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Biomedical subjects

M L Bernstein

Publications and source records attributed to M L Bernstein.

At least 37 records · Page 2Linked to original sources
PubMed1996

Skeletal and cardiac malformations with thrombocytopenia: a new syndrome?

We describe a female patient with multiple anomalies suggestive of a new syndrome. Manifestations include: VSD and ASD, mild developmental delay, conductive hearing loss, minor facial anomalies, thrombocytopenia, and radiological findings (including carpal fusion). Some of these manifestations may be present in the Keutel syndrome, IVIC syndrome, and the 10qter deletion syndrome. However, none of these syndromes can explain the spectrum of anomalies seen in our patient.

Abnormalities, Multiple↗
PubMed1996

Neuroblastoma: an enigmatic disease.

Neuroblastoma is the most common extra-cranial solid tumor of childhood. It originates in cells of the neural crest, and so can be found anywhere along the paravertebral sympathetic chain or in the adrenal gland. In the last 15 years, new developments in the genetics and biology of neuroblastoma, have led to a better understanding of the natural history and prognostic features of this cancer. The presence of identifying biochemical markers detectable in the urine of patients with neuroblastoma, as well as the remarkably inferior survival of children diagnosed at more than 12 months of age, have led some groups to screen infants for neuroblastoma, in the hope of decreasing both overall mortality, as well as the incidence of advanced stage disease. This article reviews some clinical aspects of neuroblastoma, but emphasizes the genetic and biologic features in relation to prognosis and treatment. Finally, we discuss the different screening experiences for this disease, in particular from the Quebec Neuroblastoma Screening Project.

Child↗
PubMed1996

Site-specific responses to monocrotaline-induced vascular injury: evidence for two distinct mechanisms of remodeling.

Monocrotaline (MCT)-induced pulmonary vascular injury was used to begin studying the mechanism(s) of vascular remodeling in Fischer 344 rats, using extracellular matrix (ECM) gene expression to define areas of remodeling. By day 28 after injection, pulmonary artery pressures were increased and right ventricular hypertrophy had developed compared with normal controls. Tropoelastin, fibronectin, and alpha 1(I) procollagen mRNA levels increased at least 2-fold by day 28. In situ hybridization demonstrated tropoelastin gene expression by cells adjacent to the lumen and procollagen gene expression at the medial-adventitial border in both small muscular and large elastic pulmonary arteries. This pattern of gene expression was observed as early as 1 wk after MCT injury. These observations indicated two distinct areas of remodeling, one along the vascular lumen at the site of monocrotaline-induced injury and the other at a second distinct site. To determine whether other differences may be involved at these two sites, the presence of transforming growth factor-beta (TGF-beta) was studied. Total TGF-beta protein was 4-fold higher in remodeling lungs compared with normal lungs. Gene expression for all three isoforms of TGF-beta colocalized with tropoelastin gene expression along the vascular lumen but not with alpha 1(I) procollagen gene expression. These results suggest a complex site-specific response to injury mediated by two distinct pathways in this model of pulmonary vascular remodeling.

Animals↗
PubMed1995

Femoral revision hip arthroplasty with uncemented, porous-coated stems.

One hundred seventy-five cementless femoral hip revision surgeries with extensively porous-coated stems and 161 concomitant acetabular revisions done from 1984 to 1991 were retrospectively reviewed. Average age at surgery was 62.4 years. Followup ranged from 2 to 10 years (average, 5 years). One hundred sixty-eight femoral components (96%) remain in place. Two unstable components, 1 stable fibrous component, and 1 bone ingrowth component were painful and required rerevision. Two femoral components were removed as part of resection arthroplasties for repetitive acetabular failure caused by pelvic dissociation. One component was removed for late hematogenous infection. Using the Engh radiographic criteria, femoral stems were judged to have achieved bony ingrowth in 82.8% (n = 174) of the cases. Bony ingrowth occurred more frequently when the canal was filled with the prosthesis and with lesser degrees of bone stock deficiency. Severe stress shielding occurred in 11 (7.6%; n = 144) of the bone in-grown cases and correlated with preoperative osteoporosis and larger diameter prosthesis. Significant thigh pain was less common in the bone ingrown (4.2%; n = 144) than in the stable fibrous group (18.5%; n = 27). Significant thigh pain in bone ingrown stems was more likely to occur with osteoporotic and bone stock-deficient femurs. To date, significant wear and osteolysis have not been observed.

