RARE MALIGNANT FEMALE ADNEXAL TUMOR OF WOLFFIAN ORIGIN (FATWO) WITH MULTIPLE RELAPSES AND CHEMOTHERAPY REGIMENS.

CONTEXT: Female adnexal tumors of probable Wolffian origin (FATWO) represent very rare borderline ovarian tumors with low malignant potential. Only 15 cases of malignant FATWO are described in the current literature, among which, only 5 are reported as being recurrent. OBJECTIVE: Due to the rare presentation of the recurrence of the malignant FATWO and the few cases reported in the scientific database, there are no clear therapy recommendations. This paper should help practitioners to choose the best therapy approach. DESIGN: This paper presents the 6th case of malignant recurrent FATWO and will compare all the cases available in the literature. SUBJECTS AND METHODS: We present a review of the literature comparing the therapeutic approaches and outcomes of all the five cases of recurrent malignant FATWOs. Also, we introduce the case of a stage III Wolffian origin adnexal tumor with multiple recurrences appeared after 6 years of disease free interval. RESULTS: Our case presents the longest survival reported in the literature and underwent most surgical procedures of the recurrences and more than 4 lines of chemotherapy regimens. CONCLUSIONS: This paper shows possible therapeutic approaches to be used as example by the practitioners according to the drug availability in their centers.

A systematic approach on the frequency of cleft lip/palate in pediatric patients with leukemia.

PURPOSE: The purpose of this study was to reveal the frequency between cleft lip/palate and leukemia in pediatric patients by a systematic analysis of the current literature. MATERIALS AND METHODS: Electronic search on three database (PubMed, Web of Science, Cochrane) was carried out using the following keywords: cleft lip, cleft palate, facial cleft, oral cleft, orofacial cleft, leukemia, acute myeloid leukemia, acute lymphocytic leukemia, lymphoma. Studies published until March 2020 reporting an association between leukemia and cleft lip/palate (CL/P) were included in our research. RESULTS: Five articles (2 case-controls, 3 cohorts), met the inclusion criteria. Case-control studies involved 268 patients with acute lymphocytic leukemia (ALL) and 177 patients with acute myeloid leukemia (AML), of which 9 patients had CL/P. The cohorts studies involved 10 patients with ALL, of which 6 patients with CL/P, 2 patients with cleft palate and 1 patient with cleft lip and palate. CONCLUSION: This research was able to indicate a limited evidence of the association between CL/P and leukemia. In order to draw a clear conclusion, studies with larger cohorts are needed to establish this correlation.

Reduced volumes of the CA1 and CA4-dentate gyrus hippocampal subfields in systemic lupus erythematosus.

Introduction There is evidence for hippocampal dysfunctions in systemic lupus erythematosus (SLE), which may contribute to neuropsychiatric impairments. However, fine structural alterations of the hippocampus have not been investigated in SLE. Methods We measured the volume of hippocampal subfields in 18 SLE patients and 20 healthy control individuals matched for age, gender, and education. The MRI protocol included structural T1 volumes (Philips Achieva 3T scanner, magnetization-prepared rapid acquisition gradient echo (MPRAGE)). For image processing, we used the neuGRID platform and the longitudinal pipeline of FreeSurfer v6.0 with the "hipposubfields" flag. Results Patients with SLE showed reduced volumes of CA1 (Cornu Ammonis 1) and CA4-dentate gyrus subfields relative to the control individuals. Smaller CA1 volumes were associated with worse performance on the Addenbrooke's Cognitive Examination. Conclusions These preliminary results indicate a prominent vulnerability and functional relevance of the CA1 hippocampal subfield in SLE.

Targeted deletion of histidine decarboxylase gene in mice increases bone formation and protects against ovariectomy-induced bone loss.

