Nutritional status and feeding difficulties in children up to 2 years of age with cow's milk allergy.

BACKGROUND/OBJECTIVES: To assess the nutritional status and incidence of feeding difficulties in Polish children up to 2 years of age with cow's milk allergy (CMA) on cow's milk proteins-free diet. METHODS: A cross-sectional, multi-center study included children aged 6 months to 2 years with confirmed or suspected (without oral food challenge) diagnosis of CMA on the elimination diet for at least 1 month. The primary outcomes were an assessment of proportion of children with impaired nutritional status (with the weight for length and body mass index (BMI) z-score > 1 and <-1), and feeding difficulties according to the Montreal Children's Hospital Feeding Scale. Children with confirmed and suspected CMA were assessed separately. RESULTS: A 144 children with confirmed CMA and 88 with suspected CMA were included (57 and 78% with multiple food allergies, respectively). Among children with confirmed CMA, one-third (35.5%) of participants had any nutritional status impairment regardless of definition. Among those, most of children had mild malnutrition (10.4 vs. 9%) and possible risk of overweight (11.1 vs. 9.7%; following respectively BMI for age and weight for length z-scores). Only 16.0% of children had feeding difficulties. Feeding difficulties was identified to be a risk factor for moderate malnutrition compared to children without feeding difficulties (odds ratio 10, 95% confidence interval: 4-27). CONCLUSIONS: Mild malnutrition and possible risk of overweight are concern in children up to 2 years of age on cow's milk proteins-free diet. Feeding difficulties are less common, however, may affect the nutritional status.

Comparison of the prognostic value of frailty assessment tools in patients aged ≥ 65 years hospitalized in a cardiac care unit with acute coronary syndrome.

BACKGROUND: Frailty is associated with adverse events in elderly patients with acute coronary syndrome (ACS). Our aim was to compare the prognostic value of four frailty scales in patients aged ≥ 65 years hospitalized with ACS in a cardiac care unit (CCU). METHODS: Patients aged ≥ 65 years with ACS were included. Frailty was assessed using the Fried frailty scale (reference standard), the Edmonton Frail Scale (EFS), the FRAIL scale, and the Clinical frailty scale (CFS). The primary end point was all-cause mortality and the secondary end point was unscheduled rehospitalization. RESULTS: One hundred and seventy four patients aged ≥ 65 years with ACS were recruited. The median follow-up was 637.5 days. Frailty was identified in 41.4%, 40.2%, 39.1% and 36.3% patients by the Fried frailty scale, EFS, FRAIL scale and CFS, respectively. The agreement coefficients were 0.88, 0.86, and 0.79 for the FRAIL scale, EFS and CFS, respectively. In the Cox regression model, frailty was associated with all-cause mortality regardless of the scale used (univariate: hazard ratio [HR] 95% CI = 10.5, 2.4-46.8 Fried frailty scale; 12.0, 2.7-53.4 FRAIL scale; 7.1, 2.0-25.2 EFS; 8.3, 2.4-29.6 CFS. Multivariate: HR = 5.1, 1.1-23.8 Fried frailty scale; 5.7, 1.2-26.8 FRAIL scale; 3.7, 1.0-14.0 EFS; 4.2, 1.1-15.9 CFS). The FRAIL scale had the highest HR. In the univariate analysis, frailty was associated with unscheduled rehospitalization (HR = 3.2, 1.7-6.0 Fried frailty scale; 3.4, 1.8-6.3 FRAIL scale; 3.5, 1.8-6.6 EFS; 3.1, 1.7-5.8 CFS). In the multivariate analysis, only the EFS independently predicted unscheduled rehospitalization (HR = 2.2, 1.1-4.63). CONCLUSIONS: Frailty assessed by the Fried frailty scale, FRAIL scale, EFS and CFS is associated with all-cause mortality and unscheduled rehospitalization in elderly patients hospitalized in a CCU with ACS. The adjusted HR of the FRAIL scale for all-cause mortality was the highest among the scales compared, whereas the EFS was an independent predictor of unscheduled rehospitalization. These data should be taken into consideration when choosing a frailty assessment tool.

