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Biomedical subjects

A Jankowski

Publications and source records attributed to A Jankowski.

At least 19 recordsLinked to original sources

Rheumatoid arthritis in a patient with common variable immunodeficiency: difficulty in diagnosis and therapy.

Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency syndrome in adults with equal sex prevalence. The syndrome typically presents as recurrent infections, with onset in childhood or young adulthood (between 20 and 30 years). CVID patients also have a higher prevalence of autoimmune diseases. A 38-year-old woman presented to the Rheumatology Department with polyarthralgia and fever of 39 degrees C of several months' duration. She had recurrent respiratory and gastrointestinal tract infections and pernicious anemia. Immunological studies showed decreased levels of IgG, IgM, complete IgA deficiency, increased percentage of CD8 lymphocytes, and a reduced CD4:CD8 ratio. HLA-DR typing was performed and we identified HLA-DRB1*01. Adequate intravenous immune globulin substitution as well as antibiotic and anti-inflammatory treatment resulted in the remission of arthritis. Hand radiograms repeated after 12 months showed narrowing of the intra-articular space in the right metacarpophalangeal and radiocarpal joints with multiple bone cysts and erosions. Erosions were found in both humeral heads as well. This prompted the diagnosis of rheumatoid arthritis. Arthritis can be a presenting symptom of primary immunodeficiency in adults, especially when accompanied by recurrent infections or autoimmune diseases. These patients require more advanced diagnostic procedures and therapeutic cooperation of different specialists.

Adult↗

[Imaging of adrenal ganglioneuroma: a case report].

The authors report a case of adrenal ganglioneuroma diagnosed in a young woman with melanoma. This benign hormone silent tumor arises from neural crest tissue. The imaging features at US, CT and MRI are described. Diagnosis was confirmed at CT guided biopsy.

Adrenal Gland Neoplasms↗

Inhibition of nasal polyp mast cell and eosinophil activation by desloratadine.

Nasal polyp tissue which contains mast cells and eosinophils is similar to the inflamed airway mucosa in cellular composition and mediator content. This investigation assessed the effect of desloratadine (DL), on activation of cells in nasal polyp tissue. Polyps were obtained from 22 patients with chronic rhinosinusitis [nine aspirin acetylosalitic acid (ASA)-sensitive and 13 ASA-tolerant]. Polyp tissue was dispersed by digestion, and preincubated with DL and incubated with anti-immunoglobulin E (IgE) or calcium ionophore. LTC4, eosinophil cationic protein (ECP) and tryptase concentrations in supernatants were measured by immunoassays. Desloratadine (1, 10 and 50 microM) inhibited calcium ionophore-induced LTC4 release by a mean of 29%, 50% and 63% respectively, and anti-IgE-induced LTC4 release by a mean of 27%, 35% and 39% respectively. Calcium ionophore-induced tryptase release was inhibited 60% and 69% by 10 and 50 microM of DL, respectively, and anti-IgE-induced tryptase release was inhibited 33%, 47% and 66% for 1, 10 and 50 microM of DL. Desloratadine 10 microM and 50 microM inhibited ECP release by and 45% and 48% respectively. Polyp tissue from ASA-sensitive patients when compared with ASA-tolerant patients released at baseline significantly more ECP (medians 120.0 microg/ml, range: 69.0-182.0 vs 63.4 microg/ml, range: 3.7-172.0; P <0.05), but similar amounts of tryptase and LTC4. This study demonstrated that DL inhibits activation of both eosinophils and mast cells derived from a site of airway mucosal inflammation.

Adult↗

Association of stem cell factor expression in nasal polyp epithelial cells with aspirin sensitivity and asthma.

