Single stage repair of tetralogy of fallot associated with left pulmonary artery sling and tracheal stenosis.

We report a rare case of tetralogy of Fallot (TOF) with left pulmonary artery (LPA) sling with tracheal stenosis. The patient underwent successful surgery in one stage involving intracardiac repair of TOF, LPA reimplantation and resection of tracheal stenosis with end-to-end anastomosis.

Perinatal tuberculosis: four cases and use of broncho-alveolar lavage.

Despite the high prevalence of tuberculosis in adults and children, congenital and perinatal forms of tuberculosis are rare. Four patients with perinatal tuberculosis are described. Diagnosis was made by demonstration of acid-fast bacilli (AFB) on broncho-alveolar lavage (BAL) specimens (two cases), gastric aspirate smear (one case) and lymph node fine-needle aspirate (one case). All of the above specimens were subsequently positive on culture. Two infants died of progressive pulmonary failure, and one of the mothers died, despite the institution of anti-tuberculous therapy. BAL specimen examination for AFB is useful in the diagnosis of perinatal tuberculosis, especially in infants with smear-negative gastric aspirate.

Evaluation of the immunogenicity and safety of an indigenously developed DTwP-Hib tetravalent combination vaccine (Shan 4) with EasyFourTM in Indian infants administered per EPI schedule: a phase III trial.

The study was planned to assess and compare immunogenicity and safety of an indigenous DTPw-Hib combination vaccine (Shan 4) with EasyFour, the available DTwP-Hib vaccine in India. Overall 210 healthy infants, six to eight weeks of age, were randomized to receive three doses of either Shan 4 or EasyFour at 6, 10 and 14 weeks of age. Antibodies were analyzed prior to and four to six weeks post third vaccine dose. Solicited and unsolicited local and systemic events in the follow up period after each dose were recorded. Post vaccination 100% of the infants in Shan 4 and EasyFour groups had seroprotective concentrations of Anti PRP-T IgG antibodies, IgG anti-diphtheria toxoid antibodies and IgG anti-tetanus toxoid antibodies. Following third dose of vaccination 86.99% subjects in the Shan 4 group and 73.85% subjects in the EasyFour group seroconverted for anti-pertussis antibody titres. Two Serious Adverse Events (SAE s) were reported during the course of the study, all unrelated to the respective vaccine administered. Most commonly reported adverse events in both the groups were pain at injection site, mild fever (<103°F) and minor swelling at injection site. The study proved that Shan 4 was safe and immunogenic compared to the available licensed vaccine.

Serological and molecular detection of Mycoplasma pneumoniae in children with community-acquired lower respiratory tract infections.

This study was designed to evaluate the incidence of Mycoplasma pneumoniae infection in children with community-acquired lower respiratory tract infections (LRTIs). A total of 245 patients 6 months to 12 years of age were investigated for M. pneumoniae employing serological tests, polymerase chain reaction (PCR), nested PCR, and reverse transcription PCR (RT-PCR) on throat swab samples. Forty five (59.2%) children <5 years and 31 (40.7%) children ≥5 years age group were positive for M. pneumoniae infection, and this difference was statistically significant (P ≤ 0.01).Clinical and radiological findings across M.pneumoniae-positive and -negative cases were comparable. Serology, PCR, nested PCR, and RT-PCR together detected M. pneumoniae infection in 76 (31%) patients. Sensitivity, specificity, and positive and negative predictive values of PCR were 16.18%, 95.48%, 57.89%, and 74.78%, respectively, and those of serology were 57.89%, 74.78%, 16.18%, and 95.48%, respectively. Serological and molecular detection in combination is useful for rapid and reliable diagnosis of M. pneumoniae infections in children with LRTIs.

Mycoplasma pneumoniae infection and asthma in children.

A clinical association between exacerbation of asthma symptoms and Mycoplasma pneumoniae ( M. pneumoniae) infection has long been suspected. We studied 80 children aged 5-15 years; 50 with asthma (Group 1) and 30 without an acute exacerbation of asthma (Group 2) for detection of M. pneumoniae by serology and polymerase chain reaction (PCR) on nasopharyngeal aspirates. Our study confirms that lower respiratory tract infections with M. pneumoniae are frequently associated with exacerbations of asthma in children.

