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X-linked hypophosphatemia (XLH) caused by PHEX mutations results in elevated serum FGF23 levels, renal phosphate wasting and low 1,25-dihydroxyvitamin D. The glycophosphoprotein osteopontin, a potent inhibitor of mineralization normally degraded by PHEX, accumulates within the bone matrix. Conventional therapy consisting of supplementation with phosphate and vitamin D analogs is burdensome and the effects on bone material poorly characterized. We analyzed transiliac bone biopsies from four adult patients, two of them severely affected due to no diagnosis and no treatment until adulthood. We used light microscopy, qBEI and FTIRI to study histology, histomorphometry, bone mineralization density distribution, properties of the organic matrix and size of hypomineralized periosteocytic lesions. Non-treatment resulted in severe osteomalacia, twice the amount of mineralized trabecular volume, multiple osteon-like perforations, continuity of lamellae from mineralized to unmineralized areas and distinctive patches of woven bone. Periosteocytic lesions were larger than in treated patients. The latter had nearly normal osteoid thicknesses, although surface was still elevated. The median calcium content of the matrix was always within normal range, although the percentage of lowly mineralized bone areas was highly increased in non-treated patients, resulting in a marked heterogeneity in mineralization. Divalent collagen cross-links were evident independently of the mineral content of the matrix. Broad osteoid seams lacked measurable pyridinoline, a mature trivalent cross-link and exhibited considerable acidic lipid content, typically found in matrix vesicles. Based on our results, we propose a model that possibly integrates the relationship between the observed mineralization disturbances, FGF23 secretion and the known osteopontin accumulation in XLH., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Endoscopic sinus surgery can lead to crusting or synechiae formation, which can affect the healing process., Objective: The aim of our study was to compare the influence of steroid versus antibiotic versus saline solution impregnated absorbable nasal spacers on postoperative wound healing and patient satisfaction., Methods: Eighty patients, 33 women and 47 men, were enrolled in this study. At the end of the surgery, two pieces of 4cm biodegradable material were applied in each ethmoid cavity. One of them was impregnated with saline solution, while the second one with steroid, or with antibiotic., Results: We observed statistically significant differences in the Lund-Kennedy score between the control and both treatment groups: for the Antibiotic-group on days 10 and 30 (p=0.009; p=0.009) and for the Steroid-group on day 90 (p=0.008). The extended endoscopic appearance of nasal mucosa indicated statistically significant differences in crust formation on day 10 comparing the steroid and control dressing (p=0.025), in secretion type on days 10 and 30 comparing the antibiotic and control dressing (p=0.003; p=0.016) and additionally for steroid and control on day 90 (p=0.046). On Day 90 we observed statistically significant differences in the absence of mucosal edema in the S-group compared to controls (p=0.007)., Conclusions: The results of this study reveal the significant positive influence of steroid- and antibiotic-impregnated biodegradable nasal packing on the postoperative healing process and patient satisfaction compared to the saline soaked dressing., (Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Background: The increasing population of very old intensive care patients (VIPs) is a major challenge currently faced by clinicians and policymakers. Reliable indicators of VIPs' prognosis and purposefulness of their admission to the intensive care unit (ICU) are urgently needed., Methods: This is a report from the Polish sample of the VIP1 multicentre cohort study (NCT03134807). Patients ≥ 80 years of age admitted to the ICU were included in the study. Information on the type and reason for admission, demographics, utilisation of ICU procedures, ICU length of stay, organ dysfunction and the decision to apply end-of-life care was collected. The primary objective was to investigate the impact of frailty syndrome on ICU and 30-day survival of VIPs. Frailty was assessed with the Clinical Frailty Scale (≥ 5 points on a scale of 1-9)., Results: We enrolled 272 participants with a median age of 84 (81-87) years. Frailty was diagnosed in 170 (62.5%) patients. The ICU and 30-day survival rates were equal to 54.6% and 47.3% respectively. Three variables were found to significantly increase the odds of death in the ICU in a multiple logistic regression model: SOFA score (OR = 1.16; 95%CI 1.16-1.24), acute mode of admission (OR = 5.1; 95%CI 1.67-15.57) and frailty (OR = 2.25; 95%CI 1.26-4.01)., Conclusion: Measuring frailty in critically ill older adults can facilitate making more informed clinical decisions and help avoid futile interventions.

