Precipitation of serum proteins by polyethylene glycol (PEG) in pretransfusion testing.

Polyethylene glycol (PEG) is used as a potentiator of blood group antigen-antibody interactions. Although PEG is known to precipitate immunoglobulins, we could find no reports of this reagent entrapping red blood cells (RBCs) in irreversible clumps. The patient we describe here had hyperglobulinemia with a reversed albumin:globulin ratio and a diffuse immunoglobulin peak on serum protein electrophoresis. During preparation of serologic tests, a precipitate formed that entrapped the RBCs when PEG was added. Rapid recognition of this phenomenon could prevent delay in the selection of blood for transfusion by substituting PEG-indirect antiglobulin test (IAT) with another technique such as low-ionic-strength solution (LISS)-IAT, and by increasing the number of washes prior to addition of the antiglobulin reagent.

Role of inhibition of nitric oxide production in monocrotaline-induced pulmonary hypertension.

Monocrotaline (MCT)-induced pulmonary hypertension (PH) is associated with impaired endothelium-dependent nitric oxide (NO)-mediated relaxation. To examine the role of NO in PH, Sprague-Dawley rats were given a single subcutaneous injection of normal saline [control (C)], 80 mg/kg MCT, or the same dose of MCT and a continuous subcutaneous infusion of 2 mg.kg-1.day-1 of molsidomine, a NO prodrug (MCT+MD). Two weeks later, plasma NO3- levels, pulmonary arterial pressure (Ppa), ratio of right-to-left ventricular weights (RV/LV) to assess right ventricular hypertrophy, and pulmonary histology were evaluated. The plasma NO3- level in the MCT group was reduced to 9.2 +/- 1.5 microM (n = 12) vs. C level of 17.7 +/- 1.8 microM (n = 8; P < 0.02). In the MCT+MD group, plasma NO3- level was 12.3 +/- 2.0 microM (n = 8). Ppa and RV/LV in the MCT group were increased compared with C [Ppa, 34 +/- 3.4 mmHg (n = 6) vs. 19 +/- 0.8 mmHg (n = 8) and 0.41 +/- 0.01 (n = 9) vs. 0.25 +/- 0.008 (n = 8), respectively; P < 0.001]. In the MCT+MD group, Ppa and RV/LV were not different when compared with C [19 +/- 0.5 mmHg (n = 5) and 0.27 +/- 0.01 (n = 9), respectively; P < 0.001 vs. MCT]. Medial wall thickness of lung vessels in the MCT group was increased compared with C [31 +/- 1.5% (n = 9) vs. 13 +/- 0.66% (n = 9); P < 0.001], and MD partially prevented MCT-induced pulmonary vascular remodeling [22 +/- 1.2% (n = 11); P < 0.001 vs. MCT and C]. These results indicate that a defect in the availability of bioactive NO may play an important role in the pathogenesis of MCT-induced PH.

Eccrine syringofibroadenoma surrounding a squamous cell carcinoma: a case report.

A 91-year-old man presented with a 9.0 x 7.0 cm exophytic mass on the dorsum of the right foot, surrounded by a scaling hyperkeratotic plaque-like lesion that had been present for many years. He had similar long-standing hyperkeratotic plaque-like lesions on both legs. Histopathologic examination of the exophytic mass revealed a well-differentiated squamous cell carcinoma surrounded by an eccrine syringofibroadenoma (ESFA). Histochemistry, immunohistochemistry and electron microscopy support this diagnosis. To our knowledge, this is the only reported case of ESFA being intimately associated with a malignant neoplasm.

Penectomy in diabetic patients undergoing maintenance dialysis.

Penectomy was performed to sustain life in 2 patients with insulin-dependent and non-insulin-dependent diabetes mellitus, respectively, who were undergoing maintenance hemodialysis. Both patients previously had manifested a series of serious macro- and microvascular diabetic complications. The histopathologic findings in both cases included gangrenous necrosis of penile tissue, while case 2 also evinced calcification of penile arteries. Penectomy has been reported as the result of penile malignancy, anticoagulant toxicity, self-inflicted injury, and criminal assault. Other reports document penectomies attributed to perineal infection (Fournier's syndrome) in diabetic patients with uremia. In five previously reported cases of penectomy in diabetic patients undergoing dialysis, systemwide arteriopathy was present in all. There is an association between uremia in diabetics and predisposition to an ischemic-infectious lesion of the penis that fails to respond to antimicrobial therapy.