Comparison of PCR, culture & serological tests for the diagnosis of Mycoplasma pneumoniae in community-acquired lower respiratory tract infections in children.

BACKGROUND & OBJECTIVE: Mycoplasma pneumoniae is known to be a major cause of lower respiratory tract infections in children. A specific diagnosis is important to institute the appropriate treatment. Information on diagnostic methods used for M. pneumoniae in Indian paediatric population is scarce. The study was thus conducted to compare polymerase chain reaction (PCR), culture and serology for the diagnosis of M. pneumoniae in community-acquired lower respiratory tract infections in children. METHODS: Seventy five children aged 6 months to 12 yr with signs of community-acquired lower respiratory tract infections were selected for the study. Culture of nasopharyngeal aspirates was done. The serum samples were analyzed for the detection of IgM and IgG antibodies to M. pneumoniae. A 543 base pairs (bp) region of P1 gene of M. pneumoniae was selected for amplification in PCR assay applied to nasopharyngeal aspirates. RESULTS: M. pneumoniae was isolated in culture from 4 (5.33%) children. Serological evidence of M. pneumoniae infection was observed in 16(21.3%) children. All culture positive patients were also positive by serology. Overall, PCR for M. pneumoniae was positive in 13 (17.3%) patients. All four culture positive patients were also positive by PCR. In 11 out of 13 (84.62%) PCR positive patients, serological evidence was there. Culture and/or serology and/or PCR positive results diagnosed M. pneumoniae infection in 18 (24%) of 75 patients. INTERPRETATION & CONCLUSION: A combination of culture, serology and PCR may provide diagnostic information on the aetiology of M. pneumoniae community-acquired lower respiratory tract infections in paediatric population.

Xpert(®) MTB/RIF assay for the diagnosis of pulmonary tuberculosis in children.

SETTING: A tertiary care teaching hospital in New Delhi, India. OBJECTIVE: To determine the sensitivity and specificity of the Xpert(®) MTB/RIF assay in paediatric pulmonary tuberculosis (PTB) using MGIT™ culture as gold standard. METHODS: After ethical approval had been obtained, 50 patients aged 0-14 years with suspected PTB were enrolled. Sputum/induced sputum and gastric lavage from the participants were sent for direct smear, MGIT culture and Xpert testing. Chest X-ray and tuberculin skin test (TST) were also performed. PTB diagnosis was made without considering Xpert results according to the Revised National Tuberculosis Control Programme (RNTCP) algorithm. The sensitivity and specificity of Xpert were calculated using culture as gold standard. RESULTS: Of 50 individuals with suspected PTB, 23 (46%) were diagnosed with PTB based on the RNTCP algorithm. Sixteen children from the PTB group (69.5%) were Xpert-positive. None in the 'not PTB' group were Xpert-positive. With culture as gold standard, Xpert sensitivity and specificity were respectively 91.6% (95%CI 59.7-99.5) and 86.8% (95%CI 71.1-95.05). CONCLUSION: In almost 70% of PTB cases, a definitive diagnosis could be made within 2 h using Xpert, establishing its role as a sensitive and specific point-of-care test.

Blood levels of isoniazid in Indian children with tuberculosis.

