Cytokinin from soluble RNA of Escherichia coli: 6-(3-methyl-2-butenylamino)-2-methylthio-9-beta-D-ribofuranosylpurine.

We have isolated a compound responsible for the cytokinin activity of soluble RNA from Escherichia coli. The structure, indicated as 6-(3-methyl-2-butenylamino)-2-methylthio-9-beta-D-ribofuranosylpurine, C(16)H(23)N(5)0(4)S, on the basis of low-and high-reso!ution mass spectrometry, was established by unequivocal synthesis. The mass spectra, chromatographic behavior, and ultraviolet spectra of the compounds from natural and synthetic sources were identical.

Primary nonspecific ileal ulceration as a cause of massive rectal bleeding.

A case of primary ulceration of the ileum in the absence of a Meckel's diverticulum or ectopic gastric tissue is described. Although this condition is infrequently observed, it should be considered as a potential cause of massive rectal bleeding, iron deficiency anemia of unknown cause, perforation, or partial small bowel obstruction. The diagnosis is normally made at surgery. Segmental resection with end-to-end anastomosis is the treatment of choice. Recurrence following surgery is rare.

Esophageal motor abnormalities in children and adolescents with scleroderma and mixed connective tissue disease.

To determine the frequency and nature of esophageal motor abnormalities in children and adolescents with scleroderma syndromes and mixed connective tissue disease, esophageal manometry was performed on seven patients with progressive systemic sclerosis, four patients with mixed connective tissue disease, and two patients with linear scleroderma. A total of 73% of patients with progressive systemic sclerosis and mixed connective tissue disease had symptoms of esophageal dysfunction. A significant association between the presence of Raynaud phenomenon and esophageal symptoms was noted. Esophageal motor abnormalities were detected in 73% of patients with progressive systemic sclerosis and mixed connective tissue disease; these abnormalities were characterized by decreased lower esophageal sphincter pressure and abnormal peristalsis in the distal two thirds of the esophageal body. They resemble those described among adults with progressive systemic sclerosis and mixed connective tissue disease but were not related to disease duration or to the presence of Raynaud phenomenon. Patients with linear scleroderma did not have esophageal symptoms and demonstrated only nonspecific motor abnormalities that did not worsen during several years of follow-up.

Sibling psychological status and style as related to the disease of their chronically ill brothers and sisters: implications for models of biopsychosocial interaction.

This study explores the relationship between disease type and disease activity, and the psychological status of siblings of chronically ill children. Closest-age siblings of children with Crohn's disease (CD) (n = 41) and ulcerative colitis (UC) (n = 24) were assessed for psychological disorder and psychological style, using reliable and standardized measures. The disease activity of the patients was also evaluated. Assessment occurred during outpatient visits to a pediatric gastroenterology division. Results indicated that CD siblings had more psychological disorder than UC siblings. This was not due to greater acute disease activity in the CD patients. However, as a group, siblings of the sickest CD patients displayed more "internalizing" behaviors, whereas siblings of the healthiest CD patients displayed more "externalizing" behaviors. UC siblings, who were psychologically healthier, displayed "externalizing" behaviors regardless of the patient's disease activity. These results are in accord with our previous findings on family functioning, which, together, yield a heuristic model representing the different patterns of biopsychosocial interaction for CD in contrast to UC.

Cholecystoappendicostomy in a child with Alagille syndrome.

The authors performed a partial biliary diversion on a 10-month-old child with Alagille syndrome using the appendix vermiformis as a conduit between the gallbladder and the abdominal wall skin. Three years later the patient is well, and his previously severe pruritus is controlled. The appendix has a lumen closer to that of the biliary tree, does not accumulate significant amounts of bile, and provides a smaller stoma. The small bowel is undisturbed, and there is less interference with a possible future liver transplant.

Pneumatic dilatation and operative treatment of achalasia in children.

The therapeutic approach to children with achalasia of the esophagus is controversial. Both pneumatic dilatation (PD) and Heller esophageal myotomy (EM) are considered effective, while bougienage has been discarded by most authorities. To determine the best place for each in the therapy of achalasia, 19 cases treated since 1964 were reviewed. Ages ranged from 9 months to 17 years (median 11 years), and duration of symptoms ranged from 4 months to 8 years (median 1 year). Three patients had symptoms from infancy. Two patients underwent a successful EM as their sole procedure. Two underwent bougienage as their initial therapy. Dysphagia recurred quickly and both required operation. Fifteen underwent PD under intravenous sedation with a Brown-McHardy dilator placed under fluoroscopy. Seven underwent a single dilatation; seven underwent two; and one underwent four. Relief of dysphagia was achieved in 11 patients, but four required surgery. The patients who experienced adequate relief with dilatation alone were clinically identical to those in whom it failed with respect to age, race, sex, symptom duration, and manometric data. Those who required EM following PD experienced only a brief period of relief following PD (median 1 month) compared with those who enjoyed lasting results (median 18 months). Three patients suffered prolonged chest pain or fever following PD, but without esophageal leakage and with full recovery. Two of eight operative patients developed late postoperative reflux. There were no deaths. Both PD and EM are safe and effective treatments for achalasia. Our results indicate that dilatation is the logical first therapeutic step, but rapid recurrence of symptoms may identify those patients who will require operative myotomy.

