Tibial hypo-/aplasia with preaxial syn- and polydactyly

Summary Tibial hypo-/aplasia with preaxial syn- and polydactyly is a rare autosomal dominant condition. Fewer than 20 cases have so far been described. One is presented here.     

In situ breast carcinomas: A population-based study on frequency,growth pattern,and clinical aspects

This study comprises 78 consecutive women, within situ carcinoma of the breast and no evidence of invasion, who were diagnosed and treated surgically from October, 1979 to June, 1986. Lobular carcinoma in situ (LCIS) was found in 30 patients (38%) and ductal carcinoma in situ (DCIS) was identified in 48 patients (62%). The series is a study based in the county of Funen, which has a total female population of 230,028 inhabitants.Epidemiological calculations were carried out in 5 municipalities in the area of Odense University Hospital with 77,477 female inhabitants 20+ years of age according to a predetermined referral scheme and diagnostic procedures. This sub-study revealed a frequency of 9.0 cases ofin situ carcinomas per 10⁵ woman-years and a cumulated risk of 0.53% of havingin situ carcinoma demonstrated within the age range of 20–75 years.In situ lesions made up about 6% of all newly diagnosed breast carcinomas in the region. Furthermore, in about 3% of all breast biopsies and in about 0.3% of all women having mammography due to breast symptoms, anin situ finding was the only malignant change.Pathoanatomically, a distinction could be made between 3 different growth patterns: microfocal, tumor-forming, and diffuse growths. Microfocal lesions comprised a major part (60%) of in situ carcinomas. With one exception, the microfocal cases were chance findings in an otherwise benign breast biopsy, whereas the more aggressive growth patterns (tumor-forming and diffuse) were predominantly clinical and/or mammographic findings, all belonging to the DCIS type. Excisional biopsy or partial mastectomy was performed in 85% of the women with microfocal cases, while such breast preservation procedures were used in only 27% of patients with tumor-forming or diffuse growths.

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Resumen El presente estudio comprende 78 mujeres consecutivas con carcinoma mamarioin situ y sin evidencia de invasíon, quienes fueron diagnosticadas y tratadas quirúrgicamente durante el período 1979 a 1986. Se encontró carcinoma lobular in situ (CLIS) en 30 pacientes (38%) y carcinoma ductal in situ (CDIS) en 48 pacientes (62%). La serie proviene de la región de la isla de Funen, Dinamarca, con una población de 230,028 habitantes.Se realizaron determinaciones epidemiológicas en 5 municipalidades correspondientes a la región del Hospital de la Universidad de Odense, con 77,477 habitantes mujeres de acuerdo a un esquema predeterminado de referencia y a procedimientos de diagnóstico estandarizados. Este subanálisis reveló una frecuencia de 9.0 casos de carcinomasin situ por 10⁵ mujer-años y un riesgo de 0.53% de tener carcinomain situ para las mujeres entre los 20 y los 75 años. Las lesionesin situ representaron alrededor del 6% de la totalidad de los nuevos carcinomas mamarios diagnosticados en la región. Además, en alrededor de 3% de todas las biopsias mamarias y en alrededor de 0.3% de todas las mujeres sometidas a mamografía por razón de presentar síntomas mamarios, el hallazgoin situ fue el único hallazgo de malignidad.Se pudo hacer la diferenciación anatomopatológica entre 3 diferentes patrones de crecimiento: microfocal, formación tumoral, y crecimiento difuso. Las lesiones microfocales comprenden la mayor parte (60%) de los carcinomasin situ. Con una excepción, los casos del tipo microfocal fueron hallazgos fortuitos en biopsias mamarias por lo demás benignas, en tanto que los patrones de crecimiento de tipos más agresivos (formación tumoral y crecimiento difuso) fueron predominantemente hallazgos clínicos y/o mamográficos, todos CDIS. Se realizó biopsias excisional o mastectomía parcial en 85% de las mujeres con el tipo microfocal, en tanto que tales procedimientos de conservación mamaria fueron utilizados en solo 27% de las pacientes con los patrones de formación tumoral de crecimiento difuso.

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Résumé On a étudié les résultats de soixante-dix-huit cancersin situ du sein chez la femme, sans évidence d'envahissement, diagnostiqué et traités chirurgicalement entre octobre 1979 et juin 1986. Il y avait 30 cas (38%) de cancer lobulaire (CL) et 48 cas (62%) de cancer cannalaire (CC). Cette série de patientes provenait du comté de Funen, Danemark, dont la population féminine est de 230,028.L'épidémiologie a été étudiée dans 5 municipalités dans la région de l'Hôpital Universitaire d'Odense où la population féminine est de 77,477 selon une méthode de consultation et de diagnostic standardisée. Cette étude a montré que l'incidence était de 9 cas de cancerin situ pour 10⁵ femmes-années; le risque d'avoir un cancerin situ était de 0.53% entre les âges de 20–75 ans. Six pour cent des cancers diagnostiqués dans la région étaitin situ. Un cancer in situ a été trouvé dans 3% de toutes les biopsies du sein, et 0.3% des femmes ayant une mammographie motivée par une Symptomatologie au niveau du sein.Histologiquement, on pouvait distinguer 3 types de croissance: microfocale, tumorale, et diffuse. Les lésions microfocales étaient responsables de 60% des cancersin situ. A une exception près, les microfoyers de cancer ont tous été découverts par hasard, alors que les formes tumorales et diffuses ont tous été décelés grâce à des signes cliniques et/ou mammographiques. Il s'agissait toujours d'un cancer cannalaire. Une biopsie d'exérèse ou mastectomie partielle a été effectuée chez 85% des femmes ayant un microcancer, mais seulement chez 27% des femmes ayant une forme tumorale ou diffuse.

