Protein-losing enteropathy following the Fontan operation: resolution with prednisone therapy.
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Biomedical subjects
Publications and source records attributed to J Snyder.
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Recently, the role of feeding as treatment of acute diarrhoea has received increasing attention. To assess the efficacy of early feeding in acute diarrhoea, we conducted a randomised, clinical trial of a traditional legume-based weaning diet khitchri in boys 9 to 48 months old with moderate to severe dehydration. Khitchri is composed of rice and lentils cooked with cottonseed oil. Children were randomly allocated to 2 groups: group A received only WHO ORS but no food for the first 24 hours and then khitchri and half-strength cow's milk formula freely; group B received khitchri and the half-strength formula in addition to ORS after the initial rehydration period of 4 to 6 hours. The mean period of evaluation was 3 days. 69 patients were admitted into the study, 33 in group A and 36 in group B. The initial mean purging rate for the children was greater than 200 g/kg/day. Six children did not complete the study because they developed intercurrent infections or were removed by their parents for non-medical reasons. Of the 63 patients who were evaluated, 44 (70%) were successfully treated, 21 in group A and 23 in group B. There were no significant differences in the 2 groups in mean stool output, number of stools, or weight gain, although a trend toward earlier improvement was seen in group B. These data indicate that early feeding of khitchri and WHO/ORS may be as well tolerated as WHO/ORS alone in the first 24 hours treatment of severe acute diarrhoea in young children.
Failure-to-thrive (FTT) is a chronic symptom accounting for 1% of all patients admitted to pediatric hospitals. FTT, which is traditionally attributed to organic (OFTT) and/or nonorganic (NFTT) causes, results in undernutrition. Undernutrition has potentially serious effects on child development, behavior, and cognitive skills. We undertook a study of children with FTT to determine whether multidisciplinary team treatment resulted in improved weight gain compared with children treated in a primary care setting. Fifty-three children with NFTT referred to our outpatient FTT consultative clinic and 107 children with NFTT identified as comparison subjects from our primary care clinic (PCC) were enrolled in the study. Growth outcomes over a 6-month follow-up were analyzed using growth quotient (GQ) analysis. Children followed in the multidisciplinary team clinic grew better (GQ = 1.75 +/- 0.39 SD) than did children in the PCC (GQ = 1.18 +/- 0.42 SD, p less than .001). The use of a multidisciplinary team offers special advantages in the rapid correction of undernutrition in children with NFTT.
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Spondylothoracic dysplasia, also known as short-trunk dwarfism or Jarcho-Levin syndrome, is a fatal autosomal recessive disorder characterized by vertebral and spinal defects with a short thorax. Until recently, in utero diagnosis could only be made radiographically. Sonographic criteria for antenatal diagnosis are discussed in conjunction with a review of the literature.
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Failure-to-thrive (FTT) is categorized as organic (OFTT) or nonorganic (NFTT). Traditionally, it has been taught that children with OFTT are unable to grow well in spite of adequate care (calories, nurturance, medical supervision), whereas NFTT children will grow well when given adequate care. We undertook this study to determine whether NFTT and OFTT children could grow at similar rates when treated by a specialized multidisciplinary team that provided concrete, individualized therapies including psychosocial support, medical care, and hypercaloric diets. Eighty-six children were enrolled on referral to our outpatient FTT consultative clinic; 64 had NFTT and 22 had OFTT. Growth quotient analysis was used to determine growth outcomes over a 6-month follow-up period. Growth at a rate paralleling the growth curve produces a GQ equal to 1. Growth 2 times as rapid as expected gives a GQ = 2. Children in both groups grew extremely well. For NFTT, GQ = 1.67 +/- 0.56 (SD); for OFTT, GQ = 1.81 +/- 0.37 (SD), p = NS. OFTT children grew just as well as NFTT children when given adequate calories for catch-up growth. Our data indicate that weight gain alone cannot reliably differentiate OFTT from NFTT, as has been traditionally taught. They suggest that we make every attempt to maximize caloric intake in FTT children. They also suggest that a multidisciplinary team consisting of a pediatrician, child psychiatrist, nutritionist, nurse clinician, and social worker may be successful in managing FTT children.
