Michael K. Farrell

Gastroesophageal reflux in infants: Relation to apnea

The temporal relationship between apnea and gastroesophageal reflux was examined in 14 infants with abnormal GER scores and histories of prolonged apnea. Simultaneous polysomnographic and intraesophageal pH recordings were performed for each infant. GER episodes were compared to control segments of the recording (without GER) for frequency and type of apnea. Apnea was equally likely to occur during the control segments as during the GER episodes. Brief obstructive apneic episodes were more common during the onset of GER episodes than the onset of control segments. GER duration appeared prolonged during sleep. GER and apnea were not temporally related in the majority of instances, and may be two manifestations of a more general developmental delay.

Grade I Reye's syndrome. A frequent cause of vomiting and liver dysfunction after varicella and upper-respiratory-tract infection.

In a one-year prospective study we assessed the incidence of Reyes syndrome in children presenting with the acute onset of vomiting after a prodromal upper-respiratory-tract infection or varicella, and with serum alanine or aspartate aminotransferase levels at least three times higher than normal, and a paucity of neurologic findings. Of 25 patients meeting the above criteria, 19 had liver biopsies yielding adequate tissue for diagnostic purposes. Biopsy specimens from 14 of these 19 patients (74 per cent) were diagnostic of Reyes syndrome, according to rigorous light-microscopical, histochemical, and ultrastructural criteria. None of the biopsy specimens contained evidence of other acute pathologic processes, including hepatitis. A wide spectrum of mitochondrial alterations existed at the ultrastructural level, ranging from mild to severe lesions that were indistinguishable from those seen in comatose patients with Reyes syndrome. Our findings suggest that the clinical complex of vomiting, hepatic dysfunction, and minimal neurologic impairment after varicella or an upper-respiratory-tract infection usually represents Reyes syndrome. This syndrome occurs more frequently than previously recognized.

The 13C-xylose breath test for the diagnosis of small bowel bacterial overgrowth in children

BACKGROUND We evaluated the clinical utility of the 13C-xylose breath test for the diagnosis of small bowel bacterial overgrowth in children. METHODS To determine the optimal dose of 13C-xylose, 29 healthy children, 3 to 12 years old, were randomly assigned to receive one of three doses of 13C-xylose (10, 25, or 50 mg). After an overnight fast, the oral dose of 13C-xylose was administered, and breath samples were collected every 30 minutes for 4 hours. Samples were analyzed for 13CO2 by gas chromatography with mass spectrometry. Using the 50 mg dose, we then performed nine breath tests with concurrent duodenal bacterial cultures in 6 children, 3 to 12 years old, with short-bowel syndrome (n = 2), immunodeficiency states (n = 1), and motility disorders (n = 3). RESULTS Excretion of 13CO2 in breath peaked at 2.5 hours in all three control groups. The 50-mg dose produced the highest median peak and the smallest range of 13CO2 excretion in breath within each time period. The time of peak 13CO2 excretion in breath varied among the diseased children; however, the six patients with small-bowel bacterial overgrowth (2 x 10(5)-3.5 x 10(5) gram negative rods) all had peak 13CO2 that exceeded the maximum breath 13CO2 level in breath of the control subjects at the corresponding time period (100% sensitivity). Of the three patients with negative cultures, two had negative breath test results and one had positive results (67% specificity). One subject had normalization of both duodenal culture and breath test results after antibiotic treatment of small-bowel bacterial overgrowth. CONCLUSIONS Our preliminary results suggest that with a dose of 50 mg 13C-xylose, breath test results reliably predict small-bowel bacterial overgrowth in susceptible children.

Epidemic influenza myopathy in Cincinnati in 1977.

A distinctive myopathy was observed in 24 children following influenza B infection. The abrupt onset of severe muscle pain and difficulty in walking began as the respiratory symptoms were waning. The lower extremities, particularly the gastrocnemius and soleus muscles, were involved preferentially. Nasopharyngeal cultures were positive for influenza B Hong Kong in 18 of these patients. Serum creatine phosphokinase levels were significantly elevated (mean 55.2 units) when compared to controls and nine patients with Reye syndrome. The cardiac muscle isoenzyme was detected in the serum of 17 myopathy patients. Serum glutamic oxaloacetic transaminase, but not serum glutamic pyruvic transaminase, was elevated compared to controls (P less than 0.01) but less (P less than 0.001) than the patients with Reye syndrome. Twelve patients underwent muscle biopsy; segmental rhabdomyolysis without inflammation was detected in nine patients. Myopathy is a complication of influenza infection that can be diagnosed by clinical, biochemical, and virologic examination.

