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Biomedical subjects

C Vogler

Publications and source records attributed to C Vogler.

At least 55 records · Page 3Linked to original sources
PubMed1988

Aluminum-containing emboli in infants treated with extracorporeal membrane oxygenation.

We found fibrin thrombi or thromboemboli at autopsy in 22 of 23 infants with respiratory failure who had been treated with venoarterial extracorporeal membrane oxygenation (ECMO). In addition, distinctive basophilic aluminum-containing emboli were found in 12 of the infants; the distribution of these emboli was similar to that of the thromboemboli, except that an aluminum-containing embolus was found in a lung in only 1 infant. Sixteen infants had pulmonary thrombi or thromboemboli. We also found friable aluminum-containing concretions adhering loosely to the mixing rods of heat exchangers that had been used to warm the blood flowing through the ECMO circuit; such concretions were not present on unused mixing rods. We propose that these aluminum-containing concretions developed as the silicone coating of the heat exchanger wore away and aluminum metal was exposed to warm, oxygenated blood and that fragments of aluminum-containing concretions formed emboli. This hypothesis is supported by the fact that aluminum-containing emboli were generally not present in the lungs, which are bypassed by ECMO. Although infarcts were found in 16 of the 23 infants, we cannot be certain whether any of the infarcts were caused by the aluminum-containing emboli.

Aluminum↗
PubMed1987

Electron microscopy in the diagnosis of lysosomal storage diseases.

Lysosomal storage diseases (LSD) affect cells of the conjunctiva in the absence of clinical eye findings. Consequently conjunctiva was added to skin, rectal mucosa, and peripheral blood lymphocytes as a biopsy site for electron microscopic examination in suspected LSD. The abnormally stored substrates in LSD are the result of diminished lysosomal enzyme activity. Although all cells lack the deficient lysosomal enzyme, the quantity of stored material varies with the extent of breakdown of substrate in the cell under normal circumstances. The stored material does not accumulate in all cells and may have a different cellular morphology at different sites. Although no one tissue provides universally diagnostic material for electron microscopy, skin and conjunctiva are optimal for most LSD. Rectal mucosa and peripheral blood lymphocytes are also useful in many cases. Biochemical assays have replaced brain biopsy for diagnosis but these techniques may give equivocal or non-diagnostic results, and in some diseases the enzyme defect remains undefined. Of the 359 biopsies of conjunctiva, skin, rectal mucosa, and lymphocytes we evaluated for LSD and other neurodegenerative diseases, 65 showed abnormal lysosomal storage; in 41 a specific diagnosis was made by biochemical assay or morphology. Ultrastructural examination of tissue from patients with clinically suspected storage disease may disclose pathognomonic alterations or suggest a differential diagnosis even in the absence of clinically evident involvement of the biopsied tissue. Biopsy has particular value in those diseases with incompletely characterized biochemical abnormalities.

Female↗
PubMed1987

Glomerular basement membrane and lamina densa in infants and children: an ultrastructural evaluation.

The glomerular basement membrane and lamina densa are complex structures that change in composition and thickness with age. We studied their ultrastructural morphology in 37 infants and children, from 1 day to 11 years of age, who died without clinical or morphological evidence of renal disease. The glomerular basement membrane and its lamina densa rapidly increase in width during the first 2 years of life, followed by continued, albeit slower, growth during later childhood.

Aging↗
PubMed1986

Paucifascicular congenital sensory neuropathy in identical twins.

A male infant had sensory and autonomic dysfunction, and his identical twin had a similar clinical finding. One twin was extensively studied, utilizing sural nerve, skin, and conjunctival biopsy specimens, to evaluate the status of peripheral sensory axons. The results support an antenatal neurodevelopmental disturbance in axonal growth that affects sensory neurons and limits their distal extension. Neuropathologic studies of this patient closely resemble findings in hereditary sensory neuropathy type II; clinically, however, this patient resembles patients with congenital autonomic dysfunction and universal pain loss. Investigation of proximal and distal sural nerve, skin, and/or conjunctival biopsy specimens is recommended in patients with sensory and autonomic dysfunction to help differentiate these patients to assist in genetic counselling, treatment, and prognosis. It is possible that clinical overlap in such patients may result from a common neuropathic process, but with varying degrees of involvement.

Conjunctiva↗
PubMed1986

Glomerular membranopathy in adolescents with insulin-dependent diabetes.

