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Introduction: The association of pulmonary hemosiderosis with celiac disease (Lane-Hamilton syndrome) is extremely rare., Case Details: A five-year-old female child presented with fever, cough, breathlessness, and pallor for 20 days, without any previous history of recurrent lower respiratory tract infections, tuberculosis, or cardiac disease. There was no history of pica, chronic diarrhea, bleeding, or personal or family history of repeated blood transfusions. She had tachycardia, tachypnea, severe pallor, stunting, rickets, and bilateral fine lung crepitations. Peripheral smear and blood indices revealed dimorphic anemia. Anti-tissue transglutaminase IgA antibody levels were high (>200 U/mL) and the upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of celiac disease. The child was discharged on a gluten-free diet (GFD) and oral hematinic, but her dietary compliance was poor. Interestingly, the child had persistent bilateral pulmonary infiltrates, which was initially attributed to congestive cardiac failure (CCF), which persisted even despite treatment. HRCT chest revealed interstitial thickening and bilateral alveolar shadows and bronchoalveolar lavage showed a few inflammatory cells. The child was readmitted four times with similar complaints and was given packed red cell transfusions. In the fourth admission, a lung biopsy was done, which revealed extensive pulmonary hemosiderosis. The patient was given a course of oral steroids for 6 weeks, with a gluten-free diet, following which both the anemia and the pulmonary infiltrates resolved., Conclusion: Pulmonary hemosiderosis is an important cause of anemia in cases of celiac disease and may be misdiagnosed as CCF due to severe anemia. A strict GFD, with or without corticosteroids, can reverse the clinical and radiological picture., Competing Interests: None

Introduction: There is a paucity of studies on the progression and outcome of Systemic Inflammatory Response Syndrome (SIRS) with its determinants., Aims and Objectives: To determine the predictors of the outcome and progression of pediatric sepsis and septic shock., Materials and Methods: Prospective observational study of children fulfilling criteria of SIRS and their progression to sepsis, severe sepsis, and septic shock (clinically and biochemically) was conducted at a tertiary care center., Results: Totally, 200 children were recruited over a period of 21 months (from February 2016 to October 2017). Most cases (80, 40%) were infants. Of the total, 188 (94%) cases were of an infective etiology (mostly respiratory system). Temperature and heart rate were the two commonest SIRS parameters which were deranged. Blood cultures were positive in only 25 (12.5%) cases. Out of the total 200 children, 108 progressed to sepsis, of which 26 progressed to severe sepsis, of which 22 progressed to septic shock. Abnormal leukocyte count, culture positivity and severe acute malnutrition were significantly associated with progression of SIRS patients to septic shock (P = 0.001, 0.00001 and 0.002, respectively). Factors associated with mortality were positive blood culture, multiorgan dysfunction, late hospital admissions, severe acute malnutrition, and requirement of supportive care (P values-<0.0001, <0.0001, 0.03, <0.0001 and <0.0001, respectively)., Conclusions: SIRS can progress to septic shock if not identified early. The predictors of mortality were positive blood cultures, multiorgan dysfunction, late hospital admissions, severe acute malnutrition, and requirement of supportive care. The predictors of progression to septic shock were abnormal leukocyte count, culture positivity, and severe acute malnutrition., Competing Interests: None

Intracranial hemorrhage (ICH) is rarely seen in patients with thalassemia. A seven-year-old male, known case of beta-thalassemia major, on irregular packed cell transfusions (elsewhere) and non-compliant with chelation therapy, presented with congestive cardiac failure (Hb-3 gm/dl). He received three packed red cell transfusions over 7 days (cumulative volume 40 cc/kg). On the 9th day, he developed projectile vomiting and two episodes of generalized tonic-clonic convulsions with altered sensorium. He had exaggerated deep tendon reflexes and extensor plantars. CT-scan of brain revealed bilateral acute frontal hematoma with diffuse subarachnoid hemorrhage (frontal and parietal). Coagulation profile was normal. CT-angiography of brain showed diffuse focal areas of reduced caliber of anterior cerebral, middle cerebral, and basilar and internal carotid arteries (likely to be a spasmodic reaction to subarachnoid hemorrhage). He required mechanical ventilation for 4 days and conservative management for the hemorrhage. However, on the 18th day, he developed one episode of generalized tonic-clonic convulsion and his sensorium deteriorated further (without any new ICH) and required repeat mechanical ventilation for 12 days. On the 28th day, he was noticed to have quadriplegia (while on a ventilator). Nerve conduction study (42nd day) revealed severe motor axonal neuropathy (suggesting critical illness polyneuropathy). He improved with physiotherapy and could sit upright and speak sentences at discharge (59th day). The child recovered completely after 3 months. It is wise not to transfuse more than 20 cc/kg of packed red cell volume during each admission and not more than once in a week (exception being congestive cardiac failure) for thalassemia patients., Competing Interests: There are no conflicts of interest.

