Biomedical subjects

H Steiner

Publications and source records attributed to H Steiner.

At least 109 records · Page 6Linked to original sources

PubMed1997

Reports of stress: a comparison between eating disordered and non-eating disordered adolescents.

This study utilizes a unique method to examine reports of stressful life events provided by eating disordered and non-eating disordered adolescents. Subjects (all females) participated in a standardized procedure to obtain reports of stressful life events. The Life Events and Coping Inventory (LECI) was used to categorize reported stressors. Eating disorder subjects discussed more stressors than non-eating disorder subjects only when eating disorder events were included. Furthermore, eating disorder subjects reported more events that could not be classified within the LECI and were rated as non-normative. The findings point to the importance of qualitative rather than purely quantitative approaches to stressful life events assessment with adolescents.

Adaptation, Psychological↗

PubMed1997

Sclerotic lipoma: lipomas simulating sclerotic fibroma.

AIMS: We report a series of five unusual subcutaneous lipomas characterized by prominent stromal sclerosis in a storiform arrangement. METHODS AND RESULTS: All lesions occurred in young male adults (23.0 +/- 9.5 years), three lesions were on the scalp and two on the hands. Clinically, lipoma, chondroma or some form of cyst were suspected. In contrast to sclerotic fibroma ('circumscribed storiform collagenoma') a variable number of adipocytes were interspersed within the fibrosclerotic background. These cells also showed immunoreactivity for S100 protein as well as intracytoplasmic lipid deposits ultrastructurally. None of the lesions recurred within a follow-up period of between 0.5 and 9 years. CONCLUSIONS: Our series suggests that sclerotic lipomas should be classified with fibro- and myxolipomas, angiolipomas, spindle cell and pleomorphic lipomas, as an additional characteristic subtype of lipoma. Sclerotic lipomas are often misdiagnosed as a sclerotic fibroma, but are essentially fatty tumours and are, at least in our small series, not associated with Cowden's disease.

Adolescent↗

PubMed1997

Granular cell dermatofibroma.

AIMS: To describe a series of five granular cell dermatofibromas as an unusual and rare manifestation of fibrohistiocytic tissue response. METHODS AND RESULTS: Five granular cell dermatofibromas were collected out of 136 tumours filed as granular cell tumours. Clinically, all lesions occurred on the shoulder or back of middle-aged adults (two women, three men), mostly with the clinical diagnosis of a fibrohistiocytic lesion. Histology revealed well-circumscribed, dermal to subcutaneous lesions dominated by periodic acid-Schiff (PAS) positive, granular cells. Acanthosis above, as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lymphohistiocytic infiltrate at the periphery of the lesion, indicated the fibrohistiocytic origin. Lesions showed prominent reactivity with NK1C3 (CD57), as well as for macrophage markers KiM1p and KP1 (CD68). In contrast to classic Schwannian/neurogenic granular cell tumours, granular cell dermatofibromas were S100 protein negative, but showed variable reactivity for factor XIIIa (10-50%) in 4/5, for smooth muscle specific actin (10-50%) in 2/5 and with E9 (10-30%) in 3/5 lesions. Electron microscopy in one case revealed large pools of phago-lysosomes and variably sized glycogen granules in granular cells. CONCLUSION: Our series delineates granular cell dermatofibroma as a distinct clinicopathological variant of fibrohistiocytic tissue response which needs to be distinguished from other tumours with granular cell features.

Adult↗

PubMed1997

Clear cell dermatofibroma.

This series presents six cases of a rare variant of dermatofibroma, characterized by marked clear cell change. All lesions occurred on the lower extremities of middle-aged adults (four women, two men), mostly with the clinical diagnosis of fibrohistiocytic lesion. Histological examination revealed well circumscribed, faintly stained dermal to subcutaneous lesions which were due to the overwhelming presence of clear cells (> 90%), some with prominent PAS-positive cytoplasmic granulation. Overlying epidermal hyperplasia as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lympho-histiocytic infiltrate at the periphery of the lesion indicated the fibrohistiocytic origin. Individual histopathological peculiarities included: bizarre giant cells in two cases, perifollicular arrangement and haemangiopericytoma-like features with iron deposition in one case each. Immunohistochemically three of four lesions showed moderate reactivity for factor XIIIa and two of four with an anti-metallothionen marker E9, but were otherwise negative with a broad panel of markers. Electronmicroscopy in two cases revealed large pools of glycogen beside focal, prominent endoplasmic reticulum and lysosomes in some granular cells, but only optically translucent cells in cases of clear cells. Recognition of clear cell dermatofibroma is important as the differential diagnosis includes some entities with more serious outcome/considerations such as metastases of renal cell carcinoma, xanthogranulomatous reactions, balloon cell naevus/melanoma and clear cell sarcoma.