BACKGROUND: Under the Revised National Tuberculosis Control Program (RNTCP) in India children are receiving antituberculosis treatment (ATT) as per a weight band system. In this children may be receiving antituberculosis drugs in doses which may be more or less than that recommended in mg/kg body weight doses. The recommended dose of isoniazid (INH) for intermittent therapy under the RNTCP is 8-12 mg/kg body weight and by the World Health Organization (WHO) for daily therapy is 10-15 mg/kg body weight. AIMS: To evaluate the blood levels and pharmacokinetics of INH, in children suffering from tuberculosis, at doses administered under the weight band system of the Revised National Tuberculosis Control Program (RNTCP) 2009 of India. DESIGN: Prospective, open label, non-randomized single-dose study conducted in 20 children in the age group 5-12 years attending the outpatient, chest clinic of a tertiary care hospital. RESULTS: Group I (n = 8) included children who received INH in a dose of 10 mg/kg body weight or more and Group II (n = 12) included those who received INH in a dose less than 10 mg/kg body weight. The mean peak INH concentration (Cmax) was 6.03 ± 1.4 µg/mL and this was achieved in 2 hours (Tmax). The mean serum INH concentration was significantly higher in children who received INH in dose more than 10 mg/kg (Group I) as compared to those who received INH in doses lesser than 10 mg/kg body weight (Group II) at all-time points except at 2 hours (P < 0.05). The Cmax was also lower in Group II patients in comparison to Group I patients. Area under the concentration time curve (AUC) was significantly lower in Group II patients (P value 0.002). The elimination half-life of INH was 4.3 ± 0.4 h, elimination rate constant 0.16 ± 0.01/h, the volume of distribution 44.05 ± 5.3 L and clearance 7.1 ± 0.8 L/h. CONCLUSIONS: Lower blood levels and AUC of INH were achieved in children receiving doses of INH lesser than 10 mg/kg body weight. Long elimination half-life of INH is indicative of a slower rate of metabolism. Lower INH levels despite a slower rate of drug metabolism indicate caution with the INH doses being administered to children for intermittent therapy under the RNTCP.

Indoor air quality and acute lower respiratory infection in Indian urban slums.

The present prospective study was conducted at two urban slums of Delhi, Kusumpur Pahari and Kathputly Colony, in the peak winter season from November 1994 through February 1995. We studied 642 infants to determine the incidence of acute lower respiratory infection (ALRI) and its relationship to indoor air pollution due to fuel used for cooking (wood or kerosene). In Kusumpur Pahari, there were 317 children (142 wood and 175 kerosene), including 64 controls and 78 cases of ALRI in the wood fuel group and 81 controls and 94 ALRI cases in the kerosene group (p > 0.05). Out of 316 children in Kathputly Colony (174 wood and 142 kerosene), there were 33 and 45 ALRI cases in the wood and kerosene groups, respectively (p < 0.05). Controls were children without ALRI and were used as controls in different groups. The demographic data and risk factors, namely, nutritional and immunization status, were comparable in ALRI cases and controls in both study areas. Pneumonia was the most common ailment in all the groups. Bronchiolitis was reported in 22.5% of the wood group and 27.1% of the kerosene group in Kathputly Colony versus 13.7% in the wood group and 12.1% in the kerosene group in Kusumpur Colony. Only one case of croup was reported from Kusumpur Pahari among wood users. The duration of illness was longer in the Kusumpur Pahari due to poor compliance, feeding, and child rearing habits. In conclusion, a higher incidence of ALRI was reported in kerosene users in Kathputly Colony, a high pollution area; however, the reasons for the differences observed need further elucidation.

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of two cases with fine-needle aspiration cytology.

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare but well-defined histiocytic proliferative disorder. There are only few reports or small series of cases on the fine-needle aspiration (FNA) cytologic features of this entity. Our first case was a 14-yr-old girl who presented with low-grade fever and neck swellings of 2 mo duration. FNA smears from the submandibular and cervical lymph nodes showed numerous histiocytes with evidence of lymphophagocytosis (emperipolesis) against a background of reactive lymphoid cells. The FNA cytodiagnosis was Rosai-Dorfman disease. The patient was put on steroids, with improvement. The second patient was a 3(1/2)-yr-old girl who presented with fever and left cervical lymphadenopathy. The initial FNA smear from an outside laboratory was reported as reactive hyperplasia. A repeat FNA smear showed numerous histiocytes with evidence of emperipolesis, mild nuclear enlargement and multilobation/multinucleation in a few histiocytes, and reactive lymphoid cells. The cytodiagnosis was Rosai-Dorfman disease. Review of the initial smear showed the Rosai-Dorfman (RD) cells, but in smaller number. We conclude that FNA cytology is a useful tool in the diagnosis of SHML, but the diagnosis may be missed at initial stages when characteristic cytomorphologic features are not well-developed.