Introduction: Nasal packing after endoscopic sinus surgery is used as a standard procedure. The optimum solution to minimize or eliminate all disadvantages of this procedure may be accomplished using biodegradable packs., Objective: The aim of this study was to compare patient satisfaction and clinical outcome associated with absorbable and non-absorbable packing after FESS., Methods: In total, 50 patients were included in a prospective, double-blind, randomized trial. One side was packed with polyurethane foam, while the opposite side was packed with gauze packing. On the 2nd, 10th, and 30th postoperative day, the patients were questioned with the aid of a visual analog scale. The standardized questionnaires for bleeding, nasal breathing, feeling of pressure, and headache were used. The presence of synechiae, infection, or granulation was noted and recorded with the video-endoscopy., Results: A significant difference according to lower pressure was found in the NasoPore group compared to the controls on day ten after surgery. The NasoPore packing had lower scores with respect to postoperative nose blockage on the 2nd and 10th days. Mucosal healing was better for the NasoPore group, both at day ten and 30 compared with the control group., Conclusion: The overall patient comfort is higher when using NasoPore compared to non-resorbable traditional impregnated gauze packing. Intensive saline douches applied three to four times per day are mandatory after the operation to prevent synechiae formation and fluid resorption by the packing., (Published by Elsevier Editora Ltda.)

Recently oral melatonin has been proposed as an agent for premedication. In this study, we compared melatonin with clonidine, considering its anxiolytic properties as well as the influence of both agents on the pharmacokinetic, hypnotic, and hemodynamic effects of propofol during propofol-remifentanil total intravenous anesthesia (TIVA). The dataset under analysis included 32 patients scheduled for a functional endoscopic sinus surgery. The population pharmacokinetic modeling was done with NONMEM. The PK of propofol was described with a two-compartment disposition model, whereas the BIS and AAI were described with a sigmoidal Emax model linked with the propofol concentration via the biophase compartment. The anxiolytic effect was assessed by means of the visual analog scale for anxiety (VAS-A). The population PK/PD model was successfully developed to describe the data. No significant differences in the PK/PD of propofol were noted due to the premedication with clonidine and melatonin. Melatonin was less effective than clonidine in reducing patients' anxiety at the induction of anesthesia, whereas clonidine premedication was associated with greater decrease in heart rate and blood pressure. A decreased EC50 (2.47 vs. 3.17 mg/L) and increased slope (2.71 vs. 1.30) of the sigmoidal Emax relationship was observed for the AAI index, as compared with the BIS measurements., (© 2014, The American College of Clinical Pharmacology.)

Objectives: Premedication with clonidine has been found to reduce the bleeding during endoscopic sinus surgery (ESS), therefore lowering the risk of surgical complications. Premedication is an essential part of pre-surgical care and can potentially affect magnitude of systemic stress response to a surgical procedure. The aim of this study was to compare the efficacy of premedication with clonidine and midazolam in patients undergoing sinus surgery., Methods: Forty-four patients undergoing ESS for chronic sinusitis and polyp removal were enrolled and randomly assigned to receive either oral clonidine or midazolam as a premedication before receiving propofol/remifentanil total intravenous anesthesia. The effect of this premedication choice on anesthetic requirements, intraoperative hemodynamic profile, preoperative anxiety and sedation as well as postoperative pain and shivering were examined in each premedication group., Results: Total intraoperative remifentanil requirement was lower in the clonidine group as compared to the midazolam group 503.2±147.0 µg vs. 784.5±283.8 µg, respectively (P<0.001). There was no difference between groups in required induction dose of propofol, level of preoperative anxiety, level of sedation and postoperative shivering. Intraoperative systemic blood pressure and heart rate response had a more favorable profile in patients premedicated with clonidine. Postoperative pain assessed by visual analogue scale for pain was lower in the clonidine group compared with to the midazolam premedication group., Conclusion: Premedication with clonidine provides better attenuation of hemodynamic response and reduction of intraoperative remifentanil requirements in patients undergoing ESS. Postoperative pain seems to be better controlled after clonidine premedication as well.

Background: Inadequate surgical field visualization due to intraoperative bleeding during endoscopic sinus surgery (ESS) can cause major complications. The aim of this prospective study was to compare the effect of preoperative administration of clonidine and melatonin on the quality of the surgical field visualization and selected aspects of presurgical premedication., Methods: Twenty-six patients undergoing ESS for chronic sinusitis and polyp removal were enrolled and randomly assigned to receive either oral clonidine or melatonin as preoperative premedication. During the operation, the quality of the surgical field was assessed and graded using the scale proposed by Boezaart. The evaluations were done at 15, 30, and 60 minutes after incision. The effect of this premedication choice on the intraoperative and postoperative hemodynamic profile was examined in each group. Anxiolytic effects of both premedication agents were assessed using the visual analogue scale for anxiety (VAS-A). Propofol dose and induction time (the time for bispectral index [BIS] to reach 45), anesthesia and surgical procedures time, and recovery time (the time for BIS to reach 90) were assessed in both groups as well., Results: The quality of the surgical field was consistently better in 2 of 3 time points in the clonidine group. Perioperative mean arterial pressure and intraoperative heart rate had a more favorable profile in patients premedicated with clonidine. There were no differences in other measured parameters between groups., Conclusion: Premedication with clonidine before ESS provides better quality of surgical field and more favorable hemodynamic profile as compared to melatonin., (© 2014 ARS-AAOA, LLC.)

