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Biomedical subjects
Publications and source records attributed to E Blank.
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The study of gastrointestinal motility in children has evolved during the past 25 years. Miniaturization of tools for collecting data has created opportunities to study the maturation of gastrointestinal motility patterns in infants and complaints of abdominal pain, nausea, diarrhea, constipation and distention in children. Available methods, indications for testing, and data evaluation of pediatric esophageal, gastrointestinal, and colonic motility and manometric tests are discussed.
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An infant who presented with signs of duodenal atresia had proximal duodenal obstruction by upper gastrointestinal barium study, but had a small amount of air in the distal bowel. Exploration showed an annular pancreas and microperforation of a duodenal diaphragm. Prior to concluding that pancreaticobiliary duct anomalies are the path of air into the distal bowel in patients with duodenal atresia, microperforation of a duodenal diaphragm must be excluded.
The aim of the present study was to 1) characterize nicotine-induced peristalsis in the feline esophagus and 2) determine the site of action of nicotine. Experiments were done on ketamine-sedated cats. Esophageal contractions were measured using a multilumen catheter assembly system. After recording 1 degree and 2 degrees peristaltic sequences nicotine (50-100 micrograms/kg iv) was administered. Nicotine induced a peristaltic contraction through the esophageal striated and smooth muscle part of the esophagus, which was not associated with any mylohyoid electromyogram activity or pharyngeal response, although the upper esophageal sphincter did relax. Addition of either atropine (20-50 micrograms/kg iv) or hexamethonium (10-20 mg/kg iv), a peripherally acting nicotinic antagonist, did not affect the striated muscle portion of the nicotine-induced esophageal contractile response but antagonized the smooth muscle response. However, mecamylamine (0.5-1 mg/kg iv), a ganglionic antagonist that crosses the blood-brain barrier, abolished the esophageal response to nicotine. Succinylcholine (0.5-1 mg/kg iv) abolished the striated muscle response without affecting the nicotine-induced smooth muscle contractility. Finally, the nicotine-induced peristaltic sequence was abolished after bilateral cervical vagotomy. In conclusion, nicotine, administered peripherally, activates central brain stem mechanisms that mediate a peristaltic sequence through the feline esophagus.
A 32-wk-gestation female with type II achondrogenesis-hypochondrogenesis has been studied. The clinical features were typical, and radiographs revealed short ribs, hypoplastic ilia, absence of ossification of sacrum, pubis, ischia, tali, calcanei, and many vertebral bodies; the long bones were short with mild metaphyseal flaring. The femoral cylinder index was 6.3. Comparison with previous cases placed the patient toward the mild end of the achondrogenesis-hypochondrogenesis spectrum (Whitley-Gorlin prototype IV). Light microscopy revealed hypercellular cartilage with decreased matrix traversed by numerous fibrous vascular canals. The growth plate was markedly abnormal. Ultrastructural studies revealed prominently dilated rough endoplasmic reticulum containing a fine granular material with occasional fibrils in all chondrocytes. Immunohistologic studies indicated irregular large areas of cartilage matrix staining with monoclonal antibody to human type III collagen. The relative intensity of matrix staining for type II collagen appeared diminished. More striking, however, were intense focal accumulations of type II collagen within small rounded perinuclear structures of most chondrocytes but not other cell types. These results strongly suggest intracellular retention of type II collagen within vacuolar structures, probably within the dilated rough endoplasmic reticulum observed in all chondrocytes by electron microscopy (EM), and imply the presence of an abnormal, poorly secreted type II collagen molecule. Biochemical studies (see companion paper) suggest that this patient had a new dominant lethal disorder caused by a structural abnormality of type II collagen.
The lower esophageal sphincter (LES) exhibits cyclical phasic contractile activity synchronous with phases II and III of the gastric migrating motor complex. Motilin has been implicated in this process, although the exact mechanism is unknown. The effect of motilin on LES pressure and on gastrointestinal myoelectric activity was examined in 8 unanesthetized opossums. Intraluminal pressure was recorded by a manometric assembly incorporating a sleeve device. Myoelectric activity was recorded from the stomach, duodenum, and jejunum via implanted electrodes. The opossum LES exhibited cyclical periods of phasic contractions synchronous with phases II and III of the gastric migrating motor complex cycle. Variations in the occurrence and magnitude of the phasic LES pressure waves paralleled the spontaneous cyclic fluctuations in the level of circulating plasma motilin. Pulse doses of exogenous motilin (25-400 ng/kg) elicited a contractile LES response that mimicked the spontaneous migrating motor complex-related phasic LES contractions. This effect was dose related with the maximal response occurring at a motilin dose of 100 ng/kg. The LES response to motilin was abolished by hexamethonium and significantly antagonized by atropine and 4-diphenylacetoxy-N-methylpiperidine methiodide, but was not affected by pirenzepine, phentolamine, or naloxone. The study findings support the hypothesis that cyclic increases in circulating endogenous motilin incorporate phasic LES as well as gastric contractile activity into the gastrointestinal migrating motor complex cycle. Motilin acts on the LES by the preganglionic stimulation of cholinergic nerves.
