Activity of the National Polish Bone Marrow Donor Registry--analysis of the matching process successfully completed with hematopoietic stem cell transplantation.

The activity of the National Polish Bone Marrow Donor Registry includes: (1) recruitment and typing of volunteer donors for the registry; (2) primary and confirmatory typing of patients and their families for sibling and family alternative donor transplantations; and (3) donor-recipient matching. There has been an improvement regarding the number of procedures (184 vs 201) and the successfully completed matches (102 vs 145) in 2006-2007 vs 2008-2009, respectively. The success rate of conclusive matching depended on the number of donors in a given registry and the incidence of rare HLA B-C or DR-DQ associations and specificities. In 2008-2009, Polish, German and all European registries, finally matched 129 (out of all 145) donors including 3, 18, 4 donors for patients with and 30, 68, 6 donors for patients without difficult HLA specificities and associations, respectively. Among the HLA-difficult patient group those lacking a donor in Europe and Israel were successfully matched in the USA (10/11 patients); the only one other subject was matched with a donor from Australia.

[A case of tongue neoplasm in a 15-year old patient treated with immunosuppressants for renal insufficiency]. / Przypadek raka jezyka u 25-letniego pacjenta poddanego leczeniu immunosupresyjnemu z powodu niewydolnosci nerek.

The case of lingual cancer in the 25-years old man treated in 2nd ENT Department Silesian Medical Academy in Zabrze was presented in this paper. The patient has been treated from 1989 due to chronic pyelonephritis finished with the renal insufficiency. In 1991 the renal graft was performed and in 1993 graftectomy due to its rejection. In 1991-1993 the long-term immunosuppression was taking including medicines like Cyclosporin, Imuran, Azatioprin and Encorton. The aim of this study was to consider the influence of immunosuppression therapy on lingual cancer appearance in our patient.

Folate, vitamin B12, and sulfur amino acid levels in patients with renal failure.

We examined the plasma profile of sulfur amino acids (SAA) in patients with chronic renal failure (CRF) and looked for any correlation with serum folate (FA) and/or vitamin B12. Group 1 comprised 9 patients with CRF and glomerular filtration rate (GFR) >20 ml/min per 1.73 m2, 9 patients with GFR<20 ml/min per 1.73 m2 comprised group 2, and 14 patients on hemodialysis group 3. The control group comprised 16 healthy children. Homocysteine (Hcy), methionine (Met), cysteine (Cys), and serine (Ser) were measured with gas chromatography. FA and vitamin B12 were measured using enzymatic immunoassay. Median SAA concentrations were significantly lower in controls than in the three groups of patients. Hcy concentrations were 0.8 micromol/l in controls versus 5 micromol/(group 1), 9 micromol/l (group 2), and 20 micromol/l (group 3). Met concentrations were 26 micromol/l in controls versus 26 micromol/l (group 1), 66 micromol/l (group 2), and 281 micromol/l (group 3). Cys concentrations were 10 micromol/ in controls versus 98 micromol/l (group 1), 54 micromol/l (group 2), and 122 micromol/l (group 3). Ser concentrations were 88 micromol/ in controls versus 153 micromol/l (group 1), 239 micromol/l (group 2), and 240 micromol/l (group 3). The median concentrations of FA were lower in controls than in groups 2 and 3: 5.5 ng/ml versus 8 ng/ml and 15 ng/ml, respectively. Vitamin B12 concentrations did not differ between groups. Vitamin levels did not correlate with SAA. The only difference between patients with Hcy levels in the lower and upper quartile was in Met concentration (38 vs. 263 micromol/l, P<0.02) and GFR (P<0.01). In conclusion, patients with CRF had higher SAA concentrations than healthy children. FA concentrations are higher in CRF patients than in healthy children but did not correlate with concentrations of SAA.

Clustering of isochorismate synthase genes menF and entC and channeling of isochorismate in Escherichia coli.