Targeted disruption of the histidine decarboxylase gene (HDC(-/-)), the only histamine-synthesizing enzyme, led to a histamine-deficient mice characterized by undetectable tissue histamine levels, impaired gastric acid secretion, impaired passive cutaneous anaphylaxis, and decreased mast cell degranulation. We used this model to study the role of histamine in bone physiology. Compared with WT mice, HDC(-/-) mice receiving a histamine-free diet had increased bone mineral density, increased cortical bone thickness, higher rate of bone formation, and a marked decrease in osteoclasts. After ovariectomy, cortical and trabecular bone loss was reduced by 50% in HDC(-/-) mice compared with WT. Histamine deficiency protected the skeleton from osteoporosis directly, by inhibiting osteoclastogenesis, and indirectly, by increasing calcitriol synthesis. Quantitative RT-PCR showed elevated 25-hydroxyvitamin D-1alpha-hydroxylase and markedly decreased 25-hydroxyvitamin D-24-hydroxylase mRNA levels. Serum parameters confirming this indirect effect included elevated calcitriol, phosphorus, alkaline phosphatase, and receptor activator of NF-kappaB ligand concentrations, and suppressed parathyroid hormone concentrations in HDC(-/-) mice compared with WT mice. After ovariectomy, histamine-deficient mice were protected from bone loss by the combination of increased bone formation and reduced bone resorption.

Biostimulatory windows in low-intensity laser activation: lasers, scanners, and NASA's light-emitting diode array system.

OBJECTIVE: The purpose of this study was to assess and to formulate physically an irreducible set of irradiation parameters that could be relevant in the achieving reproducible light-induced effects in biological systems, both in vitro and in vivo. BACKGROUND DATA: Light-tissue interaction studies focusing on the evaluation of irradiation thresholds are basic for the extensively growing applications for medical lasers and related light-emitting systems. These thresholds are of central interest in the rejuvenation of collagens, photorefractive keratectomy, and wound healing. METHODS: There is ample evidence that the action of light in biological systems depends at least on two threshold parameters: the energy density and the intensity. Depending on the particular light delivery system coupled to an irradiation source, the mean energy density and the local intensity have to be determined separately using adequate experimental methods. RESULTS: From the observations of different research groups and our own observations, we conclude that the threshold parameters energy density and intensity are biologically independent from each other. CONCLUSIONS: This independence is of practical importance, at least for the medical application of photobiological effects achieved at low-energy density levels, accounting for the success and the failure in most of the cold laser uses since Mester's pioneering work.

The epidemiology and the pathogenesis of inflammatory bowel disease.

The etiology of inflammatory bowel disease (IBD) is still unknown. However, a satisfactory solution cannot be far away. IBD actually encompasses two diseases, i.e. Crohn's disease (CD) and ulcerous colitis (UC). These diseases resemble each other so closely that they cannot be distinguished even pathologically, but differ from each other sufficiently to regard them as independent entities. Epidemiological observations may be helpful in identifying the true causative factors of this evasive disease. Geographically, the prevalence of the disease has a slope from North to South and, to a lesser degree, from West to East. The Western-Eastern discrepancy can be attributed to a difference in Western life styles. The incidence of the disease has been increasing world-wide of late, but its spread has been slowing down in highly affected countries. Racial and ethnic relations in different populations and immigration studies offer interesting data which can reflect genetic, inherited, environmental and behavioural factors. The disease seems to have a characteristic racial-ethnic distribution: the Jewish population is highly susceptible everywhere, but its prevalence in that population nears that of the domestic society in which they live. In Hungary, the Roma (Gypsies) have a considerably lower prevalence than the average population. This can be attributed to a genetic or environmental influence. According to age, the onset of the disease occurs more often in the second or the third decade of life, but there also is another peak in the 60s. Regarding sexual distribution, there is a slight preponderance of colitis ulcerosa in men and of Crohn's disease in women. It may correspond to the stronger auto-immune affection in the process of Crohn's disease. Environmental factors and behavioural influences also are investigated. Diet, the role of the early ages, smoking habits and the influence of hormonal status and drugs are viewed as useful contributing factors in the manifestation of the disease. Genetic studies show that one-fourth of IBD patients have an affected family member. HLAB27 histocombatibility also plays an important, but not determining role in the development of the disease. Genetic factors seem to have a stronger influence in Crohn's disease than ulcerative colitis. The existence of multiple sclerosis-IBD families may reflect the common genetic background or the similar microbial effect as well. A great number of bacterial and viral factors has been suspected of being infectious factors in IBD, mostly in CD. Mycobacteria, Yersinia, Campylobacter, Clostridium, Clamidias, etc. as well as bacteria and some viruses such as herpes and rotavirus and the primary measles virus. None of them has been proven as a real and exclusively pathogenic factor. Immunological background has an important function in the manifestation of the disease. If an individual has a genetic susceptibility to infections, the down regulation of an inflammation in the bowel wall does not occur in a proper way. This initiates the auto-immune process which is a self-increasing cycle. Extra-intestinal manifestations of IBD are of high importance because they can not only follow intestinal symptoms, but precede them by years. Hepatic and biliary disturbances (primary sclerosing cholangitis), are the most serious complications. Mucocutaneous manifestations can be the first appearance of the main disease (in the mouth). Auto-immune consequences (erythema nodosum) or complications caused even by the therapy can occur. Ocular and musculoskeletal manifestations supposedly have the same genetic background and often precede the intestinal symptoms. Considering the epidemiological, genetic and immunological data, we can conclude that ulcerative colitis and Crohn's disease are heterogeneous disorders of mutifactorial etiology in which hereditary (genetic) and environmental (microbial, behaviour) factors interact to produce the disease.