Improved survival in biliary atresia patients in the present era of liver transplantation

Therapy for patients with biliary atresia (BA) has become controversial, with orthotopic liver transplantation (OLTx) suggested in place of portoenterostomy. This is based on the unpredictable success of portoenterostomy, and the increased difficulty of the OLTx procedure following prior extensive liver surgery. The survival rate reported here for infants transplanted after unsuccessful portoenterostomy does not support this approach. OLTx was undertaken in 37 patients when end-stage liver failure followed primary portoenterostomy. Recipient age ranged from 6 months to 14 years (median, 13 months), and weight ranged from 5 to 45 kg (median, 8 kg) at the time of OLTx. Reduced-size allografts were used as the primary allograft in 25 patients (23 left lobe), and 12 received whole-organ allografts. Retransplantation was required in 5 patients, each received a reduced-size allograft. There was no increased incidence of vascular complications, primary nonfunction, irreversible rejection, intestinal perforation, biliary complications, sepsis, or lymphoma comparing the BA patients with all other non-BA patients who had undergone OLTx (all P = .16). There was no statistically significant difference in mean operative blood loss between BA patients (EBL = 1.99 BV) and non-BA patients (1.50 BV) (P = .14). Actuarial survival for the series of BA patients was 89% at 1 year, and 80% at 2 years. Following the introduction of reduced-size allografts, donor organs were selected for use with a priority on donor stability. The actuarial survival for BA patients during this time has improved to 96% at 1 year, and 91% at 2 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Gastroesophageal Reflux in Infants

Gastroesophageal reflux, the spontaneous passage of acidic gastric contents from the stomach into the esophagus, occurs frequently throughout life. In a survey of presumably normal adults, 36 per c...

Intensive nutritional support and remedial surgical intervention for extreme short bowel syndrome.

Summary: Management of extreme short bowel syndrome (SBS) has changed dramatically over the last 20 years with notable improvements in survival and quality of life in patients with this syndrome. A review of our institutions medical records over a 12-year period (1980–1992) revealed 32 patients with <100 cm (range, 14–94; median, 40) of functional small bowel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long-segment Hirschsprungs disease (5 cases), midgut volvulus (5 cases) and gastroschisis (3 cases). The mean follow-up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patients without an ileocecal valve (ICV) and with <40 cm of small bowel was noted. The absence of an ICV, however, was associated with significantly prolonged total parenteral nutrition. Follow-up surgical procedures, including intestinal lengthening, tapering enteroplasty, Martins procedure, longitudinal myectomy-myotomy, and ostomy takedown, were performed in 20 of the patients (64%). Prolonged survival and normal development can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned secondary surgical intervention. These results are also seen in patients with extreme SBS (<40 cm residual small bowel length) and no ICV.

Bone mineral content in children with short bowel syndrome after discontinuation of parenteral nutrition

To determine whether children with short bowel syndrome had evidence of metabolic bone disease, total body bone mineral content was measured by dual-energy x-ray absorptiometry in 18 patients and 36 age-, sex-, and race-matched control subjects. Children with short bowel syndrome had decreased bone mineral content compared with control subjects; however, it was not significant when adjusted for differences in weight and height. Whether these children will have normal bone accretion throughout puberty is not known.

The spectrum of bile duct complications in pediatric liver transplantation

It has been noted that reduced-size liver transplants are associated with increased rates of biliary complications, and it has been suggested that some of these complications can be handled nonoperatively. In a 6-year period, 91 orthotopic liver transplants were performed in 77 children. The medical records were reviewed to analyze the effect of reduced-size grafts on the incidence of bile duct complications and to investigate the utility of interventional radiology techniques for treatment. Forty-two children received 47 whole-organ transplants, and 35 children received 44 reduced-size transplants. The median age and weight were greater for children receiving whole-organ transplants (age, 4.25 years; weight, 16 kg) than for those receiving reduced-size grafts (age, 1.0 year; weight, 8 kg). The overall incidence of bile duct complications was 19.5% (n = 15). The incidence was not different between the whole organ group (17%) and the reduced-size group (16%). Four of the children with bile duct complications had associated hepatic artery thrombosis, two of whom had another transplant. Complications included anastomotic stricture (n = 6), anastomotic leak (n = 5), intraparenchymal biloma (n = 3), and multiple strictures (n = 1). Twelve of 15 children presented within 3 months of transplantation. Six children had initial percutaneous drainage or placement of transanastomotic stents (external). Operative repair was eventually required for all 15 children, three of whom received a second transplant. There was a 40% incidence of cytomegalovirus infection involving the liver or extrahepatic bile ducts near the time of presentation.(ABSTRACT TRUNCATED AT 250 WORDS)

Multiple purpose central venous access in infants less than 1,000 grams

The use of central venous catheters in low birthweight infants has been associated with a high rate of infectious and mechanical-related complications. We reviewed our experience with multipurpose central venous catheters in infants less than 1,000 g to determine the rate of catheter-related sepsis and mechanical catheter malfunction. From October 1981 to August 1984, 20 infants (average weight 778 g) underwent placement of 22 central venous Broviac catheters. In addition to parenteral nutrition, antibiotics, aminophylline, and replacement fluids were infused. Total catheter days were 961, with an average of 44 days per catheter. Primary catheter sepsis occurred with two catheters (9%). Mechanical complications occurred with six catheters (exposed cuff, 1; catheter break, 2; catheter reposition, 1; catheter thrombosis, 1; dehiscence of cutdown site, 1). The incidence of catheter-related sepsis was acceptably low. The high incidence of mechanical catheter malfunction (6/22, 27%) resulted in minimal morbidity to the infant and could have been avoided by better operative technique, proper positioning intraoperatively, and meticulous care of the catheter post-operatively. We conclude that multipurpose long-term central venous access can be safely utilized with the Broviac catheter in infants less than 1,000 g.