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Biomedical subjects

H Rothschild

Publications and source records attributed to H Rothschild.

At least 37 records · Page 2Linked to original sources
PubMed1981

Tropical splenomegaly syndrome in a nontropical setting.

Tropical splenomegaly syndrome, a rare complication of recurrent malarial infection thought to occur only in endemic areas, was diagnosed in a 9-year resident of the United States. The patient had splenomegaly, anemia, a history of recurrent fever since childhood, cryoglobulinemia, increased serum IgM, and elevated specific immunofluorescent antibody titers to Plasmodium falciparum. After antimalarial treatment and splenectomy, she became asymptomatic and the IgM levels and specific antibody titers returned to normal. Because of increased travel to and from endemic malarial areas, this syndrome should be considered in the differential diagnosis of chronic splenomegaly.

Female↗
PubMed1981

Heel deformity in hereditary spastic paraplegia.

The association of pes cavus deformity and neurological disorder is well known. Varus deformity of the heel is often associated with, and may even precede the development of pes cavus. Clinical and radiographic examinations of the feet of members of three kindreds of hereditary spastic paraplegia, a rare neurologic disorder, suggested that the autosomal dominant form manifests a significantly higher incidence and degree of heel varus deformity than the autosomal recessive form of the disease. Therefore, heel varus deformity may be a sign suggestive of autosomal dominant inheritance of hereditary spastic paraplegia.

Adult↗
PubMed1980

Visual evoked potentials in two forms of hereditary spastic paraplegia.

Visual evoked potentials (VEPs) were used to study two forms of hereditary spastic paraplegia. We recorded the VEP in 6 homozygotes and 5 obligate heterozygotes in one family with recessively transmitted disease and in 5 persons with dominantly transmitted hereditary spastic paraplegia. The VEPs in the affected persons from both families were similar; both had delayed conduction times and changes in the wave form. The VEPs of the obligate heterozygotes were indistinguishable from those of the unaffected control subjects. Comparison of our findings with those of other disease processes that affect the VEPs suggest that the conduction delays in our patients' VEPs may be associated with changes in myelin.

Adult↗
PubMed1979

Autosomal recessive spastic paraplegia: evidence for demyelination.

Neurological evaluation of a family living in rural Louisiana showed that six living members have splasticity, hyperactive stretch reflexes, and pathological reflexes. Ten deceased family members had been similarly affected. All affected persons were offspring of consanguineous marriages, and sex distribution was equal, indicating that inheritance was autosomal recessive. Although undetected clinically, conduction abnormalities suggestive of demyelination in the visual pathways were revealed by special tests.

Adult↗
PubMed1978

Tetanus--review of the literature and report of case.

This case presents many of the difficulties of management that are inherent in a severe case of tetanus. Also it shows a seldom-considered therapeutic modality, that of dental extraction, gingival debridement, and gingivectomy with a confirmed case of tetanus without an established portal of entry. It is well known that periodontal and periapical locations can easily give rise to an anaerobic focus. In this case, anaerobic culture of C tetani was unsuccessful, possibly because of the inherent difficulty of anaerobic transfer from an oral locus and the extreme fastidiousness of the organism. As a rule, all patients who recover from tetanus do so completely, without any residual deficit. This was true in the case discussed here. Conclusions that can be drawn from this case are the following: a diagnosis of tetanus should be considered in any case of unexplained trismus; aggressive treatment of the immunologic deficit by antitoxin should be instituted along with a course of active immunization; and aggressive symptomatic treatment should be started. The bases of the treatment of this case were management of airways, sedation, treatment of autonomic crisis, and eradication of the causative agent. The result was that a severely ill patient reponded to treatment and completely recovered from a devastating disease.

Aged↗
PubMed1977

Advocacy and compliance factors in a voluntary selective screening program.

To evaluate factors responsible for compliance with a voluntary selective screening program, we surveyed 495 participants and 212 nonparticipants in a screening program for Tay-Sachs disease. Knowledge about the program and motivation are the most important factors in compliance. The primary incentive for participation was to avoid having abnormal children in future generations.

Adult↗
PubMed1976

Isolation and characterization of a heat-inducible simian virus 40 mutant.