Kidney biopsy specimens from 15 patients ranging in age from 11 to 19 years with two- to 14-year histories of insulin-dependent diabetes mellitus were evaluated electron microscopically. Although the mean duration of disease was only eight years, the glomerular basement membrane (GBM) in these patients showed a variety of alterations typical of insulin-dependent diabetes mellitus. Saccular glomerular microaneurysms, previously little recognized, were seen in six of the specimens. These lesions, always associated with breaks in the paramesangial BM, were morphologically distinct from the ectatic capillary loops and glomerular capillary aneurysms described previously in diabetic glomerulopathy. All of the patients with such aneurysms also had other severe GBM alterations. Lytic or mechanical injury to the structurally and biochemically altered diabetic GBM may be responsible for the formation of microaneurysms.

Adolescent↗
PubMed1986

Cytomegalovirus infection and fetal death in a twin. A case report.

In a diamnionic-dichorionic male twin pregnancy, one twin was stillborn, with disseminated cytomegalovirus (CMV) identified morphologically, and the other was liveborn, without clinical or laboratory evidence of CMV infection. The placenta of the affected twin had chronic villitis; that of the liveborn was normal. Although the discordance went unexplained, it illustrates the clinical variability of congenital CMV infection. Dissimilar immune responses between the affected and unaffected twin or direct extension of uterine CMV infection to only one twin may have been responsible for the discordance.

Adult↗
PubMed1986

Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients.

The clinical and pathologic features of 14 children with rhabdoid tumors are presented. Eight patients had primary renal neoplasms and six had extrarenal tumors. The eight renal rhabdoid tumors were identified among 514 primary renal neoplasms collected at four pediatric institutions. Six patients were under 1 year of age; five children died of tumor-related causes, four of them within 4 months of diagnosis and one 17 months postnephrectomy. Another patient died of sepsis 12 days postnephrectomy. One is alive 13 months postnephrectomy, and one was lost to follow-up evaluation. The most common sites of metastasis were the lymph nodes (seven children) and the lungs (three patients). Three infants with renal rhabdoid tumors had, in addition, intracranial masses, two of which manifested clinically before the detection of the renal tumors, in one confirmed to be a primitive neuroectodermal tumor. Five of the 6 extrarenal tumors were identified among 155,926 surgical pathology specimens examined in the same children's hospitals over the same period of time; the remaining extrarenal rhabdoid tumor was received in consultation from a community hospital. The extrarenal rhabdoid tumors occurred in the dorsum of the right foot, liver, soft tissue of the right chest wall, left temporal lobe, left leg, and left thoracic paraspinal region. The ages ranged from 6 weeks to 15 years and two months. Three patients died of tumor-related causes within 4 months of diagnosis; one was a term stillborn. Two are alive, 1 month and 70 months postdiagnosis. Common sites for metastases included the lungs (three patients), and liver and lymph nodes (two children each). Patients with renal and extrarenal rhabdoid tumors are of similar age, have a similar clinical course, with early metastases and poor response to therapy. Primitive neuroectodermal intracranial tumors have been identified in several reported patients with renal rhabdoid tumors; similar brain tumors have not been documented in patients with extrarenal rhabdoid tumors. The histogenesis of this tumor remains unknown.

Brain Neoplasms↗
PubMed1986

Intramuscular hematopoiesis in hypotonic infants with type 1 muscle fiber dysmaturation.

Three term infants had severe hypotonia, a maturation defect of type 1 muscle fibers, and extramedullary hematopoiesis (EMH) in quadriceps muscle specimens obtained within one month of birth. Although predominantly myelopoietic, signs of inflammatory myopathy were absent. One patient had congenital myopathy with maturation arrest of type 1 fibers, another had transient maturation delay of type 1 fibers, and the third patient was subsequently classified as having spinomuscular atrophy. Extramedullary hematopoiesis was demonstrated in normal muscle obtained from young fetuses, but not in muscle obtained at autopsy from infants representing the third trimester and first postnatal month, or in muscle biopsy specimens from 15 other hypotonic infants with type 1 fiber size disproportion. We conclude that EMH in muscle of hypotonic infants is an abnormal persistence of a fetal state that is associated with delayed muscle maturation with diverse origins. Extramedullary hematopoiesis in muscle may indicate hypoxia, but signs of perinatal asphyxia in these babies were inconclusive. Low blood flow due to inactivity or an unidentified product of immature muscle may promote intramuscular EMH, but there is no evidence to suggest that myelopoiesis is injurious to muscle fibers. Intramuscular EMH should be distinguished from inflammation.

