Incidental Finding of Ebstein's Anomaly in an Adolescent with an Upper Respiratory Infection: A Case Report.

Ebstein's anomaly is a rare congenital heart disorder and has a varied clinical course, with detection as late as the seventh decade. We hereby describe an 11-year-old child in whom Ebstein's anomaly was diagnosed. The most common presentation in early second decade is due to an arrhythmia; however, the present case report is about a patient who presented with acute congestive heart failure due to decompensation from an acute upper respiratory tract infection, which is a rather uncommon presentation.

Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.

Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta.

Recurrent meningitis with upper airway obstruction in a child: frontonasal encephalocele- a case report.

Nasal encephalocele are rare congenital anomalies; these benign masses may be confused with nasal dermoids, hemangiomas, nasal gliomas and anterior skull base masses. These lesions have concomitant defects in the anterior cranial fossa thus this potential communication can cause recurrent episodes of meningitis and/or difficulty in breathing and cosmetic anomalies. We bring a case of a 6-year-old child who presented to the clinic with multiple episodes of meningitis which was associated with nasal discharge. The imaging studies and nasal fluid analysis confirmed it as cerebrospinal fluid; subsequently imaging findings concluded it as frontonasal encephalocele which was later resected and patient showed improvement.

Congential lumbar hernia with malrotation of left kidney and hydronephrosis in an infant: a rare presentation.

Congenital Lumbar hernia was previously known as Lumbocostovertebral syndrome. This syndrome consists of the presence of hemivertebrae, rib defects, abdominal wall anomalies and hypoplasia of the abdominal musculature. This case report describes a 40-hour-old infant with malrotation of left kidney and hydronephrosis in association with congenital lumbar hernia. Various congenital renal anomalies like renal pyelectasis, bilateral renal agenesis and pelviureteric junction obstruction, malascended kidneys, hypospadiasis have been reported in association with this disease. Malrotation has only been reported in adults so far. However ours is the first ever case in literature to report malrotation of kidney in association with this syndrome in neonatal age group. Treatment of this condition is done usually by mesh hernioplasty. Presence of this condition must prompt the surgeon to carry out all possible investigations to rule out various other congenital orthopedic, neurological, and urological anomalies.

Yokenella regensburgei infection in India mimicking enteric fever.

Yokenella regensburgei is an opportunistic human pathogen of the Enterobacteriaceae family rarely reported to cause human infections. Here, we present a case report of Y. regensburgei bacteraemia from India clinically resembling enteric fever in an apparently immunocompetent paediatric patient.

Bacteraemic Haemophilus influenzae type B pneumonia complicated with empyema--report of a case and review of literature.

A case of bacteraemic pneumonia complicated with pleural empyema due to Haemophilus influenzae type b is reported in a one-year old previously healthy child who had apparently no other associated medical condition. The organism was isolated from both the pleural fluid aspirate and blood of the patient with pneumonia. She was successfully treated with parenteral ampicillin and chloramphenicol alongwith intercostal chest tube drainage. The case is notable because it adds to the existing disease spectrum of invasive Hib diseases and brings awareness to the existing burden of the disease in Asia. In addition, it reflects the urgent need to include Hib vaccine in the current immunization program in India.

School absenteeism among children and its correlates: a predictive model for identifying absentees.

OBJECTIVES: To determine the magnitude of absenteeism and its correlates and to develop a model to predict absenteeism in school children. DESIGNS: A cross-sectional study. SETTING: three government schools in Delhi. PARTICIPANTS: 704 students, aged 10 to15 years. METHODS: students were registered and interviewed using a pre designed questionnaire. The frequency and causes of school absenteeism were ascertained by school records, leave applications and one months recall. The factors were subjected to univariate analysis and a stepwise multiple logistic regression analysis and a predictive model was developed. RESULTS: The average absenteeism of a student over 6 months was 14.3±10.2 days (95% CI 13.5 -15.0). 48% children absented themselves for more than two days per month on an average. The main factors associated with school absenteeism were younger age, male sex, increasing birth order, lower levels of parental education and income, school truancy, school phobia and family reasons. The discriminating ability of the predictive model developed was 92.4% CONCLUSIONS: it is possible to identify potential absentees in school children.

