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While the long-term evolution of disease behavior in Crohn's disease has been well described in the pre-anti-TNF era, our knowledge thereon remains scarce after the introduction of anti-TNF.Our investigation examined the long-term evolution of disease concerning Montreal classification's B-stages over time in patients enrolled into the Swiss IBD Cohort Study between 2006 and 2017.We analyzed prospectively collected SIBDCS data using a Markov model and multivariate testing for effects of treatment and other confounders on B-stage migration over time. The primary outcome was a transition in disease behavior from B1 to either B2 or pB3, or from B2 to pB3, respectively.The 10- and 15-year probability of remaining in B1 was 0.61 and 0.48, as opposed to a probability to migrate to B2 or B3 of 0.25 or 0.14, and 0.32 or 0.2, after 10 and 15 years, respectively. In multivariate testing, the hazard ratio for migrating from B1 to pB3 (HR 0.27) and from B2 to pB3 (HR 0.12) was lower in patients 40 years compared to patients 17 years. We found that immunosuppression (HR 0.38) and treatment with anti-TNF for 1 year (HR 0.30) were associated with a decreased likelihood of transitioning from stage B1 to pB3.While in the anti-TNF era most patients with Crohn's disease will eventually develop stricturing and/or penetrating complications, our data indicate that immunosuppressive and anti-TNF treatment for more than 1 year reduce the risk of transitioning from stage B1 to pB3 in the long-term run.

Focal segmental glomerulosclerosis (FSGS) is a major cause of familial nephrotic syndrome. We generated 20 induced pluripotent stem cell lines from patients diagnosed with FSGS. The iPSC lines include 8 female and 12 male lines and cover a donor age range from 31 to 78. The lines were generated from peripheral blood mononuclear cells by integration-free reprogramming using Sendai virus vectors. Cell lines were fully characterized regarding their pluripotency and differentiation potential, and quality controlled for karyotypic integrity, identity and clearance of reprogramming vectors. The generated cell lines represent a valuable tool for disease modelling and drug development for FSGS.

MIRAGE syndrome is a multisystem disorder caused by mutations in SAMD9 (sterile α motif domain-containing protein 9) with a high mortality in the first decade of life. We generated 2 human induced pluripotent stem cell lines from male children diagnosed with MIRAGE syndrome. The cell lines were generated from fibroblasts by integration-free reprogramming using the Sendai virus. Both cell lines were fully characterized regarding their pluripotent identity and differentiation potential, and quality controlled for karyotypic integrity, cell line identity and clearance of reprogramming vectors. The generated cell lines represent a valuable tool to study the disease mechanism of MIRAGE syndrome.

Background: The complement system activation and regulation have been linked to post-transplant pathologies including chronic antibody mediated rejection (cAMR) and the recurrence of IgA nephropathy (ReIgAN) but distinct mechanisms remain to be elucidated.Methods: In this retrospective single center study, the outcome of kidney transplantation was studied in 150 patients with late histological diagnosis to be either cAMR or ReIgAN, 14 stable kidney grafts at 3 months and finally 11 patients with native kidney IgAN nephropathy. To study a role of complement cascade and regulation in cAMR and ReIgAN, the RNA was extracted from available frozen kidney biopsy samples and using RT-qPCR transcripts of 11 target genes along with clinical data were determined and compared with stable grafts at 3 months protocol biopsies or IgAN native kidney nephropathy. Immunohistologically, CD46 (MCP), and C5 proteins were stained in biopsies.Results: Interestingly, there were no differences in kidney graft survival between cAMR and ReIgAN since transplantation. cAMR was associated with significantly higher intragraft transcripts of C3, CD59, and C1-INH as compared to ReIgAN (p < 0.05). When compared to normal stable grafts, cAMR grafts exhibited higher C3, CD55, CD59, CFH, CFI, and C1-INH (p < 0.01). Moreover, ReIgAN was associated with the increase of CD46, CD55, CD59 (p < 0.01), and CFI (p < 0.05) transcripts compared with native kidney IgAN. Rapid progression of cAMR (failure at 2 years after biopsy) was observed in patients with lower intrarenal CD55 expression (AUC 0.77, 78.6% sensitivity, and 72.7 specificity). There was highly significant association of several complement intrarenal transcripts and the degree of CKD regardless the diagnosis; C3, CD55, CFH, CFI, and C1-INH expressions positively correlated with eGFR (for all p < 0.001). Neither the low mRNA transcripts nor the high mRNA transcripts biopsies were associated with distinct trend in MCP or C5 proteins staining.Conclusions: The intrarenal complement system transcripts are upregulated in progressively deteriorated kidney allografts.