Acetabulum↗
PubMed1994

Reanalysis of the 12-minute walk in patients with chronic obstructive pulmonary disease.

The purpose of this study was to determine the correlation between different intervals in the 12-min walk test, to determine which of the intervals best correlated with maximal oxygen intake (VO2max) and maximal CO2 expelled (VCO2max) and to determine the degree of correlation between changes in the VO2max and changes in the walk test and spirometry. Nine elderly (age, 67 +/- 4 years) patients with moderate COPD (FEV1, 1.32 +/- 0.28 L) who were ventilatory limited were seen 6 times over a 14-week period. At each visit they performed spirometry, a 12-min walk, and a symptom-limited maximal exercise test. During the 12-min walk the distances traversed in each 2-min interval were recorded. A Borg score was obtained at the end of each 2 min. The distances that the patients walked in each 2-min interval were very similar, but the mean Borg score became higher the longer the patient walked. The Borg score after 2 min was 1.64 +/- 1.15, while the Borg score after 12 min was 5.70 +/- 3.59. The correlation coefficients between the distance walked at various intervals and the oxygen consumption (VO2)/kg (approximately 0.65) and the VCO2/kg (approximately 0.52) were very similar. However, changes in the VO2/kg were more closely correlated with changes in the 12-min walk (r = 0.72), than with changes in the 6-min walk (r = 0.64), the 4-min walk (r = 0.59), or the 2-min walk (r = 0.53). This latter observation suggests that the 12-min walk may be preferable to tests in which the patient walks 4 or 6 min for documenting changes in the exercise capabilities.

Aged↗
PubMed1994

Maternal malignancy involving the products of conception: a report of malignant melanoma and medulloblastoma.

PURPOSE: Cancer in pregnancy is not uncommon, but cases of maternal malignancy involving the placenta or the fetus are much more rare. We report two additional cases. PATIENTS AND METHODS: We report one case of malignant melanoma discovered during pregnancy and found to have metastasized to the placenta. We also describe a case of maternal medulloblastoma involving the placenta at delivery. CONCLUSIONS: Although maternal malignancy during pregnancy occurs in as many as one in 1,000 pregnancies, involvement of the products of conception is rare. Subsequent malignancy in the fetus is even more rare. We report two cases, one of melanoma and another of medulloblastoma (the first such case described). Both infants are alive and well.

Adult↗
PubMed1994

Parents' versus physicians' values for clinical outcomes in young febrile children.

