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.

Non Malignant Lymphoproliferative Disorders in Children: A Case Series.

Lymphoproliferative disorders occurs due to uncontrolled proliferation of lymphocytes that causes lymphocytosis, lymphadenopathy, and involvement of extra nodal sites (bone marrow, liver and spleen) and occur primarily due to immune dysfunction. We describe series of cases with non malignant LPD encountered in our practice and their varied clinical presentation, difficulties in diagnosis, underlying etiology, treatment and outcome. Many of these disorders are self limiting, however some are associated with significant morbidity, hence treatment must be tailored based on the underlying immune dysfunction and aggressiveness of the clone.

Hemophagocytic Lymphohistiocytosis in Children.

OBJECTIVE: To study the profile of children with Hemophagocytic Lymphohistiocytosis (HLH) in a tertiary care hospital for children. METHODS: A retrospective analysis of case records of 52 children diagnossed with HLH was performed. RESULTS: Of the 52 children 13% (n = 7) had Familial HLH and 87% (n = 45) had secondary HLH (sHLH). Common manifestations were fever (100%), organomegaly (87%), respiratory distress (54%), neurological symptoms (31%) and skin rashes (26.2%). Anemia and thrombocytopenia were present in 51% and 73% respectively. Hyperferritinemia was present in 96% and hypofibrinogenemia in 42% and high lactate dehydrogenase (LDH) in 91%. Bone marrow examination showed hemophagocytosis in 80%. Most common etiology among infections was viral infections (67%), of which Dengue was the most common (52%). Among children with sHLH 51% received supportive care only. Thirty-seven percent (n = 17) received intravenous (IV) immunoglobulin and steroids. Of these 77% (n = 35) recovered completely. Children with familial HLH were initiated on HLH 2004 protocol but all of them expired due to disease progression. CONCLUSIONS: Identifying HLH early and managing it, poses a significant challenge. Prompt recognition and initiation of immunosuppressive therapy is extremely important for the better outcome; hence high clinical suspicion and structured work up including immunological, and genetic studies is required. It may be difficult to differentiate primary and secondary HLH in many instances unless genetic analysis is done. Identification of familial HLH is necessary for early referral to Hematopoietic Stem Cell Transplantation (HSCT). Hence screening for primary HLH needs to be considered in all children with HLH.

Clinical Characteristics and Associated Congenital Lesions with Tracheomalacia in Infants.

OBJECTIVE: To describe clinical presentation, bronchoscopy findings and associated anomalies in cases of congenital tracheomalacia in infants (age <1y). METHODS: Hospital record review of 88 infants (mean age 8 mo, 57 males) diagnosed as having tracheomalacia by flexible bronchoscopy between 2012 and 2015. RESULTS: The predominant features were wheeze (57.9%), stridor (42.1%), cough (38.6%), pneumonia (29.5%) and collapse (12.5%). On bronchoscopy, malacia was observed in lower half of trachea in 51 (57.9%) infants. Synchronized airway lesions observed were laryngomalacia (30.7%) and bronchomalacia (3.4%). 15 (17%) infants had associated congenital heart disease and 21 (23.8%) required care in intensive care unit. CONCLUSION: Wheeze,stridor and cough are the main symptoms in tracheomalacia. Laryngomalacia and congenital heart diseases are the most common other anomalies associated in these infants.

Network Coded Cooperative Communication in a Real-Time Wireless Hospital Sensor Network.

The paper presents a network coded cooperative communication (NC-CC) enabled wireless hospital sensor network architecture for monitoring health as well as postural activities of a patient. A wearable device, referred as a smartband is interfaced with pulse rate, body temperature sensors and an accelerometer along with wireless protocol services, such as Bluetooth and Radio-Frequency transceiver and Wi-Fi. The energy efficiency of wearable device is improved by embedding a linear acceleration based transmission duty cycling algorithm (NC-DRDC). The real-time demonstration is carried-out in a hospital environment to evaluate the performance characteristics, such as power spectral density, energy consumption, signal to noise ratio, packet delivery ratio and transmission offset. The resource sharing and energy efficiency features of network coding technique are improved by proposing an algorithm referred as network coding based dynamic retransmit/rebroadcast decision control (LA-TDC). From the experimental results, it is observed that the proposed LA-TDC algorithm reduces network traffic and end-to-end delay by an average of 27.8% and 21.6%, respectively than traditional network coded wireless transmission. The wireless architecture is deployed in a hospital environment and results are then successfully validated.

Recurrent oral thrush.

Autoimmune polyendocrinopathy syndrome type 1 (APS-1) is characterized by the presence of at least two out of three clinical features, which include chronic mucocutaneous candidiasis (CMC), Addison's disease and hypoparathyroidism. The authors' present an one and a half year old girl with recurrent oral thrush who presented with generalised afebrile seizure. Evaluation revealed severe hypocalcemia with low parathormone and normal vitamin D level consistent with hypoparathyroidism. In view of the oral candidiasis and hypoparathyroidism, a clinical possibility of autoimmune polyglandular syndrome (type 1) was strongly considered. Her mother, on subsequent pregnancy was subjected to gene analysis of the fetus (chorionic villus sampling) and also for this child (index case). Both the fetus and index child were confirmed to have the AIRE gene mutation of APS1. After detailed counseling the parents opted for medical termination of the pregnancy. In children who present with recurrent oral thrush we need to consider but also look beyond immunodeficiency.