Early-term births are associated with high neonatal morbidity and with NICU or neonatology service admission. Evaluation of local prevalence data will assist in implementation of specific preventive measures and plans, as well as prioritize limited health care resources.

Lane-Hamilton syndrome refers to the uncommon co-occurrence of idiopathic pulmonary hemosiderosis and celiac disease (CD). Three children aged between 7 and 14 years with IPH were detected to have co-existing non-diarrheal CD. Institution of gluten-free diet in each of the three children resulted in amelioration of the pulmonary symptoms along with improvement of anthropometric parameters and hemoglobin over a short-term follow-up period of 8-17 months. Inhaled/oral steroids and immunosuppressants could be weaned off after dietary exclusion therapy in each of the three children. Gluten free diet should be instituted in all patients diagnosed with Lane-Hamilton syndrome. It ameliorates both the pulmonary as well as the intestinal symptoms although the precise mechanism of the pulmonary response is as yet unclear.

Idiopathic Pulmonary Hemosiderosis (IPH) is characterized by the triad of iron deficiency anemia, pulmonary infiltrates and haemoptysis with no recognizable cause. Since the first description of its association with Celiac Disease (CD) by Lane and Hamilton in 1971, only a few isolated cases have been reported in literature. Although it has been considered an uncommon association of two disease entities, recent reports indicate that prevalence of celiac disease is as high as one percent. Further, individually both celiac disease and IPH are known to present as refractory anemia only. We are reporting a young adult with Lane Hamilton Syndrome, who realized that he was having significant gastrointestinal complaints only when they disappeared on gluten free diet (GFD). This case report reiterates the fact that celiac disease should be considered in all patients of IPH because of the therapeutic implications. Further on review of literature, we believe that covert hemoptysis may be responsible for disproportionately severe anemia in patients of celiac disease. Thus, prevalence of this association may be more than currently believed. Further research in this regard may improve our understanding of pathogenesis of celiac disease.

Respiratory distress syndrome (RDS) is characterized by the presence of fibrin-rich exudates in the alveoli. Fibrin and its degradation products may play an important role in the pathogenesis of bronchopulmonary dysplasia (BPD). The purpose of this study was to test the hypothesis that preterm neonates with RDS have depressed alveolar fibrinolytic activity and that those with RDS progressing to BPD have an even greater impairment in alveolar fibrinolysis. Serial tracheal aspirate (TA) samples from intubated neonates--9 control and 46 with RDS--were analyzed for fibrinolytic activity. In neonates with RDS, 26 resolved, 18 progressed to BPD, and 2 died before 28 d. Plasminogen activator (PA) and its inhibitor (PAI) were identified in TA by reverse fibrin autography and immunoblotting. Net PA/plasmin activity in TA was significantly depressed on d 1 of life in patients with self-resolved RDS (median = 20.85 ng/mL, p < 0.05) and RDS progressing to BPD (median = 4.97 ng/mL, p < 0.001) compared with control patients (median = 87.1 ng/mL). In addition, neonates progressing to BPD had significantly lower PA/plasmin activity on day one of life compared with neonates with self-resolved RDS (p < 0.001). ELISA for specific PA and PAI were not significantly different. We speculate that depressed fibrinolytic activity may place preterm neonates at risk for RDS and that the degree of this depression may predict the progression to BPD. In infants < or = 30 wk of gestation at birth with RDS, a PA/plasmin activity < or = 10.0 ng/mL on the 1st d of life had a positive predictive value of 80% (12/15) and a negative predictive value of 82% (9/11) for the progression to BPD.

Steroids (corticosteroids) are anti-inflammatory drugs. Corticosteroids are used in many pulmonary conditions. Corticosteroids have a proven beneficial role in asthma, croup (Laryngotracheobronchitis), decreasing the risk and severity of respiratory distress syndrome (RDS), allergic bronchopulmonary aspergillosis, interstitial lung disease, hemangioma of trachea, Pulmonary eosinophillic disorders. Role of corticosteroids is controversial in many conditions e.g. idiopathic pulmonary hemosiderosis, bronchiolitis, hypersensitivity pneumonitis, hyperplasia of thymus, bronchiolitis, acute respiratory distress syndrome, aspiration syndromes, atypical pneumonias, laryngeal diphtheria, AIDS, SARS, sarcoidosis, meconium aspiration syndrome (MAS), pulmonary haemorrhage, bronchitis, bronchiolitis obliterans with organizing pneumonia in JRA, histiocytosis, alpha-1 antitrypsin deficiency, bordtella pertusis, pulmonary involvement in histiocytosis. However these are used empirically in many of these conditions despite lack of clear evidence in favour. There is concern about their side effects, especially on growth. Systemic steroids are associated with significant adverse effects. Pulmonary conditions have a strategic advantage that inhaled corticosteroids are useful in many of these. Although inhaled preparations of corticosteroids have been developed to maximise effective treatment of lung diseases characterised by inflammation and reduce the frequency of harmful effects, these have not been eliminated. There are situations where only systemic steroids are useful. Clinicians must weigh the benefits against the potential detrimental effects. It is recommended that standard protocols for use of steroids available in literature should be followed, always keeping a watch on the potential hazards of prolonged use.