There are two isochorismate synthase genes entC and menF in Escherichia coli. They encode enzymes (isochorismate synthase, EC 5.4.99.6) which reversibly synthesize isochorismic acid from chorismic acid. The genes share a 24.2% identity but are differently regulated. Activity of the MenF isochorismate synthase is significantly increased under anaerobic conditions whereas the activity of the EntC isochorismate synthase is greatly stimulated during growth in an iron deficient medium. Isochorismic acid synthesized by EntC is mainly channeled into enterobactin synthesis whereas isochorismic acid synthesized by MenF is mainly channeled into menaquinone synthesis. When menF or entC were separately placed onto overexpression plasmids and the plasmids introduced into a menF(-)/entC(-) double mutant in two separate experiments, the isochorismate formed was fed into both, the menaquinone and the enterobactin pathway. Moreover, in spite of a high isochorismate synthase activity menaquinone and enterobactin formation were not fully restored, indicating that isochorismate was lost by diffusion. Thus, under these conditions channeling was not observed. We conclude that in E. coli the chromosomal position of both menF and entC in their respective clusters is a prerequisite for channeling of isochorismate in both pathways.

[Stapedectomy in the treatment of otosclerosis in children]. / Stapedotomia w leczeniu otosklerozy wieku dzieciecego.

In the years 1994-2000 in the Laryngology Department Silesian Medical Academy in Zabrze 167 stapedectomy were performed in 143 patients with otosclerosis. In this group of patients 11 stapedectomies in 8 children aged from 9 to 17 years were performed. 5.6% of all the patients with otosclerosis treated in our Department were children. The purpose of this study was to analyze the audiologic results of stapedectomies in children. The postoperative evaluations were performed in the period from 6 months to 6 years after the surgical treatment. In the estimation of the hearing state we analyzed the change of the air-bone gap, an average air conduction hearing improvement, the rate of percentage hearing improvement and an average hearing loss by Bell Telephone Laboratories. In all the operated children we observed the decrease of the air conduction hearing level. Stapedectomy was successful (postoperative air bone gap within 10 dB of the preoperative bone conduction) in 63.6% of the patients. In 3 cases the air bone gap was lower than 20 dB (27.3%). In one case it was greater then 20 dB. The air bone gap closure was at the average of 5.75 dB level. The results were comparable with the other authors. Stapedectomy can be an effective procedure for correcting conducting hearing losses due to the otosclerosis in the pediatric patients.

Polysaccharides from Melittis melissophyllum L. herb and callus.

For comparison of the water-soluble polysaccharides from Melittis melissophyllum L. herb with that produced by Melittis callus cultures the polymeric carbohydrates were extracted from both sources, fractionated by IEC and GPC and the respective fractions analysed concerning sugar composition and linkage characteristics. The dominant structures found in all fractions isolated from herb material and callus were type-II arabinogalactans with a (1-->3)-galactose backbone and arabinose-galactose side chains. No significant differences were found between herb and callus polysaccharides. To optimize the Melittis cell culture systems the culture media were varried systematically. A modified Murashige-Skoog (MS) medium, supplemented with GA3, NAA and BAP was found to be most suitable for large-scale production of callus material.

[Esthesioneuroblastoma: staging system and methods of treatment]. / Nerwiak wechowy--kryteria podzialu i metody leczenia.

A rare neoplasm of the nose and paranasal sinuses--esthesioneuroblastoma was characterised. New staging system, diagnostics and therapeutic methods were analyzed. Diagnostic difficulties in esthesioneuroblastoma recognition were emphasized. The interesting case report confirming this problem was shown. Value of the immunohistochemical examinations was emphasized.

[Sulphur amino acids, vitamin B12 and folic acid in children with chronic renal failure]. / Profil amininokwasów siarkowych, witaminy B12 i kwasu foliowego we krwi u dzieci z przewlekla niewydolnoscia nerek.