Ceftaroline is the first member of a novel class of cephalosporins approved for use in the United States. Although prior studies have identified eight ceftaroline-resistant methicillin-resistant Staphylococcus aureus (MRSA) isolates in Europe and Asia with MICs ranging from 4 to 8 mg/liter, high-level resistance to ceftaroline (>32 mg/liter) has not been described in MRSA strains isolated in the United States. We isolated a ceftaroline-resistant (MIC > 32 mg/liter) MRSA strain from the blood of a cystic fibrosis patient and five MRSA strains from the respiratory tract of this patient. Whole-genome sequencing identified two amino acid-altering mutations uniquely present in the ceftaroline-binding pocket of the transpeptidase region of penicillin-binding protein 2a (PBP2a) in ceftaroline-resistant isolates. Biochemical analyses and the study of isogenic mutant strains confirmed that these changes caused ceftaroline resistance. Thus, we identified the molecular mechanism of ceftaroline resistance in the first MRSA strain with high-level ceftaroline resistance isolated in the United States.

An intervention for Gram-negative bloodstream infections that integrated mass spectrometry technology for rapid diagnosis with antimicrobial stewardship oversight significantly improved patient outcomes and reduced hospital costs. As antibiotic resistance rates continue to grow at an alarming speed, the current study was undertaken to assess the impact of this intervention in a challenging patient population with bloodstream infections caused by antibiotic-resistant Gram-negative bacteria.A total of 153 patients with antibiotic-resistant Gram-negative bacteremia hospitalized prior to the study intervention were compared to 112 patients treated post-implementation. Outcomes assessed included time to optimal antibiotic therapy, time to active treatment when inactive, hospital and intensive care unit length of stay, all-cause 30-day mortality, and total hospital expenditures.Integrating rapid diagnostics with antimicrobial stewardship improved time to optimal antibiotic therapy (80.9 h in the pre-intervention period versus 23.2 h in the intervention period, P0.001) and effective antibiotic therapy (89.7 h versus 32 h, P0.001). Patients in the pre-intervention period had increased duration of hospitalization compared to those in the intervention period (23.3 days versus 15.3 days, P = 0.0001) and longer intensive care unit length of stay (16 days versus 10.7 days, P = 0.008). Mortality among patients during the intervention period was lower (21% versus 8.9%, P = 0.01) and our study intervention remained a significant predictor of survival (OR, 0.3; 95% confidence interval [CI], 0.12-0.79) after multivariate logistic regression. Mean hospital costs for each inpatient survivor were reduced $26,298 in the intervention cohort resulting in an estimated annual cost savings of $2.4 million (P = 0.002).Integration of rapid identification and susceptibility techniques with antimicrobial stewardship resulted in significant improvements in clinical and financial outcomes for patients with bloodstream infections caused by antibiotic-resistant Gram-negatives. The intervention decreased hospital and intensive care unit length of stay, total hospital costs, and reduced all-cause 30-day mortality.

Secondary hyperparathyroidism is a well-known complication of end-stage renal disease (ESRD). Both nodular and diffuse parathyroid hyperplasia occur in ESRD patients. However, their distinct molecular mechanisms remain poorly understood. Parathyroid tissue obtained from ESRD patients who had undergone parathyroidectomy was used for Illumina transcriptome screening and subsequently for discriminatory gene analysis, pathway mapping, and gene annotation enrichment analysis. Results were further validated using quantitative RT-PCR on the independent larger cohort. Microarray screening proved homogeneity of gene transcripts in hemodialysis patients compared with the transplant cohort and primary hyperparathyroidism; therefore, further experiments were performed in hemodialysis patients only. Enrichment analysis conducted on 485 differentially expressed genes between nodular and diffuse parathyroid hyperplasia revealed highly significant differences in Gene Ontology terms and the Kyoto Encyclopedia of Genes and Genomes database in ribosome structure ( P = 3.70 × 10−18). Next, quantitative RT-PCR validation of the top differently expressed genes from microarray analysis proved higher expression of RAN guanine nucleotide release factor (RANGRF; P < 0.001), calcyclin-binding protein ( CACYBP; P < 0.05), and exocyst complex component 8 ( EXOC8; P < 0.05) and lower expression of peptidylprolyl cis/ trans-isomerase and NIMA-interacting 1 (PIN1; P < 0.01) mRNA in nodular hyperplasia. Multivariate analysis revealed higher RANGRF and lower PIN1 expression along with parathyroid weight to be associated with nodular hyperplasia. In conclusion, our study suggests the RANGRF transcript, which controls RNA metabolism, to be likely involved in pathways associated with the switch to nodular parathyroid growth. This transcript, along with PIN1 transcript, which influences parathyroid hormone secretion, may represent new therapeutical targets to cure secondary hyperparathyroidism.

Context.—Slow-growing nonchromogenic mycobacterial species are an infrequent cause of soft tissue infection. Because these organisms are rare, they are not often initially considered in the differential diagnosis of synovitis. Objective.—To evaluate the clinical and pathologic characteristics of patients with synovitis resulting from slow-growing nonchromogenic mycobacterial species. Design.—A 20-year retrospective review of records from The Methodist Hospital Microbiology Laboratory identified 18 culture-positive cases of synovitis that resulted from slow-growing nonchromogenic mycobacteria, including 14 caused by Mycobacterium avium complex, 1 caused by Mycobacterium malmoense, 1 caused by Mycobacterium haemophilum, and 2 caused by Mycobacterium nonchromogenicum isolates. In addition, a comprehensive literature search revealed an additional 48 cases of synovitis caused by slow-growing nonchromogenic mycobacteria. Results.—The historic literature described the majority of the 48 patients as previously healthy, elderly individuals with a several-month history of monoarticular pain and swelling in the small joints of the upper extremity. In contrast, the current series demonstrated the probable role of multiple chronic coexisting medical conditions in promoting disease susceptibility. These patients were also unique in their significantly younger age distribution and diversity of infection sites. Histologic examination and direct acid-fast bacteria stains generally did not aid the diagnosis. Amputation was performed in 2 patients because of delayed identification of disease. Conclusions.—The current series demonstrates that difficult identification and infrequent occurrence cause these organisms to be overlooked by physicians and laboratory personnel. A heightened clinical suspicion for slow-growing nonchromogenic mycobacterial species is necessary when routine culture and histopathologic findings do not readily isolate an organism, or when the patient does not respond to antibiotic and anti-inflammatory treatment.

