A 42-month-old male, first child of healthy non-consanguineous parents, diagnosed with bilateral hydronephrosis prenatal ultrasound and gastric dilatation.
In the early neonatal period, the only complaints were glossitis, globulous abdomen and the first meconium deposition on the fourth day of life, with absolute absence of stools.
An opaque enema showed microcolon and malrotation, which was corrected surgically.
A rectal biopsy was positive for neuronal enolase and ruled out vesicular pathology.
Parenteral nutrition was maintained at all times, with attempts of oral intake that finally tolerated at 2 months of life with a maximum volume of 7 ml/h.
The patient always had adequate diuresis, although he maintained creatinine levels in high limits of normality.
Later he underwent bilateral pyeloureterostomy improving his renal function.
At 20 days of life she developed catheter-related sepsis due to Staphylococcus epidermis, which was treated with teicoplanin and cefoxime with a good response9.
These data expand what has been published in 2004 by the Department of Neonatology and the Pediatric Surgery Department of the Central University Hospital of Asturias10.
Currently the patient has good general condition, cutaneous bilateral peristalsis, peristalsis and intestinal habit, relatively well-nourished and 96% acceptable and progressive development percentile 10 of weight and height, Waterlow index.
She received parenteral nutrition (PN) from the third day of life and currently on an outpatient basis, supplemented with continuous oral intakes, as long as tolerance to them began.
The composition of PN per kg of body weight per day is 1.8 g of amino acids, 8.6 g of glucose, 1.4 g of lipids, total mEq volume, 55.795 k 1.4 mEq, Ca 0.7, Cl 4.4, P 0.2 mEq.
PN is infused over 17 hours in steps of 40 ml/hour, with an increase and decrease in one hour, with rest time without HPN of 7 hours.
She received prophylactic treatment for bacterial overgrowth with metronidazole, in addition to trimethoprim, phenobarbital,, deoxycholic acid, ranitidine, vitamin D3 and intestinal iron against darbepoetin E.
Monthly, clinical and analytical control (complete biochemistry, renal function, blood count and acute phase reactants) and microbiological control of ostomy skin and Hickman catheter were performed.
She had episodes of abdominal obstruction, one of which, secondary to intestinal adhesions, required surgical intervention.
Complications related to PN were two: transient hepatic cytolysis, which remitted after normalizing the ratio of calories from carbohydrates to fats (60/40) (when the antibiotic treatment was normal alkaline phosphatase, alkaline phosphatase was refractory),