Microdiverticulitis: a rare but distinct cause of small bowel obstruction in early infancy.

During a 20-year period, eight term infants admitted to a large children's hospital for treatment of dehydration associated with diarrhea (6) and vomiting (2), and who ranged in age from 10 to 60 days at onset of symptoms and 18 to 75 days at presentation, developed distal small bowel obstruction following apparently successful rehydration. With the exception of a single infant who was dead on arrival, and another whose obstruction went unrecognized, all infants came to operation shortly after obstruction was confirmed. In each case, a severely inflamed area of distal ileum (7) or proximal colon (1) was found at autopsy (2) or operation (6) to be the cause; perforation was present in four of the cases. Resection of the diseased segments of intestine, and primary anastomosis, were performed in all six operated cases; reoperation was required in four of the six for leaks (3) and adhesions (1). All but two survived. Pathologically, the resected intestinal segments showed a unique pattern of injury: numerous punctate ulcers were apparent, which undermined the muscularis mucosae, without evidence of necrosis. Regenerating epithelium extended through these defects, resulting in the presence of glandular invaginations, which were surrounded by a brisk inflammatory response: hence the term "microdiverticulitis." We believe this lesion represents a beginning or furtive attempt at repair of severely inflamed, but viable intestine, and that it is a rare but true cause of small bowel obstruction in early infancy, separate and distinct from necrotizing enterocolitis.

Severe cardiomyopathy simulating hepatitis in adolescence.

Two adolescent patients referred for evaluation of severe "hepatitis" were found to have clinically unsuspected cardiac failure. Clinical profiles were characterized by anorexia, malaise, right upper quadrant pain, mild jaundice, initially predominantly indirect hyperbilirubinemia, profound elevations of transaminases, and prolonged prothrombin time. Left ventricular failure and low cardiac output were documented by clinical examination, echocardiography, and measurements of pulmonary capillary wedge pressure and cardiac index. Acute and chronic intrinsic liver disease was ruled out by appropriate tests. At autopsy, pathologic lesions of centrilobular necrosis characteristic of hepatic hypoxia were noted, and there was evidence of marked myocardial degeneration. These two cases emphasize the need for careful cardiac examination when evaluating acute hepatitis.

Recurrent abdominal pain: an update.

RAP is a broad descriptive term commonly used in pediatrics to define a heterogeneous group of patients who experience episodic attacks of abdominal pain over a period of at least 3 months. The majority of patients who seek medical attention for RAP have a functional disorder thought to be triggered by a motility or sensory disturbance of the GI tract provoked by a variety of physical and psychological stimuli. There are three distinct clinical presentations of functional abdominal pain in children and adolescents: periumbilical paroxysmal abdominal pain, dyspepsia, and irritable bowel. The medical history, physical examination, and selected laboratory, radiologic, and endoscopic evaluations allow a positive diagnosis of a functional disorder in each type of clinical presentation.

Gastroesophageal reflux in the pediatric patient.

Gastroesophageal reflux in the pediatric patient is a functional disorder with unique clinical presentations and management issues compared with the same disorder seen in adults. The goal of this article is to critically review our present knowledge regarding pediatric gastroesophageal reflux and to provide some perspective on how to evaluate and manage the individual patient.

Critical role of adrenal glands in precocious increase in jejunal sucrase activity following premature weaning in rats: negligible effect of food intake.

The effect of jejunal sucrase activity of premature weaning (PW) of rats is studied and compared with the effect of starvation. Within 24 hours after PW of rats on postnatal day 16 onto a high sucrose diet, there is a highly significant increase in sucrase activity as compared with that in nonseparated controls. During this time, food intake is minimal, the rats lose weight and there is an arrest of jejunal growth. Rats starved from day 16 onward exhibit the same increase of sucrase activity. Adrenalectomy on day 14, i.e. 2 days before PW, results in 33% mortality on the second postweaning day, i.e. on day 18, and in progressive loss of jejunal protein. Sucrase activity in adrenalectomized prematurely-weaned rats does not differ from the activity of intact suckling controls on day 18. Our experiments suggest that the increase in sucrase activity following PW is not related to food intake, but is primarily mediatd by the adrenal glands. Presented results stress the importance of the endocrine system in the intestinal "adaptive" response to PW in the rat. Endocrine factors should be considered in any evaluation of the effect of nutritional manipulation during the weaning period.