Fungi are important etiologic agents of sinusitis. However, features of fungal sinusitis including the histologic spectrum, diagnostic mishaps, incidence, and fungal types have not been systematically studied. From 1996 through 2001, a total of 788 surgical pathology sinus specimens from 384 cases was retrieved. Fungal sinusitis was diagnosed in 58 specimens (7%) from 47 cases (12%). Four histologic categories of fungal sinusitis were identified: (1) allergic fungal sinusitis in 34 cases (copious mucin, abundant eosinophils, Charcot-Leyden crystals (so-called allergic mucin), with rare noninvasive fungal hyphae); (2) mycetoma/fungus ball in 11 cases (tightly packed fungal hyphae without allergic mucin or tissue invasion); (3) chronic invasive fungal sinusitis in 1 case (tissue granulomas with fungal hyphae); and (4) acute fulminant fungal sinusitis in 1 case (fungal vascular invasion). The diagnosis was initially missed in 16/34 (47%) cases of allergic fungal sinusitis despite typical features; incorrect classification was noted in 47% of cases. Sixty-seven percent of cases had positive fungal cultures, dematiaceous fungi being the most common. Allergic fungal sinusitis accounted for the majority of fungal sinusitis. Although misdiagnosis or incorrect classification is rather frequent for fungal sinusitis, awareness of the distinctive morphologic features of this entity may prevent these errors.

Pre-operative embolisation of metastatic spinal tumours has the potential to decrease intra-operative blood loss. Intra-operative blood loss is multifactorial and one factor may be the embolisation technique used. The purpose of this study was to retrospectively analyse the effect of three different pre-operative embolisation techniques on intra-operative blood loss, complication rate and tumour aetiology in patients treated with a corpectomy and dorsoventral stabilisation at our institution.We conducted a retrospective analysis of embolisation procedures for vertebral metastases performed from January 2002 to December 2011. Only pre-operatively embolised patients treated by a single-level hemicorpectomy or corpectomy procedure from T4-L5, including posterior spinal stabilisation using pedicle screws, were included. All patient charts and examinations were analysed regarding the embolisation technique, gender, age, primary tumour, time between the embolisation and surgery, intra-operative blood loss, intra-operative transfusions and complications related to embolisation.We identified a total of 46 patients, 25 male and 21 female patients. The mean age at the time of surgery was 66 years (range 39-84 years). The tumours treated were: 15 (33%) renal cell carcinomas, six (13%) breast carcinomas, five (11%) lung carcinomas, five (11%) urothelial carcinomas, four (9%) myelomas and 11 (24%) miscellaneous types including rectal carcinoma, thymoma and melanoma. Embolisation with coils was performed in 23 patients, particles were used in six and a combination of coils and particles in 18. The mean time between the embolisation and surgery was 23 hours (range 80-4,430 minutes). The median overall intra-operative estimated blood loss (EBL) was 2,300 ml (range 500-15,000 ml). In patients embolised with coils and particles, EBL was 2,200 ml compared to 1,450 ml in patients embolised with particles and 2,500 ml in the coil group. No statistically significant differences between the three groups could be detected. There were no complications related to the embolisation techniques.Pre-operative embolisation of spinal metastases using coils, particles or a combination of both is a safe and reproducible procedure. In our cohort we reported no complications during the three different embolisation techniques. No statistically significant difference regarding blood loss between the three embolisation techniques could be detected. Our data confirm existing studies concerning the control of intra-operative blood loss using different embolisation techniques. The benefit of embolisation with a combination of coils and particles compared to embolisation with particles only is questionable.

The dematiaceous fungi appear to be an increasing cause of human disease. At The Methodist Hospital, in Houston, Texas, five cases of serious disease caused by these fungi occurred between 1987 and 1992. Cerebral abscesses with Xylohypha bantiana followed treatment for lymphoma. An infection of the lower extremity with Exophiala jeanselmei var. castellanii followed cardiac surgery. Peritoneal growth of Alternaria tenuissima was a complication of peritoneal dialysis. Cerebral abscesses with Dactylaria gallopava occurred in a liver transplantation patient. A traumatic ankle wound contaminated with dirt led to an infection with Phialophora repens. All patients except the last were immunocompromised at the time of the infection; diabetics and patients on steroids may be at particular risk.