We report a 48-day-old female infant, who developed cardiac conduction abnormalities and seizures secondary to supratherapeutic doses of oral flecainide. Flecainide was started in this infant for treatment of supraventricular tachycardia. The drug was withdrawn with successful normalization of the QRS complex and no further recurrence of seizures. The Naranjo probability score for adverse drug reaction was 8, making the causality "probable." The case restates an important message that physicians should be aware of the side effects of the drugs that they prescribe, especially of those drugs which have a narrow therapeutic window.

Our patient presented with congenital heart disease (CHD: Tetralogy of Fallot), hypocalcemia, hypoparathyroidism, and facial dysmorphisms. Suspecting DiGeorge syndrome (DGS), a fluorescence in situ hybridization (FISH) analysis for 22q11.2 deletion was made. The child had a hemizygous deletion in the 22q11.2 region, diagnostic of DGS. Unfortunately, the patient succumbed to the heart disease. DGS is the most common microdeletion syndrome, and probably underrecognized due to the varied manifestations. This case stresses the importance of a detailed physical examination and a high index of suspicion for diagnosing this genetic condition. Timely diagnosis can help manage and monitor these patients better and also offer prenatal diagnosis in the next pregnancy.

An 18-month-old female child presented to us with clinical features suggestive of nephrotic syndrome. Her physical examination and detailed family history highlighted the familial occurrence of abnormal nails, suggesting a diagnosis of the Nail-Patella syndrome. Nail-Patella syndrome is a rare cause of nephrotic syndrome in children. This case highlights the importance of a detailed history, including pedigree and a thorough examination of the patient.

The treatment of the emerging multidrug resistant (MDR) gram-negative organisms is a challenge. The development of newer antibiotics has recently slowed down. This has led to the re-emergence of the 'old forgotten' antibiotic "Colistin", whose use had almost stopped (after 1970's) due to the high incidence of nephrotoxicity and neurotoxicity. Colistin (polymyxin E) is a polypeptide antibiotic belonging to polymyxin group of antibiotics with activity mainly against the gram-negative organisms. Use of colistin has been increasing in the recent past and newer studies have shown lesser toxicity and good efficacy. Colistin acts on the bacterial cell membrane resulting in increased cell permeability and cell lysis. Colistin can be administered orally, topically, by inhalational route, intramuscularly, intrathecally, and also intravenously. Parenteral Colistin (in the form of colistimethate sodium) has been used to treat ventilator-associated pneumonia (VAP) and bacteremia caused by MDR bacteria such as Pseudomonas aeruginosa, Klebsiella pneumoniae, and Acinetobacter baumannii. Inhaled Colistin is used for treating pneumonia/VAP due to MDR gram-negative organisms and also used prophylactically in patients with cystic fibrosis. This manuscript is a brief review of Colistin and its clinical applications in the pediatric population.