Chemically stable nitroxide radicals that can be monitored by electron paramagnetic resonance (EPR) spectroscopy can provide information on structural and dynamic properties of functional RNA such as riboswitches. The convertible nucleoside approach is used to install 2,2,6,6-tetramethylpiperidin-1-oxyl (TEMPO) and 2,2,5,5-tetramethylpyrrolidin-1-oxyl (proxyl) labels at the exocyclic N(4)-amino group of cytidine and 2'-O-methylcytidine nucleotides in RNA. To obtain site-specifically labeled long riboswitch RNAs beyond the limit of solid-phase synthesis, we report the ligation of spin-labeled RNA using an in vitro selected deoxyribozyme as catalyst, and demonstrate the synthesis of TEMPO-labeled 53 nt SAM-III and 118 nt SAM-I riboswitch domains (SAM=S-adenosylmethionine)., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: During functional endoscopic sinus surgery (FESS), intraoperative bleeding can significantly compromise visualization of the surgical field. Clonidine constricts peripheral blood vessels and reduces systemic blood pressure, which in combination decrease nasal mucosa blood flow. This dual effect can potentially reduce bleeding during FESS and stabilize the intraoperative hemodynamic profile of the patient., Aim: The aim of this prospective study was to assess if the quality of the surgical field visualization during FESS was improved when clonidine was used as a premedication agent., Methodology: A group of 44 patients undergoing FESS for chronic sinusitis and polyp removal were enrolled and randomly assigned to receive either oral clonidine or midazolam as preoperative premedication. During the operation, the quality of the surgical field was assessed and graded by the operating surgeon using the scale proposed by Boezaart. The evaluations were done during surgery at 15 minutes (K1), 30 minutes (K2), 60 minutes (K3) and 120 minutes (K4) after incision., Results: The duration of the surgical procedure was significantly shorter in the clonidine group: mean time of surgery: 80 vs. 96 min in the clonidine and midazolam groups, respectively. Also better quality of surgical field was observed at all time points in the clonidine group., Conclusion: Premedication with clonidine before FESS results in shortening of the surgical time and a better quality of the surgical field.

Background: Patients with chronic kidney disease (CKD) develop renal osteodystrophy with alterations in bone turnover, mineralization, and volume (TMV). A specific skeletal complication in children is growth impairment, which currently is treated by recombinant human growth hormone (rhGH). The effects on bone material properties are poorly understood. This study assesses the effects of rhGH treatment on bone matrix mineralization., Study Design: Observational study., Setting & Participants: 18 short children and adolescents (aged 3.6-16 years) with CKD on dialysis therapy., Predictor: rhGH treatment for 1 year., Outcomes: Tetracycline-labeled bone biopsy classified according to the TMV system., Measurements: Bone mineralization density distribution (BMDD) was evaluated by quantitative backscattered electron imaging in trabecular and cortical compartments. Additional data for patients' height and biochemical bone serum parameters were obtained., Results: Prior to rhGH treatment, our cohort showed low bone turnover and high mineralization densities versus reference data: Ca(mean) (weighted mean calcium content) in cancellous bone, +3.3% (P = 0.04); Ca(mean) in cortical bone, +6.7% (P < 0.001); Ca(peak) (mode of the BMDD) in cancellous bone, +5.0% (P < 0.001); Ca(peak) in cortical bone, +8.2% (P < 0.001); Ca(width) (heterogeneity in mineralization), no significant difference for cancellous (P = 0.2) and cortical (P = 0.1) bone; Ca(high) (portion of fully mineralized bone) in cancellous bone, 5-fold greater (P < 0.001); Ca(high) in cortical bone, 14-fold greater (P < 0.001); Ca(low) (portion of low mineralized bone) in cancellous bone, +23.9% (P = 0.02); Ca(low) in cortical bone, -22.2% (P = 0.05). After rhGH treatment, height increased by 9.1 cm (P < 0.001) and bone turnover indices to normal values or beyond. Matrix mineralization was lesser and more heterogeneous compared to baseline: Ca(width) for cancellous bone, +15.3% (P < 0.001); Ca(width) for cortical bone, +34.1% (P < 0.001). Ca(mean), Ca(peak), and Ca(high) for cancellous bone and Ca(mean) and Ca(peak) for cortical bone were no longer significantly different from reference data. Ca(high) for cortical bone dramatically decreased after treatment but was still substantially greater than reference data., Limitations: Low case number per TMV subgroup, no measurements of fibroblast growth factor 23., Conclusions: Children and adolescents with CKD and growth deficiency are at risk of having low bone turnover. rhGH treatment improves height and concomitantly bone modeling/remodeling, which appears beneficial for bone matrix mineralization., (Copyright © 2013 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Little is known whether trabecular bone matrix mineralization is altered at the site of osteoporotic vertebral fractures. Bone mineralization density distribution (BMDD) was assessed in trabecular bone of acute, single-level compression fractures of the spine at various stages of fracture repair using quantitative backscattered electron imaging (qBEI). The grading of the repair stage was performed by histological methods. From 20 patients, who underwent either kyphoplasty (n=18) or vertebroplasty (n=2), a vertebral bone biopsy was taken prior to cement augmentation. Six patients took bisphosphonates (BP) prior to fracture. Three study groups were formed: N1=early-, N2=late-healing and B=BP treatment at late healing stage. In general, all groups had an altered BMDD when compared to historical normative reference data. Mean matrix mineralization (CaMean) was significantly (p<0.001) lower in all groups (N1: -5%, N2: -16%, and B2: -16%). In N2, CaMean was -13.1% (p<0.001) lower than N1. At this stage, deposition of new bone matrix and/or formation of woven bone are seen, which also explains the more heterogeneous matrix mineralization (CaWidth). Moreover, BP treatment (B2) led to a significant reduction in CaWidth (-28.5%, p<0.001), when compared to N2. Bone tissue from vertebrae with acute compression fractures reveals a large variation in matrix mineralization depending on the stage of repair. Bisphosphonate treatment does affect the mineralization pattern of tissue repair. The low mineralization values found in early stage of repair suggest that altered bone material properties may play a role in the occurrence of fragility fractures of the spine., (Copyright © 2012 Orthopaedic Research Society.)

