Pediatr. Rep., Volume 3, Issue 4 (November 2011) – 9 articles

We report the detailed long-term reconstitution of B-lymphocyte subpopulations, immunoglobulins, and specific antibody production after two courses of rituximab in a young, previously healthy girl with steroid-dependent autoimmune hemolytic anemia. B-lymphocyte subpopulations were surprisingly normal directly after reconstitution. However, there was a slower reconstitution after the second rituximab course, especially of non-switched and switched memory B-lymphocytes, and a temporary decline in IgM below age-matched reference values. Full article

We describe 2 cases of children with malignant disease who developed severe mucositis with perineal necrotizing fasciitis during severe neutropenia after chemotherapy. Treatment with topical negative pressure therapy with silver foam dressing, together with large spectrum antibiotics, resolved the problem with complete closure of the wound after 30 and 36 days of treatment, respectively. Full article

Sepsis related coagulopathy ranges from mild laboratory alterations up to severe disseminated intravascular coagulation (DIC). There is evidence that DIC is involved in the pathogenesis of microvascular dysfunction contributing to organ failure. Additionally, the systemic activation of coagulation, by consuming platelets and coagulation factors, may cause bleeding. Thrombin generation via the tissue factor/factor VIIa route, contemporary depression of antithrombin and protein C anticoagulant system, as well as impaired fibrin degradation, due to high circulating levels of PAI-1, contribute to enhanced intravascular fibrin deposition. This deranged coagulopathy is an independent predictor of clinical outcome in patients with severe sepsis. Innovative supportive strategies aiming at the inhibition of coagulation activation comprise inhibition of tissue factor-mediated activation or restoration of physiological anticoagulant pathways, as the administration of recombinant human activated protein C or concentrate. In spite of some promising initial studies, additional trials are needed to define their clinical effectiveness in adults and children with severe sepsis. Full article

Obese patients are known to have greater risks to develop hypertension, coronary vascular disease, and insulin resistance, and more attention has been recently paid to the impact of obesity on kidney. This study was conducted to investigate whether obese children have higher risk of renal injury as well as adults. Eighteen hundred and thirty school children aged 6-14 years with abnormal urinary findings on thrice occasions detected by the screening program for renal disease in Japan were enrolled. Of them, 27 children with nephritis or suspected nephritis diagnosed by persistent proteinuria with hematuria were compared to 588 without urinary abnormalities regarding their body mass index (BMI), blood pressure (BP), and serum level of total cholesterol. BMI and systolic BP (mmHg) were significantly higher in the former than in the latter. As a result, obesity may be associated with the development of renal injury even in childhood. Full article

Procedural pain is an important aspect of care in pediatrics, and particularly in pediatric oncology where children often consider this to be the most painful experience during their illness. Best recommended practice to control procedural pain includes both sedative-analgesic administration and non-pharmacological treatments, practiced in an adequate and pleasant setting by skilled staff. A nationwide survey has been conducted among the Italian Centers of Pediatric Hematology-Oncology to register operators’ awareness on procedural pain, state of the art procedural pain management, operators’ opinions about pain control in their center, and possible barriers impeding sedation-analgesia administration. Based on indications in the literature, we discuss the results of the survey to highlight critical issues and suggest future directions for improvement. Future objectives will be to overcome differences depending on size, improve operators’ beliefs about the complexity of pain experience, and promote a global approach to procedural pain. Full article

