Indian Academy of Pediatrics Consensus Guidelines on Preconception Care.

JUSTIFICATION: The preconception period is the earliest window of opportunity to ensure optimal human development.  Pregnancy and childbirth outcomes can be improved by interventions offered to support the health and well-being of women and couples prior to conception. Thus, preconception care is essential in preparing for the first thousand days of life. Adolescence, the stage of life that typically comes before the preconception stage, is characterized by various high-risk behaviors like substance abuse, sexual experimentation, injuries, obesity, and mental health issues which can adversely affect their health in adult life. Thus, a Consensus Guideline for pediatricians on providing preconception care to adolescents and young adults can go a long way in making the generations to come, healthier and more productive. OBJECTIVES: The purpose of these recommendations is to formulate an evidence-based Consensus Statement that can serve as a guidance for medical professionals to provide preconception care for young adults and adolescents. INTENDED USERS: All obstetric, pediatric, and adolescent health care providers. TARGET POPULATION: Adolescents and young adults. PROCESS: A large proportion of adolescents seek care from pediatricians and there is a lack of Consensus Guidelines on preconception care. Therefore, the Indian Academy of Pediatrics called an online National Consultative Meeting on April 03, 2023, under the chairmanship of Dr MKC Nair and the National Convenor Dr Himabindu Singh. A group of pediatricians with wide experience and expertise in adolescent health care were assigned the task of formulating evidence-based guidelines on preconception care. The group conducted a comprehensive review of existing evidence by searching resources including PubMed and Cochrane databases. Subsequently, a physical meeting was held at Amritsar on October 07, 2023 during which the consensus was reached through discussions and voting. The level of evidence (LoE) of each recommendation was graded as per the Oxford Centre for Evidence-Based Medicine (OCEBM) 2011. RECOMMENDATIONS: Every woman planning a pregnancy needs to attain and maintain a eumetabolic state. Prospective couples need to be counselled on the importance of a healthy lifestyle including a nutritious diet, avoidance of substance abuse, and timely screening for genetic disorders. Screening for and management of sexually transmitted diseases in males and females, appropriate vaccination and addressing mental health concerns are also recommended.

Comprehensive chemo-profiling of coumarins enriched extract derived from Aegle marmelos (L.) Correa fruit pulp, as an anti-diabetic and anti-inflammatory agent.

Aegle marmelos (L.) Correa is an Indian medicinal plant known for its vast therapeutic activities. In Ayurveda, the plant is known to balance "vata," "pitta," and "kapha" dosh. Recent studies suggest anti-inflammatory, anti-microbial, and anti-diabetic potential but lack in defining the dosage over the therapeutic activities. This study aims to determine the chemical profile of Aegle marmelos fruit extract; identification, enrichment, and characterization of the principal active component(s) having anti-inflammatory and anti-diabetic potential. Targeted enrichment of total coumarins, focusing on marmelosin, marmesin, aegeline, psoralen, scopoletin, and umbelliferone, was done from Aegle marmelos fruit pulp, and characterized using advanced high-throughput techniques. In vitro and in silico anti-diabetic and anti-inflammatory activities were assessed to confirm their efficacy and affinity as anti-diabetic and anti-inflammatory agents. The target compounds were also analysed for toxicity by in silico ADMET study and in vitro MTT assay on THP-1 and A549 cell lines. The coumarins enrichment process designed, was found specific for coumarins isolation as it resulted into 48.61% of total coumarins enrichment, which includes 31.2% marmelosin, 8.9% marmesin, 4% psoralen, 2% scopoletin, 1.7% umbelliferone, and 0.72% aegeline. The quantification with HPTLC and qNMR was found to be correlated with the HPLC assay results. The present study validates the potential use of Aegle marmelos as an anti-inflammatory and anti-diabetic agent. Coumarins enriched from the plant fruit have good therapeutic activity and can be used for Phytopharmaceutical ingredient development. The study is novel, in which coumarins were enriched and characterized by a simple and sophisticated methodology.

Proposed method for evaluation and categorization of functional capacity of children, adolescents, and adults with cardiac diseases to bring them in existing social justice system by creating the cardiac disability criteria.