Foreign-body osteitis of the metacarpal bone.

Foreign-body granulomas in the hand can appear secondary to an unrecognized foreign material. In a six-year-old boy, an extensive sterile osteitis of the second metacarpal bone appeared adjacent to a wood splinter.

Effect of verapamil on monocrotaline-induced pulmonary artery hypertension and endothelial cell dysfunction in rats.

Verapamil, a calcium channel blocker has been used with partial success in cases of primary pulmonary hypertension, as well as to reduce hypoxia-induced pulmonary hypertension (PH) in rats. However, its effect on monocrotaline (MCT)-induced PH in rats is not known. We studied the effect of verapamil on MCT-induced PH. Three weeks after a single injection of MCT, significant PH was noted in the MCT-injected rats compared with control (44.35 +/- 3.5 vs. 22 +/- 2.5 mmHg). MCT-injected rats on daily verapamil showed significant reduction in PH (31.5 +/- 3.4 mmHg). The main pulmonary artery of MCT-injected rats revealed subendothelial thickening, thinning and fragmentation of elastic laminae, smooth muscle cell hypertrophy and necrosis or loss of smooth muscle cells, and increased amounts of collagen in media and adventitia. In contrast, the main pulmonary artery of MCT + VP-treated rats showed less intimal thickening, some smooth muscle cell hypertrophy, but little necrosis or loss of cells in addition to disappearance of outer elastic laminae. Smaller pulmonary arteries (less than 150 microns in diameter) in MCT + VP-treated rats showed less medial thickening than MCT groups. However, diminished lung angiotensin-converting enzyme activity suggestive of endothelial cell dysfunction was noted in both MCT and MCT + VP-treated rats. This study indicates that verapamil attenuates MCT-induced PH, but has no effect on pulmonary endothelial cell dysfunction.

Severe chronic EBV infection associated with specific EBV immunodeficiency and an EBNA+ T-cell lymphoma containing linear, EBV DNA.

A patient with severe chronic Epstein-Barr virus (EBV) infection (CEBVI) of 6 years duration developed an EBV+ T-cell lymphoma. To determine whether the development of the T-cell tumor was linked to EBV, we studied this patient's EBV-specific immune response and her T-cell tumor tissue for evidence of EBV infection. Peripheral blood lymphocytes from this patient were systematically studied for immune function and response to EBV. Tumor tissue was examined for EBV genome and for evidence of EBV replication. This patient failed to develop anti-EBV nuclear antigen (EBNA) antibodies and had decreased mitogen responsiveness. Her T-cells showed a broad suppression of both autologous and allogeneic B-cells, which was coincident with clinical hypoimmunoglobulinemia. A selective cytotoxic T-cell defect toward autologous EBV-infected B lymphoblasts, which could not be corrected by the addition of lymphokine-mediated T-cell help, was also documented. A lymph node biopsy taken 5 years after her clinical presentation revealed lymph node architecture completely effaced by a diffuse CD3+, CD4+, Ia+, CR2+ T-cell lymphoma containing EBNA and linear, replicating EBV DNA. Select CEBVI patients with humoral and combined cellular aberrations in the immune response to EBV may be predisposed to the development of EBV+ T-cell tumors.

Pulmonary vasculature in monocrotaline-induced hypertensive rats on magnesium therapy.