Mast cells constitute a significant proportion of cells infiltrating nasal polyp tissue, and epithelial cells may release stem cell factor (SCF), a cytokine with chemotactic and survival activity for mast cells. We aimed to assess the expression of SCF in human nasal polyp epithelial cells (NPECs) as related to patients' clinical phenotypes. Nasal polyp tissues were obtained from 29 patients [including nine with aspirin (ASA)-hypersensitivity and 12 with bronchial asthma] undergoing polypectomy for nasal obstruction. Epithelial cells were obtained following 6-week culture of nasal polyps explants. The SCF released into the culture supernatant was assessed by enzyme-linked immunosorbent assay (ELISA) and total SCF mRNA in the polyp tissue was determined by semiquantitative reverse transcriptase polymerase chain reaction (RT-PCR). For the whole group of patients, the number of polypectomies correlated with expression of SCF mRNA (r = 0.62; P < 0.005), SCF protein in the NPECs supernatants (r = 0.39; P < 0.05) and with density of mast cells in epithelial layer (r = 0.37; P < 0.05) and stromal layer (r = 0.5; P < 0.01) of nasal polyps. The SCF/beta-actin mRNA ratios were significantly higher in ASA-hypersensitive (AH) asthmatics (median 0.97, range: 0.8-1.5) when compared with ASA-tolerant (AT) patients (median 0.5, range: 0.1-0.7; P < 0.001). The SCF protein concentration in NPEC supernatants was also significantly higher in AH asthmatics (median 1.10 pg/microg DNA, range: 0.4-1.9) when compared with AT patients (median 0.1 pg/microg DNA, range: 0.02-1.2; P < 0.001). In the subpopulation of ASA-sensitive asthmatics the number of polypectomies correlated also with the density of mast cells and eosinophils in the polyp tissue.

Adult↗

In situ measurements of the pH of mammalian peroxisomes using the fluorescent protein pHluorin.

Peroxisomes are metabolically active organelles that participate in the oxidation of long-chain fatty acids and in the biosynthesis of bile acids, cholesterol, and ether phospholipids. Even though maintenance of a stable acid-base milieu is essential for proper peroxisomal function, the determination of the peroxisomal pH (pH(p)) remains inconclusive, and little is known about its regulation. To measure the pH of intact peroxisomes in situ, we used the peroxisome-specific carboxyl-terminal targeting sequence, SKL, to deliver a pH-sensitive mutant of the green fluorescent protein (pHluorin-SKL) selectively into peroxisomes. Proper targeting was verified by colocalization with the peroxisomal marker catalase. Peroxisomes were visualized by imaging fluorescence microscopy, and ratiometric measurements were combined with calibration using ionophores or a null-point method to estimate pH(p). The pH(p) was between 6.9 and 7.1, resembling the cytosolic pH. Manipulation of the cytosolic pH in intact cells or after permeabilization of the plasmalemma with streptolysin O revealed that pH(p) changed in parallel, suggesting that the peroxisomal membrane is highly permeable to H(+) (equivalents). We conclude that peroxisomes do not regulate their pH independently, but instead their large H(+) permeability effectively connects them with the buffer reservoir of the cytoplasm and with the homeostatic mechanisms that control cytosolic pH.

Animals↗

Within the hemopoietic system, LAR phosphatase is a T cell lineage-specific adhesion receptor-like protein whose phosphatase activity appears dispensable for T cell development, repertoire selection and function.

Expression of the receptor-type tyrosine phosphatase LAR was studied in cells of the murine hemopoietic system. The gene is expressed in all cells of the T cell lineage but not in cells of any other hemopoietic lineage and the level of expression in T cells is developmentally regulated. The CD4(-)8(-)44(+) early thymic immigrants and mature (CD4(+)8(-)/CD4(-)8(+)) thymocytes and T cells express low levels, whereas immature (CD4(-)8(-)44(-) and CD4(+)8(+)) thymocytes express high levels of LAR. Among bone marrow cells only uncommitted c-kit(+)B220(+)CD19(-) precursors, but not B cell lineage committed c-kit(+)B220(+)CD19(+) precursors, express low levels of LAR. In contrast to the c-kit(+)B220(+)CD19(+) pre-BI cells from normal mice, counterparts of pre-BI cells from PAX-5-deficient mice express LAR, indicating that PAX-5-mediated commitment to the B cell lineage results in suppression of LAR. During differentiation of PAX-5-deficient pre-BI cell line into non-T cell lineages, expression of LAR is switched off, but it is up-regulated during differentiation into thymocytes. Thus, within the hemopoietic system, LAR appears to be a T cell lineage-specific receptor-type phosphatase. However, surprisingly, truncation of its phosphatase domains has no obvious effect on T cell development, repertoire selection or function.

Amino Acid Sequence↗

Natural resistance to intracellular infections: natural resistance-associated macrophage protein 1 (Nramp1) functions as a pH-dependent manganese transporter at the phagosomal membrane.

