Family Function in Pediatric Liver Transplant Recipients Residing In a Developing Country.

BACKGROUND: Pediatric liver transplantation (LT) can affect recipients' family function; however, inconsistent results between studies exist, and data from developing nations are sparse. We aimed to evaluate family function and identify factors associated with suboptimal function in pediatric LT recipients. METHODS: A cross-sectional study was performed at a teaching hospital in Bangkok, Thailand between May 2018 and December 2018. We included the families of children aged 2 to 18 years who underwent LT for at least 1 year. Chulalongkorn Family Inventory (CFI) was used to evaluate the family function in these children comparing with families of healthy children. Family function was interpreted from the total CFI score and characterized as dysfunctional, normal-functioned, and well-functioned. RESULTS: We enrolled families of 82 LT recipients and 72 healthy children. LT recipients had median age of 7.4 (interquartile range: 4.5-10.3) years. Eighteen children (22%) had a single parent, and at least one unemployed parent was reported in 25%. Most (96%) had well-functioned families, and none had a dysfunctional family. Furthermore, the total score was not significantly different between families of LT and healthy children (P = .95). LT families had a higher score in problem-solving (P < .01) and lower score in the affective involvement and general functioning dimension (P < .01 and .02, respectively). Among the LT children, postoperative bile leakage was associated with lower overall family function score. CONCLUSIONS: Even though most recipients had good family function, physicians should pay close attention to specific aspects of family function, especially in children with certain postoperative complications.

Gastrointestinal transit and anorectal manometry in children with colonic substance P deficiency.

BACKGROUND AND AIMS: Severe intractable constipation in children may be associated with a reduction of substance P (SP)- containing fibers in colonic circular muscle. The aim of this study was to characterize gastrointestinal transit (GIT), anorectal manometry (ARM) and electromyographic (EMG) changes in these children. METHODS: Seromuscular laparoscopic biopsies of the colon were obtained from 35 children with severe constipation. Immunofluorescent staining for SP and vasoactive intestinal peptide (VIP) were then performed on these specimens. The cohort of patients studied included a SP-deficient group (SPD, n = 25) who had reduced numbers of SP-immunoreactive nerve fibers. The other group consisted of patients with normal staining for both SP and VIP (SPN, n = 10). Gastrointestinal transit studies (gastric emptying, orocecal and colonic transit) suitable for analysis were available for 17 patients (SPD, n = 9 and SPN, n = 8). The colon was divided into segments and radioactivity counts in each segment were expressed as a percentage of the total colonic count at each time point (6, 24, 32 and 48 h). The geometric center (GC), ARM, EMG, clinical and demographic data characteristics of both groups of patients were compared. RESULTS: There were no differences in demographic data, gastric emptying, orocecal transit or geometric center of transit in the colon between the two patient groups. The ARM and EMG studies suggested that the SPN group have a higher mean threshold volume of balloon distension required to initiate a rectoanal inhibitory reflex, and a higher incidence of anismus; however, this did not reach statistical significance. CONCLUSIONS: These data suggest a trend that the SPN patients have a greater problem with obstructive defecation and abnormal rectal sensation than those with SPD. We were unable to confirm any defect in colonic transit in the SPD patients compared with the SPN group.

Extramedullary haematopoietic tumor producing small intestinal intussusception in a beta-thalassemia/hemoglobin E Thai boy: a case report.

The authors report a case of beta-thalassemia/hemoglobin E disease with extramedullary haematopoietic tumor which developed at the small intestine and caused intussusception. A 7 year-old boy with homozygous beta-thalassemia/hemoglobin E presented with recurrent abdominal pain. The abdominal ultrasonography showed ileo-ileal intussusception with a solid mass as the leading point. Resection of the ileal segment was performed. Pathological examination revealed an extramedullary haematopoietic tumor forming an intraluminal polypoid mass, being the leading point of the intussusception. Extramedullary haematopoiesis in the intestinal tract is rare. To our knowledge, this is the first case of extramedullary haematopoietic tumor that produced intussusception of the small intestine in a beta-thalassemia/hemoglobin E patient.

School absenteeism after upper gastrointestinal endoscopy in children.