Urodynamic evaluation in primary enuresis: an investigative and treatment outcome correlation.

A prospective study was done in pediatric out-patient department of a tertiary care hospital to evaluate the role of urodynamics in the management of primary enuresis in the 5-14-year-old children and to compare the effectiveness of multidimensional behavioral therapy with pharmacological therapy. Hundred and nineteen children between 5-14 years with primary enuresis were evaluated clinically and investigated. Three patients with obvious organic causes were then excluded. The remaining patients were given either behavioral or pharmacological treatment on the basis of urodynamic assessment. Urodynamic abnormalities were seen in 80/116 (68.9%) patients namely uninhibited bladder contraction 50/116 (43.1%), small bladder capacity 20/116 (17.2%), large bladder capacity 4/116 (3.4%), decreased bladder compliance 3/116 (2.5%) and detrusor sphincter dyssenergia 3/116 (2.5%). Combination of abnormal micturition history stating daytime urgency or frequency or dysfunctional voiding symptoms like squatting and/or abnormal voiding charts could predict abnormal results of urodynamics correctly with sensitivity of 81% and specificity of 86.2%. Ultrasound identified only 38/80 enuretics with urodynamic abnormalities although it was 100% specific. Additionally one patient who was identified as having a small bladder capacity on voiding chart was seen to have mild pelvicalyceal dilatation on ultrasound and subsequently on urodynamic assessment was found to have Detrusor sphincter dyssenergia (DSD). Behavioral therapy as compared to drug therapy produced more complete remission (17/18 vs. 14/18) and lesser relapse rate (2/17 vs. 5/14) in monosymptomatic enuretics with normal urodynamics. In patients with urodynamic abnormality, response rates with behavioral therapy, imipramine, oxybutynin and flavoxate were 73.9% (CI 56-91.8%), 89.4% (CI 75.7-100%), 94.2% (CI 84.7-100%) and 89.4% (CI 75.7-100%), respectively. Specific drug therapy as per the urodynamic abnormality was significantly more effective 49/57 [86% (CI 77-95%)] vs 17/23 [73.9% (CI 56.1-91.9%)] at P < 0.05 than behavioral therapy in patients with underlying abnormal urodynamics. Micturition history and voiding chart can be used as screening tool for enuretics. Behavioral therapy should be the first line treatment for mono symptomatic and drug therapy for polysymptomatic enuretics. Urodynamic testing may be reserved for polysymptomatic enuretics with abnormal ultrasound or those who fail to respond to first line treatment.

Age-related clinical and microbiological characteristics of enteric fever in India.

A retrospective, hospital-based study at Safdarjang Hospital, India, was undertaken between January 1999 and December 2003 to estimate age-related epidemiological, clinical and microbiological characteristics in enteric fever cases. A total of 750 blood-culture-proven cases of enteric fever were studied. The majority of cases occurred in children aged 5-12 years and 24.8% of cases were in children up to 5 years of age. Salmonella serotypes showed an age-related predilection, with paratyphoid fever more common in adults. Classically-described clinical features of the disease were comparable among patients under and above 5 years of age. Hepatomegaly, anaemia and complications in general were more frequent in children up to 5 years of age. The antimicrobial resistance pattern, irrespective of Salmonella serotype, did not reveal a statistically significant difference across age groups for the different antibiotics tested. Multidrug resistance was seen only in Salmonella enterica serotype Typhi but not in S. Paratyphi A isolates. However, resistance to nalidixic acid was comparable in both serotypes. Age-related differences of serotype isolation rates, clinical presentation and associated complications are noteworthy for better case management and policy planning. More epidemiological studies regarding reasons for age-related differential serotype patterns would enable and guide public health strategies to contain enteric fever in endemic locations.

Current perspectives of enteric fever: a hospital-based study from India.

