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.

Evidence-Based Guidelines for the Management of Allergic Bronchopulmonary Aspergillosis (ABPA) in Children and Adolescents with Asthma.

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) frequently complicates asthma. There is urgent need to develop evidence-based guidelines for the management of ABPA in children. The Evidence Based Guideline Development Group (EBGDG) of the Indian Academy of Pediatrics (IAP) National Respiratory Chapter (NRC) addressed this need. METHODS: The EBGDG shortlisted clinical questions relevant to the management of ABPA in asthma. For each question, the EBGDG undertook a systematic, step-wise evidence search for existing guidelines, followed by systematic reviews, followed by primary research studies. The evidence was collated, critically appraised, and synthesized. The EBGDG worked through the Evidence to Decision (EtD) framework, to formulate recommendations, using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. RESULTS: Seven clinical questions were prioritized, and the following recommendations formulated. (1) Children with poorly controlled asthma should be investigated for ABPA (conditional recommendation, moderate certainty of evidence). (2) Low dose steroid therapy regimen (0.5 mg/kg/d for the first 2 wk, followed by a progressive tapering) is preferable to higher dose regimens (conditional recommendation, very low certainty of evidence). (3) Oral steroid regimens longer than 16 wk (including tapering), should not be used (conditional recommendation, very low certainty of evidence). (4) Antifungals may or may not be added to steroid therapy as the evidence was neither in favour nor against (conditional recommendation, low certainty of evidence). (5) For clinicians using antifungal agents, the EBGDG recommends against using voriconazole instead of itraconazole (conditional recommendation, very low certainty of evidence). (6) No evidence-based recommendation could be framed for using pulse steroid therapy in preference to conventional steroid therapy. (7) Immunotherapy with biologicals including omalizumab or dupilumab is not recommended (conditional recommendation, very low certainty of evidence). CONCLUSIONS: This evidence-based guideline can be used by healthcare providers in diverse clinical settings.

Mini versus ultra-mini percutaneous nephrolithotomy in a paediatric population.

OBJECTIVE: To evaluate whether there would be a difference in outcome when the smaller ultra-mini 12 Fr sheath was used instead of the mini 16 Fr sheath for percutaneous nephrolithotomy (PCNL) in paediatric patients for stones less than 25 mm. METHODS: This was a prospective cohort study of patients who underwent PCNL in our hospital in a 2-year period from July 2016 to June 2018 by a single surgeon. PCNL was performed in a prone position and tract was dilated to the respective size using single step dilatation. Laser was used to fragment the stone. Stone-free outcome was defined as absence of stone fragment at 3 months on kidney, ureter, and bladder X-ray. RESULTS: There were 40 patients in each group. Mean stone size was comparable between the two groups (14.5 mm vs. 15.0 mm). The procedure was completed faster in the 16 Fr group compared to 12 Fr group (24.5 min vs. 34.6 min). Stone clearance was highly successful in both groups (97.5% vs. 95.0%). There was no difference in complications between the two groups. The decrease in hemoglobin was minimal in both groups (0.2 g/dL vs. 0.3 g/dL). CONCLUSION: We found that the success rates were similar in both mini PCNL and the smaller ultra-mini PCNL groups. No significant difference in bleeding was noted in our pilot study, however, operative time was longer in the ultra-mini group as compared to the mini sheath group.

Mini-percutaneous cystolithotripsy (mPCCL) versus transurethral cystolithotripsy (TUC) in pre-school children: Prospective comparative non-randomized outcomes over 8 years.