OBJECTIVE: To compare how parents and physicians value potential clinical outcomes in young children who have a fever but no focus of bacterial infection. METHODS: Cross-sectional study of 100 parents of well children aged 3 to 24 months, 61 parents of febrile children aged 3 to 24 months, and 56 attending staff physicians working in a children's hospital emergency department. A pretested visual analog scale was used to assess values on a 0-to-1 scale (where 0 is the value of the worst possible outcome, and 1 is the value for the best) for 22 scenarios, grouped in three categories according to severity. Based on the three or four common attributes comprising the scenarios in a given group, each respondent's value function was estimated statistically based on multiattribute utility theory. RESULTS: For outcomes in group 1 (rapidly resolving viral infection with one or more diagnostic tests), no significant group differences were observed. For outcomes in groups 2 (acute infections without long-term sequelae) and 3 (long-term sequelae of urinary tract infection or bacterial meningitis), parents of well children and parents of febrile children had values that were similar to each other but significantly lower than physicians' values for pneumonia with delayed diagnosis, false-positive diagnosis of urinary tract infection, viral meningitis, and unilateral hearing loss. For bacterial meningitis with or without delay, however, the reverse pattern was observed; physicians' values were lower than parents'. In arriving at their judgment for group 2 and 3 scenarios, parents gave significantly greater weight to attributes involving the pain and discomfort of diagnostic tests and to diagnostic error, whereas physicians gave significantly greater weight to attributes involving both short- and long-term morbidity and long-term worry and inconvenience. Parents were significantly more likely to be risk-seeking in the way they weighted the attributes comprising group 2 and 3 scenarios than physicians, ie, they were more willing to risk rare but severe morbidity to avoid the short-term adverse effects of testing. CONCLUSIONS: Parents and physicians show fundamental value differences concerning diagnostic testing, diagnostic error, and short- and long-term morbidity; these differences have important implications for diagnostic decision making in the young febrile child.

Attitude of Health Personnel↗
PubMed1994

Persistence and possible progression of a pelvic neuroblastoma detected by mass screening during 19 months.

PURPOSE: The preclinical detection of neuroblastoma by screening for elevated levels of urinary catecholamines often leads to the discovery of children with early-stage, biologically favorable disease. It is uncertain how vigorously therapy must be pursued in such cases. We report an infant whose pelvic mass was initially thought to be a fecaloma, and consequently was not treated for 19 months. CASE REPORT: A 2-month-old girl was referred for evaluation for the presence of a neuroblastoma because of elevated urinary catecholamines detected in a mass screening program. Although no mass was initially found, urinary catecholamines became increasingly elevated, and a pelvic mass was finally radiologically identified. It was resected. Histology showed it to be a neuroblastoma, POG stage C, International stage 3, with unfavorable Shimada features, although near triploid with an unamplified N-myc oncogene. Chemotherapy was given for five cycles, and the child remains well, with no evidence of disease. CONCLUSION: Although mass screening programs often detect early- stage, biologically favorable neuroblastomas that may spontaneously regress, our case had rising levels of urinary catecholamines and unfavorable histologic features at the time of resection. The unusual location of the tumor, and radiologic features of a fecaloma, contributed to the delay in definitive diagnosis, although persistence of elevated urinary catecholamines in the absence of a tumor is infrequent.

Catecholamines↗
PubMed1993

Ifosfamide with mesna uroprotection and etoposide in recurrent, refractory acute leukemia in childhood. A Pediatric Oncology Group Study.

BACKGROUND: Ifosfamide has previously been shown to be active as a single agent and in combination with doxorubicin, etoposide, and teniposide in pediatric solid tumors and adult acute leukemia. The authors performed a dose-escalation trial of ifosfamide with a fixed dosage of etoposide, with mesna uroprotection, in children with multiply recurrent acute leukemia. METHODS: Chemotherapy was administered daily for 5 days. Etoposide 100 mg/m2 was followed by ifosfamide at an initial dosage of 1.6 g/m2. The ifosfamide was escalated in 20% increments to the maximum tolerated dosage in cohorts of three patients. Mesna 400 mg/m2 was given immediately before the ifosfamide and then at 3 and 6 hours after ifosfamide in the initial patients. Subsequent patients were treated with mesna 400 mg/m2 just before ifosfamide, and then every 2 hours to a total dosage equal to the ifosfamide dosage. RESULTS: Forty-four heavily pretreated patients were entered on study. Forty were evaluable for toxicity and 36 for response as well. The maximum tolerated dosage of ifosfamide was 4.0 g/m2/d for 5 days (20 g/m2/course). Overall, 10 patients achieved complete remission, and 3 achieved partial remission. Remissions were brief, although four patients went on to bone marrow transplant while in remission. One patient is still alive. CONCLUSIONS: The combination of etoposide and ifosfamide with mesna uroprotection showed promising activity in children with multiply recurrent acute leukemia.