Enteroclysis and spiral CT examination in diagnosis and evaluation of small bowel Crohn's disease.

INTRODUCTION: During the last few decades introducing many of new radiologic methods, diagnostic conditions and facilities of Crohn's disease has became markedly improved. Appropriate using of these technics definitely modifies the management of patients with known or suspected Crohn's disease serving reliable information about extent, severity and possible complications of disease. Enteroclysis and Computed tomography are the two major and basic methods to disclose or confirme diagnosis of Crohn's disease, obtain appropriate inforination about disease either with mucosal, transmural or extraintestinal manifestation. METHODS AND PATIENTS: We evaluated 281 patients who were referred in our institution under suspition of Crohn's disease. Enteroclysis and abdominal spiral CT in all cases were carried out usually within 1 week. The 172 patients underwent abdominal spiral CT as the primary examination to evaluate diagnostic value of spiral CT in this entity, while 109 patiens had enteroclysis followed by abdominal CT. In 11 cases we also perforined CT enteroclysis with administration of 0.5% methylcellulose solution thorough nasojejunal tube controlled by electric motor driven contrast pump. Results were compared with final clinical, pathological or surgical data were available. RESULTS: From the 281 patients eventually 74 proved Crohn's disease; sensitivity and specificity of enteroclysis proved to be 96 and 98%, while spiral CT sensitivity and specificity was 94 and 95%, respectively. Enteroclysis was superior to the spiral CT in demonstration of early lesions and functional disorders, while spiral CT proved to be essential in evaluation of transmural and extraintestinal complications. CONCLUSIONS: Regarding enteroclysis and spiral CT as complementary methods, they provide excellent results in diagnosis of Crohn's disease.

Ultrasound in Crohn's disease of the small bowel.

OBJECTIVE: The purpose of this work is to prospectively evaluate high resolution ultrasonography with graded compression in the ability to detect Crohn's disease of the small bowel (CDSB) together with its complications and activity signs, compared with enteroclysis, CT and immunoscintigraphy in the mirror of the final diagnosis. METHODS AND MATERIAL: In a series of 73 consecutive patients, who were referred for enteroclysis with suspected Crohn's disease of the small bowel computed tomography (CT), ultrasound (US), immunoscintigraphy with 99mTc labeled monoclonal antigranulocyte antibody (AGAb) examinations were performed within 10 days from each other. For the final evaluation the diagnosis of CDSB was based on combination of clinical and enteroclysis findings (73 cases) and in 17 cases additional surgical and pathological data were available. The results of other modalities were blinded to the radiologists performing and reading out the exams. The diagnostic values of each modality was assessed also in those 18 patients, who had early Crohn's disease. In the group of 43 patients with proven CDSB who had all the four imaging modalities, the modalities were compared in their ability to demonstrate various pathological conditions related to CD. Increased (>500 ml/min) flow measured by Doppler US in the superior mesenteric artery and increased color signs in the gut wall seen by power Doppler sonography were compared to CDAI. RESULTS: Of the 73 patients the combination of enteroclysis and clinical tests demonstrated CDSB in 47. The sensitivity, specificity and accuracy of ultrasound were 88.4, 93.3 and 90.4%, respectively. Enteroclysis was the most accurate method. CT was more sensitive than US, but less specific. The accuracy of US, CT and scintigraphy were similar. In the group of 18 patients, who had early CDSB, the sensitivity of US decreased to only 67%, CT and scintigraphy had higher values. Intra- and perimural abscesses, and sinus tracts were also more frequently visualized by US, especially if they were small. US was superior than CT in detecting stenoses and skip lesions, but inferior to enteroclysis. US and CT detected more fistulas, than enteroclysis. Compared to CT, US detected more cases with mesenteric lymphadenopathy, equal cases with abscesses and free peritoneal fluids. In detecting mesenteric inflammatory proliferation CT, and in detecting colonic involvement CT and immunoscintigraphy were slightly superior than graded compression US. Patterns of mural stratification detected by ultrasound correlated well with the enteroclysis severity stages. There was only 59% agreement between increased superior mesenteric artery flow detected by Doppler sonography and CDAI, and 60.5% agreement between increased number of Color pixels in the gut wall measured by power Doppler and increased CDAI. CONCLUSION: High resolution graded compression sonography is a valuable tool for detecting small intestinal Crohn's disease. It has similar diagnostic values as CT. However in early disease the sensitivity substantially decreases. In known Crohn's disease for following disease course, evaluating relapses and extramural manifestations US is an excellent tool. Doppler and Power Doppler activity measurements do not correlate well with the more widespread clinical activity index.

