Congenital B-cell Acute Lymphoblastic Leukemia with Congenital Rubella Infection.

Background: Congenital B-cell Acute lymphoblastic leukemia (ALL) is a rare malignancy.

Characteristics: A newborn infant presented with purpuric spots and ecchymotic patches, blueberry muffin rash, depressed neonatal reflexes, respiratory distress and hepatosplenomegaly. Peripheral smear revealed atypical blast cells.

Scrub typhus causing neonatal hepatitis with acute liver failure-A case series.

Neonatal hepatitis with acute liver failure due to varied etiology including various infections is reported in the past. Scrub typhus as a cause of neonatal hepatitis has rarely been reported in literature. A high index of clinical suspicion is required for early diagnosis and timely treatment.

Lactate clearance as the predictor of outcome in pediatric septic shock.

Context: Septic shock can rapidly evolve into multiple system organ failure and death. In the recent years, hyperlactatemia has been found to be a risk factor for mortality in critically ill adults.

Aims: To evaluate the predictive value of lactate clearance and to determine the optimal cut-off value for predicting outcome in children with septic shock.

Perioperative Considerations in a Patient with Hemophilia A: A Case Report and Review of Literature.

Classic hemophilia or hemophilia A is a congenital bleeding diathesis in which the affected individual may present with spontaneous hemorrhage or persistent bleeding even after minor trauma. Knowledge about the disease process, multidisciplinary team approach, and timely management can lead to favorable outcome in these patients. We report management of a child with hemophilia A for suturing of lacerated upper lip mucosa following trauma.

Early Infant Feeding Practices May Influence the Onset of Symptomatic Celiac Disease.

Purpose: To study whether breastfeeding and breastfeeding status during gluten introduction influences the age at diagnosis of celiac disease (CD). In addition to study, whether the timing of gluten introduction influences the age at diagnosis of CD.

Methods: It was a hospital based observational study.

Predictors of Compliance to Gluten-Free Diet in Children with Celiac Disease.

Aim. To identify the predictors of compliance to gluten free diet in children with celiac disease. Methods.

Lipoma arborescens.

Lipoma arborescens is a very uncommon condition of the synovial joints and bursae. Although the etiology is unknown, lipoma arborescens is characterized by hyperplasia of mature fat tissue in the subsynovial layer. The knee joint, especially the suprapatellar pouch, is the most frequent site of involvement.

Clinico-epidemiological profile of children hospitalized with dengue.

Objective: To study the clinico-epidemiological profile of children hospitalized with dengue illness.

Methods: Prospective study of children hospitalized with the diagnosis of dengue illness during from September through November 2006 at a tertiary care centre in Jaipur.

Results: A total of 948 children including 671 (70.

True duplicate bladder exstrophy: a rare lesion.

A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.

Ectopia cordis associated with Cantrell's pentalogy.

Cantrell's pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100,000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken.

Congenital bronchopulmonary malformations: a single-center experience and a review of literature.

Purpose: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature.

Materials And Methods: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.

Unusual variant of Cantrell's pentalogy?

A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance.

Single-stage repair of vestibular and perineal fistulae without colostomy.

Purpose: This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute.

Methods: From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study.

Neonatal oral tumors: congenital epulis and epignathus.

Oroparyngeal tumors in neonates protruding from the mouth are extremely rare. They have the potential to cause respiratory distress, or even death of the child during the perinatal period owing to airway obstruction and may need a multidisciplinary team approach at the time of birth. Congenital epulis and epignathus are the 2 rare lesions usually presenting like this and clinical manifestations depend on their position and size.

Right lung agenesis.

Congenital pulmonary agenesis or aplasia is extremely rare. Although more than fifty percent of patients die before first five years of age, some individuals may remain asymptomatic throughout their life. A three-month-old female child with right pulmonary agenesis presented to us with severe respiratory distress.

Supernumerary testis: a case report and review of literature.

Supernumerary testis or polyorchidism is a rare anatomical and congenital anomaly, and approximately 100 cases have been reported in the literature so far. It is often associated with processus vaginalis anomalies and with increased risk of malignancy and infertility. We report a case and its management, having left-sided duplex testis and found incidentally during inguinal hernia repair, and review the literature.

Congenital urethral hypoplasia with urethral fistula without imperforate anus: report of two cases.

Two male children with ano/recto-urethral fistula without anorectal malformation were managed in our institute. These patients had variable clinical manifestations and associated anomalies including congenital urethral hypoplasia, renal agenesis and patent urachus. Both were treated successfully according to their status of disease-complexes.

Congenital microgastria with esophageal stenosis and diaphragmatic hernia.

A rare case of congenital microgastria in association with distal esophageal stenosis and left-sided congenital diaphragmatic hernia is reported. Other features included megaesophagus and asplenia. The probable causative factors are discussed.

Dengue shock syndrome in newborn: a case series.

Four cases of dengue shock syndrome were seen during an epidemic of dengue fever. Three cases recovered following appropriate management.

Rupture of the right hemidiaphragm due to blunt trauma in children: a diagnostic dilemma.

The diagnosis of right-sided diaphragmatic rupture (RDR) due to blunt abdominal trauma is often missed in the acute setting, especially in the absence of other thoracoabdominal injuries. We describe two such children. The problems associated with the diagnosis and management of RDR are discussed to emphasize the need to have a high index of suspicion for this entity.

Changing antibiotic sensitivity in enteric fever.

Emergence of drug resistance in enteric fever is a major concern for the clinician. All children hospitalized with enteric fever during the period 1 March to 31 May 2000 were studied prospectively for their clinical presentation and response to therapy, and an attempt was made to compare these results with the results of hospitalized children with enteric fever during a similar period in the years 1990 and 1995. The results revealed that there was a significant change in the response to antibiotic therapy as evidenced by significant resistance to ciprofloxacin (55.

Surgical emphysema--an unusual finding in spontaneous neonatal gastric perforation.

A neonate presented with extensive surgical emphysema of the abdominal wall and a spontaneous gastric perforation. This clinical presentation is very rare. Early diagnosis and treatment improve the clinical outcome.