Search

Background: The mechanism(s) responsible for the development of parenteral nutrition-associated liver disease (PNALD) is unknown. Recently, a number of bile canalicular transport proteins have been identified that transport bile components out of hepatocytes. One group of these genes, multidrug resistance 1 (mdr1) and mdr2, encode P-glycoproteins. Mice lacking mdr2 expression develop liver disease that appears similar to PNALD. This study investigated the alteration in the expression of these transport proteins during the administration of total parenteral nutrition (TPN)., Methods: Mice received either physiologic saline and standard chow or TPN. Mice were sacrificed on day 7, and hepatic DNA and RNA content, mRNA expression, and levels of mdr1 and mdr2 proteins were measured., Results: TPN administration led to a significant (p < .05) decline in mdr2 mRNA expression and an increase in mdr1 mRNA expression. Mdr2 protein expression declined by 66% in the TPN-treated group, and mdr1 protein expression significantly increased by 58%. Histology and biochemical parameters showed no evidence of liver injury. Serum bile acid levels were elevated in the TPN group, suggesting the development of early cholestasis., Conclusions: The decline in mdr2 and rise in mdr1 mRNA and protein expression with TPN administration occurred before the development of liver injury but during an early state of cholestasis. This suggests that alterations in mdr gene expression may be a causative factor in the development of PNALD.

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.

Background/purpose: The introduction of managed care in the 1980s caused increased pressure to reduce costs for hospitalized patients. The authors hypothesized that these market forces have resulted in a decreased hospital stay and utilization of sophisticated diagnostic testing in children treated for appendicitis. If true, the impact of this paradigm shift on patient outcome is unknown., Methods: Hospital records for 913 pediatric patients treated for appendicitis from 1974 to 1998 were reviewed retrospectively. Patients were stratified into those with perforated appendicitis (PA) and nonperforated appendicitis (NPA). Demographics, perioperative hospital course, diagnostic testing, complications, and long-term outcomes were analyzed after stratification into time intervals., Results: Over time, children with NPA were treated with shorter antibiotic courses (P<.05) and were placed on a regular diet earlier (P<.05). These changes in treatment resulted in an earlier discharge (P<.05). The amount of time to become afebrile with a normal white blood cell count (WBC) did not change over time. Children with PA exhibited similar results with shorter antibiotic courses (P<.05), earlier dietary intake (P<.05) and earlier hospital discharge (P<.05) over time. In all children with appendicitis there was no significant difference in the rate of wound infections, abscesses requiring drains, readmission, or reoperations overtime. The utilization of abdominal radiographs (83%) and ultrasonography (USN; 40%) was high and remained unchanged over time. Utilization of computed tomography (CT scan) was low (4.3%) in the early decades and was not used as a preoperative test from 1991 to 1994. Given the high diagnostic accuracy of a pediatric surgeon for this disease, Bayesian analysis indicates that USN utilization rates should be 15%., Conclusions: The market pressures of managed care have resulted in a new treatment paradigm with an earlier discharge of all children with appendicitis. There has been no concomitant increase in the complication rate in either group as a result of this paradigm shift. Bayesian analysis indicates that USN and abdominal radiographs are overutilized in our institution.

We measured the dimensions of Koch's triangle in children with normal intracardiac anatomy to determine the relation between the size of the triangle of Koch and patient age, weight, height, and body surface area. We found that the dimensions of Koch's triangle varies significantly and directly with patient age and body habitus in this pediatric population.

In a fetal autopsy series, we have explored the occurrence of renal tubular dysgenesis in twins. Renal tubular dysgenesis was found exclusively among those monozygotic twins with evidence of twin transfusion syndrome, particularly in those donor twins with oligohydramnios and growth restriction. We infer that hypotension in the donor twin of the twin transfusion syndrome pair is responsible for the failure of proximal convoluted tubule differentiation, and the disturbance of renal function is manifested as oligohydramnios prenatally, and either oliguria or tubular dysfunction postnatally.

