The Vitamin D Status in Children With Newly Diagnosed Acute Lymphoblastic Leukemia and Its Potential Impact on the Primary Symptoms of Leukemia and Course of Induction Treatment.

BACKGROUND: Vitamin D deficiency is ubiquitous within the population of children. A similar problem is recognized among pediatric patients with acute lymphoblastic leukemia. The purpose of this study was to analyze the prevalence of vitamin D deficiency and to investigate the connection between vitamin D status and the course of induction treatment of ALL. MATERIALS AND METHODS: A cross-sectional study including 59 patients with newly diagnosed ALL from May 2017 until November 2020. RESULTS: Vitamin D deficiency was found in 39% of the patients. There were no seasonal differences in vitamin D status. Patients with optimal 25(OH)D concentration presented more profound thrombocytopenia ( P =0.015) and required more frequent platelet transfusions ( P =0.018). Good prognosis factors such as B phenotype and hyperdiploidy were also more frequent among children with higher 25(OH)D concentration ( P =0.01 and 0.014, respectively). CONCLUSIONS: The study showed that patients with a higher serum concentration of 25(OH)D presented deeper thrombocytopenia and needed more frequent transfusions. Moreover, those patients showed higher rates of B-cell leukemia and hyperdiploid karyotype. We did not find any influence of the possible exposure to sunlight (defined as the season of the year on admission) on serum 25(OH)D concentration, which supports the argument for supplementing vitamin D all year round. Moreover, the supplementing of vitamin D seems to be safe and does not cause any renal complications connected to calcium and phosphorus imbalance as no correlation between their levels and 25(OH)D concentration was found.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Background and Objectives: Over the years, surgical repair of total anomalous pulmonary venous connection (TAPVC) outcomes have improved, however, morbidity and mortality still remain significant. This study aims to assess the early and long-term outcomes of surgical treatment of TAPVC children, operated on between 2006 and 2016, in one pediatric center in Poland. Materials and Methods: Diagnostics, surgical treatment, and follow-up data from 83 patients were collected. In addition, survival and risk factor analyses, control echocardiographic, and electrocardiographic examinations were performed. Results: In the analyzed group (n = 83), there were seven hospital deaths (within 30 days after the operation) (8.4%) and nine late deaths (10.8%). The mean follow-up time was 5.5 years, and, for patients who survived, it was 7.1 years. The mean survival time in patients with completed follow-up (n = 70) was 10.3 years; the overall five-year survival rate was 78.4%. Independent mortality risk factors were type I TAPVC, single ventricle physiology, time from admission to operation, intensive care unit stay, postoperative hospital stay, and temporary kidney insufficiency requiring dialysis. Conclusions: The presence of single ventricle physiology and the supracardiac subtype of TAPVC might be negative prognostic factors, while normal heart physiology presents with good post-repair results. This study indicates that cardiac arrhythmias may occur. Morbidity and mortality, related to surgical TAPVC correction, still remain significant.

Edwards Inspiris Resilia® valve for mitral replacement in an infant after mechanical valve failure.

We present the surgical implantation of the Edwards Inspiris Resilia® aortic valve in mitral position for mechanical mitral valve failure in a severely ill infant after valve replacement because of anomalous origin of the left coronary artery from the pulmonary artery. The biological valve was chosen because the child could not receive oral anticoagulation and was for several months on heparin infusion. The procedure was safely performed with good haemodynamic result.

Isavuconazole in a Successful Combination Treatment of Disseminated Mucormycosis in a Child with Acute Lymphoblastic Leukaemia and Generalized Haemochromatosis: A Case Report and Review of the Literature.