Pulmonary arteriovenous malformations are rare vascular anomalies which rarely present in childhood. We discuss a nine-year-old male child who presented with the classical triad of exertional dyspnea, cyanosis and clubbing since six years of age. Pulmonary arteriovenous malformation was suspected on chest radiography and was later confirmed on computed tomography scan. A bubble contrast echocardiography showed the presence of an extracardiac shunt. Pulmonary angiography was done to delineate the anatomy of the pulmonary vasculature and to plan embolization. The child has undergone two sittings of transcatheter coil embolization with improvement in oxygenation.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder (triad of iron-deficiency anemia, hemoptysis, and alveolar infiltrates). A 3-year-old male presented with mild fever, breathlessness, dry cough, and bluish nail discoloration for 8 days. He had required five blood transfusions in the past 1 year (last transfusion was given 4 months ago). He had a respiratory rate of 58/min with respiratory distress, cyanosis, and grade III clubbing. Respiratory system examination was normal. Several previous reports of hemoglobin were as low as 3.6 g/dl with hypochromic and microcytic anemia. There were transient increases in the hemoglobin and normalization of red cell morphology with blood transfusions. Serum iron, G6PD enzyme assay, hemoglobin electrophoresis, the sickling test, Coomb's test, stool and urine analysis, and a Meckel's scan were normal. HIV antibody and dsDNA were negative. The chest radiograph revealed symmetrical patchy infiltrates sparing lung apices (confirmed on high-resolution computed tomography). Lung biopsy diagnosed pulmonary hemosiderosis (interstitial lung disease with hemosiderin-laden macrophages scattered in the alveoli and areas of fibrosis in the alveolar septa). The patient showed marked clinical improvement in 10 days of therapy with prednisolone. IPH should be listed in the differential diagnosis of a child presenting with unexplained hypochromic, microcytic anemia and respiratory symptoms.

Oseltamivir, a selective neuraminidase enzyme inhibitor, has gained worldwide attention in view of Influenza A (H1N1) pandemic. It is one of the most important drugs effective against the novel influenza virus. Oseltamivir is used for the treatment of uncomplicated acute illness due to influenza infection. Early initiation of treatment with the drug provides greater clinical benefits. The drug can also be effectively used for prophylaxis. Oseltamivir is readily absorbed from the gastrointestinal tract and is converted to the active metabolite- oseltamivir carboxylate, which has a wider distribution in the body. Oseltamivir carboxylate is eliminated in the urine with a half-life of 6-10 h. The drug is generally well-tolerated and does not have many clinically significant drug interactions. Nausea and vomiting are the commonest adverse effects associated with its use. The standard adult dose for treatment is 75 mg twice a day for five days and the dose for prophylaxis is 75 mg once daily for at least seven days following contact with an infected individual. The dose needs to be adjusted in patients with renal failure but no adjustments are required in patients with hepatic impairment. Although most of the influenza virus strains are sensitive to oseltamivir, development of drug resistance may limit the clinical utility of the drug in the future.

Colloid cyst is a rare benign intracranial neoplasm, commonly located in the third ventricle. Though headache and visual symptoms are classical, the patients may present with sudden neurological deterioration. We present a ten-year-old male child who presented with sudden neurological deterioration due to colloid cyst of the third ventricle resulting in death. The child had intermittent headache for three months, for which medical attention was not sought. This report details the case and a short review of the condition is presented (with emphasis on the clinical features and importance of early diagnosis). Management (including surgical methods and conservative treatment) of third ventricle colloid cysts is briefly reviewed.

Morgagni hernia is a rare diaphragmatic hernia accounting for only 2% of the congenital diaphragmatic defects. A case of Morgagni hernia was diagnosed radiologically in a 12-months-old male with Down syndrome, with recurrent respiratory distress and chest deformity. The 2-dimensional echocardiography was normal. The diagnosis of Morgagni hernia was confirmed by barium studies. The patient underwent a corrective surgery at 18 months of age following which his symptoms subsided. Literature review revealed only 18 cases of Morgagni hernia with Down syndrome reported till date, with age of presentation varying from neonatal age group to 12 years of age. The mode of presentations varied from asymptomatic detection to severe respiratory distress. The possible mechanism of association and its clinical implication has been discussed. The case emphasises a need for diaphragmatic hernia to be looked for as a possible cause of respiratory distress in Down syndrome.

Autosomal translocations are rare in the patients with ovarian dysgenesis. An 18-year-old female who presented with primary amenorrhoea had hypergonadotropic hypogonadism and streak ovaries with hypoplastic uterus. Karyotype analysis revealed a balanced autosomal translocation involving chromosomes 1 and 11. The probable role of autosomal translocations in ovarian dysgenesis has been discussed.

Neurofibromatosis (NF), Noonan syndrome (NS), and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the 'Neurofibromatosis-Noonan syndrome' (NF-NS). The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported.

