Guidelines on Diagnosis and Management of Cow's Milk Protein Allergy.

JUSTIFICATION: Cow's milk protein allergy (CMPA) is increasingly being diagnosed in the West, while there is scant data on the subject from India. There is low awareness among pediatricians about its diagnosis and management; leading to improper diagnosis. PROCESS: A group of experts from the pediatric gastroenterology sub-specialty chapter of Indian Academy of Pediatrics (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition) met at Mumbai on 26 October, 2018 and discussed various issues relating to the subject. A broad consensus was reached and a writing committee was formed. They met again on 11 August, 2019 at Chennai for a detailed discussion. The statement was sent to the entire group by e-mail and their approval obtained. OBJECTIVE: To formulate a consensus statement enable proper diagnosis and management of Cow's milk protein allergy. RECOMMENDATIONS: Cow's milk protein allergy is most common in the first year of life. Gastrointestinal manifestations are usually non-IgE mediated and therefore skin prick test and specific IgE levels are not useful in diagnosis. Clinical response to elimination diet followed by a positive oral food challenge is diagnostic. In patients with only gastrointestinal manifestations, sigmoidoscopy and rectal biopsy may be considered as an alternative. Management involves strict avoidance of all forms of bovine milk protein. For infants who are artificially fed, an extensively hydrolyzed formula is the first choice. Soy formula is an alternative in those above six months of age. Since most infants outgrow the allergy, elimination diet is only for a limited period and re-evaluation should be done periodically.

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India.

Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little role of serum copper and penicillamine challenge test in the diagnostic algorithm. A new scoring system - Modified Leipzig score has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation and penicillamine challenge test have been removed from the scoring system. Differences in pharmacological approach to neurological and hepatic disease and global monitoring scales have been included. Rising bilirubin and worsening encephalopathy are suggested as indicators predicting need for liver transplant but need to be validated. The clinical practice guidelines provide recommendations for a comprehensive management of WD which will be of value to all specialties.

Preduodenal portal vein in association with midgut malrotation and duodenal web-triple anomaly?

Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause duodenal obstruction or may coexist with other anomalies. We report a neonate who presented with duodenal obstruction and was found out to have 3 coexisting anomalies, each of which can lead to duodenal obstruction independently-PDPV, midgut malrotation, and duodenal web. A duodenoduodenostomy and a Ladd procedure were done, and the child recovered uneventfully. The mechanism of obstruction, interesting metabolic aberrations observed, outcome, and relevant literature are presented.

Antenatally diagnosed ovarian cyst with torsion managed laparoscopically.

Ovarian cyst are the most common intra-abdominal cyst in female neonate. With the help of ultrasound one can make an antenatal diagnosis. We present one such neonate, she was managed by laparoscopic excision. We conclude that neonatal laparoscopy is technically feasible for management of such cysts.

Symptomatic hepatic hemangioendothelioma in a newborn.

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.

Bilateral communicating bronchopulmonary foregut malformations in a child.

Communicating bronchopulmonary foregut malformations are rare anomalies. The complex anatomy requires innovative surgical techniques. We report a child with bilateral sequestrations communicating with the lower esophagus. The sequestrations were excised through a single thoracotomy incision and the esophagus was repaired. Postoperatively the child has remained asymptomatic.