Plasmapheresis treatment of antibody-mediated rejection in an A2 donor to O pediatric liver transplant recipient.

It is safe to transplant kidneys from blood group A2 donors into O recipients if the latter have low titers of anti-A antibodies. However, in liver transplantation, O and B recipients of A2 donor livers are not routinely screened for anti-blood group antibodies because of the immuno-absorptive capacity of the liver and the low incidence of antibody-mediated rejection. Herein, we report a rare case of combined cell and antibody-mediated rejection in a pediatric blood group O recipient of an A2 donor liver, and rescue of the allograft using PP and IVIG.

Evidence by spectral karyotyping that 8q11.2 is nonrandomly involved in lipoblastoma.

We report two cases of lipoblastoma with chromosome 8-related aberrations, ie, a 92,XXYY,t(7;8Xp22;q11.2)x2 [8]/46,XY[16] in Case 1 and a 46,XY,-8,-13,add(16) (q22),+mar, +r [cp13]/46,XY[7] in Case 2. Using spectral karyotyping and fluorescence in situ hybridization techniques, the karyotype of Case 2 was redesignated as 46,XY, r(8), del(13)(q12), der(16)ins(16;8)(q22; q24q11.2)[cp13]/46,XY[7]. This report delineates a new chromosome rearrangement, ie, der(16)ins(16;8)(q22; q24q11.2) in lipoblastoma, and also confirms the t(7; 8)(p22;q11.2), reported only once previously, as a recurrent translocation involved in such a tumor. These findings provide valuable information for clinical molecular cytogenetic diagnosis of lipoblastoma. Furthermore, this report highlights the value of cytogenetic and molecular cytogenetic analysis in differential diagnosis of childhood adipose tissue tumors and adds to the number of lipoblastomas reported with chromosomal abnormalities at 8q11.2.

Prune-belly syndrome and other anomalies in a stillborn fetus with a ring X chromosome lacking XIST.

Ring X chromosomes that lack the X inactivation center and fail to be inactivated have been implicated as a cause of mental retardation and multiple congenital anomalies. We report on a stillborn fetus with karyotype mos45,X/46,X,r(X) and early urethral obstruction or prune-belly sequence, single umbilical artery, limb deficiency, horseshoe kidney, cardiac hypertrophy, persistent left superior vena cava, and axial skeleton abnormalities. Fluorescent in situ hydridization (FISH) studies confirmed that the ring chromosome is X-derived and demonstrated that it lacks the XIST locus. The findings in this fetus are discussed with regard to the spectrum of phenotypes associated with monosomy X and small ring X chromosomes.

Report of a complex karyotype in recurrent metastatic fibrolamellar hepatocellular carcinoma and a review of hepatocellular carcinoma cytogenetics.

Metastatic fibrolamellar hepatocellular carcinoma (HCC) was detected in the abdominal lymph nodes of an adolescent male after resection of the primary tumor. No dividing cells were isolated from attempted cytogenetic studies of the primary tumor. However, cytogenetic analysis of lymph node metastases detected 9 and 12 months after partial hepatectomy revealed abnormal hypertriploid karyotypes, with a suggestion of clonal evolution: 62-92 < 3n >,XX, -Y, +3, +6, +6, +7, +7, +8, +10, +13, +15, +16, +20, -21, -22, +mar1 x 2, +mar[cp6]/46,XY[8] and 78 < 3n >,XX, -Y,der(1)t(1;1)(p36.1;q21), +4, +6, +6, +7, +7,i(8)(q10), +10, +15, +20, -21, -22, +mar1 x 2, +mar2[3]/46, XY[17], respectively. Karyotypes of this variant of HCC have not been reported previously. The cytogenetics of HCC are reviewed.

Leptomyxid amebic meningoencephalitis mimicking brain stem glioma.

An 11-month-old infant presented with cranial nerve palsy and ataxia. MR revealed a large, enhancing pontine mass and small, nonenhancing parafalcial lesions; no organisms were seen in cerebrospinal fluid. After empiric treatment for brain stem glioma, the patient died. Autopsy revealed meningoencephalitis caused by leptomyxid amebae.