We have previously demonstrated that magnesium therapy attenuates monocrotaline-induced pulmonary hypertension and right ventricular hypertrophy in rats. In this report we evaluate the effect of oral magnesium therapy on the pulmonary vasculature of monocrotaline-treated rats. Lung sections were prepared for light microscopic examination, and the medial wall thickness of pulmonary arteries of less than 100 microns in diameter was measured. The mean medial wall thickness of control rats was 6.98 +/- 1.16% of the external diameter of the pulmonary artery, the monocrotaline group had a significant increase in medial wall thickness (14.61 +/- 1.52%, p less than 0.005 vs control); in contrast 75% of MCT + magnesium group revealed a significant reduction in medial wall thickness (8.13 +/- 1.26%, p less than 0.02, vs monocrotaline group). Magnesium therapy alone had no effects on pulmonary vasculature. We conclude that magnesium has significant beneficial effects on monocrotaline-induced pulmonary hypertension and the accompanying vascular lesions.

Burkitt's lymphoma in a patient with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) appear to be at increased risk for development of neoplastic disease. We describe the case of a male teenager with SLE and Burkitt's lymphoma. His presentation was similar to that of an exacerbation of his underlying SLE. We believe this to be the first case of Burkitt's lymphoma in a patient with SLE. The association of SLE and malignancy, with emphasis on lymphoproliferative states, is discussed.

Disseminated adenovirus infection with hepatic necrosis in patients with human immunodeficiency virus infection and other immunodeficiency states.

Disseminated adenovirus infection with fatal hepatic necrosis has been reported in 16 patients, 15 of whom had immunocompromising conditions. Herein we report three patients with AIDS and fatally disseminated adenovirus infection with hepatic necrosis. The median age of these 16 patients was 4.7 years, and their illness was characterized by fever (13 of 16 patients), coagulopathy (10 of 16), lower respiratory tract disease (10 of 16), and gastrointestinal hemorrhage (five of 16) in addition to clinical evidence of hepatitis. The adenoviruses isolated were the commonly found serotypes 1, 2, 3, 5, and 7 for 13 of the 15 cases for which this determination was available. With the high frequency of adenovirus infection in humans and the increasing prevalence of human immunodeficiency virus infection in children, it seems likely that this syndrome will continue to be seen.

Perinatal nonimmune hydrops: diagnostic ultrasonography and related aspects of management.

Nonimmune hydrops fetalis may become the commonest form of hydrops seen in Western countries during the perinatal period, and it has at least a 50% mortality. This report describes five infants with nonimmune hydrops associated with maternal hydramnios and with congenital fetal lesions or disorders, ie, mediastinal teratoma, pulmonary leiomyosarcoma, Beckwith-Weidemann syndrome with omphalocele, fetal tachycardia, and Down's syndrome. Three of the infants survived the neonatal period and two of these underwent surgery for resection of their tumors early in the neonatal period. The third had an omphalocele repaired at 6 hours of age. The literature is reviewed with respect to the pathophysiology of nonimmune hydrops. Its diagnosis and treatment are discussed, with special emphasis on the role of ultrasound in its early diagnosis and optimal prenatal and postnatal management, and on the morbidity seen in survivors.

Congenital oral cyst with heterotopic gastrointestinal and respiratory mucosa.

A congenital oral duplication cyst of the ventral surface of the tongue was found to contain both gastrointestinal and respiratory epithelium. The literature is reviewed, with emphasis on the possible histogenesis of this extremely rare lesion, as well on the clinical differential diagnosis.

An infant rat model of bacteremia with Brazilian purpuric fever isolates of Hemophilus influenzae biogroup aegyptius. Brazilian Purpuric Fever Study Group.

Brazilian purpuric fever (BPF) is a newly recognized fulminant pediatric infection caused by bacteremia with Hemophilus influenzae biogroup aegyptius (Hae). Following intraperitoneal inoculation, each of five disease isolates caused bacteremia more frequently than control, conjunctival isolates of Hae in complement-depleted 6-d-old rats. Sustained but self-limited bacteremia was observed in normal infant rats after inoculation with a disease strain. These rats developed meningitis and had depressed hemoglobin concentration and platelet counts. Pathologic examination showed meningitis and contiguous otitis. Pretreatment of infant rats with immune adult rat serum raised against disease isolates protected rats from bacteremia. Normal adult rat serum or immune rat serum against control strains failed to protect infant rats. Thus, strains of Hae isolated from patients with BPF are more virulent than control strains. Antibody against antigens unique to disease isolates protects infant rats from bacteremia.