Typhoid fever. Unusual hepatic manifestations.

Four cases of typhoid fever with unusual hepatic manifestations are described. Two cases had hepatitis and two had hepatic abscess. These complications are documented for the first time in the pediatric age group. Awareness of these rare manifestations may be helpful in avoiding unnecessary morbidity and mortality.

Surgical emphysema: a rare presentation of foreign body inhalation.

An 11-year-old girl with an almond lodging in the tracheobronchial tree is described. She presented with an uncommon symptom of subcutaneous emphysema The x-ray revealed left-sided pneumothorax and pneumomediastinum. Intercostal drain was inserted, but she developed respiratory failure and was ventilated. After initial stabilization for 60 hours, she deteriorated again and her x-ray revealed right-sided collapse. After removal of the foreign body, she was discharged but presented again with stridor necessitating tracheostomy. Tracheal stenosis was found and required end-to-end anastomosis. The authors feel that, while foreign bodies are uncommon in this age group with emphysema as a rarer manifestation, this cause should be kept in mind, even in the absence of forthcoming history. A high index of suspicion for tracheobronchial foreign body is required in atypical presentations of acute pediatric respiratory distress.

Diagnosis and monitoring of childhood asthma.

Diagnosis of asthma is clinical, however due to varied presentations in childhood both under and over diagnosis are possible. A good number of cases may not present with wheeze but may have a cough variant asthma. Episodic symptoms of airflow obstruction and reversibility are two very important features of asthma. Many congenital (Cystic fibrosis) and acquired conditions (foreign body) may cause wheezing in childhood and should be ruled out clinically or by specific investigations. Spirometry and peak expiratory flow rates help in objective assessment and are good tools for monitoring chronic patients. Total IgE has no role in diagnosis. There is now emphasis on co-management of asthma and patient/parents should be trained to keep symptoms records and wherever possible peak flow records. They should also be taught proper interpretation of readings for stepping up therapy in case of worsening.

Bronchiectasis: causes and management.

Bronchiectasis is a condition representing abnormal and permanent dilatation and distortion of medium sized bronchi, usually accompanied by destruction of the airway wall. Post inflammatory bronchiectasis remains very common in the developing countries as a sequel to pulmonary tuberculosis, whooping cough, and severe measles (among other causes). Cystic fibrosis is the most common cause of generalized bronchiectasis in developed countries. Symptoms primarily are chronic cough and expectoration of foul smelling sputum. Bronchography was, until recently, the investigation of choice for the diagnosis of bronchiectasis and the gold standard against which the current best imaging technique HRCT (high resolution computed tomography) has been compared. Treatment includes prompt attention to acute exacerbations, management of airway secretions and control of airway hyperreactivity. Treatment is aimed at the non progression of the disease and complete cure if possible. The role of surgical therapy has evolved from early curative resection for all patients to a more palliative approach. Patients with advanced generalized bronchiectasis should be considered for lung transplantation.

Why do some boys run away from home?

OBJECTIVE: Insufficient research has been done to look for the factors compelling children to make street, their home. The study was conducted at a Child Observation Home to which street children from all over Delhi, are brought. METHODS: Each boy admitted during the specified 6 months period was interviewed. Chi2 and Fisher's test were applied. A total of 400 boys were studied, 9.8% of these had not run away from their homes and 89.2% were "Runaways". These two groups were compared and following factors were found associated with the "Runaway" group. RESULT: Majority (55%) had left home between 10-12 year of age. They were more from "Joint" families. A higher percentage had literate fathers. A higher percentage had no parent earning and a higher proportion of "Not Runaways" had only the mother earning. Presence of a step parent, guardian other than the parents and intra-familial physical abuse were found associated with "Runaway" group. CONCLUSION: The most common reason for running away was; beating by parents/relatives, followed by a desire for economic independence (28.5%). Other reasons were maltreatment by step parent/s, being both parents dead argument with parent etc. The factors emerging can be useful for identifying high-risk families with children in pre adolescent age and hence for prevention and rehabilitation.