INTRODUCTION: Emerging epidemiological trends in India indicate the rising burden of cardiovascular diseases (CVDs) demanding a need of a social support system. Yet, the list of 21 benchmark disabilities notified by the Department of Empowerment of Persons with Disabilities, Ministry of Social Justice and Empowerment, Government of India, does not include CVDs under the newly enacted Rights of Persons with Disabilities (RPWD) Act, 2016. While the RPWD Act 2016 has acknowledged the dynamic nature of disabilities associated with congenital diseases like thalassemia, it has also provided an opportunity to bring in "cardiac disability" under its tenets. This would allow India to adopt strategies for the benefit of cardiac patients in accordance with policies adopted by developed countries such as the United States of America (USA), the United Kingdom of Great Britain (UK), and Canada. This document is to initiate a thought process of recruitment of cardiac patients in the social justice system. AIMS AND OBJECTIVES: (1) To define cardiac disability, (2) to categorize cardiac diseases/defects (groups A-C) according to severity and need for interventions, (3) to identify operated and unoperated patients with normal functional capacity and their eligibility to avail normal opportunities similar to their peer groups, (4) to create a comprehensive cardiac disability scoring (CCDS) system for disability certification based on subjective and objective evaluation of functional capacity and the corresponding heart disease category group, and (5) to create a reference literature for the issues of education, employability, insurability, and vocational counseling based on this document. METHODOLOGY: The evolution of this manuscript has been discussed in view of relevant observations made by a team of cardiologists, cardiac surgeons, intensivists, pediatricians, social workers, etc. CONCLUSION: This manuscript suggests a CCDS system to lay down criteria for disability status for eligible patients suffering from cardiovascular diseases. It intends to offer a unique scientific tool to address the psychosocial and socio-economic bias against patients with heart diseases of heterogeneous nature.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases.

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

Perioperative management of a patient with double orifice mitral valve with supramitral ring with subaortic membrane with ventricular septal defect and severe pulmonary hypertension: Report of a rare case.

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

Hepatoazygos venous shunt for Fontan completion after Kawashima operation.

Fontan completion after prior Kawashima repair for single ventricle with interruption of the inferior vena cava can be accomplished by various methods. We describe a patient who underwent the connection of hepatic to hemiazygos vein that we believe would be superior to the conventional cavopulmonary connection in our patient.

Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome.

Thoraco-abdominal viscera have unique morphological asymmetry, unlike the body's external organs. Heterotaxy syndrome is a disorder in which there is a loss of normal left to right asymmetry of thoraco-abdominal viscera and their naturally proscribed spatial relationship. It has multiple anatomical alterations, culminating into physiological and hemodynamic consequences. It is divided into two groups on the basis of morphology of the two atrial appendages. These subgroups are - 1) Isomerism of right atrial appendage (asplenia syndrome); 2) Isomerism of left atrial appendage (polysplenia syndrome); Patients from group I, usually have severe cardiac malformations and present early. They may have duct dependent lesions and eventually may undergo Fontan surgery. However, extracardiac anomalies are more common in group II. All the patients must be evaluated in detail to rule out anomalies like gut-malrotation. Patients must be provided with special care for their susceptibility to infection due to absence of spleen or presence of splenic malfunction. Majority of these patients may have genetic link and may present in families. Hence, genetic evaluation is necessary before assuming long term outcome.

Biventricular repair in heterotaxy patients.

BACKGROUND: Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. METHODS: From 2007 until 2012, a total of 20 patients (11 male) with heterotaxy syndrome underwent biventricular repair (left atrial isomerism: 10 and right atrial isomerism: 10) in our center. Their median age at surgery was 40 (range: 3-108) months. Ten patients had dextrocardia. Eleven patients presented with bilateral superior vena cava, three with inferior vena cava (IVC) draining into left atrium, and six with IVC interruption with azygos or hemiazygos continuation. Anomalous pulmonary venous drainage was present in eight patients. One had a common atrium. Atrioventricular septal defect (AVSD) occurred in nine (complete AVSD in seven) patients. Eight patients had double outlet right ventricle (DORV), one had d-transposition of great arteries (d-TGA), and two had congenitally corrected transposition of the great arteries (CC-TGA). Prior palliative procedures included pulmonary artery banding in three patients and left modified Blalock-Taussig shunt in one patient. Complex intra-atrial baffle constructions were required in all patients to direct pulmonary and systemic venous inflow to the appropriate ventricle. Complete AVSDs were corrected using two-patch technique. Intraventricular tunnel repair was done for DORV. Combined atrial and arterial switch was required to rectify abnormal connections in a child with congenitally corrected transposition with normal pulmonary valve, while a Rastelli + Senning was needed in two children with CC-TGA with pulmonary atresia (n = 1) and double outlet of the right ventricle (n = 1). RESULTS: Major early postoperative complications included intestinal gangrene in four patients for which they underwent bowel resection. Two of these patients could not be salvaged. One patient required coiling of aortopulmonary collateral for early postoperative pulmonary hemorrhage. Two patients needed a tracheostomy for prolonged mechanical ventilatory support. Five patients had a pacemaker implanted for complete heart block. There were no instances of atrial baffle stenosis. Median follow-up was 27 (range: 2-46) months. There was one late death secondary to pneumonia. CONCLUSIONS: Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.