Kartagener's syndrome is a rare autosomal recessive disorder presenting a triad of sinusitis, bronchicetasis and situs inversus with dextrocardia. It occurs in 50% of patients with situs inversus. The most important anesthetic implications of Kartegener's syndrome surgery are assessement of pulmonary and cardiac structure and function. We present a case of 43-year-old woman with chronic rhinosinusitis with polyps and bilateral sectetory otitis media. The chest radiograph and CT scans showed dextrocardia and situs inversus with chronic bronchitis without bronchiectasis. Spirometry showed forced expiratory volume in one second (FEV1) of 2.66 L and forced vital capacity (FVC) of 3.62 L. Electroechography showed no cardiac abnormalities with 55-60% of EF. The anesthetic implications of Kartagener's syndrome are varied. The regional or general anesthesia might be involved with sinus surgery, ear surgery, pulmonary surgery, infertility or abdominal and cardiac surgery. The main anesthetic considerations among patients with Kartagener's syndrome are related to the pulmonary function which include preoperative respiratory infections due to bronchiectasis. We should also monitor potentially occluded congenital heart diseases. Kartagener's syndrome is a rare disease and when the patient need an operation we have to consider surgery with regional or general anesthesia. The general anesthesia would be safe after complete preanaesthetic examination of the patient. The ECG, chest CT scans, spirometry and echocardiography are mandatory before the operation., (Copyright © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.)

Background: The aim of this study was to analyse the composition of parenteral nutrition (PN) mixtures used in the ITU., Methods: Restrospective analysis involved 2124 prescriptions for individual PN bags. They were administered over an 18-month period, to 160 ITU patients with the mean APACHE II score of 26 points (range: 5-61), calculated on admission. The mortality rate was 40%. Nutrition programs were prepared individually following the 2009 ESPEN guidelines. The prescription was modified according to the individual patient's clinical condition. One hundred and sixty prescriptions were analysed on the first day of PN (T1), 139 - on the second day (T2) and 1825 on the third and subsequent days (T3)., Results: The mean energy supplies were: 1381 kcal/day (range: 456-2612) on T1, 1467 kcal/day (range: 524-2860) on T2, and 1654 kcal/day (range: 390-2969) on T3. The mean supplies of amino acids, glucose and lipids were as follows: amino acids 68.3 g/day (range:20-120) on T1; 71.6 g/ day (range:27.5-125) on T2; 88.0 g/day (range:11-196) on T3; glucose 210.25 g/day (range: 120- 400) on T1; 218.34 g/day (range: 65-480) on T2; 278.5 g/day (range: 18-520) on T3; lipids 34.9 g/ day (range: 0-100) on T1; 38.7 g/day (range: 0-100) on T2; 52.66 g/day (range: 0-117) on T3. The percentages of non-protein energy from lipids were: 29.25 (0-73) on T1; 31.58 (range: 0-60) on T2; 33.5 (0-60) on T3. The following statistically significant differences were found: T2-T3- (p<0.05)., Conclusions: The compositions of nutrition bags prepared for ITU patients were consistent with the ESPEN guidelines. The composition varied on different days of nutrition. The differences in the supply of nutrition components indirectly confirm the need for individual prescriptions for ITU patients.