Adult↗

PubMed1997

Conservative treatment of cervical pregnancy by curettage and local prostaglandin injection.

Recently, various conservative regimens for the treatment of cervical pregnancy have been introduced to preserve fertility in young women, with methotrexate being one of the most widely used drugs. The success of conservative treatment depends on early preoperative diagnosis. We report three cases of first trimester cervical pregnancy, successfully treated by curettage to evacuate the conceptus, followed by local prostaglandin instillation to prevent severe haemorrhage. In one patient, intra-amniotic instillation of hyperosmolar glucose was necessary to terminate fetal cardiac activity. beta-Human chorionic gonadotrophin returned to normal within 3 weeks in one patient and within 7 weeks in another, both of whom became pregnant again within 1 year, resulting in term deliveries. The third patient was lost to follow-up after 1 week. The advantage of prostaglandin and curettage is the absence of major side-effects to the mother or the fetus of a subsequent pregnancy. The management strategies used in the treatment of cervical pregnancies and the results obtained since the introduction of methotrexate in 1989 are discussed in the context of previously published literature. The incidence of subsequent pregnancies among women treated medically versus women treated surgically is reported.

Adult↗

PubMed1997

Verocay body--prominent cutaneous schwannoma.

We report on eight cases of a distinct variant of cutaneous schwannoma characterized by prominent Verocay body formation (75-100% of the tumor bulk) that may cause considerable diagnostic difficulties. Like ordinary cutaneous schwannomas, these lesions preferred the head and neck region of young adults without sexual predilection and were clinically interpreted as cyst, basal cell carcinoma, or nevus. Histological examination revealed well-circumscribed nodules. Three of them consisted exclusively of nodular or ribbon-like Verocay bodies. A variable admixture of Antoni A or B type of differentiation (< 25%) was seen in five other cases. The following patterns were seen: fascicular spindle-shaped, onion-like epithelioid, myxoid-hypocellular, and degenerated ("ancient") with prominent fibrosis/hyalinosis and occasional bizarre giant cells. Immunohistochemically, the lesions were positive for S-100 protein (and vimentin) but negative for a broad panel of neurogenic and intermediate filament markers. The capsule showed focal labeling for EMA and--when it was markedly thickened--also for SMA. Labeling with E9, an anti-metallothionein marker indicative of cell activity, was negative, underscoring the slow growth potential of these lesions. No recurrence was seen in the six patients with follow-up information. The differential diagnosis includes other lesions with prominent palisading. (Amianthoid) myofibroblastoma and palisading leiomyoma are consistently positive for SMA and desmin, respectively. Palisading cutaneous fibrous histiocytoma and myofibroblastic dermatofibroma are variably positive for Factor XIIIa, SMA, and E9 and/or NK1C3 (CD57). Palisaded encapsulated neuromas are primarilly differentiated by the presence of nerve fibers with myelin sheaths.

Adolescent↗

PubMed1997

Dermal and subcutaneous variants of plexiform fibrohistiocytic tumor.

We report five cases of plexiform fibrohistiocytic tumors, three classic subcutaneous lesions and two dermal ones. Both variants had similar profiles and were clinically indistinguishable. The lesions affected the trunk more than the upper extremities and were found in children and young adults (18.4 +/- 12.8 years). They showed a marked female predominance (4:1, including both dermal variants). Clinically, they were skin-colored, hard nodules of 1-2-cm diameter that resulted in such differential diagnoses as fibroma, histiocytoma, pilomatricoma, or cyst. Interestingly, one subcutaneous case with a painful "worms in the sack" presentation was thought to represent a plexiform neurofibroma. Histology revealed well-circumscribed dermal or subcutaneous plexiform lesions with a characteristic biphasic appearance. Most of the tumor bulk consisted of spindle-shaped to stellate myofibroblasts with a variable admixture of collagen or loosening of stroma. In the center of the plexiform strands and nodules, a few osteoclast-like giant cells as well as epithelioid mononuclear cells (< 10%) were found. Myofibroblasts were positive with HHF35 and for smooth muscle actin in three of five cases. Osteoclast-like giant cells were positive with KP1 in all five cases. Both types of cells stained with the macrophage marker Ki-M1p. A broad panel of other markers was negative. This series expands the spectrum of plexiform fibrohistiocytic tumor, but it also broadens the differential diagnosis of (dermal) plexiform lesions, which at present includes spindle cell nevi, schwannomas, neurofibromas, granular cell tumors, nerve sheath myxomas (neurothekeomas), spindle cell lipomas, and tufted angiomas.

