Q: Sir, I am writing to you from Bangladesh regarding a patient I have whose details are as follows: Raiana is a 19-months-old baby who has had several episodes of diarrhoea and dysentery from six months of age. Since October 2001, she has been admitted in a pediatric-based private hospital in Dhaka seven times for severe loose motions. On 2nd June, 2002 she was admitted due to severe watery diarrhoea, huge abdominal distension, vomiting, and electrolyte imbalance. During every hospital stay she was given fluid and electrolyte replacement. A course of IV Ciprofloxacin or Selexid was given according to the pathogen isolated from her stool culture. She is suffering from malnutrition and her condition is more complicated due to her refusal of food, intolerance to milk and other supplementary foods. Raiana is a full-term baby, delivered by C-section. Her antenatal and postnatal period was uneventful. Diet at home: she was given mashed green banana, rice, vegetable oil, chicken. She is a weak and emaciated baby with a naso-gastric tube for nutrition and medication. Weight is 8 kg (weight loss is 2 kg within last two months), temperature normal, pulse 120/min, (detailed physical findings are not available). She has altered mental status manifested by periods of spasticity and mild restlessness. Chest examination showed mild crepitation with prolonged expiration, which raises the suspicion of possible bronchopneumonia. Laboratory tests showed severe electrolyte imbalance (decreased blood levels of sodium, potassium and calcium) with hypoalbuminemia and severe anemia.

A:This is a fairly complex problem and I have given some thought to this and have also discussed this with some of my colleagues. Given the clinical situation, I feel that a congenital enzyme abnormality needs to be excluded and another possibility is a-beta-lipoproteinemia. This will require further investigations (which might include an intestinal biopsy and a peripheral blood smear examination) and treatment would depend upon the diagnosis, once confirmed. Facilities for this should be available at the Apollo Indraprastha Hospital, New Delhi. However, you would probably need to plan on staying in Delhi for some time while the investigations are planned and possibly even might need to admit your daughter to hospital for some time. You would be welcome to contact me at the above e-mail address, should you wish to arrange this.