Comparative efficiency of commercial and improvised spacer device in acute bronchial asthma.

OBJECTIVE: To compare the efficacy of a commercial spacer device versus an improvised spacer device in delivering aerosolized beta-2 agonist through metered dose inhaler in an acute exacerbation of bronchial asthma. DESIGN: Randomized controlled trial. SETTING: Urban tertiary care teaching hospital. METHODS: 60 children between 1 to 12 years of age with acute asthma were prospectively enrolled and randomized into two groups. Detailed history, clinical evaluation and appropriate laboratory investigations were recorded on a pretested proforma. One group received inhaled salbutamol using metered dose inhaler via commercial spacer device (Group 1), while the other received it via improvised spacer device (Group II). The response was sequentially assessed after 20, 40 and 60 minutes of institution of therapy. RESULTS: The two groups were comparable with respect to various parameters at presentation (p > 0.05). All the outcome parameters showed a significant improvement with time in both groups (p < 0.05). There was no statistical difference between the response in the two groups (p< 0.05). CONCLUSION: Metered dose inhaler with improvised spacer device is equivalent in efficacy and a more cost effective alternative to metered dose inhaler with commercial spacer for administration of beta-2 agonist in acute asthma.

Comparative efficacy of jet nebulizer and metered dose inhaler with spacer device in the treatment of acute asthma.

OBJECTIVE: To compare the relative efficacy of jet nebulizer and metered dose inhaler (MDI) with spacer for the administration of aerosolized salbutamol in an acute exacerbation of bronchial asthma. DESIGN: Randomized prospective study. SETTING: Emergency Room. METHODS: In 60 subjects with acute asthma aged between 1 to 12 years, clinical and laboratory assessment of severity at recruitment included heart rate, respiratory rate, pulsus paradoxus, arterial blood gas analysis (all cases) and peak expiratory flow rate (wherever possible). The subjects were randomized into two equal groups to receive aerosolized salbutamol either via nebulizer (Group I) or MDI-spacer (Group II) as per the Consensus Guidelines. The response to therapy was sequentially assessed after 20, 40 and 60 minutes of institution of therapy. RESULTS: A significantly (p < 0.02) greater number of subjects in Group II presented with severe dyspnea and intercostal muscle retraction (subjective assessment). However, the objectively evaluable outcome parameters were comparable (p > 0.05) in both groups at presentation. All the outcome measures showed a significant (p < 0.05) improvement with time in both the groups. The recovery parameters were comparable (p > 0.05) at different time periods in the two groups. CONCLUSION: MDI-spacer is as effective as a nebulizer for the aerosolized administration of salbutamol in an acute exacerbation of asthma in children. However, for developing countries, distinct advantages (economic and power requirement) argue strongly for utilization of MDI-spacer in preference to nebulizer.

Clinical predictors of hospitalization in an acute attack of bronchial asthma.

The present study was undertaken to identify the clinical predictors of hospitalization in an acute attack of bronchial asthma in subjects aged 2-12 years. Seventy five children with an acute attack of bronchial asthma were evaluated. A detailed clinical history and examination was recorded and baseline investigations sent before starting therapy. All subjects were treated with injection adrenaline (two doses) and those who did not respond were hospitalized. Twenty subjects who were hospitalized were compared with 32 cases who were discharged and did not have a relapse on follow up. After multivariate analysis, the factors independently predictive of hospitalization were, pulsus paradoxus (> 10 mm Hg; OR = 1.02), younger age (below 5 years, OR = 0.98) and severe accessory muscle use (OR = 89.6). Presence of any 2 of these 3 clinical predictors has a high sensitivity (90%) and specificity (96%). The investigative variables significant after multivariate analysis were low pH (OR = 0.00) and polymorphonuclear leucocytosis (> 70%) on peripheral smear (OR = 1.12). The sensitivity of this model was similar (90%) but specificity was lower (90.6%). The addition of investigative variables to clinical model did not improve the predictability. It is concluded that it is possible to identify at presentation, children with acute bronchial asthma who require hospitalization and clinical variables are sufficient for this purpose.