Usefulness of high-frequency ultrasound to assess the healing progress of shin ulcers.

INTRODUCTION: As the average lifespan becomes longer, the number of cases of chronic shin ulcers is rising, which is slowly becoming a societal problem. Currently, there no methods for objective evaluation of ulcer healing process. AIM OF THE STUDY: The aim of the study was to assess the use of high-frequency ultrasound to monitor the healing of shin ulcers subjected to laser biostimulation. MATERIAL AND METHODS: The study included 8 patients (4 men and 4 women) aged from 46 to 81 years with 9 shin ulcers in whom the healing process did not progress within 8 weeks despite effective causal treatment. The ulcers were subjected to laser biostimulation. The induced changes were monitored using high-frequency ultrasound and photographic records. Ultrasound examination was used to assess the depth of ulceration and the thickness of granulation tissue, migrating epidermis, subepidermal low-echogenic band (SLEB) and epidermis in the immediate vicinity. The wound severity index and ulcer vascularity were also assessed. RESULTS: As a result of laser therapy, in 7 out of 9 cases the ulcers healed completely and in the remaining 2 cases ulcer size was reduced. During the ulcer healing process induced by laser therapy, ultrasound examination revealed decreasing ulcer depth, wound severity index and SLEB thickness. Granulation tissue increased in thickness and there was an epidermal formation effect (newly formed epidermis was much thicker than the one surrounding the wound and became thicker as a result of laser therapy sessions). During the healing of ulcers induced by laser therapy no significant changes were observed in ulcer vascularity and thickness of the surrounding epidermis. CONCLUSIONS: High-frequency ultrasound is a useful technique for the imaging of leg ulcers. It allows one to monitor their healing process induced by laser biostimulation. Further research on the subject needs to be pursued.

S-Detect Software vs. EU-TIRADS Classification: A Dual-Center Validation of Diagnostic Performance in Differentiation of Thyroid Nodules.

Computer-aided diagnosis (CAD) and other risk stratification systems may improve ultrasound image interpretation. This prospective study aimed to compare the diagnostic performance of CAD and the European Thyroid Imaging Reporting and Data System (EU-TIRADS) classification applied by physicians with S-Detect 2 software CAD based on Korean Thyroid Imaging Reporting and Data System (K-TIRADS) and combinations of both methods (MODELs 1 to 5). In all, 133 nodules from 88 patients referred to thyroidectomy with available histopathology or with unambiguous results of cytology were included. The S-Detect system, EU-TIRADS, and mixed MODELs 1-5 for the diagnosis of thyroid cancer showed a sensitivity of 89.4%, 90.9%, 84.9%, 95.5%, 93.9%, 78.9% and 93.9%; a specificity of 80.6%, 61.2%, 88.1%, 53.7%, 73.1%, 89.6% and 80.6%; a positive predictive value of 81.9%, 69.8%, 87.5%, 67%, 77.5%, 88.1% and 82.7%; a negative predictive value of 88.5%, 87.2%, 85.5%, 92.3%, 92.5%, 81.1% and 93.1%; and an accuracy of 85%, 75.9%, 86.5%, 74.4%, 83.5%, 84.2%, and 87.2%, respectively. Comparison showed superiority of the similar MODELs 1 and 5 over other mixed models as well as EU-TIRADS and S-Detect used alone (p-value < 0.05). S-Detect software is characterized with high sensitivity and good specificity, whereas EU-TIRADS has high sensitivity, but rather low specificity. The best diagnostic performance in malignant thyroid nodule (TN) risk stratification was obtained for the combined model of S-Detect ("possibly malignant" nodule) and simultaneously obtaining 4 or 5 points (MODEL 1) or exactly 5 points (MODEL 5) on the EU-TIRADS scale.

Avoidance of allergens as an environmental method in the prevention of inhaled allergy symptoms.