Invasive mucormycosis in immunocompromised children is a life-threatening fungal infection. We report a case of a 7-year-old girl treated for acute lymphoblastic leukaemia complicated by disseminated mucormycosis during induction therapy. Microscopic examination of surgically removed lung tissue revealed wide, pauci-septate hyphae suggesting a Mucorales infection. This diagnosis was confirmed immunohistochemically and by PCR analysis followed by a final identification of Cunninghamella sp. The patient was treated successfully with surgical debridement and antifungal combination therapy with amphotericin B, caspofungin and isavuconazole. The use of isavuconazole in a child was not previously reported. Additionally, case reports concerning pulmonary mucormycoses in paediatric population published after 2010 were reviewed. Nineteen out of 26 identified patients suffered from haematological diseases. Reported mortality reached 38.5%. By the fact of rising morbidity, unsatisfactory results of treatment and remaining high mortality of mucormycoses in immunocompromised patients, new therapeutic options are warrant. Isavuconazole, with its broad-spectrum activity, good safety profile and favourable pharmacokinetics, is a promising drug. However, further studies are necessary to confirm positive impact of isavuconazole on mucormycosis treatment in children.

Surgical Site Infection after Breast Surgery: A Retrospective Analysis of 5-Year Postoperative Data from a Single Center in Poland.

Background and Objectives: Surgical site infection (SSI) is a significant complication of non-reconstructive and reconstructive breast surgery. This study aimed to assess SSI after breast surgery over five years in a single center in Poland. The microorganisms responsible for SSI and their antibiotic susceptibilities were determined. Materials and Methods: Data from 2129 patients acquired over five years postoperatively by the Department of Surgical Oncology, Medical University of Gdansk in Poland were analyzed. Results: SSI was diagnosed in 132 patients (6.2%) and was an early infection in most cases (65.2%). The incidence of SSI was highest in patients who underwent subcutaneous amputation with simultaneous reconstruction using an artificial prosthesis (14.6%), and breast reconstruction via the transverse rectus abdominis muscle (TRAM) flap method (14.3%). Gram-positive bacteria were responsible for SSI in most cases (72.1%), and these were mainly Staphylococcus strains (53.6%). These strains were 100% susceptible to all beta-lactam antibiotics (except penicillin) but were less susceptible to macrolides and lincosamides. Conclusions: SSI is a serious problem, and attention should be focused on its prevention. Reconstruction using an artificial prosthesis or via the TRAM flap method is connected to increased SSI incidence. Further studies are required to prevent SSI following breast surgery.

Symptomatic cardiac hemangioma resected on the first day of life.

Cardiac tumors are extremely rare in neonates. We describe the case of a right atrial hemangioma in a neonate diagnosed prenatally and successfully operated on the first day of life.

Fournier Gangrene Caused by Candida albicans in an Infant After Cardiac Surgery.

Fournier gangrene is a rare, rapidly progressive, life-threatening condition. We report a 23-day-old boy with pulmonary atresia and ventricular septal defect treated surgically, who developed Fournier gangrene. Emergency surgery was performed with tissue sampling for microbiological examination. Candida albicans was confirmed; caspofungin followed by fluconazole was administered with excellent results.

Damus-Kaye-Stansel Procedure with Extracardiac Fontan: Successful Recruitment of Previously Ligated Pulmonary Valve.

We present a case of a 2.5-year-old-girl with complex congenital heart disease: tricuspid atresia (TA), bulboventricular septal defect (VSD), hypoplastic right ventricle, d-transposition of the great arteries (d-TGA) with aortic outflow from redundant RV. Due to II/III degree atrioventricular block induced after diagnostic cardiac catheterization, an epicardial pacemaker was implanted during the Glenn procedure. Because of severe left ventricle outflow tract obstruction, she was finally referred for extracardiac TCPC (extracardiac Fontan type) with recruitment of PV and Damus-Kaye-Stansel anastomosis. Intraoperatively, the pulmonary trunk stump was opened and a competent pulmonary valve with flaccid leaflets was found. Simple ligation of the pulmonary trunk with a preserved pulmonary valve enabled an effective aorto-pulmonary bridging of systemic outflow tract with the use of natural fully competent ventricle-arterial valves. The relief of single ventricle outflow tract obstruction led to final stabilization of spontaneous sinus rhythm recovery after 2 years of pacemaker stimulation.

Right Ventricle Mass Removal from Tricuspid Valve Apparatus: An Unusual Thromboembolic Complication of Severe Ketoacidosis.

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.

Antimicrobial Stewardship in Pediatrics.

Since the discovery of antibiotics in the early 20th century, significant changes have occurred in their usage principles [...].

Hybrid approach for closure of muscular ventricular septal defects.