Context.—Early diagnosis of gram-negative bloodstream infections, prompt identification of the infecting organism, and appropriate antibiotic therapy improve patient care outcomes and decrease health care expenditures. In an era of increasing antimicrobial resistance, methods to acquire and rapidly translate critical results into timely therapies for gram-negative bloodstream infections are needed. Objective.—To determine whether mass spectrometry technology coupled with antimicrobial stewardship provides a substantially improved alternative to conventional laboratory methods. Design.—An evidence-based intervention that integrated matrix-assisted laser desorption and ionization time-of-flight mass spectrometry, rapid antimicrobial susceptibility testing, and near–real-time antimicrobial stewardship practices was implemented. Outcomes in patients hospitalized prior to initiation of the study intervention were compared to those in patients treated after implementation. Differences in length of hospitalization and hospital costs were assessed in survivors. Results.—The mean hospital length of stay in the preintervention group survivors (n = 100) was 11.9 versus 9.3 days in the intervention group (n = 101; P = .01). After multivariate analysis, factors independently associated with decreased length of hospitalization included the intervention (hazard ratio, 1.38; 95% confidence interval, 1.01–1.88) and active therapy at 48 hours (hazard ratio, 2.9; confidence interval, 1.15–7.33). Mean hospital costs per patient were $45 709 in the preintervention group and $26 162 in the intervention group (P = .009). Conclusions.—Integration of rapid identification and susceptibility techniques with antimicrobial stewardship significantly improved time to optimal therapy, and it decreased hospital length of stay and total costs. This innovative strategy has ramifications for other areas of patient care.

This study focuses on the epidemiology, clinical manifestations, risk factors, diagnosis and outcome of all cases of central nervous system (CNS) fungal infections in a tertiary center. Medical records of 18 patients of culture-proven CNS fungal infections were retrospectively reviewed from 2000 to 2007, including 12 isolated from the cerebrospinal fluid (CSF) and seven from tissue biopsy. Patient demographic data included 10 males and eight females. The mean age was 55 years (range: 24-89 years). All but one patient were immunocompromised. Fungal organisms isolated from CSF included: Cryptococcous neoformans (8 patients), Coccidioides immitis (3 patients), and Aspergillus versicolor (1 patient). Histopathology of seven biopsy cases revealed groups of pigmented golden-brown fungal forms in three cases; three cases showed septate fungi, two of which had melanin in their walls; and one case showed multiple round spherules. These cases on microbiological cultures grew Coccidioides immitis (1 patient), Aspergillus fumigatus (1 patient), Cladophialophora bantiana (2 patients), Fonsecaea monophora (1 patient) and Scedosporium apiospermum (2 patients). Five of the seven fungal organisms isolated from tissue biopsies were dematiaceous fungi. Twelve patients died after a period of a few weeks to months, two were lost to follow-up, and four are alive with severe neurological sequelae. CNS fungal infections in our cohort were more common in patients post-transplant and with hematologic malignancies. In our series, rare dematiaceous fungi are emerging agents for cerebral mycosis. The outcome of CNS fungal infections is poor despite vigorous antifungal therapy.

In European countries, choledochal cysts are rare anomalies of the bile tract. At the Surgery Department of the University Hospital Ostrava, a case of a patient with cystic lesion of the pancreatic head was solved. According to ultrasonography and computer tomography it was assumed to be a pancreatic pseudocyst. Only after a complete additional examination of the pancreatico-biliary system, was it diagnosed Ia-type cystic dilatation of the common bile duct, accompanied by stenosis in its terminal part. With regard to the age of the first clinical symptoms, their character and histopathological findings, congenital origin is assumed in both of these anomalies. After provisional percutaneous transhepatic biliary drainage, a duodenohemipancreatectomy was performed with complete cyst excision. Until now, nine months later, the patient has shown absolutely no clinical difficulties.

Objective: New medical discoveries regarding genetic susceptibility to common chronic diseases, and the decoding of the human genome have increased public attention to genetics. What information is understood and what attitudes exist towards genetics and genetic research have not been well examined in underserved, culturally diverse communities. Methods: To better understand attitudes and beliefs towards genetics and genetic testing in these groups, we conducted eight focus groups with 55 patients and health care workers in New York City and Westchester, N.Y., in English, Spanish, and Chinese. Results: Focus group participants had limited understanding about genetics or genetic testing. Newborn screening was the least-known genetic issue, even among health care workers. Regardless of their cultural group, most participants expressed a desire for more information about genetics and genetic tests. Latinos and Chinese participants generally expressed positive attitudes towards genetic studies and genetic testing, with the possibility of preventing diseases cited as the main advantage. Black Americans and Non-Hispanic Whites reported mixed feelings about genetic research and genetic testing. Concerns expressed included: anxiety before receiving test results or waiting for a disease to develop, fear of genetic discrimination by health and life insurance companies and employers, not having the financial means to deal with genetic diseases in themselves or a sick child, concern that children and adults are having too many tests. Black Americans expressed the most concern for possibly harmful use of genetic information. Conclusions: Minority populations of diverse cultures have limited knowledge about genetics and genetic testing, would like to have more information, and are not well reached by the current educational approaches. Participants knew the least about newborn screening, a test that is mandatory in the New York State. While genetic knowledge by minority populations was perhaps not different from the level of knowledge of consumers in general, minority populations are at particular risk of being left behind because of historically poor access to information and services.