Bronchoscopes have markedly improved the diagnosis as well as therapy in pediatric pulmonary disorders. Two types of bronchoscopes are available; flexible and rigid, with their own advantages and disadvantages. Depending on the clinical need and availability of skills, choice is made between the two. Typically, rigid scopes are largely used by the surgeons (pediatric or otolayngologists) while flexible bronchoscope stays in the domain of the pediatric pulmonologist and intensivists. Rigid scopes may be more versatile than flexible bronchoscopes in removing the foreign bodies from the airway. Flexible bronchoscopes on the other hand can even be introduced through an endotracheal tube. At times, use of both scopes may be required in a given patient for optimal results. Bronchoscopes give us a means to visualize the inside of the airway, which can be very informative for assessing various pathologies affecting the airways. Apart from the visualization of the parts of the airway tree and their structure as well as patency, it can also be used to take tissue biopsy specimens, collect secretions from the airways and bronchoalveolar lavage which can also get cellular elements from the distal alveoli. In the past few decades, more and more instruments are being used for expanding the utility of flexible bronchoscope for interventions ranging from bronchial toilet, foreign body removal, airway stenting and lasers or cryotherapy for airway lesions. The perinatologists have opened up more vistas and thrown newer challenges for using fiberoptic bronchoscopy (FB) for in utero tracheal occlusion in cases with diaphragmatic hernia. The vast applications of this tool makes it very relevant to pulmonary investigations and therapeutics.

doi: medRxiv preprint NOTE: This preprint reports new research that has not been certified by peer review and should not be used to guide clinical practice.

Sir,We present here an infant who had acute lymphoblastic leukemia with massively enlarged kidneys due to leukemic infiltrates. A 7-month-old girl child presented with complaints of low grade fever and coryza for 6 days. The mother noticed angular deviation of mouth and tongue to right side for the past 4 days and decreased movements of right upper limb for same duration. On examination, the child had pallor and few petechie over the trunk. The vitals were HR 122/min, pulses well palpable, RR 48/min; blood pressure was 134/94 mm Hg. Her anthropometry was: weight 6.6 Kg (10th centile), length 65 cm (25 th centile), and head circumference was 41.5cm (25 th centile). Central nervous system examination revealed normal sensorium. There was left facial lower motor neuron palsy, right hypoglossal palsy and right sided hemiparesis (upper motor neuron type). There were no meningeal or cerebellar signs. She had a soft hepatomegaly (2.5 cm below costal margin) and splenomegaly (6 cm below costal margins). Bilateral kidneys were palpable and occupied most of the abdomen, their surface was smooth.The mother had undergone ultrasonography during the antenatal period and no abnormality of fetal kidneys was reported. Initial investigations revealed hemoglobin 5.8g%, TLC 7500/µl, polymorphs 35, lymphocytes 65 and Platelet count of 26X10 9 /L. The peripheral smear had normocytic normochromic to mildly hypochromic red blood cells, platelets were diminished and 3% blasts were present. The biochemistry showed blood urea 45mg/dL, serum creatinine 0.8 mg/dL, serum ALT 24 IU/L, AST 25 IU/L. albumin 4.1 gm/dL, SAP 240 IU/L. The serum sodium was 133 mEq/L, K 4.0 mEq/L, calcium 8.2 mg/ dL, and phosphates 5.4 mg/dL. A venous blood gas had a pH of 7.35, bicarbonate 21 mEq/L, base excess -5 mEq/ L. A lumbar puncture examination done showed 80 cells, polymorphs 45 and lymphocytes 25 and presence of abnormal cells; CSF sugar was 87 mg/dL and protein 62 mg/dL. Glomerular filtration rate as calculated by the Schwartz method was 45 ml/min/1.73m².An ultrasonogram of the abdomen revealed liver of normal size and echotexture. The spleen was enlarged 9.2 cm, right kidney (10.7X 5.0 cm); left kidney (10.2 X 5.5 cm), bilateral grossly enlarged kidneys with smooth outline extending from epigastrium to pelvic inlet compressing midline retroperitoneal vessels. Multiple dilated tubular structures were seen in cortex and medulla. A computed tomography of the abdomen showed enlarged smooth Acute Lymphoblastic Leukemia: An Unusual cause of Nephromegaly in Infancy kidneys with no cystic dilatation with maintained corticomedullary differentiation. An MRI of the brain was suggestive of generalized brain atrophy. A bone marrow aspirate showed presence of 21% lymphobalsts (PAS & myeloperoxidase negative blasts) in the marrow. A diagnosis of acute lymphoblastic leukemia with renal and CNS infiltrations was made. A renal biopsy was not done in view of persistent thrombocytopenia.The child was started on chemotherapy for standard risk: ALL (prednisolone, L-asparginase...