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A 59-year-old man developed Cushing's syndrome with massive bilateral adrenal macronodular hyperplasia. Plasma ACTH levels were undetectable both peripherally and in the inferior petrosal sinus. Computed tomography scans of his pituitary were normal. Hypercortisolism was not suppressed by high doses of dexamethasone. His adrenal cells were successfully isolated and grown on bovine corneal extracellular matrix. The cultured cells displayed strikingly rapid growth and hypersecretion of cortisol during incubation with control serum. Addition of the patient's serum to cultured fetal adrenal cells did not accelerate their growth. This was the first experience with our in vitro system in this rare clinical condition. The techniques described here may be used for future in vitro adrenal studies. These in vivo and in vitro data indicate that this patient's bilateral adrenal hyperfunction and growth were independent of ACTH.
Morphometric analysis of liver ultrastructure in 14 children with Reye's syndrome (RS) of varying morphologic severity was compared with that of 6 children with normal livers. Results showed reduced numbers of enlarged mitochondria in RS. Multivariant analysis identified correlations between increased mitochondrial size, decreased mitochondrial number, and severity of neurologic disease (stage). A disproportionate increase in mitochondrial area and perimeter in the RS cases with the most depressed mitochondrial number distinguished the 4 children with residual neurologic damage or death. Serum salicylate concentrations were negatively correlated with severity of morphologic alteration. Two cases of non-RS salicylate toxicity showed normal or near-normal mitochondrial size and number. In fatty liver from an autopsy specimen from a child, a child with carnitine deficiency, and a child on therapy for dermatomyositis, mitochondrial numbers were also normal. Decreased mitochondrial numbers are characteristic of RS and imply a pathophysiologic mechanism, possibly related to impaired mitochondrial replication. Synergism with other forms of mitochondrial injury, such as salicylates, hypoglycemia, or shock may influence clinical severity, progression, and outcome.
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The six (racemic or meso) isomers of 3,4-dimethyl-2,5-bis(3,4-dimethoxyphenyl)tetrahydrofuran and four corresponding desmethyl analogues were prepared and assayed as inhibitors of platelet activating factor (PAF) receptor binding to rabbit platelet plasma membranes. The inhibition by these isomers is stereodependent and varies with the gross shape of the molecules as determined by the molecular mechanics program MM2. The most potent PAF antagonist in this group of compounds is trans-2,5-bis(3,4,5-trimethoxyphenyl)tetrahydrofuran (L-652,731, 14) with an IC50 of 0.02 microM.
Five experiments examined changes in the processing of a text across reading encounters. Experiment 1 showed that reading speed increased systematically across encounters, with no loss in the extensiveness of analyses of the printed text, as indicated by the ability to detect nonword errors embedded within that passage. Experiment 2 replicated this improved reading fluency with experience and showed that it occurred even with typescript changes across trials, thus indicating that a primed visual operations explanation cannot account for the effect. The third and fourth experiments then extended the study of the familiarity effect to higher level processing, as indicated by the detection of word errors. Familiarity facilitated the detection of these violations at the syntactic-semantic levels. Finally, Experiment 5 showed that these higher level violations continued to be well detected over a series of reading encounters with the same text. The results indicate that prior experience improves reading speed, with no attenuation of analysis of the printed words or of the passage's message.
Research on social interaction has consistently documented the existence of mutual interdependencies between the behavior of 1 person and reactions to that behavior by others in the social environment. The concept of social reinforcement, although often used to explain acquisition and change in interaction patterns defined by such interdependencies, is difficult to apply to interaction observed in natural settings. On the basis of extended observation of the interaction of 2 mother-child dyads, the procedures needed to assess the effects of naturally occurring consequences on interactional patterns are detailed in this study. Reliable mother action-child reaction patterns were first identified, and the effect of maternal consequences for those patterns on the probability of their subsequent occurrence was assessed. Positive consequences were associated with increases, and negative consequences with decreases, in the probability of a child reacting to the next occurrence of the maternal action when compared to the base-rate probability of that action-reaction pattern. Thus consequences affect momentary shifts around the baseline probability of interactional patterns. Positive consequences were also associated with short-term increases, and negative consequences with short-term decreases, in the base-rate probability of interactional patterns.
Five pregnant Southeast Asian women presenting during a 14-month period with microcytic anemia, preeclampsia, and size-date discrepancies were all ultimately diagnosed as carrying fetuses with homozygous alpha-thalassemia hydrops fetalis. The perinatal complications of this hemoglobin disorder are unique to persons of this ethnic background and include uniform fatality for the affected infant, maternal preeclamptic morbidity, and retained placenta. In this report the obstetric ultrasound findings are presented and the clinical manifestations are discussed, with recommendations made to reduce this emerging public health problem in the United States.
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