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PubMed1977

A new approach to congenital posterolateral diaphragmatic hernia.

The theory is advanced that increased pulmonary vascular resistance, resulting in a state of fetal circulation, with right-to-left shunting through the ductus arteriosus, is the main reason that many patients do not survive after repair of a diaphragmatic hernia. Three patients are presented (who, by Raphaely's criteria, were destined for a fatal outcome) in whom the ductus was ligated, and vasodilator drugs were infused into the pulmonary artery. All three demonstrated definite improvement in oxygenation. Two expired after 6 days, one of whom was found at autopsy to have intestinal volvulus and gangrene, and the other multiple plumonary emboli. One case, so managed, survived. The suggestion is made that pulmonary hypoplasia is not the main reason for the high mortality rate after diaphragmatic hernia repair, and that additional laboratory and clinical investigation of the pulmonary circulation may lead to significant improvement in results.

Acetylcholine↗
PubMed2001

Do critically ill surgical neonates have increased energy expenditure?

BACKGROUND/PURPOSE: Adult metabolic studies suggest that critically ill patients have increased energy expenditures and thus require higher caloric allotments. To assess whether this is true in surgical neonates the authors utilized a validated, gas leak-independent, nonradioactive, isotopic technique to measure the energy expenditures of a stable postoperative group and a severely stressed cohort. METHODS: Eight (3.46+/-1.0 kg), hemodynamically stable, total parenteral nutrition (TPN)-fed, nonventilated, surgical neonates (5 with gastroschisis, 2 with intestinal atresia, and 1 with intestinal volvulus) were studied on postoperative day 15.5+/-11.9. These were compared with 10 (BW = 3.20+/-0.2 kg), TPN-fed, extracorporeal life support (ECLS)-dependent neonates, studied on day of life 7.0+/- 2.8. Energy expenditure was obtained using a primed, 3-hour infusion of NaH(13)CO(3'), breath (13)CO(2) enrichment determination by isotope ratio mass spectroscopy, and the application of a standard regression equation. Interleukin (IL)-6 levels and C-reactive protein (CRP) concentrations were measured to assess metabolic stress. Comparisons between groups were made using 2 sample Student's t tests. RESULTS: The mean energy expenditure was 53+/-5.1 kcal/kg/d (range, 45.6 to 59.8 kcal/kg/d) for the stable cohort and 55+/-20 kcal/kg/d (range, 32 to 79 kcal/kg/d) for the ECLS group (not significant, P =.83). The IL-6 and CRP levels were significantly higher in the ECLS group (29 +/-11.5 v 0.7+/-0.6 pg/mL [P<.001], and 31+/-22 v 0.6+/-1.3 mg/L [P<.001], respectively). Mortality rate was 0% for the stable postoperative patients and 30% for the ECLS group. CONCLUSIONS: Severely stressed surgical neonates, compared with controls, generally do not show increased energy expenditures as assessed by isotopic dilution methods. These data suggest that the routine administration of excess calories may not be warranted in critically ill surgical neonates and support the hypothesis that neonates obligately redirect energy, normally used for growth, to fuel the stress response. This is a US government work. There are no restrictions on its use.

C-Reactive Protein↗
PubMed2003

The role of intestinal transplantation in the management of babies with extensive gut resections.

BACKGROUND/PURPOSE: Modern neonatal care, surgical treatment, and total parenteral nutrition (TPN) have improved survival rate for babies with extensive gut resections. The authors examined the role of intestinal transplantation in the treatment of these patients. METHODS: The authors reviewed all pediatric intestinal transplants performed for short bowel syndrome at our center (70 transplants performed between Aug 1994 and Feb 2002). Factors affecting patient survival were analyzed. RESULTS: Older patient age at the time of transplant was a significant factor favorably affecting patient survival (P =.031). Trends toward better survival rates were observed in those transplants performed more recently (P =.063), in those patients with greater body weight (P =.084), in those not hospitalized at the time of transplant (P =.14), and in those without concomitant liver failure (P =.12). Three-year survival rate for patients greater than age 2 years and without liver failure was 90%. However, 32% of our recipients underwent transplant at age less than one year, and most in this group (75%) had concomitant liver failure. CONCLUSIONS: For babies with irreversible intestinal failure, intestinal transplantation is a life-saving option. Results, which have recently improved, are best when transplantation compliments more conservative surgical treatments and TPN. However, there is a subset of patients who have liver disease early requiring urgent transplant.

Age Factors↗
PubMed1983

Surgical implications of congenital chloride diarrhoea.