Increased myocardial collagen accompanies pressure overload of the adult left ventricle. This phenomenon is poorly understood in infants. This study compares the myocardial volume fraction of collagen in infants who did not have primary heart disease with infants with isolated pressure overload of the right ventricle (tetralogy of Fallot [ToF]), and with infants with combined volume and pressure overload (aortic valve atresia [AVA]). The distribution of collagen in the neonatal myocardium was also determined. We measured the volume fraction of collagen from right ventricular biopsy specimens of cadaver hearts in normal infants (1 to 9 months old; n = 7), infants with ToF (1 day to 9 months old; n = 9), newborns with AVA (AVA-NB) (1 to 4 days old; n = 5), and older patients with AVA (AVA-I) (5 to 8 months old; n = 5). Myocardium from 3 patients undergoing repair of ToF (6 to 8 months old) was also analyzed. Specimens were stained with Masson's trichrome and myocardial volume fraction of collagen determined by point counting. Myocardial volume fraction of collagen was significantly higher (p = 0.02) in AVA-I patients (8.0 +/- 3.5%) versus normal (3.3 +/- 2.7%), ToF (3.2 +/- 1.8%), and AVA-NB (3.5 +/- 2.3%) patients. There was a tendency for increased collagen in the subendocardium, especially in AVA-I patients (p > 0.05). We conclude that patients with AVA-I have increased collagen relative to normal subjects, patients with ToF, and patients with AVA-NB, and that this increase is greatest in the subendocardium.

We report on two sisters with an unusual form of craniosynostosis, protruding nasal spine, micrognathia, short limbs, lung hypoplasia, absent or hypoplastic gallbladder, short intestine with ileal distention, hypoplastic uterus, and intrauterine growth retardation. This combination of defects appears to be a newly recognized and probably autosomal recessive disorder.

Enterocolitis associated with Hirschsprung's disease (HEC) remains a major source of morbidity and even mortality, both before and after definitive surgical treatment. This study was undertaken to investigate whether histopathologic mucosal changes, in the absence of clinical manifestations of HEC, could predict the subsequent development of this complication. The clinical data and histopathology of 25 patients who eventually developed clinical HEC were compared with a control group of 25 age- and sex-matched patients with Hirschsprung's disease (HD) but with no clinical HEC either preoperatively or postoperatively. The histopathologic findings of tissue obtained by rectal biopsy or during laparotomy were graded from 0 to V according to severity and compared with the eventual clinical course. This study showed that (1) histopathologic findings of HEC appear to predict the clinical development, but not the severity, of future episodes of clinical HEC; (2) a histological grade of > or = II (> or = 2 crypt abscesses per high power field) should raise suspicion for subsequent occurrence of HEC, whereas a grade of > or = III (multiple crypt abscesses) places a child at high risk for development of clinical HEC; and (3) contrary to what is generally thought, histopathologic changes of HEC occur in both ganglionic and aganglionic segments. Based on these findings, it is recommended that histopathologic documentation of HEC and its grade should be an integral part of the tissue diagnosis of HD.

We reviewed seven young children (< or = 10 years) with aneurysmal bone cysts. There were four girls and three boys. Six had involvement of the long bones and one had involvement of the clavicle. The average age was 5.5 years (range 2.9-10.6 years). Initial treatment was curettage and bone grafting. There were recurrences in five of the seven children (71%). This represented 100% of children with radiographically aggressive or active lesions. The recurrences appeared rapidly, at an average of 8 months from the first procedure. The mitotic index of the initial lesion did not correlate with that of the recurrent lesion. Surgical management of the recurrences must be handled individually, but repeat curettage and grafting is only recommended when surgical resection is not possible. This high rate of recurrence in radiographically aggressive or active aneurysmal bone cysts in young children should be considered when planning treatment, and in the preoperative counseling of parents.

Survival rate in neuroblastoma, a tumor of post-ganglionic sympathetic neuroblasts, correlates with disease stage, tumor histology, and N-myc gene amplification. N-myc amplification is associated with rapid tumor progression and poor survival, but is not present in all cases of poor prognosis neuroblastoma. Moreover, overexpression of N-myc is not sufficient to cause cellular transformation. These data suggest that other genetic factors are important for neuroblastoma development. We investigated the expression of the, bcl-2 proto-oncogene in untreated cases of neuroblastoma. bcl-2 is a novel proto-oncogene that promotes cell growth by inhibiting programmed cell death (apoptosis), a form of cellular demise common during normal neurogenesis. Immunocytochemical localization using a monoclonal anti-bcl-2 antibody revealed that 16 of 40 patient specimens stained positive for bcl-2. bcl-2 was strongly associated with unfavorable histology (P = 0.002) and N-myc gene amplification (P = 0.002) and marginally associated with poor stage disease (P = 0.06). A logistic regression model evaluating the simultaneous association of stage, histology, and N-myc revealed that bcl-2 was most associated with unfavorable histology and N-myc gene amplification. These results support the notion that bcl-2 may play an important role in the genesis or progression of malignant neuroblastoma.