BACKGROUND: The complexity of ventricular septal defects in early infancy led to development of new mini-invasive techniques based on collaboration of cardiac surgeons with interventional cardiologists, called hybrid procedures. Hybrid therapies aim to combine the advantages of surgical and interventional techniques in an effort to reduce the invasiveness. The aim of this study was to present our approach with mVSD patients and initial results in the development of a mini-invasive hybrid procedure in the Gdansk Hybrid Heartlink Programme (GHHP) at the Department of Pediatric Cardiac Surgery, Pomeranian Centre of Traumatology in Gdansk, Poland. MATERIAL AND METHODS: The group of 11 children with mVSDs was enrolled in GHHP and 6 were finally qualified to hybrid trans-ventricular mVSD device closure. Mean age at time of hybrid procedure was 8.22 months (range: from 2.7 to 17.8 months, SD=5.1) and mean body weight was 6.3 kg (range: from 3.4 to 7.5 kg, SD=1.5). RESULTS: The implants of choice were Amplatzer VSD Occluder and Amplatzer Duct Occluder II (AGA Med. Corp, USA). The position of the implants was checked carefully before releasing the device with both transesophageal echocardiography and epicardial echocardiography. All patients survived and their general condition improved. No complications occurred. The closure of mVSD was complete in all children. CONCLUSIONS: Hybrid procedures of periventricular muscular VSD closure appear feasible and effective for patients with septal defects with morphology unsuitable for classic surgical or interventional procedures. The modern strategy of joint cardiac surgical and interventional techniques provides the benefits of close cooperation between cardiac surgeon and interventional cardiologist for selected patients in difficult clinical settings.

Hybrid technique for muscular ventricular septal defect closure.

Since 2008 we have performed 10 hybrid procedures for perventricular muscular ventricular septal defects (mVSD) closure. The mean age was 8.5 months (range 2.7 to 17.8), the mean diameter of mVSD was 6.3 mm (range 4 to 10 mm). The postoperative course was uncomplicated and complete closure of the mVSD was accomplished in all patients. There was neither in-hospital nor late mortality, and the longest follow-up now exceeds three years (range 2 months to 3.1 years).

Hyperthyroidism secondary to disseminated mucormycosis in a child with acute lymphoblastic leukemia: case report and a review of published reports.

Thyroiditis due to fungal infection is an extremely rare cause of hyperthyroidism. The most common etiological factor of thyroiditis is Aspergillus. Infections due to members of the Mucorales have been an increasing clinical problem in recent years, and the prognosis in generalized infections due to those fungi is usually very poor. No hyperthyroidism in a child with thyroiditis due to mucormycosis has been reported in the literature so far. We describe a clinical course of generalized mucormycosis with thyroid involvement in a 12-year-old girl treated for acute lymphoblastic leukemia. The child underwent a hyperthyroidism connected with thyroid involvement due to a fungal process. The diagnosis was based on the clinical signs, laboratory findings and typical ultrasound scan; however, later attempt to amplify the fungi DNA from the tissue block has failed. The child died because of multiorgan failure due to general fungal infection 49 days after the invasive fungal infection was diagnosed. The generalized mucormycosis is always connected with poor prognosis and the mortality is high.

Pediatrician! Do you know the symptoms of DRESS syndrome? A case report of a 4-year-old girl.

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity syndrome and may be observed after administration of many drugs. Clinical symptoms usually occur 2 to 8 weeks after drug introduction. Because DRESS syndrome is potentially life threatening, it is especially important to diagnose it early. Withdrawal of the drug which induced symptoms is the most important therapeutic option. DRESS syndrome appears mostly in adults. There are relatively few articles on the DRESS syndrome in children. The article presents a case of a 4-year-old girl with a life-threatening clinical course of DRESS syndrome with massive pulmonary involvement. The knowledge of DRESS syndrome clinical symptoms is essential for doctors of various specialties. It is especially important that general practitioners, pediatricians, and pediatric neurologists should be able to take this life-threatening syndrome into consideration for differential diagnosis.

Bloody pericardial tamponade in a child treated for pneumonia mimicking a lung tumor and infiltration of the heart.

