Red blood cell distribution width in pediatric microcytic anemias.

The RBC distribution width has been reported to be of value in the discrimination of iron deficiency anemia from other microcytic anemias, but studies in pediatric populations are lacking. A population of 734 normal children was studied to establish age-appropriate normal values for RBC distribution width. The RBC distribution width of 47 patients with microcytic anemia was then evaluated. RBC distribution width was elevated in 19 of 22 patients with iron deficiency but was also increased in six of 14 patients with thalassemia trait and two of 11 patients with anemia secondary to inflammatory disease. The resulting discrimination was better than that obtained by using Mentzer's index or the discriminant function in the patients studied. The RBC distribution width, albeit a less then perfect tool, can be of value in evaluating pediatric patients with microcytic anemia.

Coincident DiGeorge anomaly and renal agenesis and its relation to maternal diabetes.

We describe 2 cases of DiGeorge anomaly with bilateral renal agenesis-one, who also had hemivertebrae, in an infant of an insulin-dependent diabetic mother (IDDM). In a review we identified 2 other instances of this combination, both in IDDMs. The currently accepted notion that DiGeorge anomaly and renal agenesis are developmental field defects, coupled with their coincidence in IDDM, suggests that this combination is an association.

Lack of validity of standard corrections for white blood cell counts of blood-contaminated cerebrospinal fluid in infants.

An evaluation of the validity in infants of standard computations for the correction of white cell counts in blood-contaminated cerebrospinal fluid (traumatic lumbar puncture) was undertaken. In 39 infants with significant blood contamination (greater than 10(4) red blood cells/microL) and no evidence of bacterial meningitis, both calculated corrections based on simultaneously obtained complete blood counts and estimated corrections based on average values resulted in overcorrection in a majority of cases (33 of 39 and 25 of 39, respectively). The mechanism of this overcorrection could not be defined. In eight infants with significant blood contamination and proven bacterial meningitis, correction computations normalized or overcorrected the white cell count in one case. It appears that standard computations frequently overcorrect white cell counts in blood-contaminated cerebrospinal fluid and that the magnitude of the overcorrection may obscure pathologic states in some cases.

Sarcomatous renal tumor of childhood. An electron microscopic study.

A review of 106 cases of Wilms' tumor revealed five examples of a distinctive, monomorphic, sarcomatous renal tumor. Electron microscopic studies suggested that such tumors have their origin from the blastemal cap cells of the developing metanephros. These variants of Wilms' tumor are more likely to metastasize to bone and have a poorer prognosis than the usual type of nephroblastoma.

The beleaguered band count.

The detection of bandemia is reported to be valuable in the diagnosis of a number of infectious conditions. Many problems that have been identified with the assessment of bandemia, both technical and practical, raise doubts about the validity of these reports.

Quality improvement approaches. Survey of pathologists serving the pediatric patient.

A questionnaire was circulated in 1990 by the Practice Committee of the Society for Pediatric Pathology to its membership to assess the involvement of pediatric pathology departments (PPDs) in quality improvement (QI) activities. Twenty-nine PPDs responded. Analysis of responses disclosed a wide variation in plans, although the majority (22 of 29) had developed or were in the process of developing (three of 29) a specific plan. The plans varied in sophistication and complexity. Pediatric pathologists (directors or designates) were heavily involved in assuming leadership roles for the QI activities in 25 of 28 PPDs. The PPDs with plans (25) had identified the QI indicators monitored either sporadically or on an ongoing basis. The respondents had several indicators in common, such as volume, technical, and clinical indicators, as well as indicators related to turnaround times, meetings, and internal and external consultations. The plurality of the monitors reported are related to indicators in the discipline of anatomic pathology. This review is therefore limited to QI issues in anatomic pediatric pathology. In conclusion, this survey functions to shed some light on the extent of involvement of PPDs in the management of QI. In addition, it provides a model for starting or improving the existing QI plans for PPDs.

T-cryptantigen exposure in neonatal necrotizing enterocolitis.