Predictors of mortality in subjects hospitalized with acute lower respiratory tract infections.

OBJECTIVE: To identify the predictors of mortality due to acute lower respiratory tract infection (ALRI). DESIGN: Prospective cohort study. SETTING: Urban tertiary care teaching hospital. METHODS: 201 cases with ALRI between 2 weeks to 5 years of age were prospectively enrolled and followed up to determine outcome. Detailed history and clinical evaluation were recorded on a pretested proforma. Significant independent predictors of mortality were determined by comparison of dead subjects (n = 21) with surviving children (n = 180) in a multiple logistic analytic framework. RESULTS: The case fatality rate (CFR) was 10.45%. Significant independent predictors of mortality were (OR, 95% CI) age less than 1 year (23.1, 2.7-197.5), inability to feed (6.2, 1.3-30.7), associated loose stools (5.1,1.2-27.3), weight for age Z score < -3 (3.9,1.01-9.7), short duration of fever (1.2,1.0-1.5) and bandemia (1.1,1.05-1.2). The WHO guidelines identified 91% of children diagnosed as ALRI by clinical and investigative criteria. The CFR was related to severity of WHO classification ("pneumonia"-0%, "severe pneumonia"-8.7% and "very severe pneumonia"-47.0%). However, 2 of the 18 subjects with a diagnosis of "no pneumonia" expired (CFR 11.1% and 10% of total mortality). CONCLUSION: Even in settings of high case fatality, predictors of mortality can be identified in under five children suffering from ALRI. In this context, age below 1 year, inability to feed, presence of loose stools and severe malnutrition merit attention for interventional purposes.

PIP: In a prospective cohort study (1993-94) conducted at a tertiary care teaching hospital in New Delhi, India, the predictors of mortality in 201 children 2 weeks to 5 years of age admitted with acute lower respiratory infection (ALRI) were investigated. There were 21 deaths in this series, for a case fatality rate of 10.45%. Mortality was inversely associated with age. Multivariate logistic regression identified the following significant predictors of mortality: age less than 12 months (odds ratio (OR), 23.1), inability to feed (OR, 6.2), associated loose stools (OR, 5.1), weight-for-age Z score under 3 (OR, 3.9), short duration of fever (OR, 1.2), and bandemia (OR, 1.1). The clinical and investigative criteria set forth in the World Health Organization (WHO) guidelines successfully identified 91% of children with ALRI. The case fatality rate was related to the severity of the WHO classification: pneumonia, 0%; severe pneumonia, 8.7%; and very severe pneumonia, 47.0%. However, 2 of the 18 children without any pneumonia died. At present, children under 2 months of age are designated as high-risk ALRI cases and targeted for close monitoring, referral, and therapy. The finding of this study of a 23-fold increased risk of mortality in children under 12 months of age suggests this cut-off should be expanded to 12 months.

Paediatric idiopathic myelofibrosis.

Pediatric myelofibrosis is a rare disorder. It is usually secondary to other diseases. Rarely, when no underlying cause is found, it is termed idiopathic. We present here, a rare case of idiopathic myelofibrosis in a 10 year old male child. Bone marrow aspirate was dilute. Bone biopsy showed marrow fibrosis, with grade 2-3 reticulin fibres, with no evidence of granuloma, parasite or infilterative disorder. Acid fast bacillus stain was negative. Iliac lymph node biopsy showed reactive sinus histiocytosis with extramedullary hematopoeisis. Thus, diagnosis of pediatric idiopathic primary myelofibrosis was made. Idiopathic pediatric myelofibrosis should be suspected in a child with progressive pallor, hepatosplenomegaly and dry tap on bone marrow aspiration and marrow fibrosis on bone biopsy, after exclusion of secondary causes.