Background: Congenital mesoblastic nephroma (CMN) is generally considered to be a benign neoplasm requiring only total excision. Rare local recurrences have usually been related to incomplete removal, and distant metastases to the lung have been reported three times., Methods: The authors reported the first case of CMN metastatic to the brain, illustrating a comparison of the histopathology of the primary and secondary lesions., Results: The gross appearance of the renal tumor was that of a benign CMN. On histopathologic examination, there was a single nodule of more cellular tissue with focal necrosis. This area was diagnosed as within the designation "cellular" mesoblastic nephroma. The appearance of the brain metastasis corresponded to that of the cellular nodule., Conclusions: This report expands the spectrum of metastatic potential of CMN. Though a rare event, this case underscores the need for close follow-up of infants with CMN.

Eleven patients with spinal osteoid osteoma and six patients with spinal osteoblastoma treated between 1975 and 1990 were reviewed to characterize the tumors as they affect the spine and to define the important differences between the two tumors. All patients with cervical osteoid osteoma presented with pain, limited range of motion of the neck, and torticollis. Four osteoblastomas had soft-tissue components in the epidural space, necessitating dissection of the tumor from the dura. No soft-tissue component was found in any of the osteoid osteomas. Our results were similar to a metaanalysis of the clinical, radiographic, and surgical findings of all published cases of spinal osteoid osteoma and osteoblastoma. Important features that have not been emphasized in the literature are the high incidence of torticollis with cervical lesions and the frequent association of epidural invasion with osteoblastoma. Surgeons treating osteoblastoma of the spine should be prepared to dissect tumor from the dura.

To determine the usefulness of echocardiographic indexes of left ventricular (LV) function as possible predictors of cardiac rejection, 12 transplant recipients (ages 3 to 17 years) underwent a total of 52 serial echocardiographic examinations and cardiac biopsies. The results were compared to those of 12 normal children (ages 2 to 17 years). Biopsies were graded as no rejection (n = 23), mild rejection (cellular infiltrate, n = 13), and moderate rejection (myocyte necrosis, n = 16). LV dimensions, percent shortening fraction, indexed LV mass, and ejection fraction were measured from M-mode and two-dimensional echocardiography. From the mitral valve Doppler tracing, the following measurements were made: isovolumic relaxation time, peak E and peak A velocities, and the fraction of filling under the E and A waves as well as in the first third of diastole. Compared with normal subjects, transplant recipients with no rejection had higher heart rates (95 +/- 15 vs 80 +/- 17 beats/min), longer isovolumic relaxation time (68.8 +/- 11.2 vs 51.5 +/- 13.6 msec), decreased first third area fraction (0.48 +/- 0.10 vs 0.57 +/- 0.10), and similar shortening fraction, LV mass, and peak E and A velocities (p less than 0.03). Compared with transplant recipients with no rejection, patients in whom mild rejection developed also had decreased shortening fraction (31% +/- 10% vs 37% +/- 8%) and decreased peak E velocity (0.68 +/- 0.19 vs 0.88 +/- 0.15 m/s) (p less than 0.03). From mild to moderate rejection, no further changes were noted in any echocardiographic indexes measured.(ABSTRACT TRUNCATED AT 250 WORDS)