An 8-month old girl, weighing 9 kg, was brought by her parents at 8.15 am to the Emergency Department (ED) for a progressive worsening of weakness and acute respiratory failure. On admission, the baby presented with poor oral intake, a weak cry and extremely weak muscular body control. Poor gag and suck, unreactive mydriasis, hypotonia, lethargy and absence of peristalsis were noted. Laboratory data showed severe respiratory acidosis. Chest X-ray, electroencephalography, encephalic CT scan and MRI were all normal, as were cerebrospinal fluid analysis and viral tests. Orotracheal intubation and continuous mechanical ventilation were applied. The patient received fluids, corticosteroids, aerosol therapy, large-spectrum antibiotics and enteral- nutrition. Further investigation revealed ingestion of an improperly prepared homecanned homogenized turkey meal. Type A botulinum neurotoxin was identified. Trivalent botulinum antitoxin, prostigmine and oral activated charcoal were administered. Generalized flaccid paralysis, areflexic bilateral mydriasis, gastric stasis and deep coma persisted for the duration of the hospital stay, and the patient died of severe respiratory failure and cardiac arrest 12 days after ED admission. Botulism poisoning should be suspected in any infant presenting with feeding difficulties, constipation, descendent paralysis or acute respiratory failure. Supportive treatment and antidotal therapy should be performed as soon as a clinical diagnosis is made. We describe a case of foodborne botulism in an 8-month old infant caused by ingestion of an improperly prepared home-canned homogenized turkey meal, representing the youngest fatal case reported in medical literature. Full article

Today, in spite of the developments in imaging methods and antibiotherapy, childhood pleural empyema is a prominent cause of morbidity and mortality. In recent years, it has been shown that there has been an increase in the frequency of pleural empyema in children, and antibiotic resistance in microorganisms causing pleural empyema has made treatment difficult. Despite the many studies investigating thoracoscopic debridement and fibrinolytic treatment separately in the management of this disease, there is are not enough studies comparing these two treatments. The aim of this study was to prospectively compare the efficacy of two different treatment methods in stage II and III empyema cases and to present a perspective for treatment options. We excluded from the study cases with: i) thoracoscopic intervention and fibrinolytic agent were contraindicated; ii) immunosuppression or additional infection focus; iii) concomitant diseases, those with bronchopleural fistula diagnosed radiologically, and Stage I cases. This gave a total of 54 cases: 23 (42.6%) in stage II, and 31 (57.4%) cases in stage III. These patients were randomized into two groups of 27 cases each for debridement or fibrinolytic agent application by video-assisted thoracoscopic decortication (VATS). The continuity of symptoms after the operation, duration of thoracic tube in situ, and the length of hospital stay in the VATS group were of significantly shorter duration than in the streptokinase applications (P=0.0001). In 19 of 27 cases (70.37%) in which fibrinolytic treatment was applied and in 21 cases of 27 (77.77%) in which VATS was applied, the lung was fully expanded and the procedure was considered successful. There was no significant difference with respect to success rates between the two groups (P=0.533). The complication rate in our cases was 12.96% and no mortality was observed. Similar success rates in thoracoscopic drainage and enzymatic debridement, and the low cost of enzymatic drainage both served to highlight intrapleural streptokinase treatment as a reliable method in reducing the need for surgery in complicated empyema. Full article

Few cases of premature infants with classical phenylketonuria (PKU) have been reported. Treatment of these patients is challenging due to the lack of a phenylalanine (Phe)-free amino acid (AA) solution for parenteral nutrition. A boy born at 27 weeks of gestation with a weight of 1000 g was diagnosed with classical PKU on day 7 because of highly elevated Phe level at newborn screening (2800 μmol/L). Phe intake was suspended for 5 days and during this time intravenous glucose and lipids as well as small amounts of Phe-free formula through nasogastric tube were given. Because of insufficient weight gain attributable to deficiency of essential AA, a Phe-reduced, BCAA-enriched parenteral nutrition was added to satisfy AA requirements without overloading in Phe. Under this regimen, the boy started to gain weight, Phe plasma levels progressively reduced and normalized on day 19. At the age of 40 months, the patient shows normal growth parameters (height 25th percentile, weight 25-50th percentile, head circumference 50th percentile) with a normal result for formally tested psychomotor development (WPPSI-III). The good outcome of the patient in spite of over 2 weeks of extremely high Phe concentrations suggests that the premature brain may still have enough plasticity to recover. Lacking a Phe-free intravenous AA solution, successful management of premature infants with PKU depends on the child’s tolerance of enteral nutrition. Although the coincidence of PKU and prematurity is rare, there is strong need for the development of an appropriate Phe-free amino acid solution for parenteral nutrition especially in case of gastro-intestinal complications of prematurity. Full article