Activation of the alternative complement pathway in normal human serum by Loa loa and Brugia malayi infective stage larvae.

Infective stage larvae (L3) of Loa loa and Brugia malayi upon in vitro incubation with normal human serum activated the alternative complement pathway. C3 conversion products were detected on larval cuticles by eosinophil adherence and by immunofluorescence with C3c antiserum. No evidence for cuticle binding of IgG, IgA, IgM, Clq, or C4 was found by immunofluorescence. L3-induced C3 activation was inhibited by 10 mM EDTA but unaffected by 10 mM Mg++-EGTA. Human sera deficient in C2, C4, or C6 incubated with L3 resulted in C3 activation. However, sera treated with zymosan or heated for 1 h, 56 degrees C were unreactive with L3. Immunoelectrophoresis of fresh serum exposed to L3 for 1 h at 37 degrees C showed C3 cleavage products. The results indicate that these nematode L3 activate the alternative complement cascade via cuticular surface components. Larval viability was unaffected by complement activation or by adherence of eosinophils.

Parasitic infections of the central nervous system in children. Part III: Space-occupying lesions.

In the last part of this three-part review of parasitic infections of the central nervous system in children, we consider parasites which due to their size, distribution, or the nature of the host response, tend to cause focal lesions in the brain and spinal cord and therefore present as space-occupying lesions which occasionally mimic malignant tumors. As in Parts I and II, infections are grouped according to their predominant geographic area. Such infections include cysticercosis, one of the more common and important infections of the central nervous system.

Parasitic infections of the central nervous system in children. Part I: Congenital infections and meningoencephalitis.

This review article presents the epidemiology, pathogenesis, clinical presentation, laboratory and radiologic findings, and treatment of parasitic infections of the central nervous system in children. Some obscure parasitic infections are included. To assist the clinician faced with a specific case, infections are categorized first by predominant clinical manifestations: congenital disease, meningoencephalitis, focal lesions, and disseminated multisystem disease. Within the clinical categories, parasites are grouped according to the geographic area where most human infections occur. Congenital infections and those that present most frequently as meningoencephalitis are discussed in this part of the review.

Parasitic infections of the central nervous system in children. Part II: Disseminated infections.

In the second segment of this three-part review of parasitic infections of the central nervous system in children, we consider parasitic infections which typically involve various tissues and organs in addition to the brain and spinal cord. Parasites capable of dissemination in immunocompetent hosts are discussed first, and, as in Part I, organisms are grouped according to their predominant geographic location. This is followed by a discussion of the unique aspects of toxoplasmosis, strongyloidiasis and infection with microsporidia in immunocompromised patients, with an emphasis on the central nervous system.

Inflammatory (pseudosarcomatous) myofibroblastic tumor of the urinary bladder causing acute abdominal pain.

Inflammatory myofibroblastic tumor is a reactive proliferation of myofibroblasts that rarely involves the urinary bladder. The cause of inflammatory myofibroblastic tumor is unknown but may represent an initial reactive process to an infectious agent or trauma that transforms into neoplastic growth. Cases reported in children, however, often lack any preexisting bladder pathology. The authors present a case in a young child that presented as acute abdominal pain. In general, these tumors follow a benign clinical course after resection, although close monitoring is essential given the rarity of this bladder lesion.

Inverted papilloma of the urinary bladder in children: case report and review of prognostic significance and biological potential behavior.

Inverted papilloma of the urinary bladder is rare in the pediatric population. Despite several reports in the literature the prognostic significance and biological potential behavior of this lesion remain uncertain. The authors report a case of polypoid inverted papilloma of the urinary bladder in an 11-year-old boy and review its pathology. The pediatric population with this lesion is an ideal group to provide intense, long-term follow-up to define the biological behavior and prognosis significance of this lesion.

The Oddono's sulcus and its relation to the renal "junctional parenchymal defect" and the "interrenicular septum".

The anatomy responsible for the sonographic diagnosis of the renal "junctional parenchymal defect" and "interrenicular septum" is caused by perirenal fat along a line of incomplete fusion of two primary renal lobes. Studies using CT, MRI and cadaver observations are presented. "Oddono's sulcus" is suggested as a name for the changes in honor of the author who first described these anatomic findings.