UNLABELLED: Aim of the study was to: 1) estimate plasma profile of sulphur AA in children with chronic renal failure (CRF) and in children on hemodialysis (HD), and 2) to evaluate any correlation with serum folic acid (FA) and vitamin B12. PATIENTS: 32 pts with CRF: 9 with GFR > 20 ml/min/1.73 m2 (group 1), 9 with GFR < 20 ml/min/1.73 m2 (group 2), and 14 pts on HD (group 3). METHODS: plasma homocysteine (Hcys), methionine (Met), cysteine (Cys), serine (Ser) were measured with gas chromatography. Serum FA and vit. B12 were measured using MEIA method. RESULTS: median Hcys concentrations were the lowest in group 1: 5 mumol/l vs 9 mumol/l (group 2) and 20 mumol/l (group 3) (p = 0.03). Similarly, the lowest Met levels were observed in group 1--26 mumol/l, vs 66 mumol/l (group 2) and 281 mumol/l (group 3) (p = 0.001). Median Cys level in group 1 was 98 mumol/l vs 54 mumol/l (group 2), and 122 mumol/l (group 3) (p = 0.02). No differences were found in median Ser levels: 153 mumol/l (group 1) vs 239 mumol/l (group 2) and 240 mumol/l (group 3). The median concentrations of FA were 6.3 ng/ml (group 1) vs 8 ng/ml (group 2) and 15 ng/ml (group 3) (NS). Median concentrations of vit. B12 were 256 pg/ml (group 1) vs 379 pg/ml (group 2) and 322 pg/ml (group 3) (NS). There were no correlation between sulphur AA and FA and vit. B12 levels. The only difference between pts with Hcys levels remaining in lower and upper quartile concerned Met concentration (38 vs 263 mumol/l, p < 0.02) and GFR (p < 0.01). CONCLUSIONS: Hyperhomocysteinemia develops already in moderate CRF. In pts on HD levels of Met and Cys are also raised. FA and vit. B12 concentrations are normal and do not correlate with plasma concentrations of sulphur AA.

In vitro cultures of higher plants and fungi as a potential source of bioactive metabolites.

The review presents the results of investigations conducted at the Chair of Pharmaceutical Botany, Collegium Medicum, Jagiellonian University, which demonstrated a prospects to obtain biologically active metabolites representative of many chemical groups (furanocoumarins, polysaccharides and lectins, indole compounds, carotenoids) in in vitro cultures of both higher plants and higher fungi (Macromycetes) (mycelial cultures). These cultures can be a potential, rich, new source of metabolites.

Aluminum contamination of parenteral nutrition additives, amino acid solutions, and lipid emulsions.

Contamination of parenteral nutrition solutions with aluminum may result in accumulation of this element in bones and, in premature infants, may inhibit bone calcium uptake and induce cholestasis. We measured the aluminum concentration of small-volume parenterals, amino acid solutions, lipid emulsions, and special solutions containing glucose, amino acids, electrolytes, and trace elements (standard I for children with a body weight of 3-5 kg, standard II for children with a body weight of 5-10 kg). The method used was graphite furnace atomic absorption spectrometry GTA-AAS (SpectrAA-400 Plus, Varian, PtY Ltd., Mulgrave, Australia). Quality control was run with the use of control serum (Seronorm, Nycomed, Oslo, Norway). The aluminum contents of parenterally administered solutions were: pediatric trace elements, 130 micrograms/L, and pediatric trace elements, 3000 micrograms/L; phosphorus salts: K-phosphates, 9800 micrograms/L, and Na/K phosphates, 13,000 micrograms/L; 10% calcium gluconate, 4400 micrograms/L; 6.5% amino acids, 30 micrograms/L; 10% amino acids, 120 micrograms/L; 12.5% amino acids, 121 micrograms/L; 20% lipid emulsion, 30 micrograms/L; 20% lipid emulsion, 180 micrograms/L; water-soluble vitamins, 12 micrograms/L; lipid soluble vitamins, 360 micrograms/L; standard I, 55 micrograms/L; standard II, 90 micrograms/L; The aluminum intake from parenteral nutrition was 6.6-10.8 micrograms.kg-1.d-1--a dose exceeding the safety limit of 2 micrograms.kg-1.d-1. The possible association of aluminum not only with metabolic bone disease, but also with encephalopathy, dictates caution when dealing with the pediatric population on long-term parenteral nutrition. In the absence of reliable label information, it seems proper to monitor the aluminum concentration in parenteral nutrition products and to report it in professional journals.