The role of nuclear medicine in inflammatory bowel disease. A review with experiences of aspecific bowel activity using immunoscintigraphy with 99mTc anti-granulocyte antibodies.

UNLABELLED: The diagnosis of inflammatory bowel disease (IBD) needs a complex diagnostic work-up. Beside verifying the disease itself, it is fundamental to assess disease extent and activity and to detect associated complications, to find the most effective treatment and for follow up. Scintigraphy with radiolabelled leukocytes is able to provide a complete survey of the whole intestinal tract, both the small and large bowel, and detects septic complications successfully with negligible risk. Radionuclide procedures are useful in establishing or ruling out IBD in patients with intestinal complaints, in assessing disease severity, and in the evaluation of extraintestinal septic complications. Widely available radionuclide procedures are discussed, i.e. scintigraphy by 111Indium oxime or 99mTechnetium HMPAO labelled white blood cells and immunoscintigraphy with 99mTc anti-granulocyte antibodies. Advantages and disadvantages of all three methods are stressed out. PATIENTS AND METHODS: The immunoscintigraphies with 99mTc anti-granulocyte antibodies (ANTI-GRANULOCYTE(R) BW 250/183) of 27 patients with suspicion of IBD were retrospectively analysed. Planar anterior and posterior images were obtained 4 and 24 h postinjection, respectively. The bowel was divided into six segments and the activity was visually graded with reference to bone marrow in each segments. The scans were compared with the results of radiological and endoscopical investigations. The diagnosis of IBD was proved or ruled out by means of enteroclysis, large bowel enema or endoscopy. RESULTS: In the 27 patients, 74 bowel segments with increased activity were detected. In the case of 30 segments in 16 patients, bowel inflammation was revealed by the other methods (true positives). In the case of 44 bowel segments, no underlying bowel inflammation could be verified, and these activities were regarded as aspecific activity. We could not differentiate between true positive and aspecific activity based on scan pattern or intensity. DISCUSSION: These findings of aspecific bowel activity using imuunoscintigraphy are in contrast with the results of former studies, while the existence of non-specific activity decreases the reliability of the method. Based on the literature and our experiences, we conclude that 99mTc HMPAO labelling should be the method of choice for the investigation of IBD patients.

Spiral CT colonography in inflammatory bowel disease.

OBJECTIVE: Most of the studies on virtual colonoscopy are dealing with the role of detecting colorectal polyps or neoplasms. We have undertaken this study to evaluate the value of CT colonography in patients with colonic Crohn's disease. METHODS AND MATERIAL: Five patients (three males, two females, 23-51 years, mean age 42 years) with known (4) or suspected (1) Crohn's disease of the colon underwent fiberoptic colonoscopy and CT colonography in the same day or during a 1-week period. The images were evaluated with the so called zoomed axial slice movie technique and in some regions intra- and extraluminal surface shaded and volume rendered images were generated on a separate workstation. The results were compared to those of a colonoscopy. RESULTS: The final diagnosis was Crohn's disease in four patients and colitis ulcerosa in one. Total examination was possible by colonoscopy in two cases, and with CT colonography in all five cases. The wall of those segments severely affected by the disease were depicted by the axial CT scans to be thickened. The thick walled, segments with narrow lumen seen on CT colonography corresponded to the regions where colonoscopy was failed to pass. Air filled sinus tracts, thickening of the wall of the terminal ileum, loss of haustration pseudopolyps and deep ulcers were seen in CT colonography. Three dimensional (3D) endoluminal views demonstrated pseudopolyps similar to endoscopic images None of the colonoscopically reported shallow ulcerations or aphtoid ulcerations or granular mucosal surface were observed on 2- or 3D CT colonographic images. CONCLUSION: CT colonography by depicting colonic wall thickening seems to be a useful tool in the diagnosis of Crohn's colitis, which could be a single examination depicting the intraluminal, and transmural extent of the disease.