The pathogenesis of primary osteoporosis in younger individuals is still elusive. An important determinant of the biomechanical competence of bone is its material quality. In this retrospective study we evaluated bone material quality based on quantitative backscattered electron imaging to assess bone mineralization density distribution (BMDD) in bone biopsies of 25 male patients (aged 18-61 years) who sustained fragility fractures but were otherwise healthy. BMDD of cancellous bone was compared with previously established adult reference data. Complementary information was obtained by bone histomorphometry. The histomorphometric results showed a paucity of osteoblasts and osteoclasts on the bone surface in the majority of patients. BMDD revealed a significant shift to lower mineralization densities for cancellous bone values: CaMean (weighted mean Ca content, -5.9%), CaPeak (mode of the BMDD, -5.6%), and CaHigh (portion of fully mineralized bone, -76.8%) were decreased compared to normative reference; CaWidth (heterogeneity in mineralization, +18.5%) and CaLow (portion of low mineralized bone, +68.8; all P < 0.001) were significantly increased. The shift toward lower mineral content in the bone matrix in combination with reduced indices of bone formation and bone resorption suggests an inherent mineralization defect leading to undermineralized bone matrix, which might contribute to the susceptibility to fragility fractures of the patients. The alteration in bone material might be related to osteoblastic dysfunction and seems fundamentally different from that in high bone turnover osteoporosis with a negative bone balance.

Rett syndrome (RTT) is a common X-linked neurodevelopmental disorder caused by mutations in the coding region of methyl-CpG-binding 2 (MECP2) gene. Patients with RTT have a low bone mineral density and increased risk of fracture. However, very little is known if bone matrix mineralization is altered in RTT. A 17-year-old girl with a classical form of RTT with a heterozygous nonsense mutation in exon 3 in the MECP2-gene was treated in our hospital. Her femoral neck BMD is 43.3% below the 3rd percentile when compared to age and sex-matched controls. She underwent surgery for correction of her scoliosis, which provided a unique opportunity to obtain bone tissue to study bone matrix mineralization (Bone Mineralization Density Distribution-BMDD) using quantitative backscattered electron imaging (qBEI) and histomorphometry. BMDD outcomes were compared to recently published normative reference data for young individuals. qBEI analysis showed a significant shift to lower matrix mineralization despite histomorphometric indices indicate a low bone turnover. There was a reduction in CaMean (-7.92%) and CaPeak (-3.97%), which describe the degree of mineralization. Furthermore the fraction of low mineralized matrix (CaLow: +261.84%) was dramatically increased, which was accompanied with an increase in the heterogeneity of mineralization (CaWidth: +86.34%). Our findings show a significantly altered bone matrix mineralization of a typical patient with RTT. This may partly explain the low bone density seen in these patients. These results also warrant further studies on the molecular role of MECP2 in bone matrix mineralization., (Copyright 2010 Elsevier Inc. All rights reserved.)

This study evaluates possible circadian rhythms during prolonged propofol infusion in patients in the intensive care unit. Eleven patients were sedated with a constant propofol infusion. The blood samples for the propofol assay were collected every hour during the second day, the third day, and after the termination of the propofol infusion. Values of electroencephalographic bispectral index (BIS), arterial blood pressure, heart rate, blood oxygen saturation and body temperature were recorded every hour at the blood collection time points. A two-compartment model was used to describe propofol pharmacokinetics. Typical values of the central and peripheral volume of distribution and inter-compartmental clearance were V(C) = 27.7 l, V(T) = 801 l, and CL(D) = 2.73 l/min. The systolic blood pressure (SBP) was found to influence the propofol metabolic clearance according to Cl (l/min) = 2.65 x (1-0.00714 x (SBP-135)). There was no significant circadian rhythm detected with respect to propofol pharmacokinetics. The BIS score was assessed as a direct effect model with EC(50) equal 1.98 mg/l. There was no significant circadian rhythm detected within the BIS scores. We concluded that the light-dark cycle did not influence propofol pharmacokinetics and pharmacodynamics in intensive care units patients. The lack of night-day differences was also noted for systolic blood pressure, diastolic blood pressure and blood oxygenation. Circadian rhythms were detected for heart rate and body temperature, however they were severely disturbed from the pattern of healthy patients.

