Omphalocele
Occurs in 1 in 4000 to 7000 live births. Giant omphalocele refers to those defects in which the diameter exceeds 5cm and contains liver in the herniated sac or cases in which more than 75% of the liver is in the sac. Omphalocele is often accompanied by other congenital abnormalities
Defensins
small, cationic peptides that interact preferentially with phospholipids in microbial cell membranes, leading to formation of pores that lead to cell lysis. Secretion from Paneth cells is stimulated by bacteria and bacterial components, such as lipopolysaccharide and lipoteichoic acid
Complications of metoclopramide
Irritablity, drowsiness, oculogyric crisis, dystonic reaction, apnea and emesis
Probiotics
-Several meta-analyses have shown a significant reduction of severe NEC and decrease in all-cause mortality when comparing probiotics to placebo in preterm neonates
-studies that have separately analyzed propbiotic use by birth weight groups have not found a significant benefit of probiotic use for preventing NEC in ELBWs
Fistulas and anorectal malformations
Most patients with anorectal malformations have a fistulous connection either to the skin or the urinary tract. In female, the fistulous opening can be located either in the perineal body, as seen in an anterior anus, or more commonly in the vestibule of the vaginal vault outside the hymen (rectovestibular fistula). the presence of 2 orifices indicates a urogenital sinus
IVIG GALD
Pregnant woman may benefit from receiving IVIG 1g/kg at 14 weeks, 16 weeks and weekly from 18 weeks of gestation until the end of the pregnancy
GER vs GERD
GERD is differentiated from GER when there are signs or symptoms such as significant discomfort, poor weight gain, esophagitis, or airway symptoms
I-FABP
cytosolic water-soluble protein released from mature enterocytes when cell membrane integrity is compromised. I-FABP passes the glomerular filter and can be detected in the urine quickly. Urinary I-FABP levels are higher in neonates with NEC than in neonates without NEC. Levels may also correlate with severity of disease
rectouretral or rectovesical fistula managment
Best in staged approach - colostomy for enteric diversion, reconstruction, restoration of enteric continutiy via colostomy reversal
Organs affected in GALD
The liver is the most affected organ. others include pancreas, thyroid gland, heart and adrenal glands. The spleen, lymph nodes, and bone marrow are typically spared of iron deposition
Glucagon-like peptide 2
Antisecratory hormone that regulates intestinal transit. It is considered 1 of the most important hormones in intestinal adaptation. It is secreted by L cells int eh terminal ileum and colon and increases intestinal blood flow. In patients who have short bowel syndrome and retain their colon, glucagon-like peptide-2 levels are elevated
Cloacal Repair
A key principle is separation of the GI tract from the reproductive and urinary tracts. Posterior sagittal anorectoplasty is the standard approach for this repair. A laparoscopic approach has also been described. Vaginoscopy and cystoscopy are performed before reconstruction. The GI tract is separated and adequately mobilized down to the center of the sphincter complex. Although there also had been a strategy to separate the urinary and reproductive systems, this procedure was difficult and often led to postoperative complications.
Fasting and motility
For term neonates, intestinal motor activity during fasting is characterized by periods of quiescense (phase 1), irregular activity (phase 2), and regular phasic propagating activity (phase 3). However, preterm neonates have clusters of low-amplitude nonpropogating pressure waves in phase 3
Firmicutes
An increase in Proteobacteria and a decrease in Firmicutes have been seen before the onset of NEC. Fecal microbiota diversity is also reduced in NEC
Fryns syndrome
congenital diaphragmatic hernia, pulmonary hypoplasia (underdeveloped lungs), distinct facial features (coarse face, wide-set eyes, low-set ears), and distal limb hypoplasia (short fingers/toes).
cholestasis and micronutrients
reduce manganese and copper in PN and monitor levles because they can accumulate to toxic levles
Glutamine
primary stubstrate used by enterocytes. glutamine is a primitve growth factor for intestinal cells that signals via mitogen-activated protein kinases
LHR
Ratio of the contralateral area of the 2-dimensional lung to head circumference. LHR is the most widely validated marker of CDH severity. most reliable between 22 and 26 weeks. An O:E is used as LHR changes over time. An O:E less than 25% is assocated with less than 30% survival, whereas an o:E LHR greater than 46% si associated with more than 85% survival
Inter-alpha inhibitor protein
inter-alpha inhibitor proteins are serine protease inhibitors and acute-phase reactants that downregulate endogenous serine proteases that can contribute to epithelial cell necrosis and potentially to NEC. These proteins are generally found in high concentrations in human plasma but are decrease in conditions such as sepsis. Newborns with necrotizing enterocolitis have lower inter-alpha inhibitor protein levels than do those without NEC
Omphalocele with liver herniation
deliver by c-section
when does gald occur
after 18 weeks gestation, concurrent with onset of active transplacental IgG transfer, which starts during the 12th week.
Citrulline
produced by enterocytes. plasma citrulline concentrations are reduced in patients with short bowel syndrome. Changes in plasma concentrations of citrulline may reflect intestinal adaptation and potentially distinguish. Patients who have transient from those who have permanent intestinal failure
fat and tubing
tube feedings result in loss of lipids