Asthma and other inhaled allergies are some of the most common paediatric diseases. The association of exposure to allergens with induction and exacerbation of symptoms has been proven. The majority of allergens are permanently or periodically suspended in the air, which leads to impaired quality of life for sensitive patients. Therefore, many methods of prevention and therapy of allergic diseases have been developed. The method of allergen exposure avoidance is often the first and the most significant measure. The present research has been conducted to evaluate, based on scientific data, which measures have the most reliable evidence of effectiveness. Environmental allergen avoidance methods, despite limited evidence supporting their clinical efficacy, are listed as the main therapeutic approaches in most recommendations. The significance of the holistic approach is also emphasised: only simultaneous introduction of several avoidance methods can bring possibly beneficial effects for the patient.

Prospective analysis of inter-observer and intra-observer variability in multi ultrasound descriptor assessment of thyroid nodules.

Aim: The aim of this study was to evaluate the inter- and intra-observer variability and accuracy of ultrasound assessment of thyroid nodules using a descriptive lexicon. Materials and methods: A prospective study was performed on complete ultrasound examinations, including sonoelastography and color Doppler ultrasound of 18 patients with 20 thyroid nodules. A total of 20 records of thyroid nodules from these techniques were duplicated, numbered, and randomly arranged. Five radiologists assessed the recordings independently. Cohen Kappa and Fleiss Kappa statistics were used to determine the degree of intra- and inter-observer agreement. Results: Mean accuracy rates for all radiologists, for all ultrasound features, ranged from 82.7 to 87.8%. For B-mode and strain elastography, accuracies ranged from 65.0 to 100% and 47.4 to 86.8%, respectively. Concerning intra-observer variability, three radiologists demonstrated almost perfect agreement (the κ-value ranged from 0.81 to 0.86), and a substantial agreement was noted for the two remaining radiologists. The κ-values for inter-observer agreement ranged from 0.61 for macrocalcifications (substantial agreement) to 0.33 for Asteria four-point elastography scale criteria (fair agreement). Conclusions: The results suggest relatively good inter-observer and excellent intra-observer agreement in the assessment of thyroid nodules using ultrasound, and fair agreement in the case of strain elastography.

Duplicated inferior vena cava with coexisting multiple vascular anomalies and their clinical implications: a case report.

The paper presents the case of a 23-year-old man with multiple venous anomalies. The abnormalities were asymptomatic, and they were detected accidentally on routine abdominal ultrasound examination. The anomalies were found in the inferior vena cava, right testicular vein, left renal vein, and hepatic veins. Familiarity with different developmental variants within the inferior vena cava and other venous vessels plays a crucial role in ultrasound imaging. Vascular anomalies, although rare, should be taken into account in the differential diagnosis of focal lesions within the abdominal cavity. Also, variation in vascular anatomy may be a precipitating factor for blood flow disorders, and hence predispose patients to deep vein thrombosis and other pathological conditions. The reported case serves as a valuable addition to the knowledge of the vascular system that radiologists use in their everyday practice when performing diagnostic ultrasound examinations.

Histopathological Verification of the Diagnostic Performance of the EU-TIRADS Classification of Thyroid Nodules-Results of a Multicenter Study Performed in a Previously Iodine-Deficient Region.