Aims: To determine the incidence, risk factors, mortality and organisms causing nosocomial pneumonia (NP) in intubated patients in Paediatric Intensive Care Unit (PICU)., Materials & Methods: All patients with endotracheal (ET) tube with or without mechanical ventilation (MV) in a PICU of a tertiary care teaching hospital were included in this prospective study. Clinical parameters and investigations were evaluated in patients who developed nosocomial pneumonia (NP). Colonisation of the ET tube tip was studied by culture and the antibiotic susceptibility pattern of the isolates was determined., Results: Sixty-nine patients had an ET tube inserted and fifty-nine of these underwent MV. ET tube tip colonisation was seen in 70 out of 88 ET tubes inserted. The incidence of NP in patients with ET tube was 27.54% (7.96/100 days of ET intubation). NP developed only in patients undergoing MV. The main risk factors for developing NP were - duration of MV and duration of stay in the PICU. Age, sex, immunocompromised status and altered sensorium did not increase the risk of NP. The mortality in cases with NP was 47. 37%. E. coli and Klebsiella were the commonest organisms isolated from the ET tube tip cultures with maximum susceptibility to amikacin and cefotaxime., Conclusions: NP developed only in patients undergoing MV. Duration of MV and duration of stay in the PICU increased the risk of developing NP.

A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.

Penicillin-induced immune haemolytic anaemia is very rare. A ten year-old-female with rheumatic mitral stenosis on benzathine penicillin prophylaxis presented with features of haemolytic anaemia and investigations supported the diagnosis of immune haemolytic anaemia. Patient responded to discontinuation of the drug and therapy with oral prednisolone. This is first such case reported from India.

Prenatal diagnosis of cri-du-chat syndrome is described in 2 pregnancies. In Case 1, the mother was a balanced translocation carrier and had 2 previously affected off springs. Prenatal diagnosis by chorion villus sampling and cordocentesis was successful in diagnosing an affected conceptus and the pregnancy was electively terminated. Case 2 was referred for nonimmune foetal hydrops and cordocentesis revealed deletion 5p. This second case was noteworthy for the fact that deletion 5p has not been reported to cause foetal hydrops.

The present prospective study was carried out in the Paediatric Intensive Care Unit (PICU) of a tertiary care teaching hospital in Mumbai. The objective was to determine the incidence, risk factors, mortality and organisms responsible for urinary catheter related infections (UCRI). Colonization and/or bacteriuria was labelled as urinary catheter related infection (UCRI). Forty-four patients with 51 urinary catheters were studied. Incidence of UCRI was 47.06%. Age, female sex and immunocompromised status did not increase the risk of UCRI. Duration of catheter in-situ and duration of stay in the PICU were associated with higher risk of UCRI. The mortality was not increased by UCRI. Commonest organism isolated in UCRI was E. coli, which had maximum susceptibility to nitrofurantoin and amikacin.

This prospective study was carried out over a period of 6 months in the Paediatric Intensive Care Unit (PICU) of a tertiary care teaching hospital. The aim of the study was to determine the organisms causing catheter related nosocomial infections in the PICU and to study their antimicrobial susceptibility pattern. Patients with endotracheal intubation, indwelling urinary catheters and central venous catheters (CVC)/venous cutdown catheters were included in the study. Colonization of the endotracheal tube, urinary catheter related infections (UCRI) and colonization of the CVC/venous cutdown catheters was studied. E. coli was the commonest organism colonizing the endotracheal tube tip with maximum susceptibility to cefotaxime and amikacin. E. coli was also was the commonest organism causing UCRI with maximum susceptibility to nitrofurantoin and amikacin. Acinetobacter was the commonest organism colonizing the CVC/venous cutdown catheters with maximum susceptibility to ciprofloxacin. All these sites of catheter related infections considered together, E. coli and Klebsiella were the commonest nosocomial organisms. Both had maximum susceptibility to amikacin. Methicillin resistant Staphylococcus aureus (MRSA) was isolated only from one culture. All the organisms had a poor susceptibility to cefazolin and amoxycillin. A knowledge of the resident microbial flora and their antimicrobial susceptibility pattern is necessary for formulating a rational antibiotic policy in an ICU.