Chronic protracted diarrhea and jejunal atrophy in an infant. Cimetidine-associated stimulation of jejunal mucosal growth.

An infant with 21 months of chronic protracted diarrhea, associated with intestinal mucosal atrophy, decreased crypt mitotic activity, and anti-intestinal antibodies is reported. During a 4-month period, cimetidine was used in an attempt to stimulate mucosal growth. Thirty-minute postprandial serum gastrin levels rose significantly during cimetidine therapy (663 /+- 115 pg/ml, mean /+- SEM). Coincident with the cimetidine therapy, the jejunal mucosa showed progressive histologic improvement and the index of crypt mitotic activity (MI) steadily rose: pretreatment MI = 1.3 (mitoses/100 crypt cells); mid-study, 3.3; end of study, 4.5. There was a direct correlation between 30-min pp serum gastrin and MI (r = 0.989, P less than 0.005). The patient died in renal failure one month after cessation of cimetidine. At autopsy, the small bowel had returned to an atropic state. It is proposed that cimetidine may have influenced jejunal mucosal growth, possibly through meal-stimulated hypergastrinemia.

Demonstration of a difference in expression of maximal lactase and sucrase activity along the villus in the adult rat jejunum.

Lactase and sucrase are two disaccharidases that differ not only in their substrate specificity and developmental patterns, but also in their resistance to mucosal insult. In this experiment, we tested the hypothesis that there might be a dichotomy in expression of enzyme activity along the jejunal villuscrypt unit. Sectioning of the villus-crypt unit in a cryostat enabled direct comparison of the distribution of lactase and sucrase enzyme activities in the adult rat. There is a stepwise increase in mean lactase/sucrase ratio going from crypt to villus. The data indicate that unlike sucrase activity, which is expressed maximally in enterocytes along the entire villus, maximal lactase activity is not attained until midvillus. The delay in expression of maximal lactase activity might help to explain the vulnerability of this enzyme to acute mucosal insult such as occurs in viral gastroenteritis.

Bacterial peritonitis and sepsis presenting as acute gastrointestinal bleeding in patients with portal hypertension.

Four children with portal hypertension and ascites developed hematemesis, abdominal pain, and fever as the acute manifestations of bacterial peritonitis. Initial management in the emergency department was directed toward controlling the upper gastrointestinal hemorrhage, and antibiotic therapy was delayed in four of six episodes until ascitic fluid cultures grew Streptococcus pneumoniae. Gastrointestinal bleeding has not been previously reported as a presenting symptom of peritoneal infection. Our clinical experience emphasizes the need for antibiotic therapy during the initial management of children with ascites and hematemesis.

Successful treatment of achalasia in childhood by pneumatic dilatation.

Ten consecutive patients (ages 10 to 17) with achalasia of the esophagus diagnosed by radiographic, manometric, and endoscopic criteria were treated by forceful dilatations of the lower esophageal sphincter. A good to excellent response was seen in eight of the ten patients, manifested by disappearance of vomiting, improvement in dysphagia, and weight gain. A decrease in resting gastroesophageal sphincter pressure was documented in four patients tested. Short-term complications of fever or chest pain were seen following three of 18 procedures; however, barium swallow was negative for perforation and symptoms resolved spontaneously without treatment. Our findings suggest that pneumatic dilatation may produce similar results as surgical esophagomyotomy (Heller procedure) without the immediate operative morbidity, cost, and potential long-term effects.

Site of thyroxine-evoked decrease of jejunal lactase in the rat.

Localization of thyroid-mediated decrease of lactase activity along the villus-crypt unit in adult rat jejunum was studied 24 and 48 h after first injection of L-thyroxine (200 micrograms/100 g body wt) every 24 h. [3H]thymidine was also given at time of first thyroxine injection. Serum thyroid-stimulating hormone, food intake, and body weight were significantly decreased within 24 h. Total jejunal protein and villus-crypt height were unchanged during the time period studied. Lactase activity (expressed both as per tissue protein and per intestinal segment) was significantly decreased in jejunum and midjejunum within 24 h. Serial sectioning of the jejunal villus-crypt unit in a cryostat showed that the site of decrease in lactase activity at 24 h was in the apical villus and by 48 h extended along the entire height of the villus. Epithelial cell migration measured both by histoautoradiography and scintillation counting of [3H]thymidine in cryostat sections revealed no difference between control and thyroid-treated animals at both 24 and 48 h. The decrease in lactase activity at 24 h was in advance of the leading edge of radioactivity, indicating that the thyroid-evoked response occurred in mature enterocytes already on the villus.

Multiple congenital anomalies associated with infantile achalasia.

Achalasia is rare in the pediatric age group, particularly in the neonate and young infant. The symptoms are often nonspecific and may not even suggest a primary esophageal disorder. This is a report of two cases of achalasia seen in neonates, both of whom has other unusual and exceedingly uncommon congenital abnormalities.