INTRODUCTION: Bladder stones in children can often be challenging to manage. We present our experience of minimally invasive percutaneous cystolithotripsy using 'mini-perc' instruments in the treatment of the bladder stones in pre-school children. The study also compares the outcomes of mini-percutaneous cystolithotripsy (mPCCL) and transurethral cystolithotripsy (TUC). OBJECTIVE: We hypothesized that mPCCL is a new minimally invasive technique with comparable outcomes to TUC. STUDY DESIGN: After parental consent and institutional review board approval, consecutive pre-school children with bladder stones were included from January 2011-December 2018. We shifted from the TUC to mPCCL in 2014, with mPCCL introduced as a new technique. Outcomes including stone free rate (SFR) and complication rates were compared for patients aged between 1 and 5 years of age who underwent mPCCL or TUC. RESULTS: A total of 31 patients (16 patients in Group 1 (mPCCL) and 15 patients in Group 2 (TUC) underwent the procedure. The mean age, stone size and operative times were 2.4 ± 0.96 years and 3.8 ± 0.77 years; 1.86 ± 0.65 cm and 1.34 ± 0.52 cm; and 33.5 ± 8.42 min and 38.2 ± 6.76 min for groups 1 and 2 respectively. While there were no intra or post-operative complications in group 1, in group 2 one patient required conversion to mPCCL due to difficulty in fragment removal per urethra and one with residual fragment needed repeat cystoscopy and fragment removal (Clavien IIIb). All patients in both groups were discharged the following day after catheter removal and remained stone free at 1- and 6-month follow-up. DISCUSSION: Our study shows good outcomes in managing bladder stones with the use of both TUC and mPCCL. We shifted from the former to mPCCL in 2014 during which there was an overlap of both these techniques, while mPCCL was being introduced. Subsequently, all stones have been managed with mPCCL without the need to revert back to TUC. In Group 1, we performed mPCCL, where the puncture was performed under ultrasound-guidance and fragment retrieval was accomplished with the vacuum-cleaner effect through the operating sheath. Complete stone clearance was achieved in all cases with no complications or need for secondary procedures. Limitations of our study include small sample size, single-centre experience, and lack of randomization. Further prospective randomized multicenter studies may be required to validate our results. CONCLUSION: The technique of percutaneous suprapubic cystolithotripsy using mini-nephroscope is an alternate to transurethral cystolithotripsy. While both these techniques are suitable for smaller bladder stones, PCCL may be considered as the preferred management option of especially large bladder calculus in preschool children.

Insidious Risk of Severe Mycobacterium chimaera Infection in Cardiac Surgery Patients.

BACKGROUND: An urgent UK investigation was launched to assess risk of invasive Mycobacterium chimaera infection in cardiothoracic surgery and a possible association with cardiopulmonary bypass heater-cooler units following alerts in Switzerland and The Netherlands. METHODS: Parallel investigations were pursued: (1) identification of cardiopulmonary bypass-associated M. chimaera infection through national laboratory and hospital admissions data linkage; (2) cohort study to assess patient risk; (3) microbiological and aerobiological investigations of heater-coolers in situ and under controlled laboratory conditions; and (4) whole-genome sequencing of clinical and environmental isolates. RESULTS: Eighteen probable cases of cardiopulmonary bypass-associated M. chimaera infection were identified; all except one occurred in adults. Patients had undergone valve replacement in 11 hospitals between 2007 and 2015, a median of 19 months prior to onset (range, 3 months to 5 years). Risk to patients increased after 2010 from <0.2 to 1.65 per 10000 person-years in 2013, a 9-fold rise for infections within 2 years of surgery (rate ratio, 9.08 [95% CI, 1.81-87.76]). Endocarditis was the most common presentation (n = 11). To date, 9 patients have died. Investigations identified aerosol release through breaches in heater-cooler tanks. Mycobacterium chimaera and other pathogens were recovered from water and air samples. Phylogenetic analysis found close clustering of strains from probable cases. CONCLUSIONS: We identified low but escalating risk of severe M. chimaera infection associated with heater-coolers with cases in a quarter of cardiothoracic centers. Our investigations strengthen etiological evidence for the role of heater-coolers in transmission and raise the possibility of an ongoing, international point-source outbreak. Active management of heater-coolers and heightened clinical awareness are imperative given the consequences of infection.

Rowell syndrome.

Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

An infant with chronic severe neutropenia.

Neutropenia in infancy and childhood poses a diagnostic challenge as the aetiology ranges from acute life-threatening conditions to chronic benign diseases. Chronic benign neutropenia of infancy is a rare disorder occurring in 1:100,000. The neutrophil count continues to be low for a prolonged period until spontaneous resolution by the age of 3-4 years. Such infants are having higher incidences of minor infections requiring treatment with antibiotics and rare incidences of meningitis and sepsis. The authors describe an infant presenting with fever and cervical lymphadenitis, who was found to have isolated severe neutropenia and its persistence posing a diagnostic challenge. The prolonged course with minor infections and absence of serious underlying conditions finally confirmed chronic benign neutropenia of infancy.

Amitraz poisoning.

Amitraz is a formamidine insecticide and acaricide which acts on alpha 2-adrenergic receptors. There is little information available in the literature about the toxicity and treatment of poisoning by this compound. The authors report amitraz poisoning in a 13-y-old boy which was managed with supportive care with a good outcome.

Bardet-Biedl syndrome in two sisters: A rare incidence.

Bardet-Biedl syndrome is an autosomal recessive disorder characterized by retinitis pigmentosa, obesity, polydactyly, mental retardation and hypogonadism. We present two sisters with this rare genetic condition.