Adult↗

PubMed1997

Posttraumatic stress disorder in incarcerated juvenile delinquents.

OBJECTIVE: To assess the prevalence of posttraumatic stress disorder (PTSD) in severely delinquent subjects and to measure associated personality characteristics. METHOD: Eighty-five incarcerated boys (mean age 16.6, SD = 1.2) with mostly violent offenses were studied. The sample was representative of the California Youth Authority population. They received a standard psychiatric screen, a semistructured interview for PTSD, and self-report questionnaires measuring personality traits and defenses. A nonclinical sex- and age-matched group was used for comparing psychometrics. RESULTS: Subjects suffered from PTSD at higher rates than other adolescent community samples and at higher rates than those found in county probation camps. Thirty-two percent fulfilled criteria for PTSD, 20% partial criteria. One half of the subjects described the witnessing of interpersonal violence as the traumatizing event. Psychometric results converged in the predicted way: Subjects with PTSD showed elevated distress, anxiety, depression, and lowered restraint, impulse control, and suppression of aggression; they had high levels of immature defenses such as projection, somatization, conversion, dissociation, and withdrawal. CONCLUSIONS: PTSD occurs at high rates in delinquents, and this finding has implications for management and treatment. Personality characteristics that might put individuals at risk for the development of PTSD were identified.

Adolescent↗

PubMed1997

Summary of the practice parameters for the assessment and treatment of children and adolescents with conduct disorder.

This summary of the practice parameters describes the diagnosis, treatment, and prevention of conduct disorder in children and adolescents. The rationales for these recommendations are based on a review of the scientific literature and clinical consensus, which are contained in the complete document. Clinical features of youths with conduct disorder include predominance in males, low socioeconomic status, and familial aggregation. Important continuities to oppositional defiant disorder and antisocial personality disorder have been documented. Extensive comorbidity, especially with other externalizing disorders, depression, and substance abuse, has been documented and has significance for prognosis. Clinically significant subtypes exist according to age of onset, overt or covert conduct problems, and levels of restraint exhibited under stress. To be effective, treatment must be multimodal, address multiple foci, and continue over extensive periods of time. Early treatment and prevention seem to be more effective than later intervention.

Adolescent↗

PubMed1997

Practice parameters for the assessment and treatment of children and adolescents with conduct disorder. American Academy of Child and Adolescent Psychiatry.

These practice parameters address the diagnosis, treatment, and prevention of conduct disorder in children and adolescents. Voluminous literature addresses the problem from a developmental, epidemiological, and criminological perspective. Properly designed treatment outcome studies of modern psychiatric modalities are rare. Ethnic issues are mentioned but not fully addressed from a clinical perspective. Clinical features of youth with conduct disorder include predominance in males, low socioeconomic status, and familial aggregation. Important continuities to oppositional defiant disorder and antisocial personality disorder have been documented. Extensive comorbidity, especially with other externalizing disorders, depression, and substance abuse, has been documented and has significance for prognosis. Clinically significant subtypes exist according to age of onset, overt or covert conduct problems, and levels of restraint exhibited under stress. To be effective, treatment must be multimodal, address multiple foci, and continue over extensive periods of time. Early treatment and prevention seem to be more effective than later intervention.

Adolescent↗

PubMed1997

Malignant melanomas simulating various types of soft tissue tumors.

BACKGROUND: Primary malignant melanomas, recurrences, and metastases thereof can present with a wide variety of clinicopathologic aspects. OBJECTIVE: The present series describes eight primary malignant melanomas and/or 10 local recurrences/metastases (from eight patients) misdiagnosed as various types of soft tissue tumors: two dermatofibrosarcoma protuberans, two atypical fibroxanthomas, two storiform-pleomorphic malignant fibrous histiocytomas, one myxofibrosarcoma ("myxoid malignant fibrous histiocytoma"), two malignant hemangiopericytomas, and nine malignant schwannomas. METHODS: In three cases correct diagnosis was established by clinicopathologic correlation during follow-up; all the others were only discovered during a retrospective work-up of all soft tissue tumors diagnosed at the Dermatohistopathological Laboratory of the Department of Dermatology, University of Innsbruck. RESULTS: Helpful clues derived from subtle intraepidermal, irregular spread of melanocytes, focal nested arrangement of tumor cells, sparse melanin deposition, neurotropism, and prominent peripheral lymphohistiocytic response, partially forming lymph follicles. Besides clinicopathologic correlation, histology of serial sections as well as immunohistochemistry (S100 protein more important than NK1/C3; HMB45 without benefit) and electron microscopy (melanosomes) proved helpful for definitive diagnosis. In contrast to the general assumption that spindle cell/desmoplastic malignant melanomas have an unequivocal bad prognosis, our series, as well as evidence from the literature, document a better prognosis than that of other types with comparable tumor thickness (70% vs 50% 5-year survival). Moreover, labeling with E9, an antimetallothionein marker, confirmed its usefulness for prognosis being strongly positive in primary lesions followed by rapid progression, but mostly negative in those without. CONCLUSION: These cases document that malignant melanoma may mimic various types of soft tissue tumors; correct interpretation is important as prognosis and therapeutic management differ considerably between these entities.

