Quantitative monitoring of WT1 expression in peripheral blood before and after allogeneic stem cell transplantation for acute myeloid leukemia - a useful tool for early detection of minimal residual disease.

Overexpressed Wilms tumor gene 1 (WT1) has been found in a majority of patients with acute myeloid leukemia (AML). The aim of this study was to confirm the applicability of WT1 expression measurement as a marker of minimal residual disease (MRD). The expression of WT1 gene was measured by real-time polymerase chain reaction in peripheral blood (PB) according to European Leukemia Net (ELN) recommendations. The WT1 expression was related to the expression of a reference gene Abelson (ABL) and the results were calculated as a number of WT1 copies related to 104 copies of ABL gene. The upper normal limit of WT1 expression was set at 50 copies of WT1 to 104 copies of ABL. Morphological, flow cytometry and chimerism examinations were evaluated according to standard protocols.A total of 51 AML patients with overexpressed WT1 gene were analyzed. The median follow-up after transplantation was 14 (2-72) months. WT1 expression levels exceeding the upper normal limit were considered as a sign of impending hematological relapse, in accord with morphological, flow cytometry and chimerism data, as well as with the expression of the specific fusion genes. Moreover, in 7 patients the rise of WT1 expression preceded all other standard methods. Patients with high WT1 expression before allogeneic hematopoietic stem cell transplantation (allo-HSCT) had significantly worse outcome than patients with low WT1 level. Examination of WT1 expression in PB of patients with AML is a useful tool for MRD monitoring. Moreover, the WT1 gene expression before stem cell transplantation seems to be of prognostic significance.

5-azacitidine in aggressive myelodysplastic syndromes regulates chromatin structure at PU.1 gene and cell differentiation capacity.

Epigenetic 5-azacitidine (AZA) therapy of high-risk myelodysplastic syndromes (MDS) and acute myelogenous leukemia (AML) represents a promising, albeit not fully understood, approach. Hematopoietic transcription factor PU.1 is dynamically regulated by upstream regulatory element (URE), whose deletion causes downregulation of PU.1 leading to AML in mouse. In this study a significant group of the high-risk MDS patients, as well as MDS cell lines, displayed downregulation of PU.1 expression within CD34+ cells, which was associated with DNA methylation of the URE. AZA treatment in vitro significantly demethylated URE, leading to upregulation of PU.1 followed by derepression of its transcriptional targets and onset of myeloid differentiation. Addition of colony-stimulating factors (CSFs; granulocyte-CSF, granulocyte-macrophage-CSF and macrophage-CSF) modulated AZA-mediated effects on reprogramming of histone modifications at the URE and cell differentiation outcome. Our data collectively support the importance of modifying the URE chromatin structure as a regulatory mechanism of AZA-mediated activation of PU.1 and induction of the myeloid program in MDS.

[Splenic pseudocysts and their treatment]. / Pseudocysty sleziny a jejich lécení.

The article addresses the issue of splenic pseudocysts and their treatment. It reports on two patients treated over the past three years at the Department of Paediatric Surgery of the Prague-based Postgraduate Medical and Pharmaceutical Institute. While the pseudocyst healed spontaneously in one child after 9 months, the other patient underwent laparotomy with subsequent segmental resection of the splenic parenchyma involved.

[Acute osteomyelitis in children--modification of scintigraphic examination of the skeleton]. / Akutní osteomyelitida u detí--modifikace scintigrafického vysetrení skeletu.

The authors review diagnostic possibilities of acute osteomyelitis in children. They emphasize above all isotope examinations as the method of choice. In the subsequent part they focus attention on possible differentiation of acute osteomyelitis from inflammations of soft tissues-phlegmons. To this end they used a modified method of Israel published in 1987, based on a chronologically different uptake of 99mTc-MDP in normal bone and bone affected with osteomyelitis. According to this method it is possible to differentiate with a 90% accuracy acute osteomyelitis from phlegmons.

[Experience with non-surgical treatment of splenic injuries]. / Zkusenosti s neoperacním zpusobem lécení poranené sleziny.

Since 1980 in the department of paediatric surgery in Prague-Krc injury of the spleen is no longer an absolute indication for laparotomy and splenectomy. During the period from 1980-1990 46 children were admitted with an injured spleen. Of these 35 (76%) were treated conservatively, 11 children were operated. The number of conservatively treated patients increased in the course of time--during the initial period before 1985 65% children were treated conservatively, from 1986 to 1990 already 90% children. Only 16% children with an injured spleen treated conservatively needed a blood transfusion (on average 18.1 ml/kg body weight). This experience indicates that the great majority of children with an injured spleen can be treated by non-surgical methods. It is necessary to respect certain criteria which are given in the paper. Laparotomy is necessary in children with unstable vital functions, associated intraabdominal injuries and those to whom more than 30 ml blood per kg body weight must be administered.