We describe a tall-statured 14-year-old boy who illustrated the full phenotypic and radiographic features of Stickler syndrome type I. A bone biopsy showed evidence of reduced bone mass and bone turnover, such as reduced BV/TV (-43%), TbTh (-29%), and OS/BS (-48%), Ob.S/BS (-27%), and Oc/BS (-47%) compared to "age-matched" controls. Moreover, there was evidence that the mineralization process was severely disturbed. Quantitative backscattered electron imaging revealed that the bone mineralization density distribution (BMDD) of cancellous (Cn) as well as cortical (Ct) bone was shifted toward lower mineralization compared to a young control reference cohort. BMDD parameters of mean degree of mineralization, Cn Ca (-9.8%) and Ct Ca (-18.0%), were dramatically decreased. To the best of our knowledge this is the first clinical report describing bone biopsy findings in a boy with Stickler syndrome. Such a severe undermineralization of bone matrix might essentially contribute to the compromised mechanical competence of the skeleton found in this patient.

Background: Metallo-beta-lactamases (MBL) are the enzymes that are able to hydrolyse almost the full range of beta-lactame antibiotics--penicillins, cephalosporins and carbapenems. The latter are the drugs of choice for treatment of serious infections caused by Enterobacteriaceae strains, which produce extended-spectrum-beta-lactamases. The presence of MBL-producing strains markedly decreases the therapeutic possibilities in severe, life-threatening infections., Case Report: We present the case of a 61-yr-old man who underwent surgery for acute leg ischemia, and in whom a bifurcation prosthesis was implanted. The postoperative course was complicated with serious nosocomial infection, caused by MBL-positive Klebsiella pneumoniae strains. Despite multi drug treatment and intensive care, the patient died 30 days after surgery due to multi organ failure. All isolates cultured from the patient were resistant to carbapenems with their MICs exceeding 32 microg mL(-1). The presence of MBLs was detected with the double-disk synergy test. The presence of genes encoding MBLs was determined with a commercial kit, hyplex MBL ID (Bag Health Care, USA). The isolate from blood was found to carry the blaVIM-like family gene, located in a conjugative plasmid., Conclusion: The MBL-producing isolates were the first K. pneumoniae isolates of the kind identified in Poland. They present a serious danger, limiting the usefulness of carbapenems in ITU patients. We recommend that detection of MBLs in Enterobacteriaceae should be regarded as a standard in Polish hospitals.

Fibroblast growth factor 23 (FGF23) overexpression has been identified as a causative factor for tumor-induced osteomalacia (TIO) characterized by hypophosphatemia due to increased renal phosphate wasting, low 1,25(OH)(2)D(3) serum levels, and low bone density. The effects of long-lasting disturbed phosphate homeostasis on bone mineralization are still not well understood. We report on a patient with a 12-year history of TIO, treated with 1,25(OH)(2)D(3) and phosphate, who finally developed hyperparathyroidism with gland hyperplasia before the tumor could be localized in the scapula and removed. During surgery a transiliac bone biopsy was obtained. FGF23 expression in the tumor cells was confirmed by in situ hybridization. Serum FGF23 levels as measured by ELISA were found to be extremely elevated before and decreased after removal of the tumor. Bone histology/histomorphometry and measurement of bone mineralization density distribution using quantitative backscattered electron imaging were performed on the bone biopsy. The data showed important surface osteoidosis and a slightly increased osteoblast but markedly decreased osteoclast number. The mineralized bone volume (-11%) and mineralized trabecular thickness (-18%) were low. The mean degree of mineralization of the bone matrix (-7%), the most frequent calcium concentration (-4.1%), and the amounts of fully mineralized bone (-40.3%) were distinctly decreased, while the heterogeneity of mineralization (+44.5%) and the areas of primary mineralization (+131.6%) were dramatically increased. We suggest that the elevated levels of FGF23 and/or low phosphate concentrations disturb the mineralization kinetics in vivo without affecting matrix mineralization of pre-existing bone packets.

Unlabelled: INTRODUCTIONn: The Munchausen Syndrome by Proxy occurs when parents make a child believe he or she suffers from a disease while in fact this is not true. A medical diagnosis ruling out a possible disease of the child does not calm the parents down and only confirms--in their belief--the doctor's incompetence which in turn makes them seek a second opinion. This may be repeated over and over again and be accompanied by administration of medicines, overprotection, introducing a diet, limiting exercise as well as limiting motoric and mental development., Aims: Authors present diagnostic difficulties in diagnosing the Munchausen syndrome., Case Description: A 6-year old girl was hospitalized for the first time at the Developmental Age Psychiatry and Psychotherapy Ward of the John Paul II Pediatric Center in Sosnowiec, Poland (until then she had received psychiatric treatment at the Center on an outpatient basis only). Medical history provided by her mother as well as supplied documents show numerous hospitalizations at Pediatric Wards since the girl was 6 weeks old due to intensified vomiting and frequent diarrheas. Since the age of 4, the girl has been provoking vomiting as well as temporarily refusing meals and liquids., Commentary: Authors present diagnostic difficulties in diagnosing the Munchausen syndrome mainly caused by discharging the patient from the Ward on her mother's request as well as non-specific nature of the symptoms.

