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OBJECTIVES: (1) To develop methods to describe autopsy utilization patterns in a neonatal intensive care unit. (2) To identify classes of patients likely to have clinicopathologic concordance or discordance. METHODS: Five hundred forty-five consecutive neonatal intensive care unit deaths (338 autopsied, rate 62%) in a regional tertiary/quaternary care neonatal intensive care unit for referred infants (65,000 annual births) were classified in six clinical diagnostic groups (anomalies, cardiac anomalies, hypoxic ischemic encephalopathy, prematurity and its complications, infections, and other) and rated in three levels of certainty of clinical diagnosis as "gold standard" certainty, almost complete certainty, and less certain than the latter. Clinicopathologic discordances were rated in three classes using clinical, pathologic, and multidisciplinary mortality conference records. The proportions of autopsied cases, cases with major discordances, and cases with no discordances were compared and analyzed in relation to diagnostic group and level of certainty. RESULTS: Performance of autopsy was associated with clinical diagnostic uncertainty (p = 0.008). Major discordances with implications for outcome (Class I) were found in 3%, and without implications for outcome (Class II) in 15% of cases; 42% of cases had no discordances. Major discordance rate varied inversely with the degree of diagnostic certainty (p = 0.000) and varied among clinical groups. CONCLUSIONS: (1) Autopsy was used most for cases with potential for high yields. (2) Clinicopathologic discordances were more frequent and important in certain clinical diagnostic groups (prematurity, other) and with high levels of diagnostic uncertainty. When the diagnostic "gold standard" is available during life, autopsy will provide little information.

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A case-control study was undertaken to determine whether esophagitis in children correlated with exposure to parental cigarette smoking. At least one parent smoked in 77 (79%) of 97 families in the study group, compared with 42 (38.9%) of the 108 families in the control group (p < 0.001). Passive smoking is a risk factor for the development of esophagitis in children, providing added support for public health efforts to restrict childhood exposure to tobacco smoke.

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We report the cases of neonatal lupus erythematosus associated with significant hepatic involvement in three living infants and in one infant who died 3 hours after delivery. The three living infants had neonatal cholestasis as a major component of their clinical findings. Pathologic changes included giant cell transformation, ductal obstruction, and extramedullary hematopoiesis. Liver involvement has been noted incidentally in children with neonatal lupus erythematosus, but it has generally been attributed to hemodynamic compromise as a result of congenital heart block or systemic toxic reactions. We speculate that neonatal hepatitis proceeding to hepatic fibrosis may occur in neonatal lupus erythematosus, analogous to the occurrence of "idiopathic" congenital heart block. The neonatal hepatitis associated with neonatal lupus erythematosus is a form distinguishable from the "idiopathic" group. Liver involvement may be more common than was previously recognized, and prospective studies to look for maternal autoantibodies in idiopathic neonatal liver disease should be undertaken.

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We evaluated the efficacy of medical therapy, consisting of liquid bismuth subsalicylate prescribed either in combination with oral ampicillin (n = 15) or alone (n = 1), in the clearing of bacterial colonization on the antrum in 16 children with Campylobacter pylori-associated antral gastritis. We also examined the effects of medical treatment on altering the severity of associated antral inflammation. Eight patients had upper gastrointestinal tract hemorrhage, two had acute gastric outlet obstruction, and 10 had symptoms of episodic epigastric abdominal pain. Duodenal ulcers were demonstrated in 10 of the 16 patients; in the other six, C. pylori-associated antral gastritis was documented without evidence of acute peptic ulceration. Seven days after a 6-week course of medical therapy, repeat upper endoscopy plus mucosal biopsy specimens showed that C. pylori colonization of the antrum had cleared in 12 of the 16 (75%) patients. Inflammation in the antrum improved in all patients in whom colonization by C. pylori was eradicated. In contrast, in the four with persistent colonization of the antrum, the severity of antral gastritis had not improved (p less than 0.01). Clinical symptoms improved in 9 of 12 patients in whom C. pylori colonization was no longer present, whereas subjective symptoms were unaffected in those with continued bacterial colonization of the antrum (p less than 0.05). We conclude that oral bismuth subsalicylate, in conjunction with ampicillin, can eradicate C. pylori colonization of the antrum, and that clearing of C. pylori is correlated with an improvement in the associated antral gastritis and clinical symptoms. These findings provide additional support for the hypothesis that these gastric organisms could play an etiologic role in primary antral gastritis and peptic ulcer disease.

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Neonatal ascites is usually attributed to hematologic, genitourinary, gastrointestinal tract, or congenital heart disease. When these lesions have been excluded, metabolic storage disorders should be considered in the differential diagnosis. We report eight cases of neonatal ascites associated with different types of lysosomal storage disease: infantile sialidosis, Salla disease, GM1 gangliosidosis, and Gaucher disease. In each case there was a history of sibling of perinatal death resulting from the disease. In three cases the diagnosis of ascites was made in utero by ultrasound examination. These diseases are characterized by excretion in the fetal urine of abnormal catabolic products or by measurement of decreased activity of specific lysosomal hydrolases in cultured amniocytes. Thin-layer chromatography of the oligosaccharides in amniotic fluid may be indicated when a diagnosis of persistent fetal ascites has been established.

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Liver disease in children with alpha1-antitrypsin deficiency and protease inhibitor type ZZ does not necessarily carry a bad prognosis. Fourteen of our 18 patients presented with the neonatal hepatitis syndrome and four had hepatomegaly without jaundice. Although four patients have died of cirrhosis and its complications, and three have severe liver disease, most of the 11 others, of whom four are over 13 years of age, have relatively little clinical, biochemical, or histologic evidence of liver disease. Persistent elevation of SGOT during the third year of life and renal or pulmonary problems were associated with a poor prognosis. Liver biopsy early in the course of the disease was not helpful prognostically but was useful in assessment of the severity of liver disease and demonstration of alpha1AT storage, alpha1AT deficiency was found in 29% of our patients who presented with the neonatal hepatitis syndrome. One of seven apparently healthy Pi type ZZ sibs of our patients had significant liver disease which had not been suspected previously.

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