Enteropathic arthritis in the sacroiliac joint. Imaging and differential diagnosis.

OBJECTIVES: A new high resolution computed tomography (HRCT) scoring system of sacroiliac joint (SIJ) involvement in enteropathic arthritis is introduced. PATIENTS AND METHODS: SIJ's of 100 patients were studied. A total of 25 patients presented with pain syndrome, 25 with suspicious seronegative spondylarthritis, 25 with inflammatory bowel diseases and 25 without joint or bowel diseases, as a control group. HRCT was carried out in all 100 patients. For comparison, a plain film radiography (PFR), conventional CT (slices of 10 mm) and bone scan were used. RESULTS: Quantitative differences: In the pain syndrome group, there were no erosions identified neither intraarticular calcifications. Disc degeneration was seen in 12/25 cases. In 4/25 patients, vacuum phenomena appeared in the SIJ. In 3/25 patients, ventral capsular calcification occurred in the ventral sacroiliac ligament (anterior capsule complex). In the seronegative spondylarthritis group, 16/25 patients had positive findings, while PFR documented erosions only in 3/25 cases. In the bowel diseases group, erosions were detected in 17/25 cases with HRCT, while the plain film was positive only in three cases and in seven cases the findings were questionable. Intraarticular calcification with erosion was documented in three cases and in seven cases without erosion. The bone scan was positive in 7/25 of this cases, but in 5/7 there was mismatching with HRCT. Important new finding was the HRCT detected erosion which was not detected on BS but was obvious on Anti-Granulocyte-Antibody scintigraphy. In the control group, only degenerative changes were seen in 4/25 cases and no erosions. CONCLUSION: HRCT is: (1) the reliable imaging of definitive (often 'cold stage') sacroileitis; (2) gives optimal detection of erosion; and (3) appears to be the only method in the documentation of calcifications in the posterior ligamental portion of the SIJ.

[High-resolution computer tomography of diffuse infiltrative lung diseases]. / Diffúz infiltratív tüdöbetegségek nagy felbontóképességP6 komputertomográfiás vizsgálata.

During a period of 17 months, a total of 101 patients were examined by high resolution computed tomography (HRCT) whose minor focal changes revealed by chest X-ray raised the suspicion of diffuse infiltrative pulmonary disease. The disease of the interstitium was primarily caused by idiopathic pulmonary fibrosis (19%), autoimmune disease (18%), Boeck sarcoidosis (18%) as well as by specific (16%) and aspecific inflammation (12%). HRCT disclosed reticular (61%), nodular (55%), ground-glass opacity-like (48%), emphysematous (33%) and ring-like changes (17%). Also transbronchial excision was performed in 51 patients the results of which were compared to the changes observed during HRCT. Concerning fibrosis and inflammation, HRCT and histological analysis showed identical results in 70%. According to the authors observations, this technique is suitable for detection of fairly fine fibrotic, empysematous and inflammatory signs and for defining the activity of the individual disease as also for the selection of the optimal site of transbronchial excision. HRCT is a useful investigative method both for diagnosing diffuse infiltrative pulmonary disease as well as in following up the effectiveness of treatment.

Olfactory function in young adolescents with Down's syndrome.