Mutations at the natural resistance-associated macrophage protein 1 (Nramp1) locus cause susceptibility to infection with antigenically unrelated intracellular pathogens. Nramp1 codes for an integral membrane protein expressed in the lysosomal compartment of macrophages, and is recruited to the membrane of phagosomes soon after the completion of phagocytosis. To define whether Nramp1 functions as a transporter at the phagosomal membrane, a divalent cation-sensitive fluorescent probe was designed and covalently attached to a porous particle. The resulting conjugate, zymosan-FF6, was ingested by macrophages and its fluorescence emission was recorded in situ after phagocytosis, using digital imaging. Quenching of the probe by Mn(2+) was used to monitor the flux of divalent cations across the phagosomal membrane in peritoneal macrophages obtained from Nramp1-expressing (+/+) and Nramp1-deficient (-/-) macrophages. Phagosomes from Nramp1(+/+) mice extrude Mn(2+) faster than their Nramp(-/-) counterparts. The difference in the rate of transport is eliminated when acidification of the phagosomal lumen is dissipated, suggesting that divalent metal transport through Nramp1 is H(+) dependent. These studies suggest that Nramp1 contributes to defense against infection by extrusion of divalent cations from the phagosomal space. Such cations are likely essential for microbial function and their removal from the phagosomal microenvironment impairs pathogenesis, resulting in enhanced bacteriostasis or bactericidal activity.

Animals↗

[The influence of Aronia melanocapra in experimental pancreatitis].

The administration of anthocyanin dyes from Aronia melanocarpa in the rats before the intraperitoneal injections of PAF and ceruleine have a beneficial effect on the development of the acute experimental pancreatitis. It was revealed the reduction of pancreas swelling and decreasing of lipid peroxidation and adenosine deaminase activity. The examination was carried out on 149, weighing 200-250 g, female and male Wistar rats. They lived in the animal quarters with a stable temperature and humidity being fed with standard fodder (Murigan) and water ad libitum.

Acute Disease↗

[The effect of anthocyanin dye from grapes on experimental diabetes].

In the work an effect of anthocyanins of red wine type Cabernet on the course and intensity of symptoms of the experimental diabetes in the rats has been examined. The estimation of the course of experimental diabetes was based on: determination of sugar concentration in urine and blood serum, determination of concentration of the unsaturated fatty acids peroxidation in urine and blood serum, also on the change of body mass during the experiment. The examination was carried out on 80, weighing 200-250 g, rats. They lived in the animal quarters with a stable temperature and humidity being fed with standard fodder (Murigan) with water supply in ever quantity. The rats were divided into the following four group: I--control group, II--group of animals receiving intraperitoneal streptozotocin in concentration 70 mg/kg m.c., III--group of animals receiving intragastric 10 mg/kg m.c. of natural anthocyanin dye of red wine type Cabernet, IV--group of animals receiving intraperitoneal 70 mg/kg m.c. of streptozotocin and simultaneously receiving intragastric 1 mg/kg m.c. of natural anthocyanin dye. An essential increase of glucose concentration in urine was found in the rats after streptozotocin injection. A simultaneous daily administration of anthocyanins obtained from red wine type Cabernet and streptozotocin substantially decreased sugar concentration in urine and blood serum. Those anthocyanins also inhibited loss of body mass caused by the former injection of streptozotocin. Simultaneously antocyan pigment was stated to considerably prevent generation of free oxygen radicals. The decrease of peroxidation of lipids, the measure of which was lowering of the concentration of the products of unsaturated fatty acids oxidation in urine and blood serum was also observed.

Animals↗

A noninvasive fluorimetric procedure for measurement of membrane potential. Quantification of the NADPH oxidase-induced depolarization in activated neutrophils.

The electrogenic activity of the NADPH oxidase is associated with depolarization of the plasma membrane in activated neutrophils. The magnitude and consequences of this depolarization, however, remain unknown. Neutrophils are not amenable to electrophysiological determinations of membrane potential by current clamp. Instead, the occurrence of depolarization has been inferred from the use of potential-sensitive fluorescent dyes. However, such dyes partition into intracellular organelles and may yield erroneous results, particularly because the NADPH oxidase resides largely in secretory granules, where it has been claimed to become activated. We confirmed the intracellular generation of oxidase products using dihydrorhodamine, which is converted to the fluorescent rhodamine 123 when oxidized. Rhodamine 123 accumulated inside endomembrane organelles in both neutrophils and in differentiated HL60 cells, where it co-localized with the primary granule marker CD63. To estimate the surface membrane potential without interference from organelles, we devised a method based on the voltage-driven uptake of Mn(2+) across the plasmalemma. The uptake of Mn(2+) through calcium release-activated channels was measured as the rate of Indo-1 fluorescence quenching in thapsigargin-treated cells. The rate of Mn(2+) influx was found to vary when the membrane potential was manipulated using conductive ionophores and also when the NADPH oxidase was activated. A calibration curve in the positive potential range was constructed using the Na(+) ionophore SQI-Pr. Using this calibration, the membrane potential of phorbol ester-activated neutrophils was found to reach +58 +/- 6 mV, a sustained depolarization of over 100 mV compared with the resting potential. The depolarization was greatly diminished when the NADPH oxidase was inhibited with diphenylene iodonium. Together, these results indicate that the NADPH oxidase can generate a large depolarization of the plasmalemma, which should suffice to activate a variety of voltage-gated channels, including the outwardly rectifying H(+) conductance.