Magnesium aspartate hydrochloride attenuates monocrotaline-induced pulmonary artery hypertension in rats.

1. The effect of oral magnesium aspartate hydrochloride on monocrotaline (MCT)-induced pulmonary arterial hypertension was evaluated in rats. 2. A single subcutaneous injection of MCT, a pyrrolizidine alkaloid of plant origin, induces significant morphological changes in pulmonary vessels, pulmonary arterial hypertension and right ventricular hypertrophy in rats by 3 weeks. 3. Two groups of rats (Mg2+ control and Mg2+ + MCT) were started on oral Mg2+ (15.4 g/l magnesium aspartate hydrochloride dissolved in deionized water) 2 weeks before the MCT injection. The rest were given deionized water. At the start of the experiment, the control groups (deionized water and Mg2+) were given normal saline subcutaneously; the other groups (deionized water and Mg2+) were given MCT (60 mg/kg) subcutaneously. 4. Pulmonary artery pressure, right ventricular hypertrophy, lung pathology, organ weights and serum electrolytes were assessed 3 weeks after a single subcutaneous injection of MCT. Seventy-five per cent of the rats treated with MCT and oral Mg2+ (12 out of 16) showed significant reduction in pulmonary arterial hypertension, arterial pathology and right ventricular hypertrophy. 5. Our data indicate that Mg2+ attenuates experimentally induced pulmonary hypertension, possibly either by modulating the intracellular Ca2+ level and/or by directly affecting the pulmonary endothelial cell-smooth muscle cell complex involved in metabolism and maintenance of pulmonary vascular resistance.

Trisomy 18 with Cantrell pentalogy in a stillborn infant.

A 34-week stillborn infant had omphalocele, agenesis of the sternum and anterior rib cage, membranous diaphragms with eventration of the viscera, ectopia cordis with absence of the pericardium, and congenital heart defect. These findings are consistent with a diagnosis of Cantrell pentalogy. The presence of bilateral clubfeet, spina bifida, hydrocephalus, abnormal ears, and horseshoe kidneys suggested a chromosome abnormality. Chromosome analysis showed trisomy 18. Individuals with manifestations of Cantrell pentalogy deserve cytogenetic evaluation.

N-myc oncogene expression in histopathologically unrelated bilateral pediatric renal tumors.

Renal tumors of childhood occasionally exhibit histopathologic and clinical features that preclude accurate diagnosis. Molecular and cell culture techniques may be helpful in better characterizing these cases. This approach was used to examine unusual bilateral renal tumors from a young boy. The left kidney tumor was an undifferentiated neoplasm with light microscopic features suggestive of both Wilms' tumor and neuroblastoma, and the right kidney tumor was identified as multilocular cystic nephroma (MLCN). In vitro tissue culture of tumor cells and hybridization experiments with an N-myc oncogene DNA probe contributed to a revised diagnosis of intrarenal neuroblastoma of the left kidney. A cell line established from the left tumor exhibited neurite outgrowth and was positive for neuron-specific enolase and synaptophysin. N-myc was greater than ten-fold amplified in chromosomal DNA from the left kidney tumor. Measurement of N-myc RNA expression enabled distinction between benign and malignant tumor tissue. The detection of N-myc gene amplification predicted a poor prognosis which was confirmed by the patient's subsequent clinical course.

Cystosarcoma phylloides in the adolescent female.

Between 1979 and 1985, five adolescent females have undergone excision of cystosarcoma phylloides. Mean age at presentation was 15 years with a range of 13 to 18 years. Clinical characteristics unique to this diagnosis included a recent history of rapid growth and the large size of the breast mass (mean diameter 7 cm) at initial presentation. Each patient underwent "cosmetic" excisional biopsy utilizing either a circumareolar or inframammary approach. In four patients, the neoplasm was benign with no postoperative recurrence (mean follow-up 33.8 months). The remaining lesion was malignant (liposarcoma) and subsequent simple mastectomy was performed. There was no evidence of disease at 35 months.