During a large community-wide outbreak of hepatitis A in two adjoining villages in Slovakia with a total of 5,000 inhabitants we administered to schoolchildren the first commercially available vaccine against hepatitis A (HAVRIX, Smith-Kline Beecham Biologicals, Rixensart, Belgium) in an attempt to control the progress of the epidemic. Soon after the start of the vaccination programme, an abrupt decrease in the occurrence of cases in the school was observed. In the village school with 624 schoolchildren, 404 had received a first dose of 360 enzyme-linked immunosorbent assay (ELISA) units (EL.U) and 373 a second dose 1 month later. Subsequent to the start of vaccination there were eight clinical cases of hepatitis A among the 157 children without a history of hepatitis A who remained unvaccinated and only 1 case in the vaccinated school children, giving attack rates of 5.1% and 0.25% in the two groups, respectively. Among the remaining 63 children, one was found seropositive when screened and 62 had a history of hepatitis A at the start of the vaccination programme. These 63 children were not offered vaccine. No cases occurred in that group. During the epidemic, three cases occurred an average of 20 days following its administration among 19 children who received immune globulin (IG). Cases in the whole population of the villages also ceased soon after the vaccination of the children had started. The vaccine was found more effective than postexposure IG in interrupting the epidemic in the whole community.

The main indication for transcutaneous cholangioscopy through a channel of percutaneous transhepatic drainage are choledocholithiasis, hepatolithiasis and non-malignant or malignant stenosis of the bile ducts. In our department we were able to treat thus repeatedly also complete severing of the biliary pathways.Twelve patients with complete severing of the right hepatic ducts (4x) or common bile duct (8x) after cholecystectomy could not be operated on account of great surgical risk or adhesions after operation. On account of cholangitis they had percutaneous transhepatic drainage. After three weeks transcutaneous cholangioscopy was performed and recanalization by means of a needle-shaped knife. The operation was terminated by introduction of a metal prosthesis Palmaz.The method was successful in all patients, there were no complications associated with the procedure.Transcutaneous cholangioscopy and recanalization of complete severing of the biliary pathways is a new safe method. It is indicated in patients with increased surgical risk. For restoration of the continuity of the bile ducts a metal prosthesis Palmaz is inserted. Short-term results of treatment are satisfactors, long-term results call for a longer follow up.

At the Department of Neurosurgery, Hradec Králové, in the course of 50 years (1948-1997) 363 children, 199 boys and 164 girls (1.2:1) aged under 18 years were operated on for intracranial supratentorial tumours. The average age in children at the time of first operation was 9.3 years; most frequently they participated those of 8 and 11 to 14 years of age. Children aged 1, 2 and 3 years constituted 4.7%, 4.4% and 5% of operations. The tumours were located in: cerebral hemispheres 123 (33.8%), lateral ventricles 17 (4.7%), IIIrd ventricle 5 (1.4%), hypothalamus 26 (7.2%), thalamus 19 (5.2%), basal ganglia 24 (6.6%), sellar region 86 (23.7%), chiasmatic region 38 (10.5%) and pineal region 19 (5.2%). 223 of tumours (61.4%) were located in the midline and 140 of them (38.6%) laterally (in hemispheres and lateral ventricles of the brain). 268 of tumours were histologically verified (73.8%) and 95 of cases were evaluated according to the neurosurgeon's point of view and/or to the clinical and CT controls (26.2%), because of the biopsy (especially in the pre-CT era) was highly riskfull. Histological typing of tumours was retrospectively reevaluated according to the present WHO classification. Summarized 53 types of tumours were differentiated. The most frequent lesions were various variants of astrocytic gliomas (135 = 37.2%). Further on the craniopharyngiomas dominated (73 = 20.1%). The tumours were operated on through craniotomies 299 times, by primarily drainage operations 52 times, functions 6 times, stereotactically 8 times and or by combination of these operations 82 times. Reoperation was needed for postoperative complications in 1.7% (6 times) and for delayed recurrence in 11.3% (41 times). The postoperative mortality (up to 1 month after initial surgery) was in 156 children operated on in pre-CT era (between 1948 and 1977), as compared with 207 children operated on in the era of CT (between 1978 and 1997) in astrocytomas 3.8:0%, pilocytic astrocytomas 6.5:2.8%, craniopharyngiomas 15.4:0% and in all tumours 12.2:2.9%. 16 children with orbital tumours (the average age 5.8 years) operated on with orbitofrontal approach were also evaluated. 14 of them survive for 5-37 years (on the average 16.6 years). The chronological development of diagnostic and operative processes of supratentorial tumours in children's care is discussed. The prognostic elements of present histobiological classification of tumours are positively evaluated.