UNLABELLED: The last two decades have seen a change in the pattern of enteric fever with the emergence of multidrug-resistant strains (MDRS), particularly strains resistant to nalidixic acid. AIM: The aim of the study was to undertake a retrospective analysis of blood culture-confirmed cases of enteric fever diagnosed at Safdarjang Hospital, New Delhi, India from January 2001 to December 2003. METHODS: The epidemiological details, clinical features, treatment outcome and antimicrobial resistance patterns were studied. RESULTS: Of 377 blood culture-positive cases, 80.6% were Salmonella typhi and 19.4% Salmonella paratyphi A; 21.7% were children aged under 5 years and 6.1% were under 2 years. A significant decline in MDRS was observed, from 21.9% in 2001 to 12.4% in 2003 (p=0.04). There was a significant increase in nalidixic acid-resistant Salmonella (NARS) from 56.9% in 2001 to 88.9% in 2003 (p=0.0001). Complete resistance to ciprofloxacin (MIC>4 microg/ml) was detected in only two isolates, both Salmonella paratyphi A. Minimal inhibitory concentrations (MICs) of ciprofloxacin for NARS were increased (0.125-0.5 microg/ml) but were within National Committee for Clinical Laboratory Standards susceptibility ranges. NARS had a significantly longer fever defervescence time (7.7 vs 4.7 days, p<0.001) and hospital stay (12.1 vs 8.2 days, p<0.001), and higher rates of complications (55.5% vs 24.0%, p=0.014) and mortality than nalidixic acid-sensitive Salmonella (NASS). The rate of isolation of MDRS was higher in NARS than NASS (18.8% vs 7.3%, p=0.013). CONCLUSION: The high rate of occurrence of enteric fever in children <5 years and also of infections caused by Salmonella paratyphi A in India calls for critical re-assessment of vaccination strategy. Nalidixic acid resistance and rising MICs of fluoroquinolones in Salmonella spp pose a new global threat requiring debate on the optimum treatment of enteric fever.

Idiopathic myelofibrosis with generalized periostitis in a 4-year-old girl.

Idiopathic myelofibrosis, a chronic myeloproliferative disorder of unknown origin, is characterized by splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, teardrop erythrocytes, and myelofibrosis. It is a rare disorder in childhood. The authors describe a 4-year-old girl with features consistent with idiopathic myelofibrosis, who also had generalized solid laminated periosteal reaction involving all long bones. The presence of thrombocytopenia at the onset and lack of leukocytosis were in contrast to the reported features seen in children. Recent case reports describe a relatively indolent course in children. Spontaneous remissions have also been described in pediatric cases. The fulminant course of this patient without any features of malignant transformation was noteworthy in this regard.

Neonatal lupus erythematosus.

We describe case report of a 45 days old male baby with neonatal lupus erythematosus, who presented with 3rd degree congenital heart block and depigmented skin lesions on face and upper part of body. Diagnosis of the baby was confirmed by anti nuclear levels and skin biopsy.

Serum prolactin in seizure disorders.

This study aimed to determine the post-ictal prolactin (PL) response in different types of seizures and seizure-like events in children, and correlate with the post-ictal duration. Patients were divided into group I (generalized tonic-clonic seizures, complex partial seizures or simple partial seizures), group II (febrile convulsions) and group III (conditions mimicking seizures). Group IV consisted of 25 controls. Blood was collected within 2 hours of the seizure and PL levels assayed. PL levels were significantly high only within group I; highest and baseline levels were attained within 10 minutes and by 100 minutes respectively. The sensitivity and specificity of elevated PL for epileptic seizures were 64 percent and 98 percent respectively. It is concluded that a high prolactin level within 100 minutes of a seizure is suggestive that a generalized or complex partial seizure has occurred.

Congenital Langerhans cell histiocytosis: the self-healing variety.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare variant of Langerhans cell histiocytosis, presenting at birth or in the neonatal period with cutaneous lesions that involute spontaneously. Affected infants are otherwise well with no systemic illness. A case of CSHLCH, probably the first case report from India, is described. The patient presented on the third day of life with multiple papulonodular lesions over the body, with no systemic involvement. The lesions spontaneously regressed by 6 months of age, with no evidence of relapse at 1 year of age. Although CSHLCH is a benign and self-limited condition, long-term follow-up for evidence of relapse is emphasized.