Forty-three neuroendocrine neoplasms were analyzed by immunohistochemistry for tyrosine hydroxylase and chrornogranin A and by in situ hybridization (ISH) for chrornogranin A messenger RNA (mRNA) using formalin-fixed paraffin-embedded tissue sections. These included pheochromocytomas (7), medullary thyroid carcinomas (5), small-cell lung carcinomas (5), olfactory neuroblastomas (5), neuroblastomas (10), ganglioneuroblastomas (5), and ganglioneuromas (6). Tyrosine hydroxylase was detected in all groups of tumors except the small-cell lung carcinomas, whereas immunoreactivity for chromogranin A protein was detected in all groups. ISH analysis for chromogranin A mRNA agreed with the immunohistochemical findings. Beta-actin mRNA was present diffusely in tumor cells, confirming the preservation of another mRNA. There was no apparent relationship between protein or mRNA expression and the degree of differentiation in the 21 neuroblastic tumors. These results indicate that tyrosine hydroxylase may be another useful marker for neuroblastic tumors and that chromogranin A mRNA can be detected in routinely processed formalin-fixed paraffin-embedded tissue sections.

Options for mechanical support of pediatric patients with severe heart failure who are awaiting transplantation or have undergone transplantation are limited. This report examines 3 patients placed on extracorporeal life support (ECLS) while awaiting transplantation and 3 patients who underwent transplantation and suffered subsequent heart failure due to rejection or postoperative myocardial dysfunction. The overall survival rate was 2 of 6. The 2 surviving patients had a failing transplanted heart. There were no survivors among the patients placed on ECLS as a bridge to transplantation. In each case a contraindication to transplantation developed before a donor heart could be obtained. The mean time of ECLS support was 147.5 hours (range, 70 to 370 hours). The ECLS circuit did not affect cyclosporin levels or antirejection therapy. Extracorporeal life support can be used to support pediatric cardiac transplant patients with biventricular failure due to acute rejection or postoperative dysfunction. Although the results have been discouraging, ECLS may still have a role as a bridge to transplantation. However, complications can develop during ECLS that may preclude transplantation.

Fibrodysplasia ossificans progressiva is a rare disorder characterized by progressive heterotopic bone formation in connective tissue and muscle in association with congenital skeletal anomalies. The disease usually leads to extensive immobility and eventual disability. We report the magnetic resonance findings in a boy prior to ossification of his soft tissue masses.

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Prenatal ultrasonography at 24 weeks disclosed a fetus with a large mass protruding from the occiput. The mass was an invasive, undifferentiated mesenchymal neoplasm of the scalp associated with a defect in the occipital bone (cranium bifidum). The tumor may have derived from neural crest at the site of rhombencephalic closure. The tumor itself would seem to be a sporadic event, but the cranium bifidum may imply a recurrence risk of 2-3% for future pregnancies.

Alpha-1-antitrypsin (A1AT) deficiency originally was recognized as a biochemical abnormality in patients with pulmonary emphysema. Studies of the protein nature of A1AT during attempts to help explain the disease, led to the recognition of a protein polymorphism which expressed itself in several different phenotypes. As investigative work progressed, the spectrum of diseases associated with a deficiency of A1AT increased. Methods for determination of quantitative levels have been developed but these have proved lacking for the discrimination of the various phenotypes, for which specialized testing is necessary. Of the clinical states associated with deficiencies of A1AT, pulmonary emphysema and hepatic disease are the best defined. Pathogenetic implications, however, remain elusive. As a consequence, preventive aspects are debatable and the usefulness of the determination of A1AT levels as screening measures is controversial.

Each of the three patients with a type of unilateral, localized cystic disease of the kidney that did not fit into a specific category of existing classifications had numerous cysts involving a part of one kidney. Selective angiography showed stretching of arteries around cysts that remained radiolucent in the nephrographic phase, and microscopic examination demonstrated that septa among the cysts contained compressed renal elements. The condition appeared to be neither progressive nor familial. All three had hypertension. Two patients who had nephrectomy for the cystic lesion became normotensive postnephrectomy. Despite some morphologic resemblances, this condition is believed to be different from adult type polycystic kidney disease in its clinical and genetic aspects.

Two cases of probable congenital tuberculosis occurring in the ten-year period from 1966 to 1976 are reported. One infant with confirmed congenital tuberculosis also had symptomatic hypoadrenocorticism. Tuberculosis should be considered in the differential diagnosis of the seriously ill infant.