Congenital chloride diarrhoea (CCD) is an inherited inborn error of metabolism. Hydramnios, premature birth, no passage of meconium and a distended abdomen are the typical features of this disease in the neonate. Loss of Cl- from the intestines leads to urine-like diarrhoea, severe dehydration and alkalosis. Without treatment the mortality rate is high. The abdominal distension may erroneously be ascribed to an intestinal obstruction and thereby lead to unnecessary operations and delay of adequate treatment. There is an overrepresentation of intestinal volvulus in reported cases. Three of the five cases of CCD seen at our departments exemplify these surgical implications.

Chlorides↗
PubMed1995

Differential diagnosis of cyclic vomiting syndrome.

Vomiting is a nonspecific symptom and cyclic vomiting is a symptom complex that may result from a disorder of any major organ system. Children with cyclic vomiting syndrome (CVS) need careful review and investigation at their earliest presentations to exclude potentially lethal abnormalities such as intestinal volvulus, metabolic disorders, and neurologic space-occupying lesions. The range of abnormalities that may present with features consistent with CVS includes gastrointestinal obstructive, inflammatory and motility abnormalities, pancreatic disease, metabolic disease (particularly the amino acid-opathies, organic acidurias, fatty acid oxidation defects, and acute intermittent porphyria), renal disease, epilepsy, migraine, and psychiatric disorders. Careful history taking will usually provide clues to these uncommon problems, but all children should undergo baseline assessment of gastrointestinal morphology and screening tests for renal and metabolic disease.

Child↗
PubMed1978

Unusual abdominal complications of a suicidal overdose of analgesic and psychotropic drugs in an elderly patient.

A suicidal 67-year-old woman with manic-depressive psychosis took an overdose of asprin, amitriptyline and diazepam. The initial effects were pyrexia, tachycardia, hyperpnea, metabolic acidosis, electrocardiographic changes, hypoprothrombinemia, gastritis, and pancreatitis. Four to six weeks later, she was examined because of persistent abdominal pain with mausea, anorexia anemia, and possibly a malabsorption syndrome. An exploratory laparotomy was performed. The surgeon found several previous adhesions, a small intestinal volvulus, and a nodular pancreas. This suggested previous perforation of the small bowel from enteritis, causing a "blind-loop" syndrone. The invilved section of the small bowel was resected. With appropriate treatment, the patient is well three months after operation.

Aged↗
PubMedDate not supplied

Effect of intestinal resection on two juvenile horses with granulomatous enteritis.

Two horses were presented with lethargy, weight loss, anorexia, and swelling of the limbs and ventral body wall. One horse, a 12-month-old American Paso Fino colt, also had acute abdominal pain. The other horse, a seven-month-old Tennessee Walking Horse (TWH) filly passed diarrheic stools during the initial examination. Each horse had low serum protein, neutropenia, and a normal packed cell volume (3.2 g/dl, 1300 cells/ul, and 38%, respectively, for the colt, and 2.4 g/dl, 696 cells/ul, and 44%, respectively for the filly). After intravenously administering plasma, the colt's PCV dropped to 23%, and the filly's dropped to 30%. During exploratory surgery, 3.5 and 2.0 meters of thickened terminal small intestine were removed from the colt and filly respectively, and a jejunocecostomy performed. The results of histologic examination of resected intestine were consistent with a diagnosis of equine granulomatous enteritis (EGE). Both horses showed clinical improvement within two days after surgery. The colt developed a neutrophilia (20,500 cells/ul) within 24 hours of surgery. Serum protein concentrations remained stable and gradually elevated to normal or near normal values of 7.0 g/dl (colt) and 5.8 g/dl (filly) by two weeks. The colt was killed four months after surgery because of signs of abdominal pain. Postmortem examination revealed a small intestinal volvulus associated with an adhesion. The TWH filly remains clinically normal 13 months after surgery.

Animals↗
PubMed1983

[Biotin deficiency in the germ-free rat and propionic acidemia].

Biotin deficiency was induced in germ-free rats using three experimental protocols. The results showed the important role of biotin during gestation and suckling. The earlier the deprivation, the earlier the deficiency and the severer the symptoms. In this vitamin deficiency, symptoms occurred that were not observed in the control rats, such as the formation of an intestinal volvulus in rats ingesting an L-isoleucine-supplemented diet. The main biochemical anomalies characteristic of propionic acidemia (ketoacidosis and increased urinary elimination of propionic acid) due to propionyl CoA carboxylase deficiency in man were not observed in the rats that were deprived of biotin for 200 days even after a dietary load of L-isoleucine. Only a small urinary excretion of propionyl-glycine and tiglylglycine was observed. We observed a drop in enzymatic propionyl CoA carboxylase activity in the liver that was proportional to the severity of the vitamin deficiency.

