Síndrome de bazex: acroqueratosis paraneoplásica secundaria a cáncer de próstata / Bazex syndrome: paraneoplasic acroqueratosis secondary to prostatic cancer

La piel puede ofrecer el primer síntoma guía en el 1% de los pacientes con neoplasias internas. Las manifestaciones cutáneas de malignidades internas se pueden producir por invasión directa de la piel por el tumor y por diseminación metastásica, pero existen mecanismos indirectos que inducen la aparición de signos y síntomas cutáneos no relacionados con el tumor primitivo. El Síndrome de Bazex es una rara dermatosis descrita por Bazex y colaboradores en 1965. Se caracteriza por la presencia de placas eritematosas, psoriasiformes, que típicamente afectan a las manos, los pies, la nariz y los pabellones auriculares. La inexperiencia puede motivar retrasos en el diagnóstico, haciendo que la neoplasia asociada se encuentre en estadios avanzados en el momento del diagnóstico. Se discute un caso clínico de un paciente masculino de 53 años de edad, hipertenso, quien acude por presentar lesiones cutáneas tipo placas hipertróficas, hiperpigmentadas, descamativas y dolorosas con fondo eritematoso acompañados de secreción hialina amarillenta en regiones acrales y cuero cabelludo. La biopsia cutánea reportó granulocitosis y aumento del espesor de la capa córnea; en los cultivos bacteriano y micológico de tejido dérmico creció flora habitual. La biopsia prostática diagnosticó adenocarcinoma prostático Gleason 4/5, Se inició hormonoterapia y se realizó prostatectomía total, con mejoría clínica significativa posterior al inicio del tratamiento. La acroqueratosis de Bazex es un proceso muy infrecuente, con pocos casos descritos en la literatura. Su reconocimiento temprano podría permitir el diagnóstico de la neoplasia asociada en estadios más precoces y conducentes a un tratamiento más temprano(AU)

The skin can show the first symptom in 1% of patients with internal neoplasias. Cutaneous manifestations of internal malignancies can be caused by direct invasion of the skin by the tumor and by metastatic dissemination, but there are indirect mechanisms that induce the appearance of cutaneous signs and symptoms unrelated to the primitive tumor. Bazex Syndrome is a rare dermatosis described by Bazex et al. in 1965. It is characterized by the presence of erythematous, psoriasiform plaques, which typically affect the hands, feet, nose, and ear-lobes. The lack of experience may carry diagnostic delays and the associated neoplasia could be at advanced stages when diagnosed. We describe here the case of a 53-year-old male patient, who consulted for cutaneous lesions.These were hypertrophic, hyperpigmented, scaly and painful plaques with an erythematous background accompanied by a yellowish hyaline secretion in the hands, feet and scalp. A skin biopsy was done that reported granulocytosis and increased thickness of the corneal layer; in addition, bacterial and mycological culture of dermal tissue were negative. The prostate biopsy diagnosed a prostate adenocarcinoma, Gleason 4/5. Hormone therapy was started and total prostatectomy was performed, with significant clinical improvement .Bazex acroqueratosis is a very rare process, with few cases described in the literature. Its early recognition could allow the diagnosis of the associated neoplasia in earlier stages, leading to earlier treatment(AU)

Phenotypic, molecular characterization, antimicrobial susceptibility and draft genome sequence of Corynebacterium argentoratense strains isolated from clinical samples.

During a 12-year period we isolated five Corynebacterium argentoratense strains identified by phenotypic methods, including the use of matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF) and 16S rRNA gene sequencing. In addition, antimicrobial susceptibility was determined, and genome sequencing for the detection of antibiotic resistance genes was performed. The organisms were isolated from blood and throat cultures and could be identified by all methods used. All strains were resistant to cotrimoxazole, and resistance to ß-lactams was partly present. Two strains were resistant to erythromycin and clindamycin. The draft genome sequences of theses isolates revealed the presence of the erm(X) resistance gene that is embedded in the genetic structure of the transposable element Tn5423. Although rarely reported as a human pathogen, C. argentoratense can be involved in bacteraemia and probably in other infections. Our results also show that horizontal transfer of genes responsible for antibiotic resistance is occurring in this species.

Genetic variation in SIRT1 affects susceptibility of lung squamous cell carcinomas in former uranium miners from the Colorado plateau.