The opossum has served as a useful animal model for in vivo studies of lower esophageal sphincter (LES) function. Previous investigations, however, have been confined to studies on anesthetized animals. In 10 opossums we investigated LES pressure during fasting cycles of the gastrointestinal migrating myoelectric complex (MMC) and examined the influences of anesthesia and feeding on LES pressure. Intraluminal pressure from the esophageal body, LES, and gastric antrum was recorded by a manometric assembly that incorporated a sleeve device. Myoelectric activity was recorded from the gastric antrum and duodenum via implanted electrodes. MMCs were readily recorded from all animals. MMC cycle length was 86 +/- 2.9 (SE) min. The LES exhibited cyclic changes in intraluminal pressure that occurred in synchrony with the gastric MMC cycle. During phase I of the gastric MMC cycle, LES pressure was essentially stable, although intermittent spontaneous oscillations at 3-4/min were sometimes noted. Forceful phasic LES contraction started during phase II of the gastric MMC, became maximal during phase III, and disappeared during phase I. The MMC-related phasic LES contraction occurred at a maximal rate of 1.4 +/- 0.05/min with amplitudes of 60-150 mmHg and were temporally associated with spike bursts and contractions in the gastric antrum. Pentobarbital sodium-induced anesthesia abolished MMC-related phasic LES activity and caused a transient rise in basal sphincter pressure. Phasic LES activity was also inhibited by atropine and feeding.(ABSTRACT TRUNCATED AT 250 WORDS)
The ultrastructural pathology of nerve and muscle and the neurological dysfunction in children with cholestatic liver disease and vitamin E deficiency have not been previously correlated. We studied two children with this syndrome. One child, 11 years of age, had severe hyporeflexia and decreased vibratory sense. Nerve conduction was delayed. The second child, 2 years of age, was neurologically normal. Both children showed ultrastructural evidence of damage to the sural nerve and accumulation of electron-dense deposits in the muscle fibers. Abnormalities of the nerves included disruption of the myelin sheath and separation and degeneration of the inner and outer components of the Schmidt-Lanterman incisure.
This paper reports the findings in a study of the incidence of periosteal elevation in children and its possible relationship to child abuse. Two separate sets of radiographs of the skeleton of children were taken for a variety of diagnostic purposes. The suspected abuse set consisted of 59 radiographs taken for suspected child abuse. The mean age for all patients examined was 1.22 years whereas, the mean age for patients with cortical thickening was 0.5 years, suggesting that cortical thickening occurs in a relatively young population. Cortical thickening was assessed by reason for examination. A significant difference (p = .05) was detected with 6/8 (75%) of the patients with cortical thickening from the suspected child abuse groups and only 2/8 (25%) of the patients from the seizure-diagnostic category. The two infants who were noted to have periosteal elevation but were not suspected of abuse had experienced unusual circumstances, one was wearing an abduction splint and the other was a severely hypotonic premature. It is our impression that periosteal thickening is not a normal finding in infants and does not represent a consequence of normal infant care practices. In each case in which cortical thickening was detected, there was evidence to suggest that the child had experienced abnormal or rough handling. As a result of these findings, we believe that cortical thickening of the long bones detected on radiograph is an indication of child abuse.
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The case of nasal polymorphic reticulosis in a child is presented. The patient, an 11-year-old adopted boy, complained of nasal stuffiness and pain. Perforation of the nasal septum and ulceration of the hard palate were evident on examination, and an irregular tumor in the nasal cavity was demonstrated by tomography. Biopsy specimen showed atypical lymphoreticular cells within mixed inflammatory infiltrates. He was treated with radiation followed by cyclophosphamide, mercaptopurine, and methotrexate sodium. Despite regression of the palate lesion and a 12-month symptom-free interval, he died of disseminated polymorphic reticulosis 1 1/2 years after diagnosis. We are unable to find a similar reported case in a child.
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Polyhydramnios and premature delivery complicated the pregnancies of three women whose infants were born with renal tumors. In each case the tumor was a mesoblastic nephroma. The liquid of polyhydramnios enhances detection of masses in the fetal abdomen by ultrasound. In the future, mesoblastic nephroma may, therefore, be diagnosed antenatally. Tumors in these infants, which have proved in most cases to be benign, are usually cured by surgical removal. Occasionally, local infiltration and adhesions prevent removal. The fate of the infant with residual tumor is not known.
The infant born with a posterolateral defect in the right side of the diaphragm may appear normal at birth. His abdominal viscera may be normally located; the right side of the diaphragm may seem to be intact. Later, part of the liver may herniate. As it does, it displaces the lower part of the right lung, and hepatic flexure follows into a high posterior position in the right upper quadrant of the abdomen. Rarely, obstruction of portal and hepatic venous flow at the hernial ring may cause liquid to accumulate in the right side of the chest. At any time the liver may resume its interrupted passage. Increase in herniation will quickly make the patient sicker.
A boy, age 12 years, who had infantile cortical hyperostosis has continued to have occasional aches and new cortical thickenings in his arms and legs. His mandible is undergrown and his ribs have an abnormal slope. Recurrence of Caffey's cortical hyperostosis and persistent deformity have been observed in other children and young adults. Some unexplained cases of late cortical thickening and pain may be due to Caffey's disease.
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