Amino Acid Metabolism, Inborn Errors↗
PubMed2001

Ultrasonography and surgery of canine biliary diseases.

Findings of hepatic and gallbladder ultrasonography were analyzed in 12 dogs with gallbladder and/or extrahepatic biliary tract obstruction and compared with the results of exploratory laparotomy. Hepatic ultrasonography demonstrated normal liver in 2 dogs and hepatic abnormalities in 10 animals. The following ultrasonographic diagnoses were established compared to surgical findings: gallbladder obstruction caused by bile sludge (correct/incorrect: 1/2, surgical diagnosis: choleliths in one case), gallbladder obstruction caused by neoplasm (0/1, surgical diagnosis: mucocele), gallbladder and extrahepatic biliary tract obstruction due to choleliths (3/3), extrahepatic biliary tract obstruction caused by pancreatic mass (1/1) and small intestinal volvulus (1/1). Bile peritonitis caused by gallbladder rupture (4/4) was correctly diagnosed by ultrasound, aided with ultrasonographically-guided abdominocentesis and peritoneal fluid analysis. Rupture of the gallbladder should be suspected in the presence of a small, echogenic gallbladder or in the absence of the organ together with free abdominal fluid during ultrasonography. Laparotomy was correctly indicated by ultrasonography in all cases. However, the direct cause of obstruction could not be determined in 2 of the 12 dogs by ultrasonography alone.

Animals↗
PubMed1999

Perlman syndrome: four additional cases and review.

Perlman syndrome was first described in 1973 and comprises nephromegaly with renal dysplasia and Wilms tumor, macrosomia, cryptorchidism, and multiple facial anomalies. Polyhydramnios and hypoglycaemia are often found. Twelve children have been described from six different families. Five came from one family whose Yemenite Jewish parents were second cousins. Autosomal recessive inheritance has been suggested. Prognosis is severe with neonatal death in most children. We report on 4 new cases of Perlman syndrome from 3 families; all parents were non-consanguineous. Some of the observed manifestations have been described only once in this syndrome (cardiac defect, hepatic fibrosis with portoportal bridging, haemangioma) or never before (volvulus, intestinal atresia, and agenesis of the corpus callosum in 1 patient, a cleft palate in another). All children died within the first year. The 2 sibs were born prematurely with nephromegaly but without hamartomas or nephroblastomatosis. This is consistent with the hypothesis that dysplastic medullary parenchyma in preterm infants develops into nephroblastomatosis and hamartoma and eventually Wilms tumor.

Abnormalities, Multiple↗
PubMed2000

Method and complications of ileocutaneous anastomosis for collection of ileal digesta in neonatal pigs.

The purpose of our study was to develop a surgical method for collection of ileal digesta in neonatal (< 5 kg) pigs and to determine potential complications of the procedure. In 18 10-day-old pigs, we performed ileocutaneous anastomosis (ICA) via a right ventrolateral incision. The ICA was readily performed in these neonatal pigs; one pig died 24 hours after surgery because of intestinal volvulus. Pigs were monitored twice daily for development of post-operative complications. Ileal digesta were collected "free-catch" by using metabolism cages because attempts to use cannulas (diameter, 4 to 8 mm) and collection bags failed. To determine the effect of colon bypass on hydration, electrolytes, glucose, and serum enzyme activities, we collected serum biochemistry data before and 6 days after surgery. Changes in serum biochemical values included increased potassium, creatinine, total protein, albumin, and globulin and decreased ALP and glucose, but all values remained within normal ranges for neonatal pigs. ICA is tolerated well by neonatal pigs and is an easily learned and rapid technique for collection of ileal digesta. In addition, ICA is a useful alternative to "T-cannulas" and ileorectal anastomosis for nutrition research using neonatal pigs weighing < 5 kg.

Alkaline Phosphatase↗
PubMed2001

Determination of the acid-base status in 50 horses admitted with colic between December 1998 and May 1999.

The purpose of the present study was to investigate the acid-base status and the concentration of organic acids in horses with colic caused by various disorders. Blood samples were collected from 50 horses with colic and from 20 controls. No intravenous fluids had been given prior to sample collection. Identified causes of colic included gastric ulceration, small intestinal volvulus, cecal intussusception, cecal rupture, colonic impaction, left dorsal colon displacement, right dorsal colon displacement, colonic volvulus, colitis, peritonitis, and uterine torsion. Thirty-seven horses recovered from treatment of colic, 8 horses were euthanized, and 5 died. Most cases were not in severe metabolic acidosis. In previous studies, most horses presented for diagnosis and treatment of colic were in metabolic acidosis and in shock.

Acid-Base Equilibrium↗