We present the dramatic course of a female 5-year-old child with pneumonia and symptoms similar to local compression of the pericardium by a tumorous mass originating from the left lung. The child was treated with antibiotics for pneumonia with bilateral pleural effusions that required chest drainage. On the 10(th) day of therapy there was sudden anemia observed with the echocardiographic finding of acute cardiac tamponade. The child was referred for emergency life-saving surgical intervention. The chest was opened via a minimally invasive mini-incision in the area of the xiphoid process and bloody tension pericardial effusion was evacuated. The laboratory and histopathology investigations were not specific for neoplastic disease or tuberculosis infection. In the further observation the girl recovered and was discharged home two weeks after tamponade drainage. Fortunately our initial suspicion of neoplastic disease was not proved; nevertheless we would like to emphasize the need for oncologic vigilance in similar cases.

Outcomes of Transannular Repair of Tetralogy of Fallot With a Contegra® Monocuspid Patch.

BACKGROUND: Surgical repair of tetralogy of Fallot (ToF) depends on the anatomical variations of the heart defect. A group of patients with a hypoplastic pulmonary valve annulus required a transannular patch. This study aimed to evaluate the early and late outcomes of ToF repair with a transannular Contegra® monocuspid patch in a single center. METHODS: A retrospective review of medical records was conducted. This study included 224 children with a median age of 13 months who underwent ToF repair with a Contegra® transannular patch in over 20 years of observation. The primary outcomes were hospital mortality and need for early reoperations. The secondary outcomes were late death and event-free survival. RESULTS: The hospital mortality in our group was 3.1%, whereas two patients required early reoperation. Three patients were excluded from the study because follow-up data were not available. In the remaining group of patients (212 patients), the median follow-up was 116 (range, 1-206) months. One patient died because of sudden cardiac arrest at home six months after surgery. Event-free survival was observed in 181 patients (85.4%), whereas the remaining 30 patients (14.1%) required graft replacement. The median time to reoperation was 99 (range, 4-183) months. CONCLUSIONS: Although surgical treatment of ToF has been performed for more than 60 years worldwide, the optimal approach in children with a hypoplastic pulmonary valve annulus remains debatable. Among options, the Contegra® monocuspid patch can be effectively used in transannular repair of ToF with good long-term results.

Postoperative kinetics of pentraxin 3 (PTX3) after congenital heart surgery with cardiopulmonary bypass in pediatric patients.

BACKGROUND: Pentraxins are inflammatory proteins and markers of acute-phase responses. They are divided into short and long subgroups based on the length of the N-terminal region. The most studied short pentraxin is the C-reactive protein (CRP), which is known to be expressed in various inflammatory conditions, including surgical procedures. On the other hand, much less is known about the kinetics of long pentraxin 3 (PTX3) in surgical patients, especially in the pediatric population. The aim of this prospective study was to determine the early postoperative kinetics of PTX3 in relation to procalcitonin (PCT) and CRP levels in children undergoing congenital heart surgery with cardiopulmonary bypass (CPB). METHODS: A total of 21 children (9 boys and 12 girls, mean age 12 months) were included in the study. Blood samples for determination of CRP, PCT, and PTX3 levels were collected before the surgery and then immediately after its completion (postoperative day 0, POD 0) and subsequently at POD 1, 2, and 3. RESULTS: Serum PTX3 concentrations increased significantly between POD 0 and POD 1 (mean values were 12.2 and 72.4 ng/ml, respectively, p<0.001), decreased between POD 1 and POD 2 (mean values were 72.4 and 23.6 ng/ml, respectively, p<0.001), and normalized on POD 3 (the mean value was 1.2 ng/ml). CONCLUSIONS: PTX3 concentrations are markedly elevated during the first postoperative day. Under normal circumstances, PTX3 rises and falls quickly, and its second rise in the early postoperative period may be abnormal, however, further studies are necessary.

Perioperative Antibiotic Prophylaxis in Pediatric Cardiac Surgery-Simple Is Better.