Fetal Organ Maturity↗
PubMed1986

Muscle maturation delay in infantile myotonic dystrophy.

Specimens of the quadriceps femoris muscle from four infants with neonatal myotonic dystrophy had features of muscle fiber immaturity. Delayed establishment of major fiber subtypes and persistent myotubes, seen in the youngest infant, resolved in a repeated specimen obtained at the age of 4 months. Prominence of satellite cells, central nuclei, acid phosphatase activity sites, and Golgi zones diminished with age at biopsy. All four infants had type 1 fiber-size disproportion. These results substantiate the concept of delayed muscle fiber maturation in myotonic dystrophy.

Biopsy↗
PubMed1985

Solitary osteochondroma occurring on the transverse process of the lumbar spine. A case report.

We have presented a case of an osteochondroma involving the transverse process of L3 in a 17-year-old man with no evidence of other osteochondromatous lesions. The growth of the lesion was extremely rapid but demonstrated benign histology. This patient was asymptomatic and had a normal physical examination despite the size of the lesion. The benign scoliosis in our patient appears to have been unrelated to and unaffected by the osteochondroma. Whether the osteochondroma would have reached sufficient size to cause significant visceral or neurologic compression, such as in the case presented by Gokay and Bucy, if he had not been treated for scoliosis is a matter of conjecture.

Adolescent↗
PubMed1985

Morphology of skeletal muscle in children. An assessment of normal growth and differentiation.

Muscle fiber size and relative proportion and size of type 1 and 2 fibers were studied in postmortem samples of diaphragm, rectus abdominis, quadriceps, and deltoid of 28 infants and children. The rate of fiber growth varied with age, muscle, and fiber type. Type 1 fibers were smaller than type 2 fibers in preterm and term neonates and histologic identification of pathologic type 1 fiber size disproportion may thus not be possible in the young infant. Isolated groups of type 1 fibers unaccompanied by other evidence of neuropathy may be seen in normal children and may reflect normal variation in motor unit innervation patterns. Definition of normal muscle growth provides a standard for evaluating diagnostic material and allows insight into normal neuromuscular maturation.

Child↗
PubMed1981

Laryngeal fibromatosis in the neonate.

Infants with a fibromatosis that originates in the laryngeal mucosa have uniform clinical appearances and morphologic features that identify a clinicopathologic syndrome. Based on data obtained from the two cases in the present study and from previously reported cases, the lesion may appear at birth or appear later in childhood, with symptoms referable to the degree of laryngeal obstruction. Fibroblasts and myofibroblasts make up the cytologic composition of these lesions. These lesions can be identified among the fibromatoses that occur in infants and children. Fibromatosis of the larynx in infants and children may have the same behavior as other fibromatoses, eg, local invasion and potential recurrence; this behavior should be considered in the clinical management.

Diagnosis, Differential↗
PubMed1981

Primary ovarian nongestational choriocarcinoma. Report of a case in a young woman of childbearing age.

Nongestational, pure ovarian choriocarcinoma is decidedly rare; the unequivocal diagnosis is rarely established in women of childbearing age. Differentiation of nongestational from gestational trophoblastic neoplasia is crucial, the prognosis and therapy being distinct in each type. We report a case of primary ovarian, nongestational, pure choriocarcinoma in a 27-year-old woman treated with surgery and aggressive chemotherapy. The distinction of nongestational from gestational choriocarcinoma and its variants, including latent choriocarcinoma, is discussed.

Adult↗
PubMed1976

[Incidence of stress ulcer and secretion of gastric juice following truncal and selective vagotomy].

In rats development of stress ulcers by immobilization is accompanied by elevated pH, reduced amount of gastric juice, and by this with decreased amount of acid. Consequently, the acid factor has no primary importance for the development of stress ulcer. Truncal vagotomy, selective proximal vagotomy, and selective antral vagotomy result in reducing the acid secretion at elevated pH value and increased juice amount, and in a decreased ulcer rate. Greatest certainty to prevent stress ulcer gives the truncal vagotomy, whereas there is a poor effect after antral vagotomy.

Animals↗