Biomedical subjects
Publications and source records attributed to I Bhan.
A newborn male infant with multiple congenital malformations including lethal cardiac malformations and severe ophthalmic malformations is described. The infant was exposed in utero to hydantoin. There is no other identifiable cause for the malformations apparent in the infant's or his family's history.
Esophagitis in infancy and childhood produces characteristic histologic changes in the mucosa and lamina propria of the distal esophagus. The use of biopsy in addition to endoscopic evaluation considerably enhances the accuracy of the diagnosis of esophagitis, particularly when the degree of inflammatory change is mild. In the absence of severe inflammatory changes, such as ulceration or mucosal slough, esophageal biopsy appears to be essential for the accurate diagnosis of esophagitis. In children of any age with GER who have symptoms of esophagitis, antacid therapy and vigorous efforts to control reflux are indicated. Failure to respond to this program is an indication for esophageal biopsy to confirm or rule out the presence of esophagitis. The degree of inflammatory change in the biopsy is a reliable guide to expected response to antiacid therapy.
Three infants with suspected, and subsequently proven, necrotising enterocolitis (NEC) had contrast medium enemas early in the course of their disease to assist diagnosis. There were no adverse effects from the enemas. All patients had evidence of persistent spasm of segments of the colon and contour speculation, probably representing superficial mucosal ulceration. It is suggested that when early or mild forms of NEC present diagnostic problems, a carefully performed contrast enema may be of diagnostic value. Absence of deep transmucosal necrosis in such cases definitely decreases the risk of perforation during the examination.
The mechanism responsible for persistent regional pulmonary interstitial emphysema under tension, which occasionally complicates assisted ventilation, has not been explained. It is difficult to comprehend how interstitial air may cause lobar hyperinflation and not escape to the mediastinum or pleura. Histologic examination of resected emphysematous lung in two neonates with this type of interstitial emphysema revealed air dissecting from the interstitial connective tissue into markedly dilated lymphatics. It is proposed that intralymphatic air dissection causing emphysematous lymphangiectasia may be responsible for fixation of the lung in the emphysematous state.
Retroperitoneal lymphangiomas are uncommon benign tumors usually presenting in early life. They may cause significant pressure on adjacent structures, most often with superior and medial displacement of the kidney and medial deviation of the ureter. Relative radiolucency on total body opacification during excretory urography, and features of a cystic structure on ultrasonography and computed tomography should further suggest the possibility of a retroperitoneal lymphangioma. Vascular stretching and displacement without neovascularity on angiography and contrast entry into the cysts on lower extremity lymphography are additional features, but such procedures are usually unnecessary.
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Inborn metabolic errors causing lysosomal storage, such as beta-galactosidase deficiency (G(M1) gangliosidosis [G(M1)]), have well-recognized effects on cellular function and morphology. In some classically "neuronal" storage diseases, including G(M1), neuroradiologic observations of infants have suggested a delay in myelination on the basis of persistently "immature" signal intensities monitored over time. We sought to evaluate in a semiquantitative fashion the pattern and degree of myelination in two infantile G(M1) patients, one boy and one girl, autopsied at 15 months of age. We assigned myelination degrees for defined sites on an ordinal scale of 0 to 4, and compared them to published population-based values for autopsied infants. In both patients, earlier-myelinating structures were comparable in development to that expected for postconceptional age, whereas later-myelinating structures were delayed. These data correlate well with the neuroradiologic diagnosis of myelination delay in these infants and suggest that the metabolic defect has a primary influence on myelin development, in addition to effects related to neuronal storage. Furthermore, our analysis by light and electron microscopy and lectin histochemistry of both CNS and systemic tissues, several of which had not been described, add to the understanding of the stored material in different cell types.
Review of the clinical records, chest roentgenograms, and autopsy material of 17 infants dying from oligohydramnios related pulmonary hypoplasia confirmed that two roentgen signs appeared with high frequency. These were pneumomediastinum/pneumothorax (82%) and a bell-shaped chest contour (59%). The bell-shaped chest is valuable in predicting the most severe degrees of lung growth impairment in these infants, and was present in all cases in which oligohydramnios was caused by bilateral renal agenesis. Either pneumomediastinum/pneumothorax or the bell-shaped chest configuration, when present alone, are of low diagnostic value as they are relatively nonspecific signs; their combination, however, has predictive value for Potter's syndrome and may be important in evaluation infants with less typical clinical manifestations of Potter's syndrome.