Two achondroplastic infants had small foramina magna with lethal compression of the corresponding upper cervical spinal cords. The damage was histologically comparable to the hyperextension type of spinal cord injury. An anterior displacement of the foramen magnum was demonstrated in one of the two cases. The displacement is considered worthy of future verification as it may be an additional factor in susceptibility to hyperextension injury to the spinal cord. The second patient survived 6 months, demonstrating that not all upper cervical spinal cord injuries are immediately fatal. Patients who have sublethal lesions are potentially salvageable by surgery. It is also speculated that there might be more cases of nonlethal upper cervical spinal-cord damage in achondroplasia, especially in early infancy when infants are unable to hold their heads erect. The true incidence of slight upper cervical spinal cord damage is not known, although it is considered rare at present. As a result of the findings in these two cases, it is suggested that hyperextension of the head be avoided, when possible.

A gastric leiomyoblastoma can occur in any age group, but the prognosis and sex distribution are different in the child-adolescent group than in adults. In the adult, the lesion is generally considered to be benign, though in the child-adolescent group it may metastasize to regional lymph nodes. The possibility of a leiomyoblastoma is suggested by multicentric intramural gastric tumors discovered in a female child or adolescent who has had gastrointestinal hemorrhage.

Ninety-four patients underwent rectal biopsy with a suction capsule during a two year period. This painless procedure was performed in seventy-one patients for diagnosis of aganglionosis (Hirschsprung's disease). In nine patients, no ganglion cells were encountered, and subsequent surgery confirmed the diagnosis of Hirschsprung's disease in each of these patients. In twenty-three patients, biopsies were taken to search for neuronal storage disease. Six specimens contained abnormal material in the cytoplasm of the ganglion cells.

Liver biopsy was performed to exclude anatomic obstruction of the biliary tract in five prematurely born infants who had developed conjugated hyperbilirubinemia during intravenous alimentation with a protein hydrolysate. Each was being treated after having undergone a segmental intestinal resection for necrotizing enterocolitis. Bacterial and viral infections, metabolic disorders, and isoimmune hemolytic disease were excluded as possible causes of jaundice. Light microscopic and ultrastructural analysis disclosed cholestasis and hepatocellular injury without significant inflammatory reaction. Jaundice abated following permanent discontinuation of parenteral alimentation. The jaundice and cholestasis are interpreted to be hepatotoxic effects because of (1) their temporal relationship to the treatment and (2) the presence of hepatocellular damage.

Malacoplakia often is considered a benign disease. Our recent experience with renal parenchymal malacoplakia and a review of the literature have revealed the clinical and pathologic features as well as the prognosis of this renal disease. Upper urinary tract malacoplakia is neither benign nor self-limited, and bilateral renal parenchymal involvement has been uniformly fatal.

Lethal achondrogenesis in the past has been frequently confused with achondroplasia. Clinical and radiologic advances in the last decade have led to clear differentiation of this condition from other types of bone dysplasia. It is further separated into two types on the basis of radiographic and pathologic findings. Re-evaluation of the histologic features has led to the recognition of heretofore unrecognized intracytoplasmic inclusion bodies in the chondrocytes of type 1 achondrogenesis. The finding of inclusions strengthens the differentiation of the two types and may have implications for the pathogenesis of the form of chondrodystrophy.

