Plasma chitotriosidase activity in children with lysosomal storage disorders.

Chitotriosidase (ChT) is an enzyme that is selectively activated in tissue macrophage. This property of ChT makes it a potential marker for many disease process and prognostication. Present study has been carried out to know the significance of ChT as a screening marker in lysosomal storage disorders (LSDs) where tissue macrophage activation is commonly observed due to accumulation of substrate in various organs of the body. Study comprises of 20 healthy children in the age range of 10 days to 5 yrs and 56 children in the age range of 2.5 months to 13 yrs with regression of milestones, skeletal dysplasia, neuroregression and hepatosplenomegaly were selected for plasma ChT who had confirmed LSDs as carried out by specific lysosomal enzyme study from the leukocytes or fibroblasts. Plasma ChT was 55.21 ± 20.81 nmol/ml /hr in twenty healthy age matched controls. Plamsa ChT level was 42.88 to 79.78 nmol/ml/hr in thirteen of 56 (23.21%) children with LSDs like Morquio- B, Pompe, Metachromatic leucodystrophy (MLD), Sandhoff and Niemann-Pick disease type C (NPD-C). While in 43 (76.78%) children it was in the range of 213.74 to 23,511.40 nmol/ml/hr. who had LSDs like Morquio-B, Sly syndrome, MLD, GM2 Gangliosidosis, NPD-A/B and Gaucher disease (GD). Marked elevated ChT (4,000 to 23,511 nmol/ml/hr) was observed in all cases of GD (n=7) and NDP-A/B. It can be concluded from the present study that moderately raised activity of ChT can be utilized as a positive predictive test for certain LSD’s. Those with marked elevated ChT have confirmed GD or NPD-A/B making it a strong screening marker for this group of diseases.

Immunogenicity and safety of an indigenously developed DTPw hepatitis B combination vaccine in Indian infants.

OBJECTIVE: To evaluate the safety and immunogenicity of an indigenous hepatitis B, diphtheria, tetanus and B. pertussis tetravalent vaccine (Shantetra) in comparison with Tritanrix HBTM in healthy Indian infants. DESIGN: Multicentric, randomized, single blind intention-to-treat study with 12-18 weeks of follow up period. SETTING: 5 out patient departments at tertiary care referral centers across India. PARTICIPANTS: 151 infants were randomized in a 2:1 ratio to recruit 101 in the Shantetra and 50 in the Tritanrix HBTM groups respectively. A total of 136 subjects completed the study. No patients were withdrawn from the study due to any adverse effects. INTERVENTIONS: Recruited subjects were randomized to receive three doses of either of the two DTPw-Hepatitis B combination vaccines as per the EPI schedule. MAIN OUTCOME MEASURES: Monitoring the humoral immune response (seroconversion rates) induced by each antigenic component three to six weeks after the last dose of vaccine in both the groups. RESULTS: Seroprotective immune response was observed in 98.9% subjects for diphtheria, tetanus and hepatitis B components in the Shantetra group as compared to 95.5% subjects in the Tritanrix HB group. Anti pertussis antibody response was seen in 89% and 91.1% in the Shantetra and Tritanrix HB groups, respectively. The commonly observed adverse events in both the groups were, pain at injection site, mild fever and transient crying.CONCLUSION:The safety and immunogenicity of indigenously developed DTPwHepatitis B combination vaccine was demonstrated in the present study.

Antimicrobial stewardship in institutions and office practices.

Medically inappropriate, ineffective and economically inefficient use of antimicrobials is commonly observed in the health care units throughout the world especially in the developing countries. Antimicrobial stewardship programs attempt to balance the demand for these life-saving drugs with the need to preserve their future efficacy. A comprehensive evidence-based stewardship program should include elements chosen from the recommendations based on local antimicrobials use and resistance problems and on available resources that may differ, depending on the size of the institution or clinical setting. For success of antibiotic stewardship it is essential to increase awareness amongst medical professionals. Discipline in antimicrobial prescribing is most vital in clinical settings. A careful assessment of the benefits of prescribing against the risk of non-prescribing of antibiotics should be considered. It should be an endeavor of every physician to justify antibiotic prescription in case of empirical use. Integration of advanced information technology into antimicrobial stewardship programs holds the potential to both reduce antimicrobial overuse and improve outcomes.

Injection safety and Indian Academy of Pediatrics.

A major proportion of injections administered in India are unsafe. Unsafety is due to (1) steps that are associated with increased risk of spread of blood borne viruses and (2) errors related to technique of injection and faulty habits. Recommendations of Indian Academy of Pediatrics revolve round the following points: Equipment related, safe injection practices, best injection techniques, rational injection practices, prevention of needle stick injuries and reducing disease transmission recommendation for HIV-PEP (postexposure prophylaxis), HBV-PEP, disposal of syringes and sharps. The points have been discussed elaborately.

Lysosomal storage disorders.

This study was conducted to assess the variability of clinical expression of Lysosomal storage disorders (LSDs) and the selection of specific enzyme investigation to reach the differential diagnosis. Initially 150 children in the age range of 15 days to 13 years were screened for common metabolic disorder and based on screening results, clinical signs and symptoms, 30 children(4 mo-12 yr) of these were selected for the leukocyte enzyme study. Of these 21 were confirmed to have LSDs. The most common disorder was GM2-gangliosidosis (47.61%, 10/21) followed by mucopolysaccharidosis (33.33%; 7/21). All showed variable phenotypic expression. Metachromatic leukodystrophy (MLD) was observed in 9.5% (2/21) of children with arylsulphatase A enzyme deficiency, while two children had shown pseudodeficiency of arylsulphatase A. One case each of galactosialidosis and GMI-gangliosidosis were observed. We conclude that children with developmental delay, seizures, dysmorphic features and organomegaly, with or without positive urinary screening for common metabolic disorders, need to be investigated further for LSDs.Variability of clinical expression is commonly observed in LSDs which require further confirmation by specific leukocyte enzyme study.

Pertussis vaccine controversies and acellular pertussis vaccine.

Pertussis still continues to cause significant morbidity and mortality worldwide. Because of the high reactogenicity of whole cell pertussis vaccine, it had evoked public controversy in several countries. In 1970 Japan abandoned use of whole cell pertussis vaccine and mounted efforts to develop better vaccine. To date, nearly 24 acellular pertussis vaccines have been developed, using different number and quantity of components. No acellular vaccine is most or least immunogenic with respect to all included antigens. Vaccine efficacy and duration of immunity is comparable with whole cell pertussis vaccine. The adverse events are two thirds less compared to whole cell vaccine.

Thoracoscopy in management of empyema thoracis in children.

The usual treatment for empyema in children varies from a simple thoracocentesis to thoracotomy and open decortication. We studied the role of thoracoscopy in the management of empyema thoracis in 10 immunocompetent children after failure of medical management. All children recovered well with an early removal of intercostal tube and reduced postoperative hospital stay and showed complete resolution of empyema on follow up. Thoracoscopy has come as a new ray of hope for the patients with empyema, with the advantages of complete evacuation, minimal pulmonary dysfunction, reduced pain and hospital stay.