Fine‐needle aspiration of the diffuse sclerosing variant of papillary thyroid carcinoma masked by florid lymphocytic thyroiditis; A potential pitfall: A case report and review of the literature

The diffuse sclerosing variant of papillary thyroid carcinoma (DSV–PTC) is a rare tumor with aggressive behavior that requires aggressive treatment. Despite characteristic clinical and histological features that easily permit diagnosis, pre‐operative fine‐needle aspiration cytology (FNAC) diagnosis is often challenging and thus delays diagnosis. We describe the cytological features of a case of DSV–PTC diagnosed by FNAC in a 30‐year‐old woman presenting with an ill‐defined mass in her neck lasting for 2 months. Ultrasonograpy revealed a heterogeneous enlargement of both thyroid lobes suspicious for a lymphoproliferative syndrome. Flow cytometry showed a suspect B‐lymphocyte population. FNAC showed in five out of six slides an overwhelming presence of slightly atypical monomorphic small lymphocytes. The remaining slide showed syncytial tissue fragments of follicular cells with nuclear enlargement and pleomorphism, irregular nuclear membrane, grooves with scattered intranuclear inclusions, squamous metaplastic epithelium, and abundant psammoma bodies. A diagnosis of DSV–PTC was rendered and confirmed by total thyroidectomy and lymph node dissection. Our report supports the possibility of obtaining a preoperative diagnosis of DSV–PTC by FNAC. In the case of diffuse thyroid enlargement, adequate sampling of the thyroid and the presence of the combination of features described in our case permitted the diagnosis of this PTC variant. Diagn. Cytopathol. 2009. © 2009 Wiley‐Liss, Inc.     

Neuropathological profile of long‐duration amyotrophic lateral sclerosis in military Veterans

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting both the upper and lower motor neurons. Although ALS typically leads to death within 3 to 5 years after initial symptom onset, approximately 10% of patients with ALS live more than 10 years after symptom onset. We set out to determine similarities and differences in clinical presentation and neuropathology in persons with ALS with long vs. those with standard duration. Participants were United States military Veterans with a pathologically confirmed diagnosis of ALS (n = 179), dichotomized into standard duration (<10 years) and long‐duration (≥10 years). The ALS Functional Rating Scale‐Revised (ALSFRS‐R) was administered at study entry and semi‐annually thereafter until death. Microglial density was determined in a subset of participants. long‐duration ALS occurred in 76 participants (42%) with a mean disease duration of 16.3 years (min/max = 10.1/42.2). Participants with long‐duration ALS were younger at disease onset (P = 0.002), had a slower initial ALS symptom progression on the ALSFRS‐R (P < 0.001) and took longer to diagnose (P < 0.002) than standard duration ALS. Pathologically, long‐duration ALS was associated with less frequent TDP‐43 pathology (P < 0.001). Upper motor neuron degeneration was similar; however, long‐duration ALS participants had less severe lower motor neuron degeneration at death (P < 0.001). In addition, the density of microglia was decreased in the corticospinal tract (P = 0.017) and spinal cord anterior horn (P = 0.009) in long‐duration ALS. Notably, many neuropathological markers of ALS were similar between the standard and long‐duration groups and there was no difference in the frequency of known ALS genetic mutations. These findings suggest that the lower motor neuron system is relatively spared in long‐duration ALS and that pathological progression is likely slowed by as yet unknown genetic and environmental modifiers.     

Epithelioid variant of gastrointestinal stromal tumor: Diagnosis by fine-needle aspiration

Epithelioid gastrointestinal stromal tumors (GISTs) may cause significant diagnostic confusion on fine-needle aspiration (FNA) with carcinomas, neuroendocrine tumors, and melanoma, particularly when metastatic. This study characterizes the cytologic features of nine cases of epithelioid GISTs that were obtained by computerized tomographic guidance in five, by endoscopic ultrasound in three, and from an excised liver tumor in one. Six cases presented as liver masses, one as a perisplenic mass, one as an abdominal mass, and one as a gastric mass. The aspirates revealed mainly single or small clusters of epithelioid cells with a moderate amount of granular to clear cytoplasm, small uniform nuclei with mild to marked nuclear envelope irregularities. Binucleation and intranuclear inclusions were frequent findings. Collagenous stroma was seen in most cases. In three cases, a neuroendocrine tumor was the initial diagnosis. Immunocytochemical staining for c-kit (CD117) was performed on cellblocks in six cases and was positive in five cases. On the subsequent surgical specimen, CD117 was positive in the c-kit-negative cytology case. The diagnosis of GIST should be considered in aspirates of the gastrointestinal tract, liver, mesentery, or abdominal wall mass lesions when epithelioid cells are the predominant cell type. Ancillary studies such as immunohistochemical stains are usually helpful in making a definitive diagnosis.     

Purification of ovocalyxin-32, a novel chicken eggshell matrix protein

The eggshell is a highly ordered structure resulting from the deposition of calcium carbonate and an organic matrix from the acellular uterine fluid. Characterization of the individual matrix components is necessary to determine their influence upon calcite crystal shape, size, and orientation during eggshell calcification. We have purified and sequenced a novel 32-kDa protein, ovocalyxin-32 (OCX-32), which is present at high levels in the uterine fluid during the terminal phase of eggshell formation, and is localized predominantly in the outer eggshell. Database searches identified expressed sequence tags (ESTs) whose alignment yielded the complete cDNA. OCX-32 protein possesses limited identity (32%) to two unrelated proteins: latexin, a carboxypeptidase inhibitor expressed in rat cerebral cortex and mast cells, and to a skin protein that is encoded by a retinoic acid receptor-responsive gene, TIG1. The timing of OCX-32 secretion into the uterine fluid suggests that it may play a role in the termination of mineral deposition.     