Sixty-two infants with necrotizing enterocolitis were prospectively evaluated for evidence of the Thomson-Friedenreich cryptantigen (TCA) on their red cell surfaces. The TCA is exposed when bacterially derived neuraminidase acts on the surface of the red cells, cleaving off the N-acetylneuraminic acid, which hides the antigen. Seventeen patients (27%) had red cell TCA exposure as demonstrated by agglutination of their red cells by peanut lectin. This test is a simple agglutination procedure, which can be easily performed and yields results in a matter of minutes. Patients with TCA exposure require surgery 76% of the time compared with 18% of the time of those who did not demonstrate this phenomenon, a highly significant difference (P less than .01). All patients strongly positive for TCA exposure had intestinal perforations found at laparotomy. Patients with TCA exposure had clostridia cultured from blood, peritoneal fluid, or stool in 88% of the cases. Isolation of Clostridium perfringens was highly associated with strong TCA exposure and advanced disease. Since naturally occurring anti-TCA is found in normal adult plasma, hemolysis was observed in TCA-exposed patients who received plasma-containing blood products. The presence of TCA exposure in babies with necrotizing enterocolitis had prognostic and therapeutic implications. It indicates the need for antibiotic coverage against anaerobic organisms, strict avoidance of plasma-containing blood products, and the use of washed blood products if the patient is to be transfused. It also alerts the surgeon to the possibility of early operative intervention.

Placental emboli from a fetus papyraceous.

A syndrome in monozygotic twins that consists of a macerated twin fetus (fetus papyraceous) and a live-born twin with various anatomical defects has been described. The etiology is thought to be placental transfer of emboli or thromboplastic material through vascular shunts. Thromboplastic material precipitates disseminated intravascular coagulation (DIC) in the fetus, with a resultant hypercoagulable state due to relative fetal antithrombin III deficiency. Two cases of this syndrome will be discussed. The case of a live-born twin with intestinal atresia, who developed in utero with a fetus papyraceous, is reported. Emboli were demonstrated in vascular shunts of the diamniotic-monochorionic placenta. The hypothesis of intestinal atresia as a result of a vascular accident is reviewed. Another case involving a live-born twin with congenital skin defects, who developed in utero with a fetus papyraceous, is also reported. The skin defects were a congenital disruption from fetal DIC with resultant hypercoagulable state. Several other manifestations of the placental emboli syndrome will be discussed and the vascular etiology of the disruptions explained.

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 patient's 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.

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 less than 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.

Acute transverse myelopathy in childhood. A study of four cases.

Four cases and a literature survey show acute transverse myelopathy to be a dramatic but rare pediatric problem. Neurologic deficits can be significant but generally improve with the passage of time. The prognosis for complete recovery, however, is poor.

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.

Bacterial-induced RBC alterations complicating necrotizing enterocolitis.

Enzymes released from bacteria can alter the surfaces of RBCs rendering them susceptible to destruction by antibodies present in a high percentage of adult plasmas. Such RBC alterations were observed in four of 20 consecutive cases of radiologically proved necrotizing enterocolitis. Involved infants were seriously ill, three of four demonstrating bowel perforation. Bacteria of the genus Clostridium were isolated from blood or peritoneal fluid in three of four affected patients and elaborated appropriate RBC-altering enzymes in vitro. Two patients who had received plasma-containing products experienced notable hemolysis. Patients treated with washed products and plasma protein fractions lacking immunoglobulins had no hemolytic problems. The alteration of the RBC membrane is easily detected by a rapid, simple lectin agglutination test.

Rhizomelic chondrodysplasia punctata: report of a case with review of the literature and correlation with other peroxisomal disorders.

A 3-year-old girl with rhizomelic chondrodysplasia punctata (RCDP) had severe microcephaly but a normal gyral pattern, neuronal density, and cortical cytoarchitecture. The white matter was diffusely decreased in mass but normally myelinated. There was optic atrophy and cerebellar degeneration. Leukodystrophy in peroxisomal disorders is caused by elevated very long chain fatty acids. The absence of a fatty acid abnormality in RCDP explains the normal myelination. Cerebellar and retinal degeneration and possible stunted dendritogenesis may be due to plasmalogen deficiency, which is the most severe biochemical abnormality in RCDP.