Epidemiological studies of underground miners suggested that occupational exposure to radon causes lung cancer with squamous cell carcinoma (SCC) as the predominant histological type. However, the genetic determinants for susceptibility of radon-induced SCC in miners are unclear. Double-strand breaks induced by radioactive radon daughters are repaired primarily by non-homologous end joining (NHEJ) that is accompanied by the dynamic changes in surrounding chromatin, including nucleosome repositioning and histone modifications. Thus, a molecular epidemiological study was conducted to assess whether genetic variation in 16 genes involved in NHEJ and related histone modification affected susceptibility for SCC in radon-exposed former miners (267 SCC cases and 383 controls) from the Colorado plateau. A global association between genetic variation in the haplotype block where SIRT1 resides and the risk for SCC in miners (P = 0.003) was identified. Haplotype alleles tagged by the A allele of SIRT1 rs7097008 were associated with increased risk for SCC (odds ratio = 1.69, P = 8.2 × 10(-5)) and greater survival in SCC cases (hazard ratio = 0.79, P = 0.03) in miners. Functional validation of rs7097008 demonstrated that the A allele was associated with reduced gene expression in bronchial epithelial cells and compromised DNA repair capacity in peripheral lymphocytes. Together, these findings substantiate genetic variation in SIRT1 as a risk modifier for developing SCC in miners and suggest that SIRT1 may also play a tumor suppressor role in radon-induced cancer in miners.

The role of radiology coders and fee coordinators.

The coding process for radiology procedures is more than just looking at a report, writing in the codes and sending it on. In the radiology department, coders/fee coordinators play a key role in troubleshooting, since they are able to see the entire process from patient visit to billing. Vital to the billing operation is having competent and skilled coders who understand radiology from order to revenue. Responsibility for proper coding should rest with the radiology department, even when fee coordinators are paid from different sources. If other people are writing codes that can influence its bottom line, the department needs to know that coding is being done correctly. Thousands of dollars can be lost when the coding and billing process breaks down and incorrect coding is overlooked. A quality control program is vital so that mistakes are found and corrected prior to codes being entered. Insisting on accuracy also means finding and following up on errors through a troubleshooting system to prevent legal action. Codes must be able to look at reports, ask questions about inconsistencies and possible errors and compare the ordered procedure to what was actually done. Issues that affect proper coding are pointed out so that today's coders can be more watchful. Although it is labor intensive, it is important for coders to match reports with dictation from technologies and radiologists to ensure accuracy.

Coexistence of pemphigus vulgaris and ocular cicatricial pemphigoid.

We describe the case of a patient with both pemphigus vulgaris limited to the oral cavity and ocular cicatricial pemphigoid. The diagnoses were established by means of histopathologic examination and direct and indirect immunofluorescence studies and confirmed by immunoblot analysis of serum. Treatment with dapsone resulted in a prolonged remission of the ocular cicatricial pemphigoid. The pemphigus vulgaris has remained localized to the oral cavity and has responded to sublesional corticosteroid injections.

New light on old problem: population.

PIP: A central issue of the Third Asian and Pacific Population Conference, to be held in Sri Lanka in September 1982, is the need for full recognition on the part of developing countries that an integrated approach to population and development is essential. Such an approach requires the following: management of internal migration and urbanization; women's participation in development; and increased involvement in promoting family planning, family welfare, and family health programs. Of much concern is the fact that the ESCAP region's urban population is projected to grow from 660 million in 1980 to 1347 million by the year 2000. Much of this increase will occur in the largest cities. Unplanned urbanization and a growing population reservoir in rural areas will continue to spur movements to the cities, aggravating the urban malaise, yet migration is young people's only option if rural populations are to survive. These problems plague most governments. A recent conference held in Rome urged governments to formulate comprehensive population policies and programs, to specify goals on the rate of population growth and on the distribution of population between urban and rural areas, and to plan for the redistribution of population. The conference also recommended that cities pay attention to problems of the shantytown and slum dwellers who are principally rural migrants and the urban poor. In addition the region's corps of development planners and experts worry about the staggering increase in migration by 3rd world guest workers to the high income OPEC members in the Middle East and the industrialized market economy nations. The proper management of international migration should increase the reabsorptive capacity of sending countries to ensure the reintegration of returning off-loaded guest workers and should try to prevent the rural stagnation due to large scale migration by the young and the better educated from the countryside. Internal and international migration give women an opportunity to discover their great development potential. Yet, more generally, women breaking out of social structures that foster dependency and exploitation are in need of more than simple, single factor solutions. Emerging relationships between declining mortality levels and women's participation in development and economic activities will be explored at the Third Asian and Pacific Population Conference.^ieng

Morphogenesis of experimentally induced Arnold--Chiari malformation.