BACKGROUND: To validate the European Thyroid Imaging and Reporting Data System EU-TIRADS classification in a multi-institutional database of thyroid nodules by analyzing the obtained scores and histopathology results. METHODS: A total of 842 thyroid lesions (613 benign, 229 malignant) were identified in 428 patients (mean age 62.7 years) and scored according to EU-TIRADS, using ultrasound examination. In all tumors, histopathological verification was performed. RESULTS: In EU-TIRADS 2 (154 nodules) all nodules were benign; in EU-TIRADS 3, only 3/93 malignancies were identified. In EU-TIRADS 4, 12/103 were malignant, and in EU-TIRADS 5 (278 benign vs. 214 malignant). The malignant nodules that would not have qualified for biopsy were: EU-TIRADS 3, 2/3 (67%) malignancies were <20 mm, in EU-TIRADS 4, 7/12 (58%) were <15 mm. In EU-TIRADS 5, 72/214 (34%) were <10 mm; in total, 81/229 (36%) malignant lesions would have been missed. The cutoff between EU-TIRADS 3/4 had sensitivity of 100%, specificity of 25.1%. Using cutoff for EU-TIRADS 5, 93.4%, 54.6%, respectively. CONCLUSION: The application of EU-TIRADS guidelines allowed us to achieve moderate specificity. The vast majority of malignancies in EU-TIRADS 3, 4, and 5 would not have been recommended for biopsy because having a smaller size than that proposed classification.

Sex and body mass index implications on gluteofemoral subcutaneous tissue morphology visualized by ultrasonography - preliminary study.

Introduction: Since the protective value of gluteofemoral subcutaneous adipose tissue against cardiovascular risk factors has already been described in scientific reports, it is important to pay more attention to its evaluation. Aim of the study: The purpose of this study was to evaluate sex and body mass index implications on gluteofemoral subcutaneous tissue morphology visualized by ultrasonography. Material and method: A population of 40 participants between 20-50 years of age was examined. All individuals underwent the ultrasound examination of subcutaneous adipose tissue in three locations: anterior, posterior and lateral side of a thigh in the 1/3 distal part. All examinations were collected, and the following parameters were evaluated: thickness of subcutaneous adipose tissue in general, thickness of superficial and deep subcutaneous adipose tissue. Results: The study revealed significant differences in the architecture of subcutaneous adipose tissue between male and female subgroups. In the group of males, a significantly thinner layer of not only subcutaneous adipose tissue in general (0.65 vs. 1.67 cm, p <0.0001), but also in its main compartments was observed. Moreover, we observed strong positive correlation between body mass index and all subcutaneous adipose tissue layers in the female subgroup. Interestingly, there was no relation between the thickness of the subcutaneous adipose tissue layers between subgroups with a decreased and normal body mass index and an increased body mass index. Conclusions: The presented data indicates that sex is an important factor in the determination of subcutaneous adipose tissue architecture of a thigh. The ultrasound examination of this structure can be a useful prognostic tool in the assessment of cardiovascular risk.Introduction: Since the protective value of gluteofemoral subcutaneous adipose tissue against cardiovascular risk factors has already been described in scientific reports, it is important to pay more attention to its evaluation. Aim of the study: The purpose of this study was to evaluate sex and body mass index implications on gluteofemoral subcutaneous tissue morphology visualized by ultrasonography. Material and method: A population of 40 participants between 20­50 years of age was examined. All individuals underwent the ultrasound examination of subcutaneous adipose tissue in three locations: anterior, posterior and lateral side of a thigh in the 1/3 distal part. All examinations were collected, and the following parameters were evaluated: thickness of subcutaneous adipose tissue in general, thickness of superficial and deep subcutaneous adipose tissue. Results: The study revealed significant differences in the architecture of subcutaneous adipose tissue between male and female subgroups. In the group of males, a significantly thinner layer of not only subcutaneous adipose tissue in general (0.65 vs. 1.67 cm, p <0.0001), but also in its main compartments was observed. Moreover, we observed strong positive correlation between body mass index and all subcutaneous adipose tissue layers in the female subgroup. Interestingly, there was no relation between the thickness of the subcutaneous adipose tissue layers between subgroups with a decreased and normal body mass index and an increased body mass index. Conclusions: The presented data indicates that sex is an important factor in the determination of subcutaneous adipose tissue architecture of a thigh. The ultrasound examination of this structure can be a useful prognostic tool in the assessment of cardiovascular risk.

Integration of Sonoelastography Into the TIRADS Lexicon Could Influence the Classification.