Eosinophilic infiltration of the enteric neural plexuses in Hirschsprung's disease.

Inflammatory infiltrations of the enteric plexuses are uncommon and are usually lymphoplasmacytic. Within the past 15 years, nine pediatric cases in which a predominantly eosinophilic infiltrate of the gastrointestinal wall with a predilection for the myenteric (Auerbach's) and deep submucosal (Henle's) plexuses were seen at our institution. Two were neonates without gastrointestinal abnormalities who expired shortly after birth. Seven were patients with short-segment Hirsch-sprung's disease. There was a mild increase in mucosal eosinophils in the overlying mucosa and only one patient had peripheral eosinophilia. Follow-up data obtained 1 month to 7 1/2 years after biopsy revealed no development of inflammatory bowel disease, connective tissue disease, malignancy, allergic disorder, or intestinal dysmotility. The proximal location of the infiltrate suggests that it may represent a secondary finding rather than a primary cause of aganglionosis.

A quantitative evaluation of mucosal eosinophils in the pediatric gastrointestinal tract.

Evaluation of the mucosal eosinophil content is important in the interpretation of endoscopic biopsies, as high eosinophil densities might reflect allergic gastrointestinal disease. Reference ranges gleaned from the literature have an upper limit of normal varying from 6 to 20 eosinophils per 400 x high power field. Preliminary data suggest a geographic variation with higher eosinophil counts in the southern United States. We examined intestinal tract mucosa from 44 infants and children who died suddenly and unexpectedly in northeastern Texas. Subjects ranged in age from 3 wks to 17 yrs and were without known gastrointestinal disease. Using formalin-fixed, hematoxylin and eosin-stained 4-microns sections, intramucosal eosinophils were counted in ten consecutive high power fields and the mean eosinophil count determined. Twenty-three subjects (52%) had counts of > 20 eosinophils/high power field from at least one site. There was no correlation with age, sex, season, or cause of death. In a subset of 11 subjects, more extensive sampling showed the cecum and appendix to have the highest concentration of eosinophils and relatively low counts in the stomach and distal large intestine. These observations correlate with our impression that increased numbers of eosinophils, particularly in the proximal colon, are a common feature of otherwise unremarkable pediatric endoscopic biopsies. Efforts to distinguish the proportion of activated eosinophils in B5-fixed mucosal biopsies using the EG2 monoclonal antibody were unsuccessful, as this antibody does not appear specific for activation in B5-fixed tissue. Mucosal eosinophil counts should be interpreted with caution in geographic areas where a high background count is endemic.

Uptake and development of Wuchereria bancrofti in Aedes aegypti and Haitian Culex quinquefasciatus that were fed on a monkey with low-density microfilaremia.

Colonized mosquitoes of Culex quinquefasciatus (Haitian strain) and Aedes aegypti (Liverpool strain) were blood fed on a patas monkey (Erythrocebus patas) that had been experimentally infected with the Haitian strain of Wuchereria bancrofti and harbored a consistently low microfilaremia (1-3 mf per 20 mm3). Both species ingested more than twice the expected number of microfilariae (mf), i.e. 1.9 and 0.77 mf per mosquito, respectively. However, at 10-16 hours post ingestion only 4.2% of the mf had migrated from the blood meal in Cx. quinquefasciatus versus 20.7% in Ae. aegypti. Subsequently, only 3.5% of the ingested mf developed to the third stage in Cx. quinquefasciatus versus 56% in Ae. aegypti.

Craniopagus parasiticus: a case illustrating its relationship to craniopagus conjoined twinning.

A case of craniopagus parasiticus is described in which the parasitic twin is more fully developed anatomically than in any of the previous reports. Somatic and placental vascular anastomoses between the twins and hypoplasia of the umbilical cord of the parasite were also observed. These findings support the hypothesis that craniopagus parasiticus results from compromise of the blood supply to one of a pair of craniopagus conjoined twins.

Gametogenesis and sporogony of Hepatozoon mocassini (Apicomplexa: Adeleina: Hepatozoidae) in an experimental mosquito host, Aedes aegypti.