Membrane Potentials↗

Determination and pharmacokinetics of acyclovir after ingestion of suspension form.

The study describes, simple, precise, sensitive and accurate HPLC assay with spectrofluorimetric detection for the determination of acyclovir in human plasma. The method was linear over a range 25 1200 ng ml(-1). The average yield in this method exceeded 80%. Limits of quantitation and detection were 25 and 10 ng ml(-1), respectively. On the basis of reported method, a single-dose of pharmacokinetics on 24 men, in two doses (200 and 400 mg) of acyclovir suspension has been investigated. Pharmacokinetic parameters obtained from both doses of the drug were compared. The linearity of acyclovir pharmacokinetics in the investigated dose ranges has been confirmed.

Acyclovir↗

[Treatment of varicocele with laparoscopy].

To assess the efficacy of laparoscopic techniques for the operative therapy of varicocele in children and adolescents. Thirty-two children and adolescents who underwent laparoscopic varicocelectomy, between May 1995 to December 1997 were evaluated. In all patients laparoscopic therapy was effective and no major complications occurred. The laparoscopic technique is minimally invasive operation with optimal results.

Adolescent↗

[Analysis of results after primary heminephrectomy in surgical treatment of duplicated pyelo-caliceal system].

AIM: Determination of the effective diagnostics and therapeutic procedures in duplicated pyelo-calyceal system with upper pole dysfunction. METHOD: Retrospective review of operative procedures and follow-up. MATERIAL: In Department of Pediatric Surgery University of Medical Sciences in Poznań (Poland), 383 children with duplicated pyelo-calyceal system were treated between 1976 and 1997. The diagnosis was based on: IVP, MCU, scintigraphy, cystoscopy, and since 1984 on US. Conservative treatment was performed in 103 (26%) children with low VOR. The most frequent surgical procedure was Politano-Leadbetter ureteroneocystostomy carried out in 167 (44%) children. Primary heminephrectomy was performed in 79 (21%) patients with complete deterioration of the upper pole of the kidney. In order to attain upper pole parenchyma protection in 34 (9%) children the following primary procedures were performed: extirpation of the ureterocele with ureteroneocystostomy of both ureters, cutaneous uretral stoma or uretero-pyeloanastomosis. RESULTS: Among 79 children with primary heminephrectomy of the duplicated system with ureterocele, secondary operation was required only in 18 patients. From 34 children with kidney parenchyma protecting procedures in 18 patients secondary heminephrectomy or nephrectomy was performed. CONCLUSIONS: 1. Primary heminephrectomy with ureterectomy with partial ureterectomy is a method of choice in the treatment of seriously deteriorated upper pole of the duplicated pyelo-calyceal system. 2. Secondary surgical procedures were required only in 25% of children in whom primary heminephrectomy was performed. 3. Primary heminephrectomy is a save procedure with a low complication rate.

Adolescent↗

[Review of surgical treatment results for vesicoureteral reflux in children using the Politano-Leadbetter technique].

The analysis presents 622 children in the age ranged from 4 months to 14 years, operated on vesicoureteral reflux. The treatment was carried on in Department of Pediatric Surgery in Poznan between 1983 and 1992. All these children were operated on with antireflux Politano-Leadbetter technique, modified by Sarrazin, protecting reimplanted ureters with stenting catheters. Duplicated ureters were reimplanted "en bloc" according to Bettex technique, also with Sarrazin modification. Preoperative parameters included: history, radiological evaluations: voiding cystourethrogram, intravenous pyelogram or ultrasound imaging, endoscopic evaluations--cystourethroscopy (with meatal calibration in girls), laboratory blood and urine results. The reflux degree was estimated according to international classification IRSC. There were 12 (2%) children with II degree, 498 (80%) with III degree, 101 (16%) with IV degree and 11 (2%) patients with V degree reflux. In order to compare the results of operative treatment, all analyzed children were subdivided into groups: 303 (48.7%) patients with primary reflux, 77 (12%) with reflux to duplicated pyelo-ureteral systems, 17 (2.7%) with refluxing megaureters, 66 (10%) with reflux to hypoplastic kidney, 99 (15.9%) with cystic cystitis and 114 (18%) children with urethral meatal stricture. Good recovery with uncomplicated postoperative course was obtained in 606 (97.4%) patients. Early postoperative complications occurred in 16 (2.6%). In outpatient control the results of urinalysis and urine cultures, ultrasound imaging and voiding cystourethrograms were tested. Urine cultures were negative in 501 (80.5%) patients 3-4 weeks after discharge increasing to above 92% of patients 6 months after operation. In postoperative imaging control, 600 (96.5%) patients presented with normal status of upper urinary tract, in 14 (2.2%) transient dilatation of reimplanted ureters was observed and in 8 (1.3%) ureters had to be reimplanted because of secondary dilatation. After 6 months postoperative voiding cystourethrograms showed cessation of reflux in 589 (94.6%) patients, in 33 (5.2%) recurrent reflux was observed, which disappeared in 20 of them in control. In 13 patients urodynamic evaluation showed vesicourethral dysfunction, medically curable.