Background: X-linked hypophosphatemia (XLH) is the most prevalent heritable form of rickets. It is a dominantly inherited disorder, characterized by renal phosphate wasting, abnormal vitamin D and PTH metabolism, and defective bone mineralization. Inactivating mutations in the gene encoding PHEX (phosphate-regulating gene with homologies to endopeptidases on the X chromosome) have been found to be associated with XLH., Methods: We report about a 54-year-old male patient who exhibited the typical features of XLH, and in whom mutational analysis using PCR and sequencing was performed. Additionally, extensive laboratory and radiological investigations were carried out., Results: A 1-bp deletion in exon 2 of the PHEX gene was detected (177delC), which, to the best of our knowledge, has not been reported yet. This deletion results in a premature stop codon (C59X), suggesting a truncation of the PHEX protein. Furthermore, elevated FGF23 and PTH levels as well as an increased axial bone mineral density score were measured., Conclusions: We present a male patient with XLH, who harbors a novel mutation in the PHEX gene, which might be the cause for his disease. Our data support previous findings and therefore contribute to the decipherment of the pathogenetic pathways of XLH., (Copyright 2007 S. Karger AG, Basel.)

A case of the 11-year-old boy with erythrodermic symptoms continuing since neonatal period, atopic dermatitis, asthma, structural hair disorders and nanism has been described.

From 1973 to 1988 in the Dermatological Surgery Unit of the 1st Department of Dermatology of the Silesian School of Medicine in Katowice 82 patients were treated surgically for keratoacanthoma. In 76 patients the defect was closed by simple bringing closer the edges or by local plastic surgery, in 6 patients it was covered with free full-thickness skin grafts. The preparations of the excised tumors from various places were examined histologically. In three cases the texture of carcinoma spinocellulare was found in the base of the tumor. In 98% of the patients the wounds healed by first intention.

To obtain information about the regulating nervous mechanisms of the pig alimentary canal the activity of monoaminooxidase (MAO) and acetylcholinesterase (AChE) in duodenum, jejunum and colon was studied during the embryonal and postnatal growth. The activity of monoaminooxidase was measured manometrically according to the method prepared by Kolb and that of acetylcholinesterase colorimetrically according to Hestrin's method. It was found that the activity of MAO and AChE in different parts of the pig alimentary canal was considerably differentiated during ontogenesis. Presence of these enzymes was already shown in the embryonal period. However, their activity was poor and almost on the same level in all the examined parts of intestines. Rapid increase of activity of both enzymes was immediately after pigs birth at the time between parturition and fifth day of postnatal life. In the later period of life (6-11 days after birth) there was a decrease of activity of both MAO and AChE only in duodenum. But in jejunum and colon there was a further, slight though, increase of the activity of both enzymes. Comparing the activity of MAO and AChE in the individual parts of the mature pig intestine with the embryonal and postnatal periods, it was found that in spite of the considerable increase of activity of both enzymes after birth, their level was smaller than that in the mature animals.

AChE activity (EC 3.1.1.7) in the pig brain was examined by spectrophotometric method and histochemically in the light and electron microscope. The results obtained correlate to a high degree with the existing data concerning the activity of the studied enzyme in other mammals. In the pig, the highest activity was encountered in the striatum nucleus accumbens and nucleus amygdale lateralis: the lowest activity was found in encephalon and cerebellum. In comparison with other mammals AChE activity in the pig brain is considerably lower. No comparable proportions have been encountered between the activities of AChE and ChAT in particular brain regions in the mammals examined so far and also in the pig. The results of histochemical examination both in the light and in the electron microscope correspond to the results obtained by other authors. The discussion deals with the problem of correlation between the level of AChE activity in particular regions of the pig brain with respect to their phylogenetic growth.