Aim: Numerous TIRADS (Thyroid Image Reporting and Data System) classifications have been developed, and various ultrasound (US) parameters are employed in different countries. The aim of our study was to introduce risk classification and management in a native population based on the Guidelines of Polish National Societies Diagnostics and Treatment of Thyroid Carcinoma but with the addition of sonoelastography. Materials and Methods: We examined prospectively 208 patients with 305 thyroid lesions employing B-mode ultrasound and sonoelastography (SE). Nodule composition, echogenicity, margins, shape, presence or absence of calcifications, thyroid capsule, nodule size were assessed using B-mode ultrasound. Moreover, sonoelastography results were presented using the Asteria scale. Results: In univariate analysis, the following US features were significantly associated with malignancy: >50% solid /solid component, marked hypoechogenicity, ill-defined margins, micro and macrocalcification, taller-than wide shape, no/partial halo pattern, infiltration of the capsule and an Asteria score of 4. Multivariate logistic regression analysis of B-mode features revealed that ill-defined margins (OR 10.77), markedly hypoechogenicity (OR 5.12), microcalcifications (OR 4.85), thyroid capsule infiltrations (OR 3.2), macrocalcifications (OR 3.01), and hard lesion in SE (OR 6.85) were associated with a higher Odds Ratio (OR) for malignancy. Multivariate logistic regression analysis revealed that combining two features increases the OR and the best combination was irregular margins and Asteria scale 4 (OR 20.21). Adding a third feature did not increase the OR. Conclusions: Sonoelastography increases the value risk of predicted malignancy, with consequent different approach to further clinical investigation and management. A solitary feature (Asteria 4) in a solid tumor can result in its categorization as TIRADS 4, but coexistence with high risk features allows it to be upgraded to TIRADS 5. The irregular margin was the strongest single feature which allowed for the assignment of a solid tumor into TIRADS 5 category. The highest accuracy was found by combining the features of age, margin, echogenicity (markedly hypoechoic), capsule infiltration, microcalcifications and sonoelastography (Asteria 3,4) of the tumors.

Risk factors for local complications in children with community-acquired pneumonia.

AIM: The aim of this study was to evaluate the factors that could predict the development of local complications (parapneumonic effusion/pleural empyema, necrotizing pneumonia, and lung abscess) in children with community-acquired pneumonia (CAP). METHODS: Demographic, clinical, and laboratory data were prospectively collected and compared in children with noncomplicated and complicated CAP. RESULTS: Two-hundred and three patients aged from 2 months to 17 years were enrolled. There were 141 and 62 children with noncomplicated and complicated CAP, respectively. Significantly longer duration of fever and a higher level of acute phase reactants were demonstrated in complicated when noncomplicated to complicated CAP. Asymmetric chest pain as well as prehospital treatment with ibuprofen and acetaminophen were significantly more common in patients with complicated CAP (P < .001, P = .02 and P = .003, respectively). Preadmission cumulative dose of ibuprofen exceeding 78.3 mg/kg (median dose for the entire group) was associated with 2.5-fold higher odds ratio (OR) for CAP complications [OR 2.54 CI (1.31-4.94); P = .008)]. In contrast, pneumococcal vaccination was associated with lower odds ratio [OR.03 CI (.23-.89); P = .03] for local complications. CONCLUSIONS: Some clinical and laboratory data including chest pain, longer duration of fever, higher acute phase reactants, and especially preadmission treatment with ibuprofen or acetaminophen were associated with local complications of CAP. The results of this study highlight the association between the dose of ibuprofen and local CAP complications.

Relative cost-effectiveness of using an extensively hydrolyzed casein formula containing the probiotic Lactobacillus rhamnosus GG in managing infants with cow's milk allergy in Poland.