The administration of a single dose of vitamin A to pregnant hamsters, early during the morning of their 8th day of gestation, induces types I and II Arnold--Chiari malformation (ACM), as well as various types of axial skeletal-dysraphic disorders known to be associated with the human disease. This new model provides a means of carrying comparative studies between the axial skeletal defects and neurological anomalies of this complex developmental malformation with those which characterize the other induced disorders related to it. Study of this experimental model has demonstrated that the basichondrocranium of fetuses with ACM is shorter than normal and slightly elevated (lordotic) in relation to the axis of the vertebral column. The shortness of the basichondrocranium of these fetuses is caused by the underdevelopment of the occipital bone specially noticeable in its basal component (basioccipital). This basic defect has resulted in a short and small posterior cerebral fossa which is inadequate to contain the developing nervous structures of that region. The developing cerebellum is displaced downward to an anomalous position just above the foramen magnum; and, the developing medulla is compressed or crowded into the small posterior cerebral fossa of affected fetuses. The lordotic elevation of the basichondrocranium is also responsible for the reduction of the pontine flexure and the increased angle of the cervical flexure of the hindbrain found in these fetuses. All of these neurological anomalies, which are characteristic and diagnostic of clinical ACm as well, are considered here to be secondary to the axial skeletal defects rather than primary abnormalities, as is generally believed. The peculiar type of protrusion of the odontoid process into the cranial cavity found in fetuses with ACM, as well as in those with cranioschisis aperta and occulta, is also considered to be caused by the slight depression of the underdeveloped basioccipital and therefore, comparable to the so-called basilar impression often described in clinical ACM. This study has emphasized various developmental features which are closely related with the morphogenesis of ACM, including: the somitic origin of the occipital bone, and the late growth of the cerebellum which is predominantly postnatal in almost all experimental animals. It has been pointed out that some developmental defects involving the occipital bone and the caudal vertebral column, such as those which characterize ACM type II, may be more closely related than previously recognized. It has been also pointed out that the so-called cerebellar herniation into the cervical spinal canal described in the human disease represents a late addition to this disorder which is related to the relatively late growth of the cerebellum...

Cardiovascular abnormalities in thoracopagus twins.

A detailed anatomopathologic study of the various developmental malformations encountered in a set of female thoracopagus twins is presented. Special emphasis has been devoted to the analysis and description of the complex cardiovascular abnormalities associated with the conjoined heart and liver of this set of twins. The heart was conjoined with two sets of great arteries. The great arteries of one twin were d-malposed and originated from a single right ventricle; a normally placed aorta originated from a single left ventricle and the hypoplastic pulmonary trunk originated from a rudimentary outlet chamber of the other twin. One twin had two atria with the systemic veins returning to the right one and an anomalous pulmonary venous return terminating in the ductus venous of her liver. The other twin had a single right atrium plus an accessory atrial chamber to which both the pulmonary and the systemic veins returned. There were two umbilical arteries originating from the internal iliac arteries of one twin and two umbilical veins which fused into one as they entered into the ductus venous of the other twin. The cardiovascular abnormalities of our case have been compared with those encountered in 25 well-documented cases of thoracopagus twins previously reported in the literature. It was concluded that the majority of thoracopagus twins (approximately 75%) had conjoined heart with associated cardiovascular abnormalities making them unsuitable for surgical separation even at the cost of the life of one of them. In addition, an embryological interpretation concerning the possible origin of the cardiovascular abnormalities of our case is presented and discussed.

Developmental abnormalities of the occipital bone in human chondrodystrophies (achondroplasia and thanatophoric dwarfism).

Specific developmental malformations have been demonstrated in the occipital bone of two chondrodysplastic disorders (achondroplasia and thanatophoric dwarfism). Analysis of these malformations indicates that the occipital bone is primary affected in these disorders. In both cases, the endochondral-derived components of the occipital bone (the basioccipital, the two lateral parts, and the planum nuchale of the squama occipitalis) have failed to grow properly and are smaller and shorter than normal. On the other hand, the planum occipitalis of the squama, which derives from intramembranous ossification, is unaffected. In addition, the nature of these abnormalities indicates that the occipital synchondroses, together with the epiphyseal plates of other bones, are primarily affected in these two chondrodysplasias. The components of the occipital bone formed between the affected synchondroses failed to grow normally. The resulting malformation of the occipital bone is undoubtedly the cause of the shortening of the posterior cerebral fossa and of the considerable narrowing of the foramen magnum often described in these chondrodysplasias. It is postulated that growth disturbances between the affected occipital bone and the unaffected central nervous system results in the inadequacy of the posterior cerebral fossa and the foramen magnum to accommodate the growing brain. Consequently, compression of the brain at the posterior cerebral fossa or the foramen magnum levels could occur and thus lead to neurologic complications such as hydrocephalus and compression of the brain stem. It is suggested that the surgical removal of the fused posterior border of the lateral parts of the occipital bone (partial nuchalectomy) for the purpose of enlarging the narrow foramen magnum may be indicated in those chondrodysplastic children who develop these types of neurologic complications.

Kalinga-Apayao's BVM program.

PIP: In the poor, densely-populated north Luzon province of Kalinga-Apayao, the provincial government and the Philippine National Volunteer Service Coordinating Office jointly sponsor a program of para-medical service to remote areas called Barangay Volunteer Medic (BVM). Volunteers, who are generally "arbularyo," retired military personnel, high school dropouts and graduates and former girl scouts and boy scouts, work in their own free time, and receive a small monthly stipend. The volunteer's most important attribute is his spirit and his dedication to volunteerism. During their month-long training, BVMs are instructed in prescription and administration of medicine for ailments common among the Kalingas. Increasingly, the BVM has become involved in family planning education and dispensation, but traditional motivations to have large families are very strong.^ieng