Pediatric cardiac surgery requires perioperative antibiotic prophylaxis (PAP) to reduce the risk of surgical site infections. However, the complexity of these procedures and the metabolic immaturity of children impede the establishment of PAP regimens that are both efficacious and in line with antimicrobial stewardship (AMS). In this study, we compared two PAP regimens: cefazolin with gentamicin (in a retrospective group) and cefazolin only (prospectively) in children undergoing elective cardiac surgery. In the prospective group, additional elements of AMS were introduced, i.e., restricted access to cefazolin and more diligent use of empirical antibiotics proceeded by consultation with an AMS team. The rate of surgical site infections (SSI), the scope of PAP deviations, and the postoperative use of antibiotics other than PAP within 30 days after surgery were analyzed. There were no significant differences in the rate of SSIs between the groups (3.9% vs. 1.2% in the prospective and retrospective groups, respectively (p = 0.35)). However, in the prospective group, the PAP violation was significantly reduced compared with the retrospective group (full compliance with the PAP regimen was 45.5% vs. 4.8%, p < 0.001, respectively). In addition, a reduction of postoperative antibiotic use was observed in the prospective group (0.991 vs. 1.932 defined daily doses, respectively).

Recurrent or persistent salivary gland enlargement in children: When is it Sjögren's?

OBJECTIVES: To describe characteristic features in children with recurrent or persistent salivary gland enlargement and to propose a diagnostic algorithm with specific consideration for Sjögren's disease (SD). METHODS: In this single-center, prospective study, 45 patients < 18 years, with recurrent or persistent salivary gland enlargement of unknown etiology were enrolled from 2006 to 2019. We collected detailed clinical information to characterize this group of patients including specific details of their major salivary gland signs and symptoms. We compared clinical, laboratory and radiological parameters between 4 groups based on the results of labial salivary gland biopsy (LSGB) and between patients who met existing SD criteria or not. RESULTS: 44 patients, with a mean age of 6.8 years and female to male ratio 21:23 were observed over a mean of 3.8 years. Characteristics of salivary gland swelling episodes varied considerably between individuals, but the majority experienced ≤5 episodes per year, lasting ≤ 1 week, with swelling affecting either or both glands. Ocular and oral dryness symptoms were observed only in 25% and 59% patients, respectively. The majority were positive for ANA, but negative for SD-specific antibodies. A total of 75% patients fulfilled at least one of the existing SD criteria. CONCLUSION: SD is a major cause of recurrent salivary gland enlargement in children. For children meeting adult criteria, the diagnosis of SD is clear. However, for the many children without dryness symptoms, objective dryness, or SD-specific antibodies, further workup including a combination of salivary gland imaging and histopathological examination can help establish the diagnosis of SD.

Prognostic relevance of demographics and surgical practice for patients with gastric cancer in two centers: in Poland versus Germany.

BACKGROUND: Although studies comparing the surgical treatment of gastric carcinoma in Japan and Western industrialized countries have revealed differing survival rates, no studies to date have been performed comparing Western and Eastern Europe. This study aimed to compare demographics and surgical practice as well as the related prognostic impact on gastric cancer patients treated in Poland and Germany. METHODS: This retrospective study included gastric cancer patients treated between 1999 and 2004 by surgical departments in Gdansk (Poland) and Cologne (Germany). Univariate and multivariate analyses of demographic, histopathological, surgical, and prognostic data were performed. RESULTS: Included were 117 patients from Gdansk and 130 patients from Cologne. The Cologne patients showed higher incidence rates of serious comorbidity, pT1 cancer, and distant metastasis than those from Gdansk. Indications for and frequency of selected surgical procedures differed significantly. D2-lymphadenectomy was performed in 89% of the Cologne patients, while D1-lymphadenectomy was done for 85% of the Gdansk patients. Univariate analysis yielded a 5-year survival rate of 28.3% for the Gdansk patients, and 40.3% for the Cologne patients (p = 0.056). Independent prognostic factors were pT category (p = 0.002), pN category (p < 0.001), pM category (p = 0.027), residual tumor (R) category (p = 0.004), age (p = 0.012), and number of resected lymph nodes (p = 0.005). CONCLUSIONS: Significant differences of clinical and surgical parameters exist between gastric cancer patients treated in Poland and Germany. In addition to established independent prognostic factors, we found that survival improved with each additionally resected lymph node.