One-stage midline unifocalization and anatomic correction of corrected transposition with pulmonary atresia and absence of central pulmonary arteries.

A concomitant double-switch procedure and unifocalization were performed in a child with congenital corrected transposition of the great arteries, ventricular septal defect, pulmonary atresia, absence of central pulmonary arteries and major aortopulmonary collateral arteries. Predischarge echocardiography showed no residual shunts with laminar flow in baffles and outflow tracts. Follow-up computed tomographic angiography revealed good neo-main pulmonary artery confluence with satisfactory pulmonary blood flow.

Right aortic arch with isolation of the left innominate artery in a case of double chamber right ventricle and ventricular septal defect.

Herein, we report an unusual case of right aortic arch with isolation of the left innominate artery in a case of double chamber right ventricle with ventricular septal defect. The blood supply to the innominate artery was by a collateral arising from the descending aorta. The embryological development of this anomaly can be explained by the hypothetical double aortic arch model proposed by Edwards with interruption of the arch at two levels.

Surgical repair of a submitral aneurysm in a three-year-old child.

Submitral aneurysm (SMA) is a rare anomaly occurring adjacent to the posterior leaflet of the mitral valve. It usually presents in young adults and it is uncommon in the pediatric age group. We report one case of SMA in a three-year-old child and its successful surgical management.

Spectrum of kawasaki disease.

The authors report 22 patients of Kawasaki disease, diagnosed and treated over a period of 3 years at a tertiary care centre in New Delhi. Ten cases fullfiled the criteria of a "classical" case while 12 cases were "incomplete" cases. Echocardiography was performed in all cases and coronary artery involvement was found in 8 (36%) cases. All cases received high dose Intravenous Immunoglobulins (IVIG) as standard therapy. In two cases, a repeat dose of IVIG was required for defervescence to occur. All cases recovered after therapy. Those with coronary artery involvement were planned for a 2 years follow up from the time of diagnosis.

Isolation, molecular characterization and growth-promotion activities of a cold tolerant bacterium Pseudomonas sp. NARs9 (MTCC9002) from the Indian Himalayas.

A bacterium that grows and expresses plant growth promotion traits at 4 degrees C was isolated from the rhizospheric soil of Amaranth, cultivated at a high altitude location in the North Western Indian Himalayas. The isolate was Gram negative and the cells appeared as rods (2.91 x 0.71 microm in size). It grew at temperatures ranging from 4 to 30 degrees C, with a growth optimum at 28 degrees C. It exhibited tolerance to a wide pH range (5-10; optimum 8.0) and salt concentrations up to 6% (wt/vol). Although it was sensitive to Rifampicin (R 20 microg mi-1), Gentamicin (G 3 microg mi-1), and Streptomycin (S 5 microg mi-1), it showed resistance to higher concentrations of Ampicillin (A 500 microg mi-1), Penicillin (P 300 microg mi-1), Polymixin B sulphate (Pb 100 microg mi-1) and Chloramphenicol (C 200 microg mi-1). The 16S rRNA sequence analysis revealed maximum identity with Pseudomonas lurida. The bacterium produced indole Acetic Acid (IAA) and solubilizes phosphate at 4, 15 and 28 degrees C. It also retained its ability to produce rhamnolipids and siderophores at 15 degrees C. Seed bacterization with the isolate enhanced the germination, shoot and root lengths of thirty-day-old wheat seedlings by 19.2, 30.0 & 22.9% respectively, as compared to the un-inoculated controls.