Acute Disease↗
PubMed1993

A phase I trial of fazarabine in refractory pediatric solid tumors. A Pediatric Oncology Group study.

Fazarabine is a synthetic analog of cytosine arabinoside and 5-azacytidine that incorporates structural features of both compounds. Xenograft studies showed good activity against a variety of transplanted tumors. Initial studies in adults employed both a continuous infusion schedule and a daily bolus x 5 schedule. Myelotoxicity, especially neutropenia, was dose-limiting, with excessive myelotoxicity seen on the daily bolus x 5 at 72 mg/M2/day. Since short infusions may be administered in Ringer's lactate rather than either dimethylsulfoxide or dimethylacetamide required for continuous infusion, this study examined a daily x 5 schedule in children with refractory solid tumors. The initial dosage was 30 mg/M2/day, 80% of the maximum tolerated dosage in adults, with subsequent 30% dosage escalations. A total of 18 patients were enrolled, with a wide spectrum of pediatric solid tumors. Myelosuppression was the only significant toxicity, and was excessive at 78 mg/M2/day. Therefore, on this bolus regimen, 65 mg/M2/day for 5 days was the maximum tolerated dosage. One patient with medulloblastoma had stable disease for 65 days. No other responses were seen.

Antineoplastic Agents↗
PubMed1993

Role of the complete blood count in detecting occult focal bacterial infection in the young febrile child.

Previous studies of the value of the complete blood count (CBC) in distinguishing viral from bacterial infection in young febrile children have failed to exclude children with clinically evident bacterial infection and thus have inflated the positive predictive value of the test for occult focal infection. We prospectively studied 2492 children 3-24 months of age who presented to a children's hospital emergency department between March 1989 and August 1990 with fever (> or = 38.0 degrees C) of acute (< or = 4 days) onset but no evident bacterial focus of infection, 433 (17.4%) of whom received a CBC. We also carried out an 8-year retrospective analysis to estimate prior, or pre-test, probabilities (prevalences) and examine CBC results for rare occult bacterial infections (meningitis, osteomyelitis, and septic arthritis). Estimated prior probabilities for the four most common categories of infection that can be diagnosed at the initial visit were: non-pneumonitic viral infection, 88.6% in boys and 86.0% in girls; pneumonia, 8.5% in both sexes; urinary tract infection (UTI), 3.0% in boys and 5.5% in girls; and bacterial meningitis, 0.0066% in both sexes. The likelihood (sensitivity) of a total white blood cell (WBC) count > or = 15,000/mm3 was 25.5, 64.5, 62.5, and 50.0% for viral infection, pneumonia, UTI, and meningitis, respectively. Among children with a high total white blood cell count, neither a total polymorphonuclear count > or = 10,000/mm3 nor a band count > or = 500/mm3 was associated with significantly elevated likelihoods for occult pneumonia or UTI, a finding confirmed by multiple logistic regression analysis.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1993

Relief of sickle cell priapism by hydralazine. Report of a case.

We report a 16-year-old Tanner IV male with homozygous hemoglobin S who presented with recurrent episodes of priapism unresponsive to standard therapy with hydration, analgesia, and exchange transfusion. He had a complete resolution of his symptoms with hydralazine therapy. We therefore suggest a trial of vasodilator therapy for recurrent sickle cell priapism before attempting surgical therapy.

Adolescent↗
PubMed1992

Cinnamon-induced stomatitis venenata, Clinical and characteristic histopathologic features.

Fourteen new cases of cinnamon-induced stomatitis are reported. Ten of these fourteen cases were first detected on the basis of histopathologic changes, which included hyperkeratosis, chronic lichenoid mucositis with plasmacytic infiltration, and marked chronic perivasculitis. Six cases of false-positive histopathologic findings are presented for comparison. It is recommended that when the histopathologic features described are recognized, cinnamon stomatitis should be considered.