BACKGROUND AND AIMS: To examine the functional impact of upper gastrointestinal endoscopy as a day procedure, particularly in relation to subsequent school attendance. METHODS: Symptoms and morbidities were prospectively recorded from school-aged children during observation in hospital and for 3 days at home after endoscopy by using a structured questionnaire. Reasons for school absence were identified. RESULTS: Sixty children (31 boys, 29 girls) were enrolled in the study (mean age 10.6 +/- 2.8 years, range 6.1-16.2 years). Following the procedure, symptoms were reported at home in 68.3% (same day), 56.7% (day 1) and 20% (day 2).The commonest symptoms were sore throat, tiredness and dizziness. Twenty-nine children (48.3%) did not attend school on the day following the procedure but most (26 of 29) had returned to school by the second day. The main reason for their absence was residual physical discomfort related to the procedure (55.2%). CONCLUSIONS: Persisting physical discomfort and school absenteeism are common following upper gastrointestinal endoscopy in children.

Neonatal hepatic propranolol elimination: studies in the isolated perfused neonatal sheep liver.

Using the isolated perfused neonatal sheep liver model, we examined the disposition of propranolol (n = 8, age 0.25-10 days) and compared our findings with our previous study from the perfused near-term fetal sheep liver (Ring JA, et al. 1995. Drug Metab Dispos 23:190-196). Within 45 min of dosage, perfusate propranolol levels had fallen by three orders of magnitude to be less than the limit of detection. Perfusate disappearance curves were monoexponential in six experiments and biexponential in two experiments. The mean shunt-corrected hepatic extraction ratio was 0.92 +/- 0.09, much greater than that seen in the fetal sheep liver (0.26 +/- 0.13, P < 0.0001) but still less than values in the adult sheep (0.97). At the conclusion of the perfusion, 4-hydroxypropranolol was the major metabolite present and 5-hydroxypropranolol and N-desisopropylpropranolol were minor metabolites. We conclude that the isolated perfused neonatal sheep liver is a useful model with which to study the maturation of neonatal hepatic drug oxidation. Our study shows that propranolol is rapidly eliminated by the neonatal liver to form several metabolites at rates far greater than in the fetal liver, but rates of elimination have not yet reached that reported in the adult sheep liver.

Conjugation of para-nitrophenol by the isolated perfused neonatal sheep liver.

We examined the metabolism of para-nitrophenol (PNP) in the isolated perfused neonatal sheep liver (n = 8, 0.25-11 days) and compared the findings with our previous data from the perfused near-term fetal sheep liver (Ring, J. A., et al. Drug Metab Dispos 1996, 24, 1378). A three-step dosage regimen was used (72, 144, and 288 micromol of PNP). At the end of each dosage phase, PNP had fallen below detectable levels, and 101 +/- 16% of the dose was accounted for as PNP conjugates. Elimination of PNP from perfusate varied with dose. Elimination was first order with the 72-micromol dose; with the 144-micromol dose, elimination was first order in four livers but Michaelis-Menten kinetics in the remaining four. With all the 288-micromol doses, elimination was Michaelis-Menten and gave the following biochemical parameters: K(m) = 255 +/- 138 microM (fetal = 14.7 microM, P < 0.01), V(max) = 515 +/- 285 nmol/min/g liver (fetal = 34.3 nmol/min/g liver, P < 0.01), and intrinsic hepatic clearance = 2.36 +/- 1.21 mL/min/g liver (fetal = 4.74 mL/min/g liver, P > 0. 05). The mean shunt-corrected hepatic extraction ratio of PNP was 0. 82 (range, 0.40-1.0) and strongly correlated with neonatal age (r = 0.90, P < 0.05). We conclude that PNP is highly extracted by the isolated perfused neonatal sheep liver at much higher efficiency than in the near-term fetal sheep, reflecting a maturation of conjugation that progresses further in the early neonatal period.

An unusual cause of rectal bleeding in a patient with cystic fibrosis.

Here we identify a previously unreported cause of rectal bleeding (juvenile polyposis) in a patient with cystic fibrosis (CF). We believe this patient most likely has two coexisting genetic diseases. It also raises many issues about organ transplantation in a patient with medical conditions that individually increase the risk of gastrointestinal malignancy and stresses the diagnostic value of endoscopy in CF patients with rectal bleeding.

