Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Experience with cholelithiasis in patients with sickle cell disease in Nigeria

Sickle cell anemia, like other chronic hemolytic states, predisposes to cholelithiasis. However, it seems that other factors, apart from the consequent hyperbilirubinemia, contribute to this predisposition. This concept is supported by the fact that while cholelithiasis is very common among North American sickle cell patients, it is not so common in African patients. It is now believed that African sickle cell patients are protected from developing gallstones because of their low dietary intake of cholesterol and low intake of refined fiber-depleted foods. A review of all the published work on cholelithiasis, in Nigerians in general and sickle cell patients in particular, is presented in this article. A comparison is made with similar studies carried out in North America.     

Leukoreduction in patients with severe pertussis with hyperleukocytosis

When pertussis is associated with hyperleukocytosis, mortality approaches to 80%. Immature leukocytes have been identified in pulmonary arterioles, small arteries and venules. Techniques aimed at reducing leukocyte mass might improve the prognosis of these patients. We report our experience with 3 patients in whom a leukoreduction was performed in the context of severe pertussis and hyperleukocytosis.     

Motor impairments in children with epilepsy treated with carbamazepine

Nineteen children with epilepsy were tested on two occasions, first during treatment with carbamazepine (CBZ) and then 6 months later without treatment. Plasma drug concentrations were within the therapeutic limits in all children. The children were examined with a standardized test of gross- and fine- motor functions, the Bruininks-Oseretsky test of motor proficiency. Significant improvements were found in response speed ( p < 0.05), in composite fine-motor tests ( p < 0.01) and in the total test battery ( p < 0.05) after the treatment had been withdrawn. A tendency to improvement was found in the fine-motor subtest of upper limb coordination ( p = 0.08). Another group of 12 children was tested twice during treatment with CBZ with an interval of 6 months. No difference was found in this group except for an impairment of the results in the subtest of visual-motor control on the second test occasion ( p = 0.05).     

Infections with viridans group streptococci in children with cancer

Infection with viridans group streptococci (VGS) causes morbidity and mortality in children with cancer. Incidence of these infections has increased over time. Neutropenic patients with acute myeloid leukemia and those receiving high-dose cytarabine or undergoing stem cell transplantation are at highest risk. One-third of infected patients develop a shock syndrome despite prompt antibiotic therapy. Host defense mechanisms contribute substantially to colonization and tissue damage, but the origin of the shock syndrome is not well understood. VGS infection may be accompanied by neurological complications, myocarditis, and acute respiratory distress syndrome. Routine systemic antimicrobial prophylaxis against VGS infection has not been proven effective. Current recommendations include appropriate antibiotic therapy and intensive supportive care.     

Hyponatremia in patients with nocturnal enuresis treated with DDAVP

Treatment of nocturnal enuresis with DDAVP is associated with a low incidence of adverse effects. The only reported serious adverse effect is seizure or altered level of consciousness due to water intoxication. We reviewed 14 articles that reported data on serum sodium in patients treated with DDAVP for nocturnal enuresis and 11 articles that reported patients who developed a seizure or altered level of consciousness during treatment with DDAVP for nocturnal enuresis. Excess fluid intake was identified as a contributing factor in 6 of the 11 case reports.     

Compliance with immunizations in splenectomized individuals with hereditary spherocytosis

Overwhelming infection by encapsulated bacteria is a significant risk of splenectomy. Strict adherence to immunizations against encapsulated organisms is recommended. We performed a cross‐sectional study to determine immunization status after splenectomy in the hereditary spherocytosis (HS) population. As HS is most often autosomal dominant, we used a strategy of ascertaining older affected individuals by expanding pedigrees from patients in our pediatric hematology center. Only 26% of asplenic patients reported receiving all recommended post‐splenectomy vaccines. This study demonstrates that pediatric hematology clinics have a unique opportunity to provide or recommend catch‐up and booster vaccinations to splenectomized adult relatives of their patients. Pediatr Blood Cancer 2009;52:865–867. © 2009 Wiley‐Liss, Inc.     

Myoclonic epilepsy with photosensitivity in infants with Pallister-Killian Syndrome

IntroductionPallister-Killian Syndrome (PKS) (OMIM #601803) is a rare genetic disorder caused by a mosaic tetrasomy of the short arm of chromosome 12. Epilepsy is a frequent concern in PKS patients.Methodswe report 3 PKS patients, with early-onset myoclonic epilepsy and photosensitivity. In these children, we analysed epileptic history and the EEG phenotype.ResultsEpilepsy onset was in the first 2 years of life in all patients and in 2 of them myoclonic seizures were the only seizure type. In all children photosensitivity was observed and myoclonic seizures were mainly related to low-frequency (1–6 Hz) intermittent photic stimulation. Levetiracetam was effective and well tolerated in the 2 treated patients.Conclusionsearly-onset myoclonic epilepsy is a possible clinical manifestation of PKS. Low-frequency photosensitivity is a peculiar bioelectrical marker in these children.     

Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis

BACKGROUND: Infants with cystic fibrosis (CF) can develop episodes of hyponatremic hypochloremic dehydration with metabolic alkalosis when they sweat excessively, which is not caused by sweating in normal infants. We investigated the incidence of the metabolic alkalosis with hypoelectrolytemia in CF infants, the possible risk factors for its occurrence and the importance of the manifestation in the diagnosis of CF. METHODS: In order to evaluate the incidence and the risk factors for the development of this sweat-related metabolic disorder in CF, we reviewed the records of all children diagnosed as having CF before the age of 12 months in a 10-year period. Data analysis included medical history data, clinical features, biochemical parameters (blood pH, serum bicarbonate, sodium, chloride and potassium levels), sweat chloride test values, as well as genetic analysis data. RESULTS: The prevalence of metabolic alkalosis in association with low serum electrolyte concentrations (hyponatremia, hypochloremia, and hypokalemia) in infant CF population in our region was 16.5%. We found no season predilection in its occurrence. Early infant age, breast-feeding, delayed CF diagnosis, heat exhaustion and the presence of severe CF transmembrane conductance regulator mutations are predisposed factors for the development of metabolic alkalosis with hypoelectrolytemia. CONCLUSIONS: The results from our study suggest that metabolic alkalosis with hypoelectrolytemia is a relatively common manifestation of CF in infancy. The possibility of CF should be seriously considered in any infant with this metabolic disorder.     

Difficulty in defecation in infants with gastroesophageal reflux treated with smaller volume feeds thickened with rice cereal

We prospectively evaluated the incidence of difficulty in defecation in infants with gastroesophageal reflux who were treated with smaller volume feeds thickened with rice cereal and also assessed the effect of changing the cereal to oatmeal. We evaluated 53 thriving infants with uncomplicated gastroesophageal reflux who were treated with smaller volume feeds thickened with rice cereal. Parents maintained records of bowel movements for 7 days. Rice was substituted by oatmeal cereal in those infants developing difficulty in defecation and another 7 days' record was kept. Of the 53 infants enrolled, 34 (64%) reported no difficulty in defecation, 8 (15%) reported mild difficulty, and 11 (21%) reported severe difficulty in defecation during rice-based feedings. In these symptomatic 19 infants, after rice was substituted by oatmeal cereal, 10 infants (52.6%) reported no symptoms, 6 (31.6%) had mild symptoms, and 3 (15.8%) continued to have severe symptoms. We conclude that difficulty in defecation is common during treatment of infants with gastroesophageal reflux with smaller volume feeds thickened with rice cereal. Substitution of rice with oatmeal cereal results in partial or complete resolution of symptoms in most of these infants.