Robert W. Novak

Hemolytic-Uremic Syndrome and T-Cryptantigen Exposure by Neuraminidase-Producing Pneumococci: An Emerging Problem?

Hemolytic anemia, thrombocytopenia, and renal failure form a constellation of complications observed in patients infected with neuraminidase-producing pneumococci. The circulating enzyme causes exposure of the T-cryptantigen on cell surfaces to which most people possess a naturally occurring antibody. Antigen-antibody interaction activates effector systems that lead to the clinical manifestations. The syndrome is most frequently seen in infants, in whom it carries more than a 50% mortality rate. T-cryptantigen activation can be detected by demonstrating red cell agglutination by the peanut lectin Arachis hypogea. Plasma exchange and avoidance of blood products containing immunoglobins are of value in the management of this devastating complication of pneumococcal infection.

Histologic Analysis of Placental Tissue in First Trimester Abortions

The value of histologic evaluation in the analysis of material from first trimester abortions is not completely defined. We prospectively analyzed placenta and decidua from 75 first trimester, spontaneous abortions to ascertain if morphologic features were predictive of karyotype. The histologic features analyzed included hydropic villus change, villus fibrosis, villus scalloping with trophoblastic invaginations, atypical stromal cells, aggregates of lymphocytes in placenta or decidua, and acute inflammation of placenta or decidua. Normal karyotypes were observed in 44 cases and abnormal karyotypes were demonstrated in 31. The presence of villus scalloping with trophoblastic invagination was significantly associated with abnormal karyotypes, particularly triploidy, and the demonstration of acute inflammation was seen significantly more often in cases with normal karyotypes. We conclude that histology can provide only a suggestion as to the likelihood of an abnormal karyotype; the findings are not specific enough to obviate the need for karyotyping in the individual case.

Autologous Blood Transfusion in a Pediatric Population: Safety and Efficacy

A program for both preoperative collection and intraoperative salvage of autologous blood was established for a pediatric population. Eighty two patients, 16 years old or younger, participated during a 2-year period. Thirty four had blood collected preoperatively and 48 utilized salvaged blood only. Only 5 percent of preoperative donations were associated with any adverse reaction and all reactions were mild. Salvaged blood provided an average of 48 percent of all blood needs in those patients in whom it was used alone; in only 10 percent of cases did it provide for all of the patients blood needs. Preoperatively collected blood met an average of 74 percent of blood needs when it was used alone; in half of the cases it was the only blood the patient required. The combination of preoperatively collected and intraoperative salvaged blood was highly successful, meeting an average of 94 percent of blood needs and supplying all blood needs in 77 percent of these patients.

The Pathobiology of Red Cell Cryptantigen Exposure

This article has defined in part the circumstances in which red cell cryptantigen exposure occurs and its significance in children. Bacteria-induced cryptantigens (T and Tk) are the most commonly encountered and, when present, suggest a guarded prognosis, a complicated clinical course, and a need for care in transfusion management with particular attention to the avoidance of plasma-containing products. Nonbacterial-induced cryptantigens (Th and Tn) are much less commonly seen and are encountered as a complicating feature of a serious hematologic condition and may be a potential source of confusion in the neonate.

Undifferentiated rhabdomyosarcoma with lymphoid phenotype expression

Poorly differentiated rhabdomyosarcomas are traditionally distinguished from lymphomas by their absence of lymphoid markers such as immunoglobulin or CD20 expression. We have encountered three alveolar rhabdomyosarcomas that were initially diagnosed as lymphoid neoplasms because of the expression of a lymphocytic phenotype in morphologically undifferentiated tumor cells. Subsequent cytogenetic analysis revealed a t(2; 13) in two cases. All cases recurred in the chest wall and showed positivity for muscle markers, such as muscle-specific actin, myoglobin, MyoD1, and/or desmin on subsequent immunohistochemistry. The findings in these three cases lead us to conclude that the presence of a lymphoid phenotype does not absolutely exclude the diagnosis of rhabdomyosarcoma.

