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Biomedical subjects

R D Craver

Publications and source records attributed to R D Craver.

At least 37 records · Page 2Linked to original sources

Laboratory evaluation of pediatric bone and soft-tissue tumors.

This article discusses how specimens are approached and handled by the laboratory. Basic histology is reviewed. Contributions by electron microscopy and immunoperoxidase staining are briefly discussed. Current uses of DNA indices, cytogenetic analysis, and new molecular diagnostic advances are highlighted. Proper communication between the pathologist and the orthopedist is the cornerstone for optimal use of laboratory resources.

Biopsy↗

Nephroblastoma and end-stage renal failure with bilateral cystic kidneys.

A child of 10 years 5 months presenting with chronic renal failure had bilateral cystic kidneys. Biopsy of a right lower-pole solid mass revealed nephroblastoma. At bilateral nephrectomy, both kidneys were both replaced by variable-sized cysts with a unifocal nephroblastoma on the right. Renal failure with nephroblastoma is uncommon and is usually either a manifestation of the Drash syndrome or a complication of chemotherapy. The need to assess both kidneys in a child with any other renal abnormality in addition to a renal mass should always be considered.

Child↗

Dermatofibrosarcoma protuberans with 46,XY,t(X;7) abnormality in a child.

A 9-year-old child with dermatofibrosarcoma protuberans demonstrated a balanced translocation, 46,XX,t(X;7)(q21l2;q11.2), in the untreated tumor, an abnormality not previously reported. Unlike seven of eight other reports of dermatofibrosarcoma protuberans, no ring chromosomes were present.

Child↗

Curvularia urinary tract infection: a case report.

A healthy 5-year-old child had recurrent symptomatic urinary tract infections, the last of which was accompanied by black specks in her urine. These specks were identified as Curvularia species, a dermatiaceous mold. Symptoms resolved and fungi disappeared with long-term hydration, without specific antifungal treatment.

Child, Preschool↗

Quantification of hemoglobin variants by capillary isoelectric focusing.

Capillary isoelectric focusing (cIEF) was used to identify and quantify major and minor hemoglobin (Hb) variants. Whole blood (approximately 10 microL required) hemolysate was analyzed with a commercial instrument equipped with a 50 microns (i.d.) x 27 cm coated capillary filled with 20 g/L ampholytes (pH 6-8) in 4 g/L methylcellulose (MC). Cathode and anode solutions were 20 mol/L NaOH and 100 mol/L H3PO4 in MC, respectively. Samples (approximately 40 nL) were applied via autosampler by low-pressure injection, focused for 3 min at 30 kV, and mobilized by simultaneous voltage and low pressure past the detector, where absorbance at 415 nm was analyzed by an automated data acquisition system. Blood from subjects with sickle cell trait, Hb S/C disease, and various beta-thalassemias were analyzed by cIEF in < 15 min. cIEF was used to separate Hb S from Hb D-Los Angeles. Assay precision determined with commercial controls gave CV < 2% for Hb A and S, and 1-11% for minor Hb variants A2, F, and A1c. Results obtained by cIEF for patients' samples agreed well with values determined by conventional assays (r2 > 0.95). The results demonstrate that cIEF is a rapid, sensitive, high-resolution automated method for routine quantitative clinical analysis of Hb variants.

Capillary Action↗

Delicate longitudinal nuclear grooves in childhood ependymomas.

Nuclei of ependymal tumor cells have a pattern of delicate uniform, linear, longitudinal, excentric grooves or clefts, usually single, involving the nuclear membrane and extending at least half the nuclear diameter, producing a notched mitten-shaped nuclear outline, forming a pattern not seen in other neural tumors. Histologically, 13 of 14 childhood ependymal tumors had nuclear clefts. Using these clefts with other histologic criteria, ependymal tumors were identified at the time of surgery 11 of 11 times, with no false-positive results. Cytologically, eight of 10 ependymal and seven of 32 other brain tumors had clefts. These seven other tumors had either additional nonuniform convolutions or folds (n = 5) or had only rare clefts (n = 2). This uniform pattern of nuclear clefts may help identify poorly differentiated ependymal tumors on permanent sections and may help recognize ependymal tumors at surgery, guiding the surgeons' resection.

Brain Neoplasms↗

Glomerulocystic disease: unilateral involvement of a horseshoe kidney and in trisomy 18.

Two occurrences of glomerulocystic kidney disease (GCD) in children younger than 1 year are described. One child was 3 months old with trisomy 18; the other child was 6 months old with GCD localized to one side of a horseshoe kidney. Lectin and immunohistochemical studies in tissue from the second child suggested that the entire nephron may be affected in GCD. There may also be overlap of morphological features between GCD and early stages of autosomal dominant polycystic kidneys.

Chromosomes, Human, Pair 18↗

Isolation of parainfluenza virus type 3 from cerebrospinal fluid associated with aseptic meningitis.

Parainfluenza virus type 3 has been isolated from the cerebral spinal fluid (CSF) from six individuals--four children and two adults--over a 10-year period. All had fever, and four had signs of meningitis. All recovered uneventfully, including one child undergoing chemotherapy for medulloblastoma. The clinical presentation of this child who developed parainfluenza virus type 3 meningitis is described, and the cases of five other individuals with parainfluenza virus type 3 isolated from the CSF are briefly reviewed. The paramyxovirus parainfluenza type 3, in addition to mumps virus, may be considered capable of infecting the central nervous system.

Adult↗

Congenital cutis laxa: a case report and review of loose skin syndromes.