Uptake and excretion of sodium taurocholate by the isolated perfused neonatal sheep liver.

We present a model for perfusion of the isolated perfused neonatal sheep liver which allows examination of drug disposition by the intact organ. We studied the disposition of sodium taurocholate (TC) in seven neonatal lambs (ages 2-11 days) and compared the results with earlier data from the perfused fetal sheep liver (Ring, J. A. et al. Biochem. Pharmacol. 1994, 48, 667-674). Measurements of perfusion pressure, oxygen consumption, lactate:pyruvate ratio, bile flow, and liver histology indicated that the preparation was both viable and stable over a 2 h period. [14C]-labeled TC was added to the reservoir by constant infusion (30 micromol/h) and the ductus venosus shunt quantitated by injection of [153Gd]-labeled microspheres. Shunt-corrected hepatic extraction ratio of TC was 0. 56 +/- 0.14 (fetal 0.23 +/- 0.16, p < 0.005) and clearance of TC was 0.92 +/- 0.35 mL/min/g liver (fetal 0.44 +/- 0.23 mL/min/g, p < 0. 01). We conclude that the isolated perfused neonatal sheep liver is a useful experimental model which will facilitate the study of the developmental physiology and pharmacology of the liver. There is considerable maturation of the biliary excretion of TC between the late fetal and early neonatal periods in the lamb.

The role of prostacyclin (PGI2) and thromboxane A2 (TXA2) in pathogenesis of dengue hemorrhagic fever (DHF).

In previous studies it has been demonstrated that the levels of plasma 6 keto-prostaglandin F1 alpha (6-K-PGF1), the stable metabolite of PGI2 were elevated in DHF patients during shock. In this study it is hypothesized that excessive PGI2 production plays a very important role in developing serious clinical manifestations of dengue shock syndrome (DSS) patients. In addition, an attempt was made to determine whether TXA2 has any significant role in such patients. Plasma 6-K-PGF1 and thromboxane B2 (TXB2), the stable metabolites of TXA2 were determined in 43 normal healthy children (NC) and 54 DHF patients without shock (DHF-N) and 33 DHF patients with shock (DHF-S). Subjects aged between 2 and 14 years. Plasma 6-K-PGF1 and TXB2 were measured by radioimmunoassay and the ratio of TXB2/6-K-PGF1 were also calculated. In 43 NC the values of plasma TXB2, 6-K-PGF1 and TXB2/6-K-PGF1 ratio were (mean +/- SE) 372.3 +/- 17.1, 150.1 +/- 2.4 and 2.52 +/- 0.12 pg/ml, respectively. In 54 DHF-N patients the corresponding values were 409.1 +/- 16.0, 278.4 +/- 11.6 and 1.54 +/- 0.06 pg/ml; whereas those in 33 DHF-S patients were 254.3 +/- 26.2, 349.1 +/- 20.5 and 0.757 +/- 0.073 pg/ml, respectively. Plasma 6-K-PGF1 levels of DHF-N and DHF-S patients were significantly greater than those in normal children (p < 0.001, p < 0.01 respectively). The plasma 6-K-PGF1 levels seem to be greater in DHF-S patients than in the DHF-N patients, however the difference in values were not statistically significant (p > 0.05). These findings indicate that plasma PGI2 level is significantly increased in DHF particularly during shock. Plasma TXB2 levels of DHF-N had no significant statistical difference from those of NC (p > 0.05); however, those in DHF-S patients were significantly lowered (p < 0.001) than those of NC and DHF-N patients. The findings suggest the important role of TXA2 to compensate for excessive PGI2 secretion in DHF patients. The failure or inadequate TXA2 production may eventually lead to shock. The ratios were significantly reduced in both DHF-N and DHF-S patients when compared to those of NC (p < 0.001 both). The ratio in DHF-S patients was also significantly lowered than that in DHF-N patients (p < 0.001). It is suggested that the imbalance between TXA2 and PGI2 production exists during DHF infection. The more reduction of plasma TXA2/PGI2 ratio leads to more overt and serious clinical manifestations of the disease.