Significance of Placental Findings in Early-Onset Group B Streptococcal Neonatal Sepsis

Assessment of placental pathology and its relationship to historical data, initial laboratory parameters, and outcome was undertaken in 22 cases of early-onset group B streptococcal sepsis of the neonate. Fourteen (64%) of the placentas demonstrated chorioamnionitis, six (27%) funisitis, and in nine (41%) gram stain demonstrated organisms within the membranes. Focal villous edema was observed in five (23%) cases and diffuse villous edema in four (18%). No placenta demonstrated chorangiosis. Placental inflammation was significantly (p < 0.05) associated with prematurity, prolonged rupture of membranes, and onset of symptoms at less than 3 hours of age. No placental change was significantly associated with outcome or with neutropenia, which was the only parameter assessed that appeared to have prognostic value.

Fibrillary Glomerulonephritis with Hepatitis C Viral Infection and Hypocomplementemia

Fibrillary glomerulonephritis (FGN) is a relatively rare cause of renal disease, found in only 0.6–1.5% of native renal biopsies. The pathogenesis of FGN is not well described, and very few associations with disease processes other than hepatitis C virus (HCV) have been made. We describe a case that provides evidence in support of the FGN-HCV association, as well as introduces the association of FGN-HCV and hypocomplementemia. The case is a 53-year-old African-American female demonstrating a classical presentation of FGN complicated by a concomitant HCV infection. Treating an HCV infection with alpha-interferon has been shown to result in subsequent improvement in the nephrotic syndrome and renal function. However, this patient is unique in that she is complicated with hypocomplementemia, creating a complex treatment situation.

Pyloric Duplication Presenting with Hemorrhagic Ascites

From the Departments of Pathology and Surgery, Children’s Hospital Medical Center, Akron, Ohio. Correspondence to: Robert W. Novak, M.D., 281 Locust Street, Akron, OH 44308. Received for publication October 1982 and accepted January 1983. PYLORIC DUPLICATION is an uncommon form of gastric duplication, accounting for only one of 83 cases in a recent review of duplications of the stomach.’ The rare case reports of this entity have stressed that pyloric duplications are seen as gastric outlet obstructions with findings reminiscent of hypertrophic pyloric stenosis. 2.3 Experience with a re-

Optimal Diagnostic Testing for Urinary Tract Infection in Young Children

Urinary tract infection (UTI) is a major concern in young febrile children. Current recommendations favor use of urine obtained by bladder catheterization or aspiration, but opinion varies as to the best ancillary tests to predict a positive culture and guide initial management. The utility of dipstick leukocyte esterase, blood, and nitrite; unspun urine leukocyte count; gram stain of cytocentrifuged urine; and standard spun sediment examination was evaluated in 142 febrile, < 5-year-old children seen in the Emergency Department, 25 of whom had culture-proven UTI. Using sensitivity and negative predictive values as criteria for performance, unspun urine leukocyte count and gram stain of cytocentrifuged urine used in parallel was the best approach but still failed to detect some UTI. Analysis of the nature of the specimens evaluated provided explanation of differences from previous observations. Adoption of this modified approach to prediction of urinary tract infection appears to improve prediction but has operational implications and creates potential problems for the laboratory.

Papillomavirus Infection and its Pathologic Correlate in Sexually Active Adolescents

We investigated the prevalence of human papillomavirus (HPV) and its pathologic correlation in an adolescent clinic population (13-20 years, mean 16 years) over a 2-year period. 413 cervical specimens were obtained and analyzed cytologically and by a Southern Blot (SB) method for HPV DNA. 277 specimens from 210 patients could be fully analyzed. 23 patients (10.9%) were positive for HPV DNA by SB. Cytologic findings in these 23 patients demonstrated changes compatible with low-grade squamous intraepithelial lesions (LGSIL) and HPV-associated changes in 4 cases (17%). Cervical biopsies obtained in 3 cases with abnormal cytology demonstrated LGSIL in all cases. 6 patients were retested for HPV 2 to 6 months after the initial positive, two showed persistence of the initial virus, one was positive for a different HPV type and three were negative for HPV DNA.