[Pediatric trauma score in clinical practice: retrospective study]. / Pediatric trauma score v klinické praxi: retrospektivní studie.

In traumatological practice there is so far no uniform view as regards the classification of injuries. The authors recommend to do away with the vague traumatological terminology and replace in by a similar classification: to use the term "monotrauma" when one bodily system is affected and the term "associated injury" when two or more systems are effected; to use for more accurate definition of the severity of injury a scoring system. For paediatric traumatological practice the Pediatric Trauma Score P.T.S./is simple and suited best. The authors evaluated in a retrospective study a group of 257 children associated injuries. Based on the results of this investigation the authors consider P.T.S. a suitable scoring system for the objective evaluation of the general condition of the injured child and recommend its introduction into practice.

[Classification and evaluation of pediatric injuries]. / Klasifikace a hodnocení detských poranení.

The authors discuss the classification and evaluation of child injuries. In our literature so far no uniform conclusion was reached how classify injuries; the term "polytraumatism" or "multiple" is not accurately defined and is not used in the Anglo Saxon literature. The authors recommend therefore to differentiate only isolated injuries and "associated" injuries (injuries of two or more areas of the body). The severity of "associated" injuries can then be determined by some scoring system. For paediatric traumatological practice the Pediatric Trauma Score (PTS) is most suitable because it takes into account also the child's body weight, and thus indirectly his age, and is very simple.

[Present status of care of pediatric injuries in the Czech Republic]. / Soucasný stav v osetrování detských traumat v Ceské republice.

The great majority of child injuries are treated at the outpatient department and only in case of serious injury the child is admitted to hospital. In addition to the absolute number of injuries in our Republic it is also important to know how child injuries are treated, i. e. by whom, what are the material conditions for treatment in different departments and what is the standard of treatment. In an attempt to answer at least partly some of these questions the author sent questionnaires to 82 surgical and orthopaedic departments. Replies were obtained from 65 departments (80% of those addressed). From the answers ensued lack of uniform care of child injuries in out-patients' as well as in-hospital care. The majority of child injuries is treated by general surgeons (some 80%) and children with injuries are hospitalized in surgical, orthopaedic and paediatric departments.

Klion in the prevention of early complications following appendectomy.

The authors observations with prophylactic administration of metronidazole manufactured by the Gedeon Richter Company, Hungary (trade mark Klion) in children with phlegmonous appendicitis are reported. The drug was administered in three doses to 51 children. Results of bacteriology could be assessed in 42 children. The results of prophylactic administration of Klion in phlegmonous appendicitis were excellent. In our group there were no inflammatory wounds and/or intraperitoneal complications. For comparison, in a cohort of 1.008 children with phlegmonous appendicitis, without prophylaxis the rate of inflammatory complications was 3.62%. Klion has minimal side effects and is well tolerated and seems to be a superb drug for prophylaxis.

[Surgical treatment of cryptorchism in scrotal hypoplasia]. / Chirurgické resení kryptorchismu pri hypoplazii skrota.

In the submitted paper the authors give an account of their experience with the treatment of a patient with severe scrotal hypoplasia which is a very rare inborn defect. In a group of 1403 boys operated in the last ten years on account of cryptorchism this anomaly recorded only once, i.e. in 0.071%. The authors recommend a two-stage operation. In the first stage they use a V-Y plastic operation to create a neoscrotum. In the second stage orchidopexy is performed. Collaboration with a child endocrinologist is important. The aim of endocrinological examination is not only evidence of the presence and function of the gonads by a hormonal test, but in case of associated anomalies, also the assessment of genetic, genital and gonadal sex.

[Spontaneous ascent of testes originally located in the scrotum]. / Spontánní vzestup puvodne skrotálne ulozeného varlete.

The authors draws attention to possible postnatal changes the position of the testicle in the sensent of ascent. According to data in the literature and the author's own experience this ascent usually occurs as late as during school age. Causes of the altered position of the testicle are discussed. The condition is nowadays accepted but it is rare. From the practical aspects it is, however, necessary to examine the genitals of boys repeatedly and not only by a single examination. In case of a permanently inguinal position of the testicle the boy should be referred to a surgical department to prevent secondary damage of the testis by its incorrect position.

[Abnormal placement of the testes]. / Polohové anomálie varlat.

The authors defined a uniform classification of abnormal positions of the testes. They recommend also a uniform therapeutic procedure: after the first year of life treatment with human chorionic gonadotropin in one or two curves, if orchiopexy fails.

[Prophylactic administration of Klion in acute appendicitis]. / Profylaktické podání Klionu u akutní apendicitidy.