Congenital cutis laxa is a deforming disease that may present for plastic surgical consultation during childhood. Failure to differentiate cutis laxa, with near normal wound healing, from the other forms of hyperelasticity syndromes with poor wound healing, has historically led to conflicting recommendations regarding the surgical management of patients presenting with loose skin. A face-lift and direct nasolabial fold excision was performed in a 10-year old patient with congenital cutis laxa with a good result. The other major syndromes presenting with loose skin as a clinical feature are reviewed and distinguished from cutis laxa. Plastic surgical procedures can be aesthetically and psychologically beneficial in children with congenital cutis laxa, and can be recommended without overt fear of wound disruption, poor scarring, and medical catastrophies that can occur with other hyperelasticity syndromes.

Child↗

Necrotizing funisitis.

Necrotizing funisitis is an umbilical cord lesion characterized by perivascular bands of necrotic Wharton jelly containing inflammatory cells in various stages of degeneration. Sixty cases were reviewed histologically. Clinical information was available in 45. Forty-five age-matched infants with acute (nonspecific) funisitis only were used as controls. Infants with necrotizing funisitis had more stillbirths, birth weights below the tenth percentile (small for gestational age [SGA]), infectious complications, and necrotizing enterocolitis. No consistent infectious agents or predisposing maternal factors were found. Cord neovascularization correlated with SGA infants. Necrotizing funisitis occurred in 0.1% of deliveries greater than 20 weeks' gestation. The perivascular bands, likened to the pattern of an Ouchterlony diffusion plate, suggest the presence of a diffusible toxin in the amniotic fluid. The stillbirths and SGA infants may represent the toxin's effect on the fetus. The lack of perivascular necrotic bands around vessels on the placental surface suggests neutralization or more effective clearing of the agent in this region, for reasons as yet undetermined. The factors underlying the cord lesion may contribute to superimposed acute nonspecific vasculitis and chorioamnionitis.

Acute Disease↗

Hematopoietic elements in cerebrospinal fluid in children.

Immature hematopoietic elements have been identified in eight Wright-stained cytocentrifuge preparations of lumbar puncture-derived cerebrospinal fluid (CSF) from infants and children. These immature hematopoietic elements probably represent contamination from vertebral bone marrow. Recognition that the nucleated cells in the CSF are a contaminant and not indicative of infection or leukemia is important to the immediate care of the child. The only adverse clinical sequela of this contamination identified has been a transient headache. Bone marrow contaminants are one of several abnormalities of CSF found in childhood diseases and may or may not elevate the CSF white blood cell count. This supports the need for pathologist review of all CSF cytocentrifuge preparations from children.

Bone Marrow Cells↗

Cow's milk-induced pulmonary hemosiderosis.

Cow's milk protein-induced pulmonary disease is a serious condition which, if recognized, can be treated effectively by avoidance of milk protein; untreated it may lead to early death. It should be considered in any child who suffers recurrent episodes of pneumonia and anemia.

Child, Preschool↗

Cytogenetic abnormalities among spontaneously aborted previable fetuses.

We have reviewed spontaneously aborted fetuses of 9 to 20 developmental weeks. All fetuses with either external or internal developmental defects and those with maternal history of repeated spontaneous abortion were studied cytogenetically. Among 723 fetuses the cytogenetically proven prevalence of chromosome abnormalities was 5.8%. Inclusion of suspected chromosome abnormality based on morphologic findings resulted in an overall prevalence of only 7.1%. This prevalence and restricted spectrum of chromosome abnormalities, which included monosomy X, triploidy, gonosomal aneuploidy, trisomies 13, 18, and 21, resembled the prevalence and spectrum found among perinatal deaths and differed both in prevalence and type from those chromosome abnormalities found in spontaneous abortions occurring during the embryonic period. Monosomy X was the most common chromosome abnormality among previable fetuses. Both monosomy X and triploidy were more frequently detected in the early fetal period than in perinatal deaths. The combined prevalence of autosomal trisomies was equal to that which has been reported in the perinatal deaths. Chromosomal structural abnormalities, seen in perinatal deaths, were not found in our study.

Abortion, Spontaneous↗

Desmoplastic infantile ganglioglioma.

We present the clinical, anatomic, and laboratory findings in a 4-month-old child with desmosplastic infantile ganglioglioma. Microtubule-associated protein-2 (AP18) and neuron-specific B-tubulin (TUJ-1) were more sensitive in detecting immature neural elements than synaptophysin. Despite the immature neuroblastic component, focal intermediate proliferation indices, microinvasion, presence of secondary features (extension into Virchow Robin spaces, perineuronal satellitosis), and subtotal resection, the child has done well, with striking improvement of the magnetic resonance imaging (MRI) image, head size improvement, no tumor recurrence, and minimal neurological deficits.

Brain Neoplasms↗

Primary malignant fibrous histiocytoma of the lung in a child: a case report and review of literature.

Malignant fibrous histiocytoma (MFH), an aggressive high-grade soft tissue sarcoma, usually occurs in the elderly during the fifth to seventh decade of life. It commonly arises in the retroperitoneum, extremities, and head and neck region. Primary pulmonary MFH is extremely rare and is frequently fatal. We present the youngest known case, a 9-year-old boy with a primary left lung grade II inflammatory MFH, stage II. He underwent a left upper lobectomy for tumor resection. After completing radiation therapy, he was started on vincristine, actinomycin D, and cyclophosphamide alternating with vincristine, doxorubicin, and cyclophosphamide every 3 weeks. After five such cycles, he had a histologically proven local recurrence. He then received chemotherapy consisting of ifosfamide (2 g/m2) and etoposide (VP-16) (100 mg/m2) given daily for 3 days every 3 weeks. The patient attained complete remission (CR) after five such cycles and completed treatment without any major complications. He received a total of 16 courses and is continuing in CR 36 months off treatment. Ifosfamide and etoposide (VP-16), known for their usefulness in treatment of adult soft tissue sarcomas, can be used as salvage chemotherapy for patients with MFH who fail the front-line conventional chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