Twins were diagnosed by ultrasound in a 29-year-old woman. The cesarean delivery revealed at first a mature girl with a large benign sacrococcygeal teratoma and then a healthy boy. X-ray and CT examinations of the teratoma showed pelvic and lower limb bones. A mature teratoma-tridermoma (weight 475 g) containing the underdeveloped lower half of a human body was confirmed during surgery and verified morphologically. The fraternal twins, i.e., the girl operated upon and her brother, have been followed for 5 years and are without any complaints. They have normal neurological function.

A cyst of the choroid plexus of the left lateral ventricle with intermittent blockage of the foramen of Monro and initially with invagination of the III ventricle in a child is described. In a 6-week-old boy a ventriculoatrial shunt was implanted for correction of an active asymmetrical hydrocephalus of unknown origin. When he was 3 months of age a water-soluble contrast CT ventriculography revealed a noncolloid cyst localised predominantly in the upper portion of the III ventricle. At that time the ventricular catheter obstructed with choroid plexus was removed; new bilateral catheters in a parieto-occipital region were implanted. In the course of the next 4 years, first the atrial catheter had to be extracted and then the peritoneal catheter was changed, in both cases because of obstruction. Periods of normal life alternated with periods of transient and intermittent symptoms of increased intracranial pressure, papilloedema, and myoclonic jerks. Repeated computed tomography (CT) and magnetic resonance imaging (MRI) showed stabilised hydrocephalus with an enlarged left lateral ventricle. When the boy was 16 years old MRI revealed a choroid plexus cyst in the left lateral ventricle 2 cm in diameter, with a ball-valve type of obstruction of the foramen of Monro. CT stereoendoscopic resection of the wall of a large cyst filled with cerebrospinal fluid was performed, and two additional adnexal small cysts were coagulated using the bipolar coagulator, Diomed 25 laser and scissors; the symptoms then regressed, except for superior bilateral altitudinal anopsia. Light and electron microscopy of the cyst wall is reported. The cyst was composed of collagenic connective tissue lined with a basal lamina lacking in epithelial cells. The preoperative and postoperative MRI are presented. Choroid plexus cysts localised in the anterior part of lateral ventricles are very rare, and all reported cases have been in male patients. According to the literature our case is only the third ever described in a child.

In the world literature findings of morphological abnormalities in the area of the brain and cerebellum in children with pervasive developmental disorders are encountered. The authors of the present article describe in three case-records morphological abnormalities of the brain and cerebellum found in subjects with markedly expressed or only indicated signs of child autism. At the same time they draw attention to the possible determination of experience and behaviour of subjects with discrete morphological abnormalities, even when the pervasive developmental disorder was not diagnosed but where specific determination of premorbid experience and behaviour, as well as the clinical picture of a possible mental disorder or disease.

The second part of the study "Computerized tomography of the brain in children" is a continuation of the first one published in Supplement for the Collection of Scientific Works by Charles University's Medical Faculty in Hradec Králové from 1990. The former is issued from analyzing 1283 pathological CT findings investigated in newborns and children up to 15 years of age. The present study is concerned with not only the precise diagnosis of the lesion site but also with the type diagnosis and observation of further development of either affected or operated on children brain. When investigating ventriculomegalies, altogether with critical approach to the CT possibilities in diagnosing various types of hydrocephaluses and atrophies, which have been the most frequent pathological findings in this set, the authors also proceeded by monitoring reparative capacities of the brain tissue or post-surgery drainage complications. The problems of children CT investigation are similar to those in adults as far as the brain abscesses, empyemas and non-bacterial encephalities are concerned. A possibility of repeated examinations is of importance in selecting therapeutical approaches and observing their effect. A considerable amount of viral inflammations and the toxoplasmosis appear to be specific for children's age with yet intrauterine course of several of them. They result in severs cerebral damage, hydrocephalus and atrophy with frequent both periventricular and parenchyma calcifications. The acute meningitis did not show unambiguously positive CT findings, though they are frequent to result in mainly the obstructive or hyporesorptive types of hydrocephalus as well as atrophic changes. Only the 8 per cent of post-inflammatory intracranial patients showed normal time-delayed CT finding. From neurocutaneous syndromes, those most frequently occurring were cerebral manifestations of tuberous sclerosis. Apart from periventricular calcifications within the hamartomatous node, the three patients showed large pseudotumorous formations responsible of the deformity of lateral ventricles. In the frame of neurofibromatosis, the authors diagnosed the stenosis of aqueducts, conditioned with adjacent gliosis, and the dysplasia of the base of medial cranial fossa which caused the temporal lobe to prolapse into the fossa subtemporalis and the orbit. The exceptional finding in this set of patients was that of angioreticuloma within the ponto-cerebellar angle in a 10 year-old boy. According to literature, this type of hamartoma does not occur in children of prepubertal age. In 37 children of our set, the extra- or intraparenchymal cavities were diagnosed. Those most frequently occurring were arachnoidal cysts at the pole of temporal lobe as well as porencephalic intraparenchymal cysts.(ABSTRACT TRUNCATED AT 400 WORDS)

Fifty-five patients with Mycobacterium kansasii isolates (47 pulmonary and eight disseminated) were identified at a large Texas hospital from 1975 to 1985. The mean age of patients was 60 years, and there was a slight male predominance. Isolation of M. kansasii usually represented disease. The great majority of patients with pulmonary infection due to M. kansasii had underlying pulmonary diseases, and 70% had nonpulmonary predisposing factors. M. kansasii pulmonary disease clinically and radiographically resembled pulmonary tuberculosis. Disseminated M. kansasii infection occurred in severely immunocompromised patients, who frequently had pulmonary predispositions as well. Disseminated infection most of ten involved the lung, reticuloendothelial system, bone, joint, and skin and presented with signs and symptoms related to these organs. Despite only moderate in vitro susceptibility of M. kansasii to routine antituberculous drugs, most patients responded to rifampin-containing regimens. The prognosis of patients with M. kansasii disease was determined primarily by their underlying diseases.