Aged↗

PubMed1997

Cutaneous epithelioid hemangioendothelioma, epithelioid cell histiocytoma and Spitz nevus. Three separate epithelioid tumors in one patient.

We report the unusual history of a young lady who, over a period of 9 years, developed 3 epithelioid neoplasms of different histiogenetic origin: a cutaneous epithelioid hemangioendothelioma, an epithelioid cell histiocytoma, and a Spitz nevus. To the best of our knowledge, such an unusual combination of neoplasms has not been reported previously and raises the question of an underlying "epithelioid" reaction pattern responsible for the epithelioid appearance in all 3 tumors; the likelihood of such a combination of tumors occurring in a single patient would be less than 1 in 1 billion.

Adult↗

PubMed1996

Dynorphin regulates D1 dopamine receptor-mediated responses in the striatum: relative contributions of pre- and postsynaptic mechanisms in dorsal and ventral striatum demonstrated by altered immediate-early gene induction.

Dynorphin, an endogenous kappa opioid receptor ligand, acts in the striatum to regulate the response of striatonigral neurons to D1 dopamine receptor stimulation. We investigated the relative contributions of both presynaptic kappa receptors on dopamine terminals and postsynaptic kappa receptors on striatal neurons by analyzing opioid regulation of D1 effects in the absence of presynaptic kappa receptors, after 6-hydroxydopamine depletion of striatal dopamine. D1-receptor-mediated immediate-early gene induction was measured by using in situ hybridization histochemistry. First, repeated treatment with the D1-receptor agonist SKF-38393 (2 mg/kg/day, 3-14 days) was used to increase dynorphin levels in rats with dopamine depletions. In the nucleus accumbens, increased dynorphin expression was accompanied by reduced induction of the immediate-early genes c-fos and zif 268 by SKF-38393. In contrast, in dorsal/lateral aspects of the dopamine-depleted striatum, this D1 response was sustained despite a large increase in dynorphin expression. These results are consistent with a requirement of dopamine terminals (presynaptic kappa receptors) for the inhibitory action of dynorphin in the dorsal/lateral striatum, but not in the ventral striatum. Second, the kappa receptor agonist spiradoline (1-10 mg/kg) reduced c-fos and zif 268 induction by SKF-39393 (2.5 mg/kg) preferentially in ventral parts of the dopamine-depleted striatum, which contain higher levels of kappa receptor mRNA and binding. These results also indicate that postsynaptic kappa receptors contribute to the inhibition of the D1 response at least in the ventral striatum. Together, these results indicate that dynorphin in the striatum functions to regulate dopamine input to striatonigral neurons, acting at both pre- and postsynaptic sites, and that the relative contributions of these mechanisms differ between dorsal and ventral striatal regions.

Animals↗

PubMed1996

Heme binding to a conserved Cys-Pro-Val motif is crucial for the catalytic function of mitochondrial heme lyases.

Covalent attachment of heme to the apoforms of mitochondrial cytochromes c and c1 requires the activity of cytochrome c heme lyase (CCHL) and cytochrome c1 heme lyase (CC1HL), respectively. The two enzymes differ in their cytochrome specificity, but they are related in sequence, and both contain conserved Cys-Pro-Val (CPV) motifs. By using various in vitro assays we investigated whether heme can bind directly to heme lyases and whether the CPV motif may be involved in heme binding. Heme stabilized CC1HL, as a model protein, in a folded, protease-resistant conformation, stimulated the refolding of CC1HL after urea denaturation, and inhibited the import of the CC1HL precursor into mitochondria. These effects were not observed with a point mutant, CC1HLSPV, in which cysteine was replaced by serine, and with CC1HLDeltaCPV, in which the motif was deleted. These results show that heme lyases can bind heme directly, and they identify the CPV sequence as a structural element important for this interaction. The phenotype of a yeast mutant expressing CC1HLSPV is in good agreement with such a role of the CPV motif. The mutant cells accumulate the heme-free intermediate form of cytochrome c1 and display a severe deficiency in the holo form. We suggest that the CPV motif forms a crucial part of the substrate binding site for heme.

