Gulshan Rai Sethi

Benefit of gluten‐free diet in idiopathic pulmonary hemosiderosis in association with celiac disease

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. Pediatr Pulmonol. 2011; 46:302–305.

Sinus histiocytosis with massive lymphadenopathy (Rosai‐Dorfman disease): Report of two cases with fine‐needle aspiration cytology

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai‐Dorfman disease is a rare but well‐defined histiocytic proliferative disorder. There are only few reports or small series of cases on the fine‐needle aspiration (FNA) cytologic features of this entity. Our first case was a 14‐yr‐old girl who presented with low‐grade fever and neck swellings of 2 mo duration. FNA smears from the submandibular and cervical lymph nodes showed numerous histiocytes with evidence of lymphophagocytosis (emperipolesis) against a background of reactive lymphoid cells. The FNA cytodiagnosis was Rosai‐Dorfman disease. The patient was put on steroids, with improvement. The second patient was a 3½‐yr‐old girl who presented with fever and left cervical lymphadenopathy. The initial FNA smear from an outside laboratory was reported as reactive hyperplasia. A repeat FNA smear showed numerous histiocytes with evidence of emperipolesis, mild nuclear enlargement and multilobation/multinucleation in a few histiocytes, and reactive lymphoid cells. The cytodiagnosis was Rosai‐Dorfman disease. Review of the initial smear showed the Rosai‐Dorfman (RD) cells, but in smaller number. We conclude that FNA cytology is a useful tool in the diagnosis of SHML, but the diagnosis may be missed at initial stages when characteristic cytomorphologic features are not well‐developed. Diagn. Cytopathol. 2001;24:42–45.

Why do some boys run away from home

Objective. Insufficient research has been done to look for the factors compelling children to make street, their home. The study was conducted at a Child Observation Home to which street children from all over Delhi, are brought.Methods : Each boy admitted during the specified 6 months period was interviewed. ϰ2 and Fisher’s test were applied. A total of 400 boys were studied, 9.8 % of these had not run away from their homes and 89.2% were “Runaways”. These two groups were compared and following factors were found associated with the “Runaway” group.Result : Majority (55%) had left home between 10–12 year of age. They were more from “Joint” families. A higher percentage had literate fathers. A higher per centage had no parent earning and a higher proportion of “Not Runaways” had only the mother earning. Presence of a step parent, guardian other than the parents and intra-familial physical abuse were found associated with “Runaway” group.Conclusion : The most common reason for running away was; beating by parents/relatives, followed by a desire for economic independence (28.5%). Other reasons were maltreatment by step parent/s, being both parents dead argument with parent etc. The factors emerging can be useful for identifying high-risk families with children in pre adolescent age and hence for prevention and rehabilitation.

Study of pulmonary function tests in thalassemic children.

The present study aimed to investigate pulmonary function tests (PFTs) in children with thalassemia and to assess the relation between the degree respiratory impairment with the body iron status. High resolution computed tomography of chest (CHRCT) and bronchoalveolar lavage (BAL) was performed to study the cause of pulmonary dysfunction. Thirty-one children with thalassemia over 8 years were included. PFTs were studied including lung volumes and carbon monoxide diffusion capacity (DLco). Patients with abnormal PFTs and/or impaired DLco were further subjected to CHRCT and BAL. Total cell count was measured; differential count was performed on Giemsa and PAP smears. Iron laden macrophages were identified on Perls stain. PFTs were normal in 51.61%, diffusion capacity impaired in 41.16%, restriction in 16.12%, while obstruction in 3.22% of cases, respectively. There was significant inverse correlation between DLco and serum ferritin. Through multivariate regression analysis, ferritin was found to be a strong predictor for forced vital capacity and total lung capacity. Bronchial dilatation and areas of air trapping were the predominant CHRCT findings. Iron laden macrophages were demonstrated in 14 of 15 patients in BAL. A significant correlation between serum ferritin and DLco, forced vital capacity, total lung capacity, and the presence of iron laden macrophages in BAL indicates that iron plays a major role in the etiopathogenesis of these abnormalities.

Detection of Mycoplasma pneumoniae in children with lower respiratory tract infections

Mycoplasma pneumoniae is known to be a major cause of lower respiratory tract infections (LRTIs) in children. We studied 75 children who had been hospitalized for community-acquired LRTIs for the detection of M. pneumoniae by serological analysis and polymerase chain reaction (PCR) to amplify a 277-base pair region of 16S rDNA gene of M. pneumoniae applied to throat swab specimens. Serological and/or PCR positive results diagnosed M. pneumoniae infection in 23 (30.7%) patients.

Case report: Isolated unilateral pulmonary vein atresia diagnosed on 128-slice multidetector CT

Unilateral pulmonary venous atresia is an uncommon entity that is generally believed to be congenital. Most patients present in infancy or childhood with recurrent chest infections or hemoptysis. Pulmonary angiography is usually used for definitive diagnosis. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report two cases diagnosed using 128-slice CT angiography. On the CT angiography images both these cases demonstrated absent pulmonary veins on the affected side, with a small pulmonary artery and prominent bronchial or other systemic arterial supply.

Public Health Implications of Obstructive Sleep Apnea Burden

ObjectiveTo assess the implications of obstructive sleep apnea (OSA) burden among Indian children.MethodsMonteCarlo simulations were performed in order to estimate the number of OSA related obesity cases among Indian children (1–14 y of age) and the number of cases of stroke, coronary heart disease (CHD) and type 2 diabetes, considered as main adverse outcomes of OSA related childhood obesity, according to untreated and treated [adenotonsillectomy (AT) alone and AT associated to continuous positive airway pressure (CPAP)] pediatric OSA. Data used to perform MonteCarlo simulations were derived from a review about current literature exploring OSA related obesity.ResultsThe analysis on the number of adverse outcomes according to treated and untreated obesity related to OSA showed that treatments reduce the number of obesity cases, resulting in a great reduction of the amount of stroke, CHD and type 2 diabetes cases. However, the cost for treating adverse outcome was higher in patients treated for obesity related to OSA compared to those not receiving any treatment.ConclusionsThe reduction in the number of adverse outcomes due to treatment of obesity related OSA implicates the urgent need for public health policies in providing screening for OSA among children population: an early detection and a consequently prompt reaction to pediatric OSA could improve the burden of OSA related obesity.

Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads.

Megacystis, microcolon, intestinal hypoperistalsis is an uncommon condition presenting in neonatal age with features of intestinal obstruction and bladder evacuation abnormalities. We present here an infant girl with the diagnosis consistent with this entity.

Pulmonary Diseases and Corticosteroids

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, á-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.

Acute renal failure in children in a tertiary care center

In this retrospective study, records of all patients aged one month to 12 years who presented with acute renal failure (ARF) between May 2005 and August 2010 were retrieved. Clinical details, biochemistry, need for renal replacement therapy (RRT), cause of ARF and outcome at discharge were recorded. During this period, 230 children presented with ARF; their median age at presentation was 30 months (range: five-144 months); 120 (52.2%) were males. The causes of ARF were acute tubular necrosis (ATN) in 121 (52.6%), glomerular disorders in 5.7%, structural anomalies of the urinary tract in 9.6% and hemolytic uremic syndrome in 27 (11.7%). The mean duration of hospital stay was 17.8 ± 7.6 days. RRT was required for 54 patients (23.6%); peritoneal dialysis in 49 and hemodialysis in five patients. Complete recovery was noted in 99 study patients (43.2%) and sequelae remained in 84 patients (36.7%). Forty-six patients (20.1%) with ARF died. ATN secondary to septicemia was the most common cause of ARF in our study.