Adult↗
PubMed1992

Screening for neuroblastoma in North America. 2-year results from the Quebec Project.

The Quebec Neuroblastoma Screening Project was initiated to assess the clinical and biological aspects of screening infants for the presence of neuroblastoma in North America. All children born in the province of Quebec from May 1, 1989 to April 30, 1994 are eligible for participation. This report provides results from 22 months' accrual of infants who were screened using urine-saturated filter paper for determination of the catecholamine metabolites vanillylmandelic acid (VMA) and homovanillic acid (HVA). More than 157,000 infants have been screened to date at 3 weeks of age, representing 92% of the entire birth population of Quebec. Over 98,000 infants have been screened a second time at 6 months of age, which made up 76% of the Quebec birth cohort. After a two-stage initial screening, 340 (0.13%) infants (182 at 3 weeks and 158 at 6 months) required second laboratory examinations because of elevated levels of urinary VMA, HVA, or both. Twenty infants from the 3-week screening (0.01%) and nine from the 6-month screening (0.01%) were subsequently referred to one of four Quebec pediatric oncology centers for neuroblastoma evaluation. Seven of 20 children from the 3-week screening and two of nine children from the 6-month screening have been identified as having neuroblastoma. During the same period, 14 additional children in the birth cohort were diagnosed clinically with neuroblastoma; eight were diagnosed prior to screening at 3 weeks of age, three children had negative results at 3 weeks of age, two had negative results at 3 weeks and at 6 months of age, and one had never been screened.(ABSTRACT TRUNCATED AT 250 WORDS)

Algorithms↗
PubMed1992

A population-based study of neuroblastoma incidence, survival, and mortality in North America.

PURPOSE: The purpose of this study was twofold: (1) to provide a population-based estimate of neuroblastoma incidence, disease stage and age distribution, and survival and mortality rates in North America; and (2) to compare these figures in the province of Quebec at a time shortly before the institution of province-wide screening with those in a population-based control group, the Greater Delaware Valley (GDV) Pediatric Tumor Registry. MATERIALS AND METHODS: In Quebec, the four major pediatric teaching hospital records were searched for children with a diagnosis of neuroblastoma. Tumor board registry data and information supplied to the Division of Vital Statistics were also reviewed. Birth statistics were obtained from the population registry. The GDV Pediatric Tumor Registry is a population-based registry of pediatric cancer covering all of Delaware and parts of New Jersey, Pennsylvania, and Maryland. Age, stage of disease, and follow-up data were obtained through December 31, 1989, with Evans neuroblastoma staging data used for all comparisons. RESULTS: One hundred thirty children with neuroblastoma were identified in Quebec and 165 in the GDV, in a combined population of 3,178,736 children. The annual incidence of neuroblastoma was 10.95/10(6) under the age of 15 years and 27.75/10(6) between the ages of 0 and 4 years. The annual mortality rate due to neuroblastoma was 4.89/10(6) and 9.10/10(6) for the age groups 0 to 14 and 0 to 4, respectively. The overall 10-year survival rate for the 295 cases of neuroblastoma was 55%. The 10-year survival rates for patients with Evans stage I-IV and IVS disease were 88%, 90%, 63%, 21%, and 81%. There was no significant difference observed in the incidence, mortality, or survival in the two populations. CONCLUSION: These data represent the first large, population-based description of the clinical presentation and outcome of patients with neuroblastoma in North America, with no significant differences noted between Quebec patients and the GDV patients.

Adolescent↗
PubMed1992

Metastatic neuroblastoma presenting as a mandibular mass.

Neuroblastoma is the third most common type of cancer seen in children, after leukemia and tumours of the central nervous system. Although bony metastasis to the skull and the orbits has been well described, metastasis to the mandible is exceptional; 32 cases have been reported. Two more are presented here, along with a short review of the topic emphasizing the radiographic features and the differential diagnosis.

Abdominal Neoplasms↗