OBJECTIVE: To estimate the cost-effectiveness of using an extensively hydrolyzed casein formula (eHCF) containing the probiotic Lactobacillus rhamnosus GG (eHCF + LGG; Nutramigen LGG) as an initial treatment for cow's milk allergy compared with eHCF alone and amino acid formulas (AAF) in Poland from the perspective of the Polish National Health Fund (Narodowy Fundusz Zdrowia [NFZ]) and parents. METHODS: Decision modeling was used to estimate the probability of cow's milk allergic infants developing tolerance to cow's milk by 18 months. The model also estimated the cost to the NFZ and parents (Polish Zloty [PLN] at 2013-2014 prices) for managing infants over 18 months after starting one of the formulas as well as the relative cost-effectiveness of each of the formulas. RESULTS: The probability of developing tolerance to cow's milk by 18 months was higher among infants who were fed eHCF + LGG (0.82) compared with those fed eHCF alone (0.53) or an AAF (0.22). An infant who is initially managed with eHCF + LGG is expected to consume fewer health care resources than infants managed with the other formulas. Hence, the estimated total health care cost incurred by the NFZ for initially feeding infants with eHCF + LGG (PLN 5,693) was less than that of feeding infants with eHCF alone (PLN 7,749) or an AAF (PLN 24,333). However, the total cost incurred by parents for initially feeding infants with an AAF (PLN 815) was marginally less than that of feeding with eHCF + LGG (PLN 993), which was less than that of feeding with eHCF alone (PLN 1,226). CONCLUSION: Using eHCF + LGG instead of eHCF alone or an AAF for first-line management of newly diagnosed infants with cow's milk allergy affords a cost-effective use of NFZ-funded resources, since it improves outcome for less cost. Whether eHCF + LGG would be viewed as being cost-effective by parents is dependent on their willingness to pay an additional cost for additional tolerance acquisition to cow's milk.

Natural history of Hymenoptera venom allergy in children not treated with immunotherapy.

BACKGROUND: Differences in treatment approach still exist for children after systemic sting reactions. In addition, there are still some doubts about when systemic reactors should be treated with venom immunotherapy (VIT). OBJECTIVE: To determine the rate of sting recurrence and natural history of Hymenoptera venom allergy (HVA) in children not treated with VIT. METHODS: A total of 219 children diagnosed as having HVA who were not treated with VIT were identified in 3 pediatric allergology centers. Survey by telephone or mail with the use of a standardized questionnaire was conducted. The number of field re-stings, subsequent symptoms, and provided treatment were analyzed. RESULTS: A total of 130 of the 219 patients responded to the survey, for a response rate of 59.4%. During the median follow-up period of 72 months (interquartile range, 52-85 months), 44 children (77% boys) were stung 62 times. Normal reactions were most common, occurring in 27 patients (62%). Severe systemic reactions (SSRs) occurred in 8 (18%) of those who were re-stung. The subsequent reaction was significantly milder (P < 0.001), especially in the case of patients re-stung by the same insect (P < .001). None of the children with prediagnostic large local reactions and negative test results for venom specific IgE developed SSRs after re-sting by the culprit insect (P = .03). In children with SSRs, median time from diagnosis to re-sting was 2 times longer than that in those with large local reactions and normal reactions (P = .007). CONCLUSIONS: Most children with HVA not treated with VIT reported milder reactions after a re-sting. Probability of SSR to re-sting increases along with the severity of initial reaction.

Shear wave elastography in medullary thyroid carcinoma diagnostics.