Adolescent↗

[Intravesical instillation of atropine in treatment of detrusor overactivity in children].

UNLABELLED: We present our own experiences with intravesical Atropine instillation in patients with voiding dysfunction. We used concentration of 10(-6) Atropine in 14 patients with detrusor overactivity. In 12 patients neurogenic voiding dysfunction was caused by a meningomyelocoele. In 2 dysfunction was non-neurogenic. Patients were selected on the base of urodynamic investigation in which low bladder cystometric capacity was caused by detrusor overactivity and poor bladder compliance. This type of therapy was started in patients who had not responded to oral oxybutynin therapy, had major side-effects, or oral therapy had not been possible. All were on clean intermittent catheterisation. The effects on the functional parameters were estimated in urodynamic investigations. With the aim of estimating systemic side-effects blood pressure and heart rate was monitored during Atropine infusion. RESULTS: 6 patients responded to intravesical instillation of Atropine. The increase in bladder cystometric capacity was caused by the decrease in bladder overactivity. No effect on bladder wall compliance was noted. No systemic side-effects were observed. CONCLUSIONS: 1. Intravesical Atropine instillation was useful method of treatment in some patients with detrusor overactivity. 2. No systemic side effects were noted. 3. Because of limited effectiveness and low cost, this method should be considered as an alternative way of decreasing bladder overactivity.

Administration, Intravesical↗

[Electrostimulation in treatment of neurogenic and non-neurogenic voiding dysfunction].

UNLABELLED: The efficiency of transcutaneous and intravesical temporary electrostimulation were evaluated in patients with neurogenic and non-neurogenic voiding dysfunction. Non-invasive electrostimulation was performed in 42 children with voiding dysfunction, at the age of 4 months to 15 years. In 5 children rectal electrode and impulse frequency of 50 Hz was used because of incompetent urethral function. In 9 children with detrusor overactivity rectal electrode and impulse frequency of 5 Hz was used. In 12 girls with discoordinated voiding electrostimulation with rectal electrode was used in biofeedback training. In one case intravesical electrostimulation was used in a girl with lazy bladder syndrome. In 15 patients with neurogenic voiding dysfunction intravesical electrostimulation was used to improve bladder sensation. The efficiency of the treatment was evaluated on the base of urodynamic investigation and patients' observations. The improvement was observed in 3 children from the group with urethral incompetence, both in urodynamic parameters and subjective patients' observations. One girl claimed no change in continence despite improvement in cystometric and profilometric testing. In one girl no change in urodynamic evaluation and continence was observed. Good results were observed in the group of 9 girls in which electrostimulation with rectal electrode was a part of the biofeedback training in discoordinated voiding. In the group of 9 children in whom electrostimulation with rectal electrode was used because of detrusor overactivity in 5 cases improvement in cystometric capacity was estimated. The estimation of usefulness of the intravesical electrostimulation in patients with neurogenic voiding dysfunction is not possible because of too short time of observation and the age of patients. We had to stop stimulation with rectal electrode in one patient because of inflammation of the skin in perineal region caused by cystitis. In two children stimulated with intravesical electrodes a new infection of urinary tract was observed. CONCLUSIONS: 1. Electrostimulation is a non-invasive, save method in the treatment of voiding dysfunction. 2. Electrostimulation with rectal electrodes is an effective method of treatment in cases of urethral incompetence, and an alternative method of treatment for detrusor overactivity. 3. Electrostimulation with rectal electrodes is an additional tool in biofeedback training of discoordinated voiding.

Adolescent↗