Percutaneous transhepatic biliary duct drainage followed in nearly one quarter of the group of patients with percutaneous transhepatic cholangiography for malignant and non-malignant diseases. A total of 147 drainage operations were performed, thereof 126 (85.7%) successful ones. Drain was successfully introduced in 96 (88.2%) out of 107 patients. In 78 patients, successful or unsuccessful attempts were made once, in 19 cases drainage was performed twice, and in 9 cases three attempts were made. In 1 female patient the attempt had to be repeated 4 times. There were 11 cases of complication in connection with the puncture proper (7.5%), 2 cases required emergency laparotomy, the rest were handled conservatively. There was no lethal complication. In one quarter of the drains introduced, complete loss of function occurred in the course of drainage (slipped-out drain, block). Septic complications threatened unless drainage was restored in time. Proper management of the catheter in place is crucial for the uncomplicated course of drainage. Also important are the patient's cooperation and the nursing personnel's experience. Internal drainage was used in 15 cases. An endoprosthesis was introduced transcutaneously in 1 case. The longest functional drainage ina case of malignant involvement lasted 288 days, in a non-malignant case 330 days and for the next 334 days endoprosthesis was introduced. The average quantity of bile was 1,009 ml/24 hrs. in malignant cases, and 644 ml/24 hrs. in non-malignant cases. The average period of time from the introduction of drainage until surgical operation was 18 days in malignant cases, 11 days in non-malignant cases.(ABSTRACT TRUNCATED AT 250 WORDS)

The author presents a brief account on the non-surgical treatment of obstructive jaundice by transcutaneous insertion of a duodenobiliary endoprosthesis. He describes the case of a patient with obstruction of the biliary pathways which could not be treated by surgery where external drainage was used with transcutaneous extraction of concrements and transcutaneous insertion of a duodenobiliary endoprosthesis.

To evaluate the acute and chronic effects of diuretic monotherapy with 3 mg piretanide bid, 46 patients (pts) with congestive heart failure (NYHA II-III) secondary to coronary artery disease were studied. Within 3 weeks of therapy, the patients lost 1.6 kg body weight. Forty-four patients reported a subjective feeling of improvement. Echocardiographically, a highly significant (p less than 0.001) reduction of diastolic and systolic diameters was found, as well as an increase of fraction shortening. Chest x-ray indicated a reduction of heart volume from 1012 +/- 263 ml to 936 +/- 233 ml (p less than 0.001). The serum potassium level remained unchanged. A subgroup of 26 pts underwent invasive hemodynamic examinations. IV injection of 6 mg piretanide resulted in an acute reduction of pulmonary wedge pressure (pc) from 20.2 +/- 5.3 mmHg to 11.9 +/- 5.0 mmHg (p less than 0.001); simultaneously a slight decrease of cardiac index from 3.2 +/- 0.6 l/min/m2 to 3.0 +/- 0.4 l/min/m2 was observed. Invasive control after 3 weeks of oral therapy showed no decline of the piretanide effect. The exercise tolerance increased clearly from 135 +/- 161 Wmin to 249 +/- 268 Wmin (p less than 0.05). A control group of further 14 pts was treated with placebo only and did not show any significant changes of pc (20.0 +/- 6.4 mmHg vs. 22.8 +/- 19.2 mmHg), exercise tolerance, or other clinical parameters. Thus, the diuretic monotherapy of congestive heart failure with piretanide is highly effective and shows a significant improvement in all clinical and hemodynamic parameters in the absence of any remarkable side effects.

A man with acquired immunodeficiency syndrome developed a generalized rash and bilateral dendritic epithelial keratitis without retinitis. Cytologic examination of superficial corneal scrapings showed many megalosyncytial giant cells that were highly characteristic of cytomegalovirus (CMV) infection. Viral cultures yielded CMV from 2 separate specimens obtained by corneal epithelial debridement from both eyes. The slightly elevated, opaque, branching, nonulcerative epitheliopathy recurred after corneal scrapings and persisted despite oral and topical antiviral therapy. Stromal keratouveitis subsequently developed. This case report confirms that CMV can produce corneal involvement and suggests that CMV keratitis may be an emergent complication of acquired immunodeficiency syndrome.

The basis of olfactory signatures mediating human kin recognition was investigated in two experiments. The odors of mothers and offspring were correctly matched (by subjects unfamiliar with the stimulus individuals) at a greater than chance frequency. In contrast, subjects were not able reliably to match the odors of husbands and wives. These data support the hypotheses that characteristic individuals odors are genetically mediated and that kin recognition should be facilitated by the similarity of such familiar odors among close relatives.