Amino Acid Sequence↗

PubMed1996

Pain responses, anxiety and aggression in mice deficient in pre-proenkephalin.

Enkephalins are endogenous opioid peptides that are derived from a pre-proenkephalin precursor protein. They are thought to be vital in regulating many physiological functions, including pain perception and analgesia, responses to stress, aggression and dominance. Here we have used a genetic approach to study the role of the mammalian opioid system. We disrupted the pre-proenkephalin gene using homologous recombination in embryonic stem cells to generate enkephalin-deficient mice. Mutant enk-/- animals are healthy, fertile, and care for their offspring, but display significant behavioural abnormalities. Mice with the enk-/- genotype are more anxious and males display increased offensive aggressiveness. Mutant animals show marked differences from controls in supraspinal, but not in spinal, responses to painful stimuli. Unexpectedly, enk-/- mice exhibit normal stress-induced analgesia. Our results show that enkephalins modulate responses to painful stimuli. Thus, genetic factors may contribute significantly to the experience of pain.

Aggression↗

PubMed1996

Mitochondrial and cytosolic branched-chain amino acid transaminases from yeast, homologs of the myc oncogene-regulated Eca39 protein.

We have isolated a high copy suppressor of a temperature-sensitive mutation in ATM1, which codes for an ABC transporter of Saccharomyces cerevisiae mitochondria. The suppressor, termed BAT1, encodes a protein of 393 amino acid residues with an NH2-terminal extension that directs Bat1p to the mitochondrial matrix. A highly homologous protein, Bat2p, of 376 amino acid residues was found in the cytosol. Both Bat proteins show striking similarity to the mammalian protein Eca39, which is one of the few known targets of the myc oncogene. Deletion of a single BAT gene did not impair growth of yeast cells. In contrast, deletion of both genes resulted in an auxotrophy for branched-chain amino acids (Ile, Leu, and Val) and in a severe growth reduction on glucose-containing media, even after supply of these amino acids. Mitochondria and cytosol isolated from bat1 and bat2 deletion mutants, respectively, contained largely reduced activities for the conversion of branched-chain 2-ketoacids to their corresponding amino acids. Thus, the Bat proteins represent the first known isoforms of yeast branched-chain amino acid transaminases. The severe growth defect of the double deletion mutant observed even in the presence of branched-chain amino acids suggests that the Bat proteins, in addition to the supply of these amino acids, perform another important function in the cell.

Amino Acid Sequence↗

PubMed1996

Trichoplusia ni attacin A, a differentially displayed insect gene coding for an antibacterial protein.

The mRNA differential display method was used to isolate antibacterial defense genes from Trichoplusia ni. The mRNA population in last-instar T. ni larvae injected with bacteria was compared to that of untreated larvae. Using a PCR amplified probe corresponding to an induced mRNA, we were able to clone an attacin homolog from a lambda cDNA library from vaccinated larvae. The corresponding protein showed 63% identity to Hyalophora cecropia acidic attacin. The induction kinetics of T. ni attacin A gave optimal mRNA levels at 20 h post-infection. Genomic analysis showed this to be a single-copy gene with two introns.

Amino Acid Sequence↗

PubMed1996

A targeted mutation of the D3 dopamine receptor gene is associated with hyperactivity in mice.

While most effects of dopamine in the brain are mediated by the D1 and D2 receptor subtypes, other members of this G protein-coupled receptor family have potentially important functions. D3 receptors belong to the D2-like subclass of dopamine receptors, activation of which inhibits adenylyl cyclase. Using targeted mutagenesis in mouse embryonic stem cells, we have generated mice lacking functional D3 receptors. A premature chain-termination mutation was introduced in the D3 receptor gene after residue Arg-148 in the second intracellular loop of the predicted protein sequence. Binding of the dopamine antagonist [125I]iodosulpride to D3 receptors was absent in mice homozygous for the mutation and greatly reduced in heterozygous mice. Behavioral analysis of mutant mice showed that this mutation is associated with hyperactivity in an exploratory test. Homozygous mice lacking D3 receptors display increased locomotor activity and rearing behavior. Mice heterozygous for the D3 receptor mutation show similar, albeit less pronounced, behavioral alterations. Our findings indicate that D3 receptors play an inhibitory role in the control of certain behaviors.

Amino Acid Sequence↗