Isolation, molecular characterization and growth-promotion activities of a cold tolerant bacterium Pseudomonas sp. NARs9 (MTCC9002) from the Indian Himalayas

A bacterium that grows and expresses plant growth promotion traits at 4°C was isolated from the rhizospheric soil of Amaranth, cultivated at a high altitude location in the North Western Indian Himalayas. The isolate was Gram negative and the cells appeared as rods (2.91 x 0.71 μm in size). It grew at temperatures ranging from 4 to 30°C, with a growth optimum at 28°C. It exhibited tolerance to a wide pH range (5-10; optimum 8.0) and salt concentrations up to 6 percent (wt/vol). Although it was sensitive to Rifampicin (R 20 μg mi-1), Gentamicin (G 3 μg mi-1), and Streptomycin (S 5 μg mi-1), it showed resistance to higher concentrations of Ampicillin (A 500 μg mi-1), Penicillin (P 300 μg mi-1), Polymixin B sulphate (Pb 100 μg mi-1) and Chloramphenicol (C 200 μg mi-1). The 16S rRNA sequence analysis revealed maximum identity with Pseudomonas lurida. The bacterium produced indole Acetic Acid (IAA) and solubilizes phosphate at 4, 15 and 28°C. It also retained its ability to produce rhamnolipids and siderophores at 15°C. Seed bacterization with the isolate enhanced the germination, shoot and root lengths of thirty-day-old wheat seedlings by 19.2, 30.0 & 22.9 percent respectively, as compared to the un-inoculated controls.

Consensus guidelines on pediatric acute rheumatic fever and rheumatic heart disease.

JUSTIFICATION: Acute rheumatic fever and rheumatic chronic valvular heart disease is an important preventable cause of morbidity and mortality in suburban and rural India. Its diagnosis is based on clinical criteria. These criteria need verification and revision in the Indian context. Furthermore, there are glaring differences in management protocols available in literature. These facts prompted Indian Academy of Pediatrics to review the management of rheumatic fever. PROCESS: Management of Rheumatic fever was reviewed and recommendation was formulated at national consultative meeting on 20th May 2007 at New Delhi. OBJECTIVES: To formulate uniform guidelines on management of acute rheumatic fever and rheumatic heart disease in the Indian context. Guidelines were formulated for the management of streptococcal pharyngitis, acute rheumatic fever and its cardiac complication as well as secondary prophylaxis for recurrent episodes. RECOMMENDATIONS: (1) Streptococcal eradication with appropriate antibiotics (Benzathine penicillin single dose or penicillin V oral or azithromycin). (2) Diagnosis of rheumatic fever based on Jones criteria. (3) Control inflammatory process with aspirin with or without steroids (total duration of treatment of 12 weeks). (4) Treatment of chorea according to severity (therapy to continue for 2-3 weeks after clinical improvement). (5) Protocol for managing cardiac complication like valvular heart disease, congestive heart failure and atrial fibrillation. (6) Secondary prophylaxis with benzathine penicillin and management of anaphylaxis.

Comparison between transcatheter closure and minimally invasive surgery for fossa ovalis atrial septal defect: a single institutional experience.

BACKGROUND: Although, conventional surgical closure of atrial septal defect (ASD) provides excellent results with very low mortality and morbidity, it leaves the scar of incision and postoperative pain. Newer treatment modalities like minimal invasive surgery and percutaneous closure are being increasingly used nowadays where available. AIM: To compare the patient population, success, safety, and efficacy of transcatheter closure of ASD (Group A) with that of minimally invasive surgery (Port Access) (Group B). METHODS: In this retrospective non-randomized study, a record of a total of 640 patients with diagnosis of ASD secundum between May 1997 and October 2006 were reviewed. A total of 470 out of 640 patients were selected for transcatheter closure (Group A) while 170 patients were taken for surgical closure by minimally invasive port access surgery (Group B). The safety and efficacy of two groups was evaluated on the basis of morbidity and mortality, duration of intensive care unit (ICU) stay, total duration of hospital stay, post-procedural complications, residual sequel at time of discharge, and residual flow across the ASD. RESULTS: Success rate in two groups was 97.1% and 99.4%, respectively and had no statistically significant difference. Similarly major complication rate also had no difference in statistical significance (1.8% and 2.9% for Group A and B, respectively). Group B patients had longer hospital stay. A small but significant number of patients were not found suitable for device closure. This number is likely to decrease as experience with technique increases. Port access surgery is currently not possible in small children (femoral artery diameter 35 mm) due to difficulty in cannulation. CONCLUSION: Percutaneous device closure of ASD can be offered as a treatment option in suitable patients. Port access is minimally invasive and an equally safe and effective alternative choice in ASDs with deficient rim in patient with appropriate age and weight.