Nineteen cases of chondrodysplastic short-limbed dwarfism in early infancy were studied in the context of current clinical classification based on established radiographic criteria. The histopathologic findings were sufficiently distinctive in most categories to provide additional diagnostic criteria and to contribute to the understanding and delineation of these disorders. 1. Homozygous achondroplasia is distinguished by markedly disturbed endochondral ossification that differs from the pattern seen in typical heterozygous achondroplasia. The physeal chondrocytes contain abundant granules of glycogen. 2. Achondrogenesis, the severest form of chondrodysplasia, exists in 2 types, in which the histopathologic findings appear to be distinctive and diagnostic. Type 1 is characterized by severe disturbance in endochondral ossification. The zone of resting cartilage contains relatively normal matrix, and the chondrocytes contain intracytoplasmic inclusions. The cartilaginous matrix in type 2 is markedly deficient, and the chondrocytes have a large, primitive, mesenchymatous appearance. 3. Thanatophoric dwarfism is associated with disorganized endochondral ossification similar to that in homozygous achondroplasia, but there is no accumulation of glycogen within chondrocytes. Type 2 thanatophoric dwarfism is differentiated from classical type 1 by the presence of cloverleaf skull and histologically by many bone-lined, penetrating vascular canals in the physis and by hyperactive osteoblasts and osteoclasts in the metaphysis. 4. Asphyxiating thoracic dysplasia of Jeune is differentiated histologically into 2 types. Type 1 is characterized by patchy distribution of endochondral ossification in the physis, irregular physeal-metaphyseal junction and large islands of poorly mineralized cartilage in the metaphysis. Type 2 is characterized by uniform distribution of endochondral ossification that is disorganized and is accompanied by advancing cartilage forming latticelike meshwork in the metaphysis. 5. Chondroectodermal dysplasia, which is radiographically similar to asphyxiating thoracic dysplasia, is marked by the presence of large islands of poorly mineralized cartilage in the spongiosa of vertebral bodies. Disorganized endochondral ossification is, however, uniformly distributed, and there is no latticelike advancing cartilage in the metaphysis. 6. Chondrodysplasia punctata is characterized by myxoid and cystic degeneration of physeal and epiphyseal cartilage with focal calcification. Type 2 (Conradi-Hünermann) has a nearly normal pattern of endochondral ossification, even though there may be severe disturbance and retardation of the process. The columnization of physeal chondrocytes is normal. Type 1 (rhizomelic) differs by having markedly retarded and disorganized endochondral ossification. 7...

Elevations in serum immunoglobulins, frequently monoclonal in nature, are known to occur in patients with the adult form of Gaucher's disease. We describe amyloidosis in a 46-year-old woman of Italian ancestry with Gaucher's disease, who also had 3100 mg/dl of monoclonal IgA. She died of restrictive cardiac disease. A 50-year-old sister, also with Gaucher's disease, had 1300 mg/dl of polyclonal IgM but no evidence of amyloidosis. A glucosyl sphingosine-Sepharose affinity column provided no evidence that the large amount of immunoglobulin present has specific affinity for glucosyl ceramide. This, to our knowledge, is the first report of amyloidosis complicating Gaucher's disease.

A 15-year-old girl was found to have severe liver fibrosis on liver biopsy at the time of cholecystectomy, 5 1/2 years following a modified Fontan procedure (right atrial-right ventricular conduit) for tricuspid atresia. Postoperative right atrial pressures were consistently elevated above 13 mm Hg and this, in part, may have been due to progressive mild conduit stenosis. Because of increasing symptoms, the patient underwent successful revision of the conduit at the age of 15 years. It is suggested that sustained systemic venous hypertension caused the striking morphologic changes in the liver and that this serious complication may significantly affect the long-term prognosis of patients surviving the Fontan procedure.

Torulopsis glabrata is a yeast of low virulence and commensal within the female genitourinary tract. The first case of congenital infection with Torulopsis glabrata was reported in 1980. An additional example of perinatal Torulopsis infection which is associated with maternal sickle cell anemia is reported. The most common source of congenital fungus infection is Candida albicans. Differences in morphology between T glabrata and C albicans are apparent, particularly the pathologic response within the placenta. Pathogenicity in this case may be associated with increased susceptibility to infection in patients with sickle cell anemia.

A malignant cystic neoplasm in the liver of a 6-month-old girl is reported. The neoplasm has unique clinical and morphological features, and despite ultrastructural and immunohistochemical studies, the tumor could not be clearly classified as any known primary hepatic neoplasm of infancy. A differentiated cystic component of the neoplasm had features of cystic peritoneal mesothelioma. Alpha-1-antitrypsin and alpha-fetoprotein were demonstrated in solid, anaplastic portions of the recurrent tumor by immunohistochemistry. The neoplasm recurred after surgical resection and proved fatal 11 months following diagnosis, despite multiple courses of chemotherapy.

The gray-scale ultrasonic features of Wilms tumor are reported with pathologic comparison. The most consistent ultrasonic features are large size, sharp margination, and echogenic heterogeneity. The frequently observed anechoic areas correlated with hemorrhage and necrosis. A rare case of mesoblastic nephroma is included for comparison. Pitfalls in diagnosis are discussed.

A report of a primary adenocarcinoma of the lip, arising from minor salivary tissue, is presented. Adenocarcinomas of the lips are unusual and account for only 1% of the reported adenocarcinomas of oral tissues.