The epidemiology of genital white piedra was investigated in 166 young men with a variety of genital complaints. Trichosporon beigelii was isolated from sixty-six (40%) of the cultured scrotal hairs. Infection was more common among black patients--54% of those examined, compared to 16% of white patients and 30% of "others" (p less than 0.001). There was no relationship between infection and foreign travel. White piedra was also found in young women, but less frequently than in young men (14% vs 40%, respectively). Transmission rarely occurred from person to person. Occasionally multiple body sites were simultaneously infected. A relationship may exist between T. beigelii and Corynebacterium concurrently infecting genital hair shafts. Eradication of infection was extremely difficult, despite in vitro sensitivity to antifungal agents. Spontaneous remissions occurred, however, in some patients.

The effect of high doses of ascorbic acid (AA) (200 mg/kg body weight, s. c.) on the values of some biochemical parameters has been studied in guinea pigs (glucose, lactic acid, SH substances and GSH in blood, proteins and urea, and the LDH, MDH, ASAT, ALAT, and gamma-GT activities in the serum, the acid phosphatase activity in the liver, the gamma-GT activities in the liver and the kidney, and on the levels of SH substances and GSH in various organs). Ascorbic acid was administered to the animals in a single dose or in dialy doses for different periods (4 days, 2 weeks, and 7 weeks). The majority of the studied parameters showed a transient increase or decrease of the values which, however, after long term AA administration mostly returned to normal values. After 7 weeks, the level of urea in the serum remained increased (by approximately 30%) contrary to the decreased level of lactic acid, the gamma-GT activity in the serum, and the GSH level in some organs. In the experimental series carried out in the different seasons of the year, the results differed in some cases especially after short term AA administration. However, in a few cases, even the values obtained from the control animals tended to differ. If similar changes are to be expected in man after AA administration, then on the basis of our results it can be assumed that in the indicated cases the therapeutic long-term application of large AA doses does not present any hazard to the patient.

The role of olfactory cues in maternal recognition of neonates was investigated. Mothers were able to identify the garments worn by their own infants (in comparison to garments worn by unfamiliar infants) through odor alone within the first 6 days postpartum. In a second experiment, mothers who had only limited pre-test exposure to their infants recognized the odor of their infant's garment during tests conducted at 20.5-41.7 hr after delivery. Olfaction may be an especially salient modality for recognition of infants.

Progress in modern medicine has resulted in a drastic reduction in infant mortality. Almost 95% of children live through their 1st year, after which their lives are as safe as those of adults. The civilized countries of the world restrict the birth rate in order to prevent overpopulation, maintain high standards of living, and give women the time and opportunity to pursue professional careers. Due to birth control and family planning, Czechoslovakia has no overpopulation problem. Under these circumstances, a gynecologist should not assume the role of a priest or a judge by condemning a woman who wants to terminate her pregnancy. Instead, physicians should inform, advise, and, in some cases, act as family doctors. Birth control should not inhibit sexuality. Gynecologists should not consider sex a sin and should be deeply involved in various sex education programs, including programs for the selection of safe and effective contraceptives.

A series of experiments investigated the salience of newborn infants' facial-visual features for recognition and sex identification. Within 33 hours post-partum, mothers recognized photographs of their own offspring when presented with those of unrelated neonates. Furthermore, adult subjects were able to match photographs of unfamiliar mothers and their infants, and determine the sex of neonates, at a greater than chance level of accuracy. Although recognizable facial features are presumably genetically determined, maternal recognition of offspring probably results from brief exposure and familiarization as well as physical resemblance between the infant and other familiar family members, including the mother herself.

A melanotic neuroectodermal neurocranial tumor of infancy of extra-intra-subdural right temporal location, examined by CT and successfully operated in a 33-month-old boy is reported in context with the literature. Out of 30 cases (including our case) of melanotic neuroectodermal neurocranial and intracranial tumors (MNNIT) 18 were located in the neurocranium (MNNT) and 12 intracranially, i.e. in the brain and cerebellum (MNIT). 23 tumors were located medially (11 at the anterior fontanel, one at the posterior fontanel, one in the 3rd ventricle, one in the sella turcica, one in the pineal region, 8 in the cerebellar vermis) and 7 laterally (five involved the temporal bone, one of which with intracranial propagation (our case), one in the temporal lobe of the brain, one in the skull posterior to the mastoid process). Twenty-eight cases were children (16 boys, 8 girls, four not stated), two adults. The location is important in respect to biology and surgery: MNNIT can be grouped as to its relation to the arachnoid barrier layer into malignant and benign. MNIT localised medially subarachnoidally are malignant. MNNT of small dimensions localised medially and laterally extraarachnoidally are benign. MNNT of extreme dimensions localised medially extra-intradurally are also benign, but difficult to treat. In the MNNT when diagnosed early and operated radically, the mortality can be lowered. Our case, presented on CT (1978) as first case of combined extra-intra-subdural location, represents the 173rd MNT and the 18th related to neurocranium.