The studies have been carried out in vitro on the colons of 25 rabbits. The material was collected according to the Garry and Gillespie technique. The preparations consisted of the distal region of the colon together with the branches of parasympathetic nerves (pelvic ones) and with the sympathetic nerve (hypogastric). The conductivity was studied by the method of stimulation of these nerves branches with the 10 V impulses, 5 s long frequency 50-100 Hz, every 2-4 minutes. Spontaneous colon motoricity was registered on the kymograph in the chamber with oxyzenized Krebs fluid, according to the Magnus method. 0.3-10.0 ml of the bile per 1 l of the Krebs fluid was administered to the chamber. The same quantities of the physiological salt solution were administered to the chamber as the control. As for the chemically pure biliary acids, the cholic, deoxycholic and chenodeoxycholic acids (Light) were administered in quantities 500-1500 mumoles/l of the Krebs solution, as well as litocholic acid (Serva) in the concentration 300-500 mumoles/l. The control was carried out exactly as previously described. It has been found out that bile can inhibit or stimulate the colon motoricity and the nerves conductivity, depending on its concentration. At the small concentration (0.3 ml/l of the Krebs fluid) the bile stimulated the spontaneous colon motoricity and the conductivity of the sympathetic as well as the parasympathetic nerves. The high concentration (10 ml/l) had the inhibition effect on the colon motoricity and suppressed the nerves conductivity. As for the biliary acids, the strongest inhibitory effect had the litocholic acid, the weaker inhibitory effect had the cholic and deoxycholic acids. The chenodeoxycholic acid suppressed the nerves conductivity but stimulated the colon motoricity and increased the constriction amplitude.

The authors present three patients with the relatively rare location of basal-cell epithelioma in the axilla. The patients were surgically treated.

The studies were carried out in vitro on the jejunum of 10 pigs, 10 rabbits and 10 guinea pigs. The activity monoaminooxidase (MAO) was determined manometrically in Warburg's apparatus, that of acetylcholinesterase (AChE) spectrophotometrically after Ellman's method modified by urek and Syrowatka. The obtained results were statistically analysed. It was shown that in the animals studied the bile acids exerted a considerable effect on the activity of enzymes metabolising neurohormones. Under the influence of bile the activity of monoaminooxidase was inhibited. Stimulating, but less distinctly, bile effected the activity of acetylocholinesterase. Significant differences in the action of bile on the activity of these enzymes were found in the jejunum of the animal species studied. The highest decrease in the activity of MAO under the influence of bile was found in the jejunum of the rabbit (by about 65%), a weker in the pig (by 37.5%) and guinea-pig (by 18.5%). However, the highest increase in the activity of AChE under the influence of bile occurred in the pigs (by about 21.6%), a weaker in the rabbit (by 16.4%) and guinea-pig (by about 11.5%). Among the bile acids, the strongest action on both the activity of MAO and AChE was exerted by lithocholic acid, desoxycholic, weaker by cholic and chenodesoxycholic acid. MAO activity under the influence of lithocholic acid decreased (in the pig by about 63%, rabbit by 55.7% and guinea pig by 34.8%). After incubation of the jejunum in desoxycholic acid a decrease in MAO activity occurred (in the rabbit by 43.8%, pig by about 39.4% and guinea-pig by 15.7%). AChE activity under the influence of lithocholic acid increased (in the pig on average by 62.2% rabbit by 21.8 and guinea-pig by about 10.2%). After incubation of the jejunum in desoxycholic acid an increase in the activity of acetylcholinesteras occurred (in the pig by about 13.5%, guinea-pig by 7.7% rabbit 7.3%).

The aim of our study was to describe the effects of bile and bile acids action on the spontaneous motor activity of the rabbit small and large intestines. The following fragments of intestines were used: duodenum, jejunum, ileum, caecum and colon of 25 rabbits of both sexes, weighing 2-3 kg. The motor activity of the isolated fragments of the intestines was recorded by the suspension method in a 300 ml container with oxygenated Krebs' solution, temp. 37 degrees C (constant), pH 7.8. The intestine was connected with the miograph constructed according to the Gillespie method. Vesicular bile was given in the amounts 0.3-10 ml per 1 l of the Krebs' solution. Chemically pure bile acids were also given as follows: cholic acid (Light) and chenodeoxycholic acid (Light) in the amount 500-1500 mumol/l of the Krebs' solution and lithocholic acid (Serva)--300-500 mumol/l. 0.33 ml/l of the Krebs' solution of the bile caused the threshold reaction, and only in the jejunum caused distinct inhibition of the motor activity. In duodenum, ileum and colon the inhibition time was very short, intestines quickly regained initial activity, and, what is more, frequency and amplitude of spasms were even greater. Big doses of the bile usually inhibited the motor activity of the studied intestine fragments, eliminating completely--with the exception of caecum--the spasms' amplitude, without releasing any relaxation reaction. As for the bile acids, the strongest inhibiting effect had lithocholic acid but, unlike bile, it caused the relaxation reaction.