We have isolated a new type of temperature-sensitive mutant of simian virus 40 (SV40) that is capable of productive infection in permissive cells but not of maintenance of viral DNA integration in transformed cells at the conditional temperature. Virus development is induced when cells transformed by this mutant are shifted to temperatures above 39 degrees C, but is not induced below this temperature. The plaque-purified, temperature-sensitive mutant virus confers heat inducibility to new host cells, indicating that the conditional function is a property of the viral genome. Unlike previously described temperature-sensitive SV40 mutants, in (ts)-1501 is capable of productive infection in permissive cells at the conditional temperature. The morphology, growth, and oncogenicity of in (ts)-1501-transformed cells at 37 degrees C are similar to those of cell lines transformed by wild-type SV40. HK10-c2(in(ts)-1501), a cloned cell line, transformed at 37 degrees C by the mutant virus, exhibits a transient increase in DNA synthesis before cell death at the conditional temperature. Many properties of in(ts)-1501 are analogous to those of the heat-inducible mutants of bacteriophages in which a heat-inactivated protein is responsible for the stable integration of the prophage in the bacterial chromosome.

Antigens, Viral↗
PubMed1976

Regulation of the beta- and delta-hemoglobin genes. A family with hereditary persistent fetal hemoglobin and beta-thalassemia.

We have studied a 41-year-old black male with the simultaneous occurrence of hereditary persistence of fetal hemoglobin (HPFH) and beta-thalassemia, and his two postadolescent sons, each heterozygous for one of the traits. The son heterozygous for beta-thalassemia had an elevated Hb A2, but the index case did not. The data from this pedigree indicate that the delta-allele trans to the beta-thalassemia gene was reponsible for the increased delta-chain production. Evidence from other cases of combination HPFH and beta-thalassemia indicates that regulation of the beta- and delta-chain production in beta-thalassemia is heterogeneous with respect to mechanism.

Adolescent↗
PubMed1976

An immunological investigation of a family with chronic mucocutaneous candidiasis.

Chronic mucocutaneous candidiasis in two siblings of consanguineous parents suggested an autosomal recessive transmission of the disease. We evaluated the two affected persons and 21 members of their kindred for an inherited immunological defect. Six members of the kindred, including both patients, had negative skin-delayed hypersensitivity to Candida. The lymphocytes of both patients and three asymptomatic relatives had diminished in vitro blastogenic response when cultured with Candida albicans. Because the defect occurred in clinically unaffected relatives, we concluded that the lack of blastogenic response to C. albicans was not the only determinant for or may be unrelated to the clinical manifestations of the disease.

Antigens↗
PubMed1975

Biochemical genetic studies of cycloheximide resistance in Neurospora crassa.

Genetic analysis of a number of cycloheximide-resistant mutants of Neurospora crassa has shown that resistance is controlled by several genes. Two of these appear to be located on linkage group V. Resistance to the antibiotic is dominant in wild-type-mutant heterokaryons. Two types of cycloheximide-resistant mutants were isolated: one type exhibited colonial morphology only when grown in the presence of cycloheximide and the other type maintained normal morphology even at high concentrations of the antibiotic. Reconstitution experiments with supernatant solutions and 80S monosomes prepared from wild-type and resistant mutant strains indicated that the property of cycloheximide resistance most likely is associated with the ribosomes. No electrophoretic or serological differences were found between the ribosomal proteins of the wild-type and resistant mutants.

Amino Acids↗
PubMed1975

A review of deaths due to suspected lactic acidosis at a large metropolitan hospital.

A review of 2,647 consecutive deaths over a 19-month period at a large metropolitan hospital revealed 27 cases (1%) to be coded as metabolic acidosis. In ten of these deaths, a presumptive diagnosis of lactic acidosis could be made. Eight of the ten were diabetic, and all eight were treated with phenformin at the time of their last admission. Although phenformin could not be incriminated as the sole cause of lactic acidosis in these cases, a contributory role of the drug seems probable. The indiscriminate use of phenformin in diabetic patients should be discouraged and the contraindications to the use of phenformin should be stressed.

Acidosis↗
PubMed1975

An in vitro method to study the participation of various components in autoimmune hemolytic anemia.

A method is described which allows for the in vitro evaluation of various etiological components in autoimmune hemolytic anemia. As an example of the applicability of the technique, erythrocytes and serum from a patient with an autoimmune hemolytic anemia suspected to be due to poliovirus III were tested with poliovirus III and complement. Incubation of the patient's cells with complement, poliovirus and autologous serum was required for optimal in vitro fragility. The method is proposed as a practical and convenient in vitro technique for evaluating the participation of individual components in the pathogenesis of autoimmune hemolytic anemias.

Anemia, Hemolytic, Autoimmune↗