The authors present their experience with the prophylactic administration of metronidazole of Richter Co. Hungary (trade name Klion) in children with phlegmonous appendicitis. The preparation was administered in three doses to 51 children. The bacteriological results were evaluated in 42 children. The results of Klion administration in phlegmonous appendicitis were excellent. In the investigated group there were no wound inflammatory nor intraabdominal complications. For comparison the authors mention a group of 1008 children with phlegmonous appendicitis without prophylactic provisions where the number of inflammatory wound complications was 3.62%. Klion has minimal side-effects, was well tolerated and proved excellent for prophylactic administration.

Acute phase proteins and prognostic inflammatory and nutritional index (PINI) in moderately burned children aged up to 3 years.

In 27 children (12 boys and 15 girls) aged 3 months to 3 years, the plasma levels of albumin, prealbumin, orosomucoid and C-reactive protein (CRP) were determined after burn trauma. The results of these measurements were used to calculate the prognostic inflammatory and nutritional index (PINI). The children were assigned to two groups: a group with burns covering less than 10 per cent of the body surface area (BSA) (minor burns), and those with burns covering 11-20 per cent BSA. The lesions involved were either dermal burns (Class IIB) or full skin thickness burns (Class III). While the group with minor burns showed only a small decrease in albumin and prealbumin levels, the decrease in children with extensive burns was well below values on the first 7 days. On days 2, 5 and 7 after burning, the differences between both groups were statistically significant. Orosomucoid levels were somewhat elevated in the group with extensive burns. This difference, however, was not statistically significant compared with the group with minor burns whose values were slightly below the normal range. The levels of C-reactive protein were statistically significantly increased in children with extensive burns compared with those with burns. The PINI value correlated positively with the burn area given in per cent (for day 5 of follow-up, the regression line equation was y = 9.346 + 1.65 x). These data indicate that there are no pronounced changes in plasma protein levels in the group with minor burns.(ABSTRACT TRUNCATED AT 250 WORDS)

[Volvulus of the appendix]. / Volvulus apendixu.

The authors describe the case-history of a volvulus of the appendix which developed as a result of an inborn defect in the appendiceal mesenteriolume. The surgical finding did not suggest primary appendicitis but rather strangulation of the aboral part of the appendix.

[Immunologic response to inflammation and trauma in children]. / Immunologická odpoved na zánet a trauma u détí.

Inflammation and injury influence plasma proteins and immunoglobulins. They respond to noxious agents by a rise of c-reactive protein (CRP), orosomucoid, fibrinogen, haptaglobulin, alpha-1 antitrypsin, ceruloplasmin, C 3 and C 4 components of complement. The rate of rise and return to normal values varies. CRP reacts most'promptly. The following substances react by a decline of the levels: albumin, prealbumin, transferrin, alpha-1 lipoprotein and immunoglobulins. Investigation of the patient's immunological state is of prognostic value. CRP, orosomucoid, albumin and prealbumin are considered the most valuable indicators; from their values the so-called PINI index can be calculated and the patients can be divided into five prognostic zones. To threatened patients all-round care must be devoted incl. aimed provision of ATB and immunoprotective substances.

[Follow-up of burn injuries using the PINI index]. / Sledování prubehu popálenového traumatu u detí PINI indexem.

In the submitted paper the authors evaluate the influence of medium extensive burns injury on levels of selected plasma proteins in 27 children aged three months to three years. The assessed albumin, prealbumin, C-reactive protein and orosomucoid were used for calculation of the so-called PINI index [prognostic inflammatory and nutritional index]. The PINI index summarizes in a suitable way the impact of injury on the metabolism of plasma proteins in burned children under three years of age. It differentiates the group with mild injuries from the group with extensive burns. It drop correlates with the improving clinical condition of the patient and it can be used for the monitoring of the burned child.

[Concurrent acute appendicitis and incarcerated inguinal hernia in an infant]. / Koincidence akutní apendicitidy a uskrinuté tríselné kýly u kojence.

The authors present the case-history of a six-week old boy born during the 30th week of gestation with the coincidence of an incarcerated right-sided inguinal hernia and acute appendicitis. On operation it was found that the appendix was intra-abdominally fixed. The authors draw attention to the difficulty of diagnosis of an inflammatory acute abdomen in neonates and infants.

[Levels of C-reactive protein after surgery in fractures in children]. / Hladiny C-reaktivního proteinu po operacích detských zlomenin.

The authors investigated the levels of C-reactive protein (CRP) after operations of fractures in the area of the elbow in children. Regardless whether antibiotics were administered preventively or not, there was a rise of CRP levels with a maximum on the third day after operation. By the 7th day CRP levels were normal. In the control group of children with mere manual reposition of the fracture of the wrist there was no significant rise of the CRP level. If the elevated CRP level persists after the 7th postoperative day, a local inflammatory complication is suspected.