     

Inhibition of 2-nitropropane-induced rat liver DNA and RNA damage by benzyl selenocyanate

We observed that pretreatment of male F344 rats with benzyl selenocyanate, a versatile organoselenium chemopreventive agent in several animal model systems, decreases the levels of DNA and RNA modifications produced in the liver by the hepatocarcinogen 2- nitropropane. To clarify the mechanisms involved, we pretreated male F344 rats with either benzyl selenocyanate, its sulfur analog benzyl thiocyanate, phenobarbital or cobalt protoporphyrin IX; the latter is a depletor of P450. We then determined (1) the ability of liver microsomes to denitrify 2-nitropropane, (2) effects on 2-nitropropane- induced liver DNA and RNA modifications and (3) amount of nitrate excreted in rat urine following administration of the carcinogen. Pretreatment with benzyl selenocyanate or phenobarbital increased the denitrification activity of liver microsomes by 217 and 765%, respectively, increased liver P4502B1 by 31- and 435-fold, respectively, decreased the levels of 2-nitropropane-induced modifications in liver DNA (29-70% and 17-30%, respectively) and RNA (67-85% and 30-50%, respectively), and increased the 24-h urinary excretion of nitrate by 157 and 209%, respectively. Pretreatment with benzyl thiocyanate had no significant effect on any of these parameters. Pretreatment with cobalt protoporphyrin IX decreased liver P4502B 1 by 87%, decreased the denitrification activity of liver microsomes by 76%, decreased the 24 h urinary excretion of nitrate by 88.5%, but increased the extent of 2-nitropropane-induced liver nucleic acid modifications by 17-67%. These results indicate that the metabolic sequence from 2-nitropropane to the reactive species causing DNA and RNA modifications does not involve the removal of the nitro group. Moreover, they suggest that benzyl selenocyanate inhibits 2-NP-induced liver nucleic acid modifications in part by increasing its detoxication through induction of denitrification, although it is evident that other mechanisms must also be involved.      

Magnetic resonance imaging of the brain in childhood herpesvirus infections

We used magnetic resonance imaging (MRI) to evaluate nine children with neurologic disorders caused by infections with members of the herpesvirus family. MRI studies were abnormal in eight children and demonstrated a wide range of central nervous system lesions, including cystic encephalomalacia, ventricular enlargement, cerebral atrophy and focal parenchymal lesions. When compared with conventional computed tomographic scanning, MRI was more sensitive in detecting abnormalities of white matter and in defining the extent of parenchymal lesions. These studies indicate that MRI scans are highly useful in children with herpesvirus infections involving the central nervous system.     

Local and central sympathetic reflex control of blood flow in skeletal muscle and subcutaneous tissue in normal man

Summary. The effect of age and sex on relative changes in blood flow and vascular resistance in skeletal muscle and subcutaneous tissue during postural changes and during local increase in transmural pressure was studied in 33 healthy subjects. The intra-individual variation was studied in five subjects. Blood flow was measured by the local ¹³³Xenon wash-out method. No relation to age or sex was seen in the centrally elicited sympathetic vasoconstrictor responses in subcutaneous tissue and skeletal muscle and in the locally elicited vasoconstriction in subcutaneous tissue. A small, but statistically significant, correlation to sex and age was found in the local sympathetic vasoconstrictor response in skeletal muscle. The age correlation was caused only by an attenuated response in the young subjects below 40 years of age and may be fortuitous. The intra-individual variation was acceptably small. Based on the present results, a reduction in blood flow in skeletal muscle and subcutaneous tissue during centrally or locally elicited sympathetic vasoconstriction of 10% or less should be considered abnormal. The local ¹³³Xenon wash-out method is of value in examining patients suspected of dysfunction in the sympathetic part of the autonomic nervous system.     

Pulse methotrexate therapy in rheumatoid arthritis. A controlled prospective roentgenographic study

Study Objective: To assess whether weekly pulse methotrexate therapy alters radiographic progression of joint disease in patients with rheumatoid arthritis. Design: Prospective, controlled study. Hand, wrist and foot roentgenograms obtained before, at the onset of, and during methotrexate treatment were scored for degree of joint-space narrowing and erosions by three rheumatologists using a standard method. Patients: Sequential sample of 24 patients with active definite or classical rheumatoid arthritis and previous unsuccessful treatment; of these, 3 were excluded due to drug ineffectiveness; 2, due to side effects; and 1, due to refusal to take methotrexate. Interventions: Treatment with nonsteroidal anti-inflammatory drugs and prednisone was continued. Methotrexate was given weekly to control clinical evidence of disease in patients. Measurements and Main Results: After having had an average of 30 months of therapy, the 18 patients who continued to receive methotrexate therapy showed significant (p less than 0.05) clinical improvement, as evidenced by their decreased joint counts and joint scores, duration of morning stiffness, pain scales, and sedimentation rates. Despite patients' prolonged clinical improvement, the mean rate of development of erosions and joint-space narrowing during methotrexate therapy was not significantly different from the rate of radiographic progression before methotrexate therapy (0.043 compared with 0.041; p greater than 0.05). Conclusions: Weekly pulse methotrexate is effective for the long-term management of clinical disease activity in patients with refractory rheumatoid arthritis but may not be a disease-modifying agent by roentgenographic criteria.