UNLABELLED: Shear wave elastography (SWE) is a modern method for the assessment of tissue stiffness. There has been a growing interest in the use of this technique for characterizing thyroid focal lesions, including preoperative diagnostics. AIM: The aim of the study was to assess the clinical usefulness of SWE in medullary thyroid carcinoma (MTC) diagnostics. MATERIALS AND METHODS: A total of 169 focal lesions were identified in the study group (139 patients), including 6 MTCs in 4 patients (mean age: 45 years). B-mode ultrasound and SWE were performed using Aixplorer (SuperSonic, Aix-en-Provence), with a 4-15 MHz linear probe. The ultrasound was performed to assess the echogenicity and echostructure of the lesions, their margin, the halo sign, the height/width ratio (H/W ratio), the presence of calcifications and the vascularization pattern. This was followed by an analysis of maximum and mean Young's (E) modulus values for MTC (EmaxLR, EmeanLR) and the surrounding thyroid tissues (EmaxSR, EmeanSR), as well as mean E-values (EmeanLRz) for 2 mm region of interest in the stiffest zone of the lesion. The lesions were subject to pathological and/or cytological evaluation. RESULTS: The B-mode assessment showed that all MTCs were hypoechogenic, with no halo sign, and they contained micro- and/ or macrocalcifications. Ill-defined lesion margin were found in 4 out of 6 cancers; 4 out of 6 cancers had a H/W ratio > 1. Heterogeneous echostructure and type III vascularity were found in 5 out of 6 lesions. In the SWE, the mean value of EmaxLR for all of the MTCs was 89.5 kPa and (the mean value of EmaxSR for all surrounding tissues was) 39.7 kPa Mean values of EmeanLR and EmeanSR were 34.7 kPa and 24.4 kPa, respectively. The mean value of EmeanLRz was 49.2 kPa. CONCLUSIONS: SWE showed MTCs as stiffer lesions compared to the surrounding tissues. The lesions were qualified for fine needle aspiration biopsy based on B-mode assessment. However, the diagnostic algorithm for MTC is based on the measurement of serum calcitonin levels, B-mode ultrasound and FNAB.

[Results of immunosupressive therapy in children with severe aplastic anaemia. Report of the Polish Paediatric Haematology Group]. / Wyniki leczenia ciezkiej postaci anemii aplastycznej u dzieci. Raport Polskiej pediatrycznej grupy do spraw nienowotworowych chorób ukladu krwiotwórczego.

INTRODUCTION: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given. AIM: evaluation of results of immunosupressive therapy in children with severe aplastic anaemia. MATERIAL AND METHODS: SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy. RESULTS: complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients. CONCLUSIONS: 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia (probability of incidence was 3.8%).

[Acute lymphoblastic leukemia in children with initial leucocytosis above 50,000/mm3: summary of treatment results of Polish Pediatric Leukemia/Lymphoma Study Group]. / Ostra bialaczka limfoblastyczna u dzieci ze wstepna leukocytoza powyzej 50,000/mm3: podsumowanie wyników leczenia Polskiej Pediatrycznej Grupy ds. Leczenia Bialaczek i Chloniaków.

Initial leucocytosis, presence of t(9;22) and t(4;11) translocations and poor response to therapy with steroids or induction chemotherapy are still included to poor risk factors group. From 1981 to 1986, children with acute lymphoblastic leukemia (ALL) and initial WBC above 50,000/mm3, achieved significantly worse treatment results than children with lower WBC: over 6-year disease-free survival were respectively 33% and 60%. In attempt to improve treatment results in children with hyperleucocytosis, modified American protocols called: New York (1987), New York I (1997), and New York II (1999) were introduced consecutively in the centers of Polish Pediatric Leukemia/ Lymphoma Study Group. Actually treatment results obtained with those protocols in three groups of patients: group I: 214 children (1987-1996), group II: 58 children (1997-1999), and group III: 77 children (1999-2001) are presented. The observation was completed in March 31, 2004. In evaluated groups the first complete remissions (CR) were achieved in 91%, 95%, and 96% of patients, respectively. Relapses occurred in 72 patients of group I (37%), in 12 patients of group II (21%), and in 13 patients of group III (18%). The 5-year overall survivals were: 62%, 79%, and 78% (p=0.05) respectively; 5 year event-free survivals (EFS) were: 52%, 74%, and 69% (p=0.01) respectively. A significant improvement in treatment results in second compared with first group was achieved. Treatment results obtained with New York II are comparable with results obtained with New York I. The analysis of treatment results achieved shows the improvement of the prognosis in children with ALL and initial WBC above 50 000/mm3 in comparison with patients treated before 1987. There is strong necessity of unification of risk group qualification criteria in childhood ALL in term of comparable estimation treatment results achieved in different centers all over the world.