The sexual life cycle of the hemogregarine Hepatozoon mocassini was studied in Aedes aegypti, an experimental mosquito host, using transmission electron microscopy. Gamonts were observed leaving the host snake erythrocyte as early as 30 min after mosquitoes ingested infected blood, and some gamonts had penetrated the gut epithelial cells by this time. Six hours post-feeding, gamonts were identified within cells of the abdominal fat body. Twenty-four hours post-feeding, gamonts were often entrapped within the peritrophic membrane, but were no longer observed within the gut wall. Parasites pairing up in syzygy and undergoing sexual differentiation were observed within fat cells at this time, and by 48 hours post-feeding, well-developed macro- and microgametocytes as well as microgametes were discernible. Developing zygotes observed 3 days post-feeding were enclosed within a parasitophorous vacuole. By day 6, multinucleate oocysts with crystalloid bodies in the cytoplasm were seen. Sporozoites developing within sporocysts appeared by day 12. Seventeen days post-feeding, mature oocysts with sporocysts containing approximately 16 sporozoites were observed upon dissection of mosquitoes. Large crystalloid bodies no longer bound by rough endoplasmic reticulum were located anterior and posterior to the sporozoite nucleus. Free sporozoites were not observed.

Chemistry test ordering patterns after elimination of predefined multitest chemistry panels in a children's hospital.

Predefined multitest chemistry panels (PMCPs) have constituted a large proportion of laboratory tests and patient charges, even in pediatric settings, despite the absence of documented clinical utility for PMCPs and the general availability of random access analyzers that do not require predefined test combinations. We eliminated PMCPs in our tertiary children's hospital but placed no other restrictions on ordering, and observed a 32.7% reduction in the number of automated chemistry tests ordered. All 23 tests in the previous PMCPs showed a decline in utilization, >50% for 8 of the tests and 20-50% for 13 others, and this change was sustained throughout an 8-month follow-up period. The total number of orders for one or more tests increased by 8.2%, but the variety of combinations that were ordered increased by 280%. The most substantial changes included a decrease in the number of orders for combinations of >15 tests, and increases in the number of orders for single tests and combinations of 2 to 5 tests. Orders for combinations identical to all of the former PMCPs declined, with the exception of the 4-test electrolyte panel. There was a marked decline in orders for a 7-test panel identical to the recently defined HCFA-AMA Basic Metabolic Panel, and orders for combinations identical to the HCFA-AMA Liver Function and Extended Metabolic panels were vanishingly rare and nonexistent, respectively. The calculated reduction in patient charges was much greater than actual cost savings, but the reduction in total tests and increase in the variety of test combinations suggest that significant savings can be realized if clinicians are encouraged to order only the tests or combinations they need without imposing procedural, financial, and regulatory burdens.

Therapy associated changes in childhood tumors.

Contemporary treatment regimens for the common solid tumors of childhood have led to increased numbers of post-treatment pathologic specimens from survivors. Current therapeutic strategies for childhood cancers in North America require an accurate pathologic diagnosis and stratify patients based on combinations of clinical, biological, and pathologic features. In several tumor systems, the pathologic response to therapy also modifies the treatment regimen. Accurate pathologic interpretation of such specimens is critical in providing useful prognostic information for therapeutic decisions. Standardized handling of post-therapy pathologic specimens, appropriate use of molecular and genetic studies, consideration of the differential diagnoses, and assessment of the potential biologic significance of therapy-induced pathologic changes are, therefore, critical for patient management and determination of treatment protocols.

Acute renal failure: unusual complication of Epstein-Barr virus-induced infectious mononucleosis.

A 17-year-old boy with juvenile rheumatoid arthritis presented with jaundice, confusion, hemolytic anemia, thrombocytopenia, and acute renal failure secondary to titer-confirmed acute Epstein-Barr virus (EBV). Renal biopsy specimen revealed interstitial nephritis with an inflammatory infiltrate composed of cytotoxic/suppressor T cells, and interstitial mononuclear cell nuclei expressed EBV encoded RNA-1 (EBER-1) mRNA. Methylprednisolone treatment resulted in rapid improvement.