Further delineation of the classical Smith-Lemli-Opitz syndrome phenotype at different patient ages: clinical and biochemical studies.

We present here extensive clinical and biochemical data on thirteen SLOS (type I) patients with proven defect in cholesterol biosynthesis for further delineation of the classical SLOS phenotype at different patient ages.

[Rhinomanometry of nasal patency in children with complete and secondary cleft palate]. / Rynomanometryczna ocena droznosci nosa u dzieci z calkowitym rozszczepem podniebienia pierwotnego i wtórnego.

The authors evaluated the morphology of nasal cavities and nasal patency using active anterior rhinomanometry in 94 children (mean age 10.2 years). Complete bilateral primary and secondary cleft palate was confirmed in 28 children (29.8%), unilateral in 66 children (70.2%), where complete right-side primary and secondary cleft palate was observed in 19 children (28.8%) and complete left side primary and secondary cleft palate in 47 children (71.2%). Measurement methods were used according to Rhinomanometric Standardization Committee. Significant relationship between nasal patency examined using active anterior rhinomanometry and complete cleft palate was confirmed. High, symmetric resistance of nasal airflow was observed in children with complete bilateral primary and secondary cleft palate. Children with complete unilateral primary and secondary cleft palate were found to have asymmetric nasal airflow, higher on the cleft side. In this group mean nasal airflow resistance was higher on non-cleft side than in population without defects, whereas on clefted side it is approximal to population without defects. In children with complete bilateral primary and secondary cleft palate mean resistances distribution was symmetric but higher than in population without defects.

[Anthropometric assessment of the nose and nasal patency by means of anterior rhinomanometry method in children with complete primary and secondary cleft palate]. / Ocena antropometryczna nosa i jego droznosci wyznaczona metoda przedniej rynomanometrii u dzieci z calkowitym rozszczepem podniebienia pierwotnego i wtórnego.

This study was carried out as an attempt to evaluate the relationship between the results of nasal patency examinations and selected anthropometric measurements of nasal cavities in children with complete primary and secondary cleft palate. The examined group consisted of 130 children with cleft palate treated by means of surgery in early childhood owing to their clefts. Concerning the diagnostic methods the study group was divided into two subgroups. Group I consisted of 75 children in whom face photographs in frontal and axial projections towards the external nasal aperture were analysed. Group II consisted of 55 children in whom by means of P-A teleradiography projection the total area, the maximum height and width of nasal cavity were measured separately for the side with cleft and without cleft. The assessment of nasal patency tests using the objective method--active anterior rhinomanometry was carried out.

[Laryngeal trauma: clinical aspects]. / Urazy krtani--aspekty kliniczne.

The authors presented 11 cases trauma to the larynx. Surgical treatment was applied. It dependent on kind of injury of the larynx and other organs. In 90% of cases the larynx was reconstructed and free passage obtained.

[Tuberculosis of the larynx in patients treated in laryngological clinic No. 2 in Zabrze]. / Gruzlica krtani u chorych leczonych w II Klinice Laryngologii Sl. AM w Zabrzu.

3 cases of tuberculosis of the larynx were presented in this paper. Provisional diagnosis was tumor of the larynx. Further specialistic investigations e.g. MDL and biopsy were done and revealed proper diagnosis tuberculosis of the larynx.