The health of the Roma people: a review of the published literature

BACKGROUND: The Roma people originated in northern India and have been known in Europe for nearly a thousand years. For much of that time they have been the subjects of discrimination and oppression, culminating in the extermination of half a million Roma in the Nazi death camps. While it is widely believed that the health of Roma people is often poorer than the majority population, these inequalities remain largely unresearched. METHODS: Published literature on the health of the Roma people was identified using Medline. Opinion pieces were excluded, as were papers relating to anthropometry and to genetic markers. The resultant papers were analysed by country of study and by disease type or care group. RESULTS: Some 70% of papers identified related to just three countries; Spain and the Czech and Slovak Republics. Much literature concentrates upon communicable disease or reproductive health. The limited evidence suggests increased morbidity from non-communicable disease, but there is little published on this topic. Evidence on health care, though fragmentary, suggests poorer access to health services and uptake of preventative care. DISCUSSION: Published research on the health needs of the Roma population is sparse. The topics that have received attention suggest a focus on concepts of contagion or social Darwinism, indicating a greater concern with the health needs of the majority populations with which they live. There is a need for both further research into the health of Roma people; with particular emphasis on non-communicable disease; and also for interventions that improve Roma health. Such research must, however, be handled with sensitivity, recognising the social and political context of the society concerned.     

Mechanical ventilation in preterm infants: neurosonographic and developmental studies.

Surviving preterm infants of less than 34 weeks' gestation who were selected on the basis of serial cranial ultrasonographic findings during their nursery course had repeated neurologic and developmental examinations during late infancy and early childhood that established the presence (n = 46) or absence (n = 205) of spastic forms of cerebral palsy. Of the 205 infants without cerebral palsy, 22 scored abnormally low on standardized developmental testing during early childhood. The need for mechanical ventilation beginning on the first day of life (n = 92) was significantly related to gestational age, birth weight, Apgar scores, patent ductus arteriosus, grade III/IV intracranial hemorrhage, large periventricular cysts, and the development of cerebral palsy. In the 192 mechanically ventilated infants, vaginal bleeding during the third trimester, low Apgar scores, and maximally low PCO2 values during the first 3 days of life were significantly related to large periventricular cysts (n = 41) and cerebral palsy (n = 43), but not to developmental delay in the absence of cerebral palsy (n = 18). The severity of intracranial hemorrhage in mechanically ventilated infants was significantly associated with gestational age and maximally low measurements of PCO2 and pH, but not with Apgar scores or maximally low measurements of PO2. Logistic regression analyses controlling for possible confounding variables disclosed that PCO2 values of less than 17 mm Hg during the first 3 days of life in mechanically ventilated infants were associated with a significantly increased risk of moderate to severe periventricular echodensity, large periventricular cysts, grade III/IV intracranial hemorrhage, and cerebral palsy. Neurosonographic abnormalities were highly predictive of cerebral palsy independent of PCO2 measurements.(ABSTRACT TRUNCATED AT 250 WORDS)     

Health policy-making in central and eastern Europe: lessons from the inaction on injuries?

The burden of disease due to injuries has elicited virtually no public health response in the countries of central and eastern Europe, even though injuries have long been a much greater problem in the east of Europe than in the west, with children especially affected. This paper seeks to identify factors that have inhibited policy development on this topic and to draw lessons for health policy development in this region more generally. Several factors emerge. Deaths from injuries have had low visibility. Data have not been assembled in a way that would facilitate identification of the burden of disease that they constitute. Those organizations responsible for public health, whether within government or at local level, were typically very weak with little capacity either to identify the nature and scale of threats to the health of their populations or to develop strategies to address them. There was uncertainty about ownership, with fragmentation of responsibility but no tradition of intersectoral working. Non-governmental organizations, which have placed injuries on the health policy agenda in the west, are weak or non-existent. International donors, who could have had a role, have focused on issues such as health care reform. This analysis provides a potential framework for examining policy responses, or lack thereof, to other health challenges in this region. It highlights the need for a better understanding of the potential for using available data, which, in turn, requires a major strengthening of capacity. However, in many countries, there is a need for new ways of working, involving a broadening of the sense of ownership, with clearly designated responsibilities but designed in ways that encourage rather than inhibit intersectoral action. There is also a need to develop non-governmental organizations that have sufficient capacity to undertake their own analyses and to place issues on the agenda.     

The use of an implicit standard for measuring discrimination thresholds

We measured thresholds for comparing the separation between lines, using either the method of constant stimuli (MCS) or the method of single stimuli (MSS). In the MCS an explicit standard is presented on each trial, whereas in the MSS the standard is the mean of the set. The thresholds for the MSS procedure were nearly identical to those with the MCS procedure, whether or not feedback was used. A statistical model is presented showing how the threshold error estimated by MSS varies according to the number of past stimuli used by the observer to calculate the mean of the set. If the model is an accurate representation of human processing, our observers were averaging over the last 10-20 trials to estimate the implicit standard. Our results show that the explicit standard in the MCS procedure is generally superfluous. Provided that the test range is small, and that the observer is given some practice trials, thresholds measured with MSS procedure are just as precise as those measured with the traditional MCS procedure.