Decreased ability to smell is present in adults with Down's syndrome, many of whom are known to have brain pathology analogous to that seen in Alzheimer's disease. Because olfactory loss is well documented in Alzheimer's disease, the question arises whether young adolescents with Down's syndrome, who have no clear Alzheimer's disease-like neuropathology, also exhibit olfactory dysfunction. To consider this issue, standardised tests of odour discrimination and identification were administered to 20 young adolescents with Down's syndrome (mean age (SD) 13.89 (1.98) years) and their test scores were compared with 20 mentally retarded and 20 non-mentally retarded control subjects matched to the patients with Down's syndrome on the basis of cognitive ability. No significant differences in olfactory function were found among the three study groups. These findings, along with those from studies of olfactory function in older patients with Down's syndrome, suggest that Down's syndrome related olfactory dysfunction occurs only at ages when Alzheimer's disease-like pathology is present.

Radiological aspects of hard metal disease.

The results of 30 years screening of exposed workers in the hard metal industry are presented in a retrospective study. There were 11 workers who had radiographic signs of pulmonary fibrosis. No correlation was found between intensity and/or duration of exposure and the stage and progression of pulmonary fibrosis. In 45% of the cases there was progression of the fibrosis after cessation of the exposure. Steroid therapy in three cases relieved the subjective symptoms but there was no regression of the fibrosis.

[The day-care unit as a framework for successful community rehabilitation].

Due to rapid advances in psychiatric treatment, increased sophistication of welfare services, and increasing life expectancy, the number of psychiatric patients is increasing rapidly. Many of them do not have the necessary adaptive capacity to deal satisfactorily with their daily functions, such as housework, financial arrangements, and medical treatment. These patients could be assisted in day-care treatment units. We present the ideology and the different models of psychiatric day-care units which are becoming widely used in treatment, especially for rehabilitation of chronic mental patients.

Photochemical effects of laser irradiation on neuritic outgrowth of olfactory neuroepithelial explants.

The photochemical effect of low-intensity laser irradiation (LILI) on the maturation and regeneration of olfactory-immature estrus day 15 (E15) and olfactory-mature estrus day 22 (E22) rat fetuses was studied. Neuritic outgrowths of olfactory bipolar receptor cells were quantified in olfactory neuroepithelial explants. Explants in the experimental groups were irradiated with a helium-neon laser using different incident energy densities (IEDs). Explants in another group were exposed to fluorescent light. Control explants did not receive laser or fluorescent light irradiation. Neuritic outgrowths were analyzed on a regular basis for 12 days. Analysis of variance was used to evaluate the data. The parameters of neuritic outgrowth in E15 fetuses showed a significant increase of 30% to 50% vs. the control with a single laser irradiation of 0.5 J/cm2 IED. The rate of neuritic outgrowth observed in the E22 fetuses was less than in the E15 fetuses. The parameters of neuritic outgrowth in E22 fetuses showed a significant and substantially greater percentage increase than in the E15 fetuses with daily laser irradiations of 0.05 and 0.5 J/cm2 IED when compared to the control. The magnitude of these increases appears to be of biological significance as well as statistical significance.

Smell and taste disorders, a study of 750 patients from the University of Pennsylvania Smell and Taste Center.

Smell and taste disorders are common in the general population, yet little is known about their nature or cause. This article describes a study of 750 patients with complaints of abnormal smell or taste perception from the University of Pennsylvania Smell and Taste Center, Philadelphia. Major findings suggest that: chemosensory dysfunction influences quality of life; complaints of taste loss usually reflect loss of smell function; upper respiratory infection, head trauma, and chronic nasal and paranasal sinus disease are the most common causes of the diminution of the sense of smell, with head trauma having the greatest loss; depression frequently accompanies chemosensory distortion; low body weight accompanies burning mouth syndrome; estrogens protect against loss of the sense of smell in postmenopausal women; zinc therapy may provide no benefit to patients with chemosensory dysfunction; and thyroid hormone function is associated with oral sensory distortion. The findings are discussed in relation to management of patients with chemosensory disturbances.

[The radiation reaction of the lung following the treatment of breast carcinoma with telecesium--the correlation with the dose-time relationship]. / Die Strahlenreaktion der Lunge nach Behandlung des Mammakarzinoms mit Telecaesium--Zusammenhang mit der Dosis-Zeit-Beziehung.

51 mamma carcinoma patients underwent postoperative telecaesium irradiation (50 Gy). Chest radiograms were taken preceding the treatment and following it by eight to ten weeks. By reason of the results it can be supposed, that the unavoidable lung reaction due to radiotherapy can be decreased and partly avoided by adequate fractionation.