant lymphoma remained high. Kaposi's sarcoma (KS) continued to occur more frequently in patients whose risk factor for human immunodeficiency virus infection (HIV) was homosexuality or bisexuality, but the death rate from KS was greatest for patients with a risk factor of blood exposure to HIV. Survival was shorter and deaths from tuberculosis more common in patients with a history of intravenous drug use. Overall survival of patients in other AIDS risk groups increased over time, particularly in those treated with antiretroviral therapy. The organ system distribution of major opportunistic infections and neoplasms was similar throughout the years of the study. The lung was the most frequent organ involved by AIDS-associated diseases leading to death, followed by the gastrointestinal tract and the central nervous system. CONCLUSIONS: The causes of death in AIDS have evolved since 1988 following the widespread use of prophylactic and antiretroviral therapies in patients with HIV infection. This has occurred primarily from changes in overall frequency and death rates from infections. Organ system involvement by AIDS-associated diseases has not changed significantly over time.
Primary lymphoma of the kidney.
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Opportunistic intraocular infections in AIDS.
In conclusion, this clinicopathologic study has shown that CMV ocular infection is present in about 16% of terminal AIDS patients. The treatment of CMV retinitis reduces the number of CMV-infected nonocular organs and may also lessen the severity and control the spread of concurrent nonocular infection, both of which may prolong survival in AIDS patients. Other opportunistic infections, involving primarily the choroid, were also seen in a number of patients, some of whom had concurrent intraocular infections with CMV and P carinii, M avium-intracellulare, C neoformans. In addition, all of these choroidal infections were components of disseminated infection, underscoring the increasingly important role of the ophthalmologist in the diagnosis and treatment of disseminated opportunistic infections in AIDS.
Pseudallescheria boydii infection in the acquired immunodeficiency syndrome.
Pulmonary and renal infection developed in a 44-year-old Hispanic man due to the fungus Pseudallescheria boydii. Pseudallescheria boydii has been associated with cutaneous infection known as mycetoma but occurs very infrequently in extracutaneous sites. To our knowledge, this is the second reported case of P boydii in a patient with the acquired immunodeficiency syndrome and the first case with pulmonary and/or renal involvement.