Solitary Rectal Ulcer Syndrome: A Case Series.

A retrospective analysis of the clinical profile, endoscopic features and management of 22 children (age 18 months – 18 years) diagnosed as solitary rectal ulcer syndrome is presented. The majority (81.8%) were ≥8 years of age. Rectal bleeding was the presenting feature in all the children. Mucorrhea, constipation, tenesmus and rectal prolapse were observed in 77.3%, 63.6%, 59% and 13.6% children, respectively. Colonoscopy showed classical single rectal ulcer in 68.2% and multiple ulcers in 22.7%. Polypoidal and erosive lesions were documented in 4.5% each. The medical management comprised of bowel training and high fibre diet for all children. The other modalities included oral 5-amino salicylate (59%), sucralfate enema (4.5%) and rectal mesalamine in 9%. 64% children recovered and 13.6 % had recurrence of symptoms.

Primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy . We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.

Profile of viral hepatitis A in Chennai.

This retrospective study compares the clinical features, laboratory profile and complications of anti HAV IgM positive acute viral hepatitis A in 138 children between 1-15 year (1-5 year: n=31; 5-10 year: n=85; and 10-15year: n=22). We found that older children presented with HAV (hepatitis A virus) infection with more atypical manifestations (ascites and splenomegaly) and complications.

Immunogenicity and safety of live attenuated hepatitis A vaccine: a multicentric study.

OBJECTIVE: To evaluate immunogenicity and tolerability of single dose live attenuated injectable hepatitis A vaccine in four metropolitan cities of India. METHODS: Live attenuated hepatitis A vaccine was administered to 505 children aged 18 to 60 months in four centers across India. Immunogenicity of the vaccine was assessed by estimation of anti-HAV antibody titer at 6 weeks and 6 months following administration of the vaccine. Safety evaluation of the vaccine was also done during the visits. RESULTS: At 6 weeks, 480 subjects (95%) came for the follow-up and 411 (81.4%) subjects reported at the end of 6 months. The geometric mean titer (GMT) of anti-HAV antibody of the subjects who did not have the seroprotective titer at the baseline were assessed at 6 weeks and 6 months which was 81.04 mIU/ml and 150.66 mIU/ml respectively. At 6 weeks, 95.1 % seroconverted and at the end of 6 months, 97.9 % had seroconverted. Both solicited and unsolicited vaccine-induced local and systemic adverse events were insignificant at all the centers, except swelling and induration in a few. CONCLUSION: Live attenuated injectable hepatitis A vaccine was immunogenic and tolerable with minimal reactogenecity, in this study of single dose schedule. Safety profile was also satisfactory in the study population.

Evaluation of chronic abdominal pain--a test of "patience".

A 4 year old girl with chronic abdominal pain of 6 months duration was provisionally diagnosed as eosinophilic gastroenteritis based on endoscopic biopsy This case is reported for its rarity.

Gastrointestinal bleeding in a toddler secondary to chronic renal failure and hyper-gastrinemia.

A 3-year-old girl with H. pylori negative duodenal ulcer with hypergastrinemia secondary to chronic renal failure presenting with upper gastrointestinal bleed as the cardinal manifestation is unusual in toddlers and the case is presented for its rarity.

Primary Sjö-gren's syndrome presenting with distal, renal tubular acidosis and rhabdomyolysis.

Primary Sjögren's syndrome (PSS) is rare in India. Clinically manifest renal disease in PSS is uncommon and is usually an autoimmune tubulointerstitial nephritis presenting with distal renal tubular acidosis (dRTA) or a urinary concentrating defect. Hypokalemic paralysis due to dRTA in PSS is rare but well documented in medical literature. Rhabdomyolysis as a consequence of hypokalemia in PSS is exceptional. We report a case of PSS with dRTA and rhabdomyolysis causing prolonged respiratory failure and quadriparesis.

Crohn's disease.

Crohn's disease is a chronic inflammatory disease of the gastrointestinal tract and is an important cause of morbidity in children and adolescents. In India Crohn's disease (CD) was considered a rare disease, however, during the last 10 years CD in adults is being reported from several centers especially in Southern India. CD is characterized by transmural granulomatous inflammation involving any part of the gastrointestinal tract in a discontinuous manner. The peak incidence of Crohn's disease occurs during the adolescent and young adult years. The clinical presentation and complications are varied and several extraintestinal manifestations have been recognized. The understanding of the pathophysiology has opened new avenues in the management. The recognition of this problem in children and adolescents by pediatricians is necessary for proper diagnosis and management.

Crohn's disease presenting as palatal ulcer.

Crohn's disease (CD) in children younger than 5 years of age is termed as early onset inflammatory bowel disease (EO-IBD). We report a 4 yr 6 mo-old child with EO-IBD, who presented with palatal ulcer, an extra intestinal manifestation of Crohn's disease as the dominant feature.

Richner Hanhart syndrome.

Richner Hanhart syndrome is a rare inherited disorder involving the metabolism of tyrosine, a semi-essential amino acid and it should be considered in the differential diagnosis of a child presenting with ocular and skin lesions. We report a case of Richner Hanhart syndrome in a 19-month-old child, who presented with ocular and skin lesions.

Pediatric Crohn's disease in South India.

Crohn's disease is a chronic inflammatory bowel disorder characterized by discontinuous, transmural, granulomatous inflammation involving any location of the gastrointestinal tract. A retrospective analysis of 10 children diagnosed as Crohn's disease (CD) is presented from Chennai, South India. The children were between 5-15 years of age and majority had primary colonic involvement. Complications such as stricture and fistula were identified. These children were managed medically except one who underwent surgery.

Esophageal tuberculosis.

Esophageal tuberculosis is a rare clinical entity even in adults. Esophageal tuberculosis, can be either primary or secondary, the former is less common as compared to the latter. The authors present a 14-year-old boy, who presented with vomiting, cough, low-grade fever and anorexia for two months. He had a positive mantoux with history of contact to Tuberculosis. Upper GI scopy revealed an irregular ulcer in the mid esophagus and the biopsy was suggestive of tuberculosis. The CT scan of the chest showed consolidation left lower lobe with hilar and mediastinal adenopathy. He responded well to ATT.

Kikuchi's disease.

An 11-year-old girl presented with high grade, intermittent fever and cervical lymphadenopathy. She had multiple enlarged left cervical lymph nodes. The examination of other systems was normal. Workup for sepsis, malignancy and autoimmune disease were negative. VA-IgM